Pain is a well-recognized and important non-motor manifestation in Parkinson disease (PD). Painful or unpleasant sensations in PD can be classified as musculoskeletal, dystonia, akathisia, radicular, and central or primary pain; the last two are associated with neuropathic pain. Particularly, neuropathic pain in PD has not been fully clarified; therefore, it goes somewhat unnoticed, and the affected patients do not receive adequate pain treatment. The main purpose of this literature review was to identify the incidence of neuropathic pain in PD and the involvement of dopamine of this type of pain by the integration of different lines of investigation. In this review, a search was conducted using PubMed, ProQuest, EBSCO, Medline, EMBASE, and the Science Citation index for studies evaluating pain in patients with PD. The inclusion criteria were as follows: original articles that evaluated incidence and possible mechanism of neuropathic, central, and radicular pain in PD. Nine studies related to the incidence of neuropathic pain in PD suggest the activation of cerebral areas, such as the cortex, striatum, amygdala, thalamus, raphe nuclei, and locus coeruleus. Neuropathic pain is related to altered levels of dopamine, serotonin, and norepinephrine; these neurotransmitters are related to the sensitive and emotional dimensions of pain. Dopamine could cause hypersensitivity to pain, either indirectly through modulatory effects on affective pain processing and/or directly by affecting the neural activity in key areas of the brain that modulate pain. A considerable proportion of patients with PD suffer neuropathic pain; however, it has been disregarded, this has led to an inability to achieve an adequate treatment and a decrease in pain to improve the quality of life of these patients. We consider that neuropathic pain in PD is possibly induced by neurophysiological changes due to the degradation of dopaminergic neurons.

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Background and Objective: Current recommendations prescribe either nicardipine or labetalol as the first-line treatment for acute hypertension due to ease of use, availability, and low price. However, it is unclear if these drugs have different effectiveness and safety profiles. This systematic review and meta-analysis aimed to compare the efficacy and safety of labetalol and nicardipine in patients with acute stroke. Materials and Methods: MEDLINE via PubMed, Scopus, Embase, and Google Scholar databases were electronically searched for the eligible publications from inception until March 2022. All full-text journal papers in English which compared the efficacy of nicardipine with that of labetalol on lowering blood pressure (BP; or treating hypertension) in all subtypes of acute stroke were included. The Cochrane Collaboration tool was used to assess the risk of bias. Data were analyzed using specific statistical methods. Results: Following the abstract and full-text screening, this meta-analysis included five retrospective cohorts and one prospective pseudorandomized cohort. Nicardipine's effect on time at goal BP was significantly superior to that of labetalol in patients with acute stroke (0.275 standardized mean difference [SMD], 95% confidence interval [CI]: 0.112–0.438, P = 0.001). The incidence of adverse events was significantly higher in the nicardipine group than that in the labetalol group. The pooled odds ratio (OR) was 1.509 (95% CI: 1.077–2.113, I² = 0.00%, P = 0.757). The quality of included studies was found to be low. Conclusion: More prospective, comparative trials are needed to investigate the efficacy of BP management as well as clinical outcomes in acute stroke patients receiving continuous labetalol and nicardipine infusions.

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Introduction of international consensus criteria (2015 IPND criteria) for neuromyelitis optica spectrum disorders (NMOSDs) has improved diagnostic accuracy for aquaporin 4 (AQP4)-IgG-associated and seronegative NMOSDs. This study aimed to review relevant publications related to the incidence and prevalence of NMOSDs and provide an updated review of the global epidemiology of NMOSDs in the light of new diagnostic criteria. A comprehensive literature search was performed from January 2015 to June 2021 by using appropriate keywords in PubMed, Scopus, and Web of Science. Relevant papers that fulfilled inclusion criteria were shortlisted and reviewed. Twenty-one papers were selected for this review. Incidence of NMOSDs was 0.04–0.25/100,000 in predominantly white and 0.34–1.31/100,000 in nonwhite populations. Prevalence was 0.70–1.91/100,000 in white and 0.86–4.52/100,000 in nonwhite populations. The 2015 IPND criteria significantly improved the incidence and prevalence rates for NMOSDs when compared to the Wingerchuk 2006 criteria. Incidence of MOG-IgG-associated NMOSDs was 0.12–0.13/100,000, with prevalence in children 0.03–1.4/100,000 and in adults 0.65–2/100,000. In this systematic review, studies that used uniform diagnostic criteria and confirmed cases after testing for AQP4-IgG were included. The prevalence of NMOSDs was estimated to be <5/100,000 globally. A clear bias was seen in favor of nonwhite and indigenous populations. This review highlights the need for prospective population-based epidemiological studies and the importance of surveys in nonwhite populations around the globe.

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Background: Post-traumatic stress disorder (PTSD) is a common and debilitating illness that accompanies many neurological disorders, including stroke. Objective: The aim of this systematic review was to identify and critically appraise all published studies that have reported the frequency, severity, and time course of PTSD after stroke, the factors associated with its development, and its impact on patients' lives. Material and Methods: The PubMed EMBASE, PsycINFO, and Ovid Nursing databases were searched for studies published in English that had recruited at least 10 patients (>18 years old) after stroke and who were also diagnosed with PTSD. Results: Twenty studies covering a total of 1785 patients met the study inclusion criteria. The frequency of PTSD ranged from 3% to 31%, with a weighted proportion of 16.5%. PTSD runs a chronic course. PTSD after stroke was associated with premorbid neuroticism, negative affect, and maladaptive coping styles. Comorbid depression and anxiety also increased the risk of PTSD. Psychological factors such as negative appraisal and perceived high risk of recurrence and distress were associated with PTSD. Good social support reduced the risk of PTSD. PTSD reduced patients' quality of life, physical functioning, and medication compliance. Conclusions: PTSD is common after stroke. Further research is needed to clarify its time course and identify the neurochemical factors and brain circuits associated with the development of post-stroke PTSD. Randomized controlled treatment trials targeting PTSD in stroke are warranted.

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Background: Intrasaccular flow diverters are a new modality in the treatment of intracranial aneurysms. The Contour device has been designed to offer a variety of advantages over existing endovascular treatment options for wide-necked bifurcation aneurysms. There is no available literature describing its use in treating ruptured aneurysms. Objective: To assess the safety, outcome, and efficacy of the Contour device as a primary modality in treating ruptured intracranial aneurysms. Materials And Methods: A retrospective analysis of 14 aneurysms in 13 patients treated with the Contour device was studied. Data pertaining to demographics, clinical presentation on arrival, aneurysmal characteristics, and follow-up outcome was collected. The Hunt–Hess scale and modified Rankin scale were used to grade severity of clinical presentation. Results: Of the 14 aneurysms treated, one was unruptured and 13 were ruptured. Six middle cerebral artery aneurysms, six anterior communicating artery, one basilar top and one distal anterior cerebral artery aneurysm were treated. Coiling was required in two patients due to the large size of their aneurysms. No thromboembolic complications or intraoperative rupture was reported following device placement. Two patients required antiplatelet therapy for 6 weeks. A median follow-up at 3 months revealed stable placement of the device within the aneurysm and no sign of residual aneurysm or rebleed. Conclusion: Initial experience with Contour in treating ruptured aneurysms has shown favorable immediate and short-term follow-up outcome. However, long-term follow-up studies are required to assess its safety and long-term efficacy.

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Background: Management of foot-drop following stroke can be addressed with ankle-foot orthosis (AFO) or functional electrical stimulation (FES) of the peroneal nerve. There is limited evidence regarding the efficacy of FES as a substitute for a conventional ankle-foot orthosis. Objective: The aim of this study was to compare efficacy of FES against AFO in management of foot-drop in patients following stroke. Materials and Methods: Twenty patients (ten per group) were enrolled in this prospective crossover trial. Group A patients received gait training with AFO during first week followed by training with FES during second week and vice-versa for group B. Outcome parameters following AFO/FES training included Ten-meter, Six-minute walk test and spatiotemporal gait parameters. Patient satisfaction level was assessed using feedback questionnaire. Friedman test and Wilcoxon signed-rank test were performed to compare outcomes between barefoot, AFO and FES. The P value < 0.05 was considered statistically significant. Results: Nineteen males and one female aged 45.5 ± 9.45 years were recruited. Statistically significant improvement was observed in Ten-meter and Six-minute walk tests, gait speed, Timed up and go test (TUG), stance-swing ratio and single-limb support among users of FES as compared to AFO and barefoot. There was no statistical difference observed in other gait parameters. Physiological cost index (PCI) showed trend in improvement among FES users. Patient satisfaction scores were higher for FES users. Conclusions: Quantitative and qualitative results were in favour of FES as compared to AFO and barefoot suggesting that FES can be a potential orthotic intervention in hemiplegic patients.

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Objectives: The objective of this study was to evaluate the effect of early bedside arm and leg cycle ergometer exercises as compared to routine physiotherapy on sitting and standing ability in hospitalized acute stroke patients. Materials and Methods: Thirty-four consecutive patients with acute stroke were included in the randomized controlled trial. Patients were divided into two groups based on 1:1 simple randomization Experimental group (n = 18) and control group (n = 16). Experimental Group received arm and leg cycle ergometry along with conventional physiotherapy exercises, while the patients in the control group received conventional physiotherapy exercises. Both the groups received treatment for a total duration of 50 min session, twice a day for 7 days. Preintervention and postintervention measurements were taken for both groups using performance-oriented mobility assessment, postural assessment scale for stroke, Motricity Index, and Trunk control scale. Results: Statistically significant improvement (P < 0.05) was observed in the experimental group and control groupafter intervention among all the outcome measures. Conclusions: Early bedside intervention of cycle ergometer along with routine physiotherapy is effective in improving the sitting and standing abilities, trunk control, and motor function in acute stroke survivors.

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Background: Myasthenia gravis (MG) is an immune-mediated disorder of the neuromuscular junction. About 10% are refractory to immunosuppressive therapy. Aims: To analyze the response of patients with generalized MG to rituximab. Methods and Materials: A retrospective review of patients with MG who received rituximab was carried out (n = 13, M:F = 6:7, mean age: 44.84 ± 15.73 years). Myasthenia Gravis Foundation of America (MGFA), MGFA post-intervention status (MGFA-PIS), and Myasthenia Gravis Status and Treatment Intensity (MGSTI) were assessed before and after rituximab. Results: The duration of MG was 104.07 ± 92.25 months. Before rituximab, the MGFA was IIA/IIB/IIIA/IIIB/IVB/V in 1/1/2/6/2/1 patients and MGSTI was four in eight patients and six in three patients. The mean duration of follow up was 20.92 ± 14.06 months (range, 4 to 42 months). Dose reduction or discontinuation of cholinesterase inhibitors could be achieved 12 patients. Complete stable remission (CSR) and pharmacologic remission (PR) were achieved in one and four patients respectively and five patients had minimal manifestations. Most patients attained level 0, 1 or 2 MGSTI at last follow up. No rituximab infusion-related adverse events were noted. Three patients had exacerbation of MG between one to five weeks after rituximab administration. Three patients died, one each due to a cardiac event unrelated to MG or treatment, complications related to myasthenic crisis, and coronavirus disease. Conclusions: Rituximab was effective in bringing about remission in MG and can be considered as a first-line agent. However, it has to be administered under close supervision as some patients develop exacerbation of MG akin to steroid-induced worsening.

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Background: India, with a total population of 1,309,053,980, has 0.29 psychiatrists, 0.00 child psychiatrists, and 0.80 mental health nurses per 100,000 population. The mental health expenditure per person is just 4 INR as per Mental Health ATLAS 2017 (World Health Organization). The treatment gap for mental disorders still remains very high. These raise issues to our mental health status post coronavirus disease 2019 (COVID-19). Higher levels of anxiety, stress, and depression after the stay-at-home order post COVID-19 could lead to further psychological trauma besides mental health. A younger age, the female gender, and the caregiver status have a greater degree of stressfulness because of the pandemic. Objective: To assess the mental health status of the general population post COVID-19 in India. Material and Methods: An online survey was conducted using Depression Anxiety Stress Scale (DASS) 21 in the month of July, 2020. Results: The results of the online survey using DASS 21 conducted in the month of July, 2020, in India support the mental distress in the general population too. Conclusions: Systematic and regular surveys need to be conducted to allow for monitoring of the mental health impact of COVID-19 from time to time and prepare ourselves so as to prevent the second wave of mental health crises post the COVID-19 pandemic.

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Basal ganglia encephalitis is a part of the spectrum of autoimmune basal ganglia disorders. We are reporting a child who had a fever with focal seizures followed by behavioral problems, rigidity, bradykinesia, and dystonia. His parkinsonism-like features were increasing day by day up to the level that the child was non-ambulatory. His initial Magnetic Resonance Imaging (MRI) of the brain showed asymmetrical T2 hyperintensities involving both the caudate nuclei and putamina. Later, with progressive symptoms, repeat MRI revealed a swelling and symmetrical signal change in both the caudate nuclei and putamina in the form of T2 and Fluid-attenuated inversion recovery (FLAIR) hyperintensities. In addition, there was T2 hyperintensity involving bilateral substantia nigra. Serum basal ganglia antibody, Leptospira Immunoglobulin M (IgM) antibody was positive, and Cerebrospinal Fluid (CSF) oligoclonal band was positive. So, the child was diagnosed with post-leptospirosis autoimmune basal ganglia encephalitis. He was managed with immunomodulatory agents and significant improvement in the symptoms with mild residual extrapyramidal symptoms were noted.

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Background: Although epilepsy is a common neurological condition, there is paucity of nationwide data on treatment patterns and sociodemographic and clinical factors affecting treatment decisions in India. Objective: To assess clinical profiles, usage pattern of antiepileptic drugs (AEDs), and seizure control among patients with epilepsy in India. Methods: This was a cross-sectional, observational, multicenter study on adult patients with epilepsy who were on AEDs for at least six months before enrollment. Data were collected from patient interviews and medical records. Results: Out of 800 enrolled patients, a majority (69.0%) had generalized onset seizure in the six months before enrollment. The median age at epilepsy onset was 20.0 (1.0–64.0) years; 40.0% of the patients were females, 48.5% were married, 99.1% were literate, and 67.0% belonged to the lower or upper-middle socioeconomic class. Overall, 459 patients (57.4%) received AEDs as combination therapy. Most patients received levetiracetam (37.0%), sodium valproate (18.5%), carbamazepine (17.3%), or phenytoin (13.8%) as monotherapy, and clobazam (59.7%), levetiracetam (52.9%), carbamazepine (26.4%), sodium valproate (24.8%), or phenytoin (24.0%) in combination therapy. Quality of life was comparable for first- and third-generation AEDs. Adverse drug reactions were mostly attributed to dose modification or switching between drugs. No serious adverse drug reactions or new safety concerns were identified. Conclusions: Findings from this large, cross-sectional, observational, multicenter study indicate that first-generation AEDs sodium valproate and phenytoin continued to be used in a substantial number of patients on monotherapy and combination therapy in India, even though an increasing trend toward use of second-generation AEDs was noted in clinical practice.

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Background and Objectives: Tension type headache is one of the costliest primary headaches which can cause a significant impact on an individual's life. Healthcare professionals are exposed to multiple trigger factors which give rise to an increase in headache frequency, most notable reasons being sleep disturbances, stress and untimely food habits. With this background, we conducted this study of detailed clinical profile and trigger factors among the 2050 subjects (MBBS students: 909, BDS students: 323, nursing students: 268, postgraduates students and staff: 550) in our medical institute. Materials and Methods: Subjects were given questionnaires on headache and were instructed to give the details of their clinical symptoms along with relevant questions on trigger factors. The assessment tools used were the Visual Analogue Scale (VAS) and Migraine Disability Assessment Scale (MIDAS). Results: Out of 2050 subjects, 464 patients suffered from tension type headache. Overall prevalence of tension type headache was 22.6%. Prevalence was higher in females (57.9%) as compared to males (42.1%). Headache experienced by majority of the student population was unilateral (31.4%), pulsating type (51.6%) and of moderate intensity (77.1%). Common associated symptoms were nausea associated with other factors (40%) and photophobia and phonophobia (18.6%). Stress (72.1%) and decreased sleep (49.4%) were the most common triggering factors. Practice of self-medication was reported by 80.2% of subjects. Conclusion: Our study noted a high frequency of trigger factors in medical professionals, the most common triggering factors being stress and disturbed sleep. To our knowledge this is the first large study to evaluate headache and specifically tension type headache among medical professionals.

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Background: Approximately, 80% of civilian cranial gunshot injuries in India are mainly due to unlicensed mostly country-made guns, called the “Desi-Kattas,” manufactured by the traditional gunsmiths. These injuries constitute a unique subgroup, with respect to the make and design along with the unique wound ballistics compared to the factory-made firearms. Objective: This study is aimed at defining the prognostic factors in predicting the outcomes related to cranial gunshot injuries due to these desi-kattas. Methods: Ten patients with kata-related cranial gunshot injury underwent surgical intervention at our institution, between 2014 and 2018. Their clinical status, imaging features, and outcomes were retrospectively analyzed. Results: Ten patients with kata-related gunshot injuries to the head were identified. Homicidal injuries were documented in six cases. Five had a GCS of 13–15 at presentation. Exit wounds were noted in 50% of the suicidal cases. Multilobar involvement was seen in 70% of cases, with the bullet crossing the midline in only 40% cases. Decompressive craniectomy was required in five cases. Eight patients had a Glasgow Outcome Score of four or more at 6 months of follow-up. There was one death during the follow-up period, while the other patient remains to be in vegetative state. Conclusion: The Katta-related cranial injuries constitute low-muzzle velocity injuries with the majority of the patients sustaining nonfatal injuries, especially homicidal. The presence of positive CT findings and the crossing of the bullet across the midline on CT brain predicts dismal prognosis. Optimal neurosurgical approach should consist of minimal local debridement and attain water seal dural closure.

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Background: Many potential causes of optic nerve inflammation exist, including typical and atypical causes, which require different management strategies. Objective: The objective of this study is to identify red flags that help differentiate typical from atypical optic neuritis (ON). Materials and Methods: This prospective study included 66 patients (100 eyes) with immune-mediated ON from January 2016 to June 2019, carefully excluding the nonimmune causes. The clinico-radiological features, investigations, therapy, and outcome were analyzed. Results: We evaluated 33 cases each of typical and atypical ON. The typical group included 29 idiopathic ON and four associated with multiple sclerosis. Atypical ON included 19 neuromyelitis optica (NMO), seven MOG-associated ON (MOG-ON), and others due to Sjogren's syndrome, granulomatous polyangiitis, sarcoidosis, and IgG4 disease. Atypical ON occurred significantly and more frequently with extremes of ages (<10 or >70 years), bilateral simultaneous or severe vision loss with early disc pallor, multiple attacks, symptoms/neuro-imaging indicating non-MS disease e.g., long segment ON/myelitis, large confluent lesions, the involvement of optic tract, chiasma, area postrema or diencephalon, and (pachy) meningitis. Systemic involvement and poor outcomes despite steroids and second-line immunosuppression were observed more often in the atypical ON. Conclusions: The red flags indicating atypical ON are onset at extremes of age, multiple attacks, bilateral simultaneous or severe to very severe vision loss, early disc pallor, neurological symptoms, or imaging abnormalities suggesting non-MS disease, systemic involvement, and poor steroid responsiveness. The awareness might help the clinician promptly identify and escalate therapy to ensure a better outcome.

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Background: Carotid endarterectomy (CEA) has been the standard therapy for carotid artery stenosis (CAS). Modified eversion carotid endarterectomy (mECEA) was recently introduced to treat CAS. However, the short-term safety and long-term efficacy of mECEA are still controversial among studies. This systematic review aims to summarize the current literatures about safety and efficacy of mECEA in treating CAS. Methods: A systematic review of mECEA was conducted in the main bibliographic databases in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement. Clinical studies on the safety and efficacy of mECEA in treating CAS with clinical results of transient ischemic attack (TIA), stroke, death, and restenosis were included. Results: The initial search and screening found 15 references from the main databases, and 7 studies were finally included after full-text article assessment, which consisted of 3 single-arm studies and 4 comparative studies. The risks of postoperative complications including TIA, stroke, myocardial infarction (MI), and death ranged from 1.1% to 2%, 0% to 2.5%, 0% to 4.4%, and 0% to 2.32%, respectively. The mECEA was significantly related to lower risk of carotid artery occlusion, incision numbness, and shorter lengths of stay in hospital and average scar when separately compared with conventional CEA (cCEA), CEA with patch closure (pCEA), and eversion CEA (eCEA). Conclusions: The mECEA is a promising surgical option for CAS with acceptable clinical outcomes. In order to prove its safety and efficacy, future practices need to be conducted by more medical workers in more large-scale trials.

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Diaphragmatic flutter (DF) is an unusual movement disorder with involuntary and repetitive contractions of the diaphragm with or without other abdominal muscle involvement. The disorder is known to occur across all ages including newborns. The etiology is diverse and so are the therapeutic options. Reaching an etiological diagnosis is considerably delayed. Response to therapy is variable and is governed by the underlying etiology. We describe three children with diaphragmatic flutter. The etiology was diverse with unrelated pathologies such as hypocalcemia, striatal necrosis, and idiopathic. All three children responded promptly and completely to calcium, high dose thiamine and biotin, and clonazepam, respectively. Our case series underscores the importance of clinical identification of such rare movement disorders. It also emphasizes that directed etiological evaluation may lead to successful amelioration of DF which is otherwise considered refractory to therapy.

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Introduction: The unprecedented challenges during the COVID pandemic and the subsequent lockdown had resulted in a delay in treatment metrics for acute stroke. There is a rising concern that COVID-19 co-infection can adversely affect stroke outcome. We aim to investigate the impact of COVID-19 in the management of stroke patients. We also compared the differences in stroke manifestations, etiological pattern, treatment course, and outcome of acute stroke patients in COVID-19 confirmed cases. Methodology: A single-center retrospective study was done at the Stroke Unit, Government Medical College, Thiruvananthapuram. Consecutive patients of acute stroke confirmed by imaging, presenting within 24 hours of the onset of symptoms in May to July 2020 and May to July 2019, were included. The primary data variables included baseline demographics, risk factors, admission NIHSS, stroke timings, thrombolysis rate, TOAST etiology, mRS at discharge, and in-hospital mortality. Results: Strokes with higher NIHSS, arrival blood sugar and blood pressure, and delays in door-to-CT and door-to-needle time were more during the pandemic. Intravenous thrombolysis was less and mortality was higher in COVID-19 strokes during the pandemic. COVID-19-positive stroke patients had more hemorrhagic strokes, more severe strokes with low CT ASPECTS, more hemorrhagic transformation, high in-hospital mortality, and poor functional outcome at discharge and 3 months. Conclusion: Our study was a hypothesis-generating study with a limited number of patients. This study has reconfirmed the higher severity of the stroke, with a higher mRS score and mortality during the pandemic, especially among COVID-19-positive stroke patients.

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Background: Bacterial meningitis (BM) is an inflammation of the meninges, associated with the invasion of bacteria. The etiologic agents vary by age group. BM because of Group B streptococcus (GBS) is common for the neonatal period but considered as rare in adult patients. Acute BM can have various presentations and adverse effects, such as ischemic stroke in 10% to 29% of the cases. Objective: This study aimed to present a rare case of GBS meningitis presented with cerebral infarction (CI) in an adult patient and to make a brief review of the etiology and incidence of GBS infections in adults. Case Report: We present a case of a 62-year-old female who presented with acute onset of central lesion of the right facial nerve, mild hemiparesis on the right, and partial sensorimotor aphasia. There were no signs of meningoradicular irritation. The pupils were equal, with slow reaction to light, and unaffected eye movements. There was a central lesion of the right facial nerve and mild hemiparesis on the right. Tendon reflexes were unremarkable and Babinski's sign was negative bilaterally. Discussion: This review shows an increasing incidence of cases in elderly patients. A higher risk of GBS is found in adults with more medical comorbidities. CI, as a rare adverse effect in BM, is both a sign for severity and a predictor of a poor clinical outcome with a high lethal rate. GBS infections are a growing problem in older adults and those with chronic medical conditions. The involvement of the central nervous system as meningitis is a less common manifestation with a high lethal rate. Conclusions: CI is a rare adverse effect of neuro infections leading to an even worse clinical outcome. Early recognition of the infection and appropriate antimicrobial therapy are the crucial moments of successful management of GBS disease.

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Background: Huntington's Disease (HD) is an autosomal dominant, progressive neuropsychiatric illness caused by CAG repeat expansion. The high penetrance of the mutation and limited treatment options make it challenging for patients and caretakers. Proper counseling enables families to cope better and make informed life choices. Objective: To explore some complex issues in genetic counseling and testing (GCAT) in HD. Materials and Methods: Vignettes of patients who underwent genetic testing along with pre and post-test counseling at our GCAT clinic. Results: Case 1: Diagnosis of juvenile HD meant that the healthy parent was an obligate carrier of the mutation. Case 2: Consanguinity resulted in a dense prevalence of HD and >50% risk for the progeny. Case 3: Predictive testing in youth with healthy parents but affected uncles and aunts revealed a HD expansion. Conclusions: HD can present with complex inheritance patterns and proper counseling is necessary for better outcomes.

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Background: Stroke affects the quality of life through its devastating effects on physical, psychological, social, and economic domains. Patient-reported outcome measures help to capture the patient's perspective to changes in the quality of life. The available stroke-specific outcome measures of stroke do not comprehensively measure all the domains. Objective: We describe the development and validation of the Stroke Impact Assessment Questionnaire (SIAQ) an interviewer-administered new instrument developed using patient-centered approaches to assess the long-term problems of stroke survivors. Materials and Methodology: We used a sequential exploratory mixed methods approach (QUAL → QUAN) to develop the SIAQ. The design involves an initial qualitative phase aimed at generating the item pool and a subsequent second phase aimed at testing of items using quantitative techniques (Psychometric evaluation). Result: SIAQ, a thirty-item tool, under the eight domains (sensory, motor, social, economic, emotional, behavioral, cognitive, and communication) emerged from Principal Component Analysis. The factor loadings ranged from 0.421 to 0.880. The intraclass coefficient in test–retest reliability r is 0.958 (95% CI 0.9249-0.9799) and in interobserver reliability is 0.9473 (95% CI 0.9125 – 0.9733). The internal consistency Cronbach's alpha for the final 30 item tool was 0.88. Conclusion: SIAQ is a culturally appropriate psychometrically robust questionnaire assessing the long-term outcome of stroke. However, SIAQ is applicable only in stroke survivors for up to 65 years.

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Background: Central or atypical skull base osteomyelitis (CSBO) often presents with severe unrelenting headache and progressive mono or polyneuritis cranialis. MRI and CT are used as initial imaging techniques but have a poor specificity and sensitivity. Objective: To analyze our cohort of CSBO. Materials and Methods: Over a 5-year period [2015–2020], we retrospectively analyzed the records of all patients with CSBO who had undergone a 3T MRI Brain, MR angiography, regional FDG PET-CT, and skeletal scintigraphy with 99mTc MDP/SPECT-CT. Surgical biopsy specimens were sent for bacterial and fungal cultures. Results: In total, 17 patients with CSBO were identified. Typically, 88% of patients presented with severe unilateral headache. All patients had at least a cranial mono or polyneuritis. The majority of patients were diabetic [64%]. MRI was normal in 42% of patients, whereas PET-CT and with 99mTc MDP scan and SPECT-CT were abnormal in all patients. Conclusion: Our series of CSBO showed a 40% mortality rate with significant morbidity and relentless progression. Patients required repeated PET CT and bone scans to detect regression of disease activity. The average duration of IV therapy ranged from 3 weeks to 9 months and oral therapy for around 2–3 months. Cure was defined after taking into account the original diagnosis, symptom resolution, and concordant reduction of tissue uptake on PET CT and 99mTc bone scan. The combination of MRI, FDG PET CT, and 99mTc bone scan with concurrent SPECT CT was able to detect disease and disease progression in all patients.

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Background: Snakebite envenomation (SE) is an important tropical disease in India, causing significant morbidity and mortality among patients. The hormonal deficiencies due to the involvement of the pituitary in case of SE can present in either acute or delayed setting. Hypopituitarism (HP) is often an underrecognized and relatively rarely reported complication of this neglected disease. Methods: We present here the data of 15 patients diagnosed to have HP following systemic SE and are being currently followed-up in the Endocrinology outpatient department of a tertiary care hospital of South India. The study was approved by the Institute ethics committee, and informed onsent was taken from all the study patients. The study was a record-based retrospective analysis of the patients with HP following SE. Clinical data including lag time in diagnosis and type of snake were determined. Further, hormonal data including all the anterior pituitary functions (thyroid stimulating hormone, free T4, cortisol, insulin-like growth factor (IGF-1) luteinizing hormone, follicular-stimulating hormone, testosterone; prolactin) and water deprivation test to determine diabetes insipidus (DI) in patients with polyuria on follow-up were extracted from the records and the hospital information system. An experienced neuroradiologist examined the magnetic resonance imaging (MRI) findings of the pituitary. Results: The mean age of the patients was 43 ± 9 years and 80% were male. Around 90% of patients belonged to upper–lower socioeconomic status according to the modified Kuppuswamy scale. The commonest snake species reported was Russell's viper. Thirteen patients had delayed HP. The median duration from snakebite to onset of HP symptoms was 1 year (range 0.33–10 years). However, the median time from snakebite to the diagnosis of HP was 7 years (range 1–13 years). Central hypothyroidism and hypogonadism were present in all subjects. However, central hypocortisolism was noted in 93% of patients. Low IGF-1 was noted in all the six patients where data were available. One patient had partial central DI. Thirteen out of 15 patients had reduction of pituitary volume in MRI. Conclusion: HP in patients with SE can appear slowly and the diagnosis is frequently delayed for years. Following snakebite, multiple pituitary hormone deficiencies associated with radiological abnormalities like a significant reduction in the pituitary volume are common.

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Background and Aims: We evaluated dynamic changes in neurophysiology of Guillain-Barré syndrome (GBS) at different time points and the role of demyelination and axonal burden in predicting outcome. Methods: Nerve conduction study (NCS) was done in 44 GBS patients at admission and at 1 and 3 months, and were categorized into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), equivocal and in-excitable motor nerve (IMN). The demyelinating and axonal burden on motor NCS at admission, 1 and 3 months were computed and correlated with disability at 3 and 6 months. Disability was assessed using Clinical Grading Scale. Results: Twenty-four (54.3%) had AIDP, 5 (11.4%) AMAN, 12 (27.3%) equivocal and 3 (6.8%) had IMN at admission. Maximum instability was noted in equivocal group; majority of whom became AIDP at three months. Neurophysiological subtypes at different time points did not correlate with 6 months disability, but demyelination burden at admission (r = -0.42; P = 0.005) and axonal burden at one month (r = 0.43; P = 0.04) correlated with six months disability. Conclusion: Inverse correlation of axonal burden at one and three months with disability suggests role of secondary axonal damage in predicting outcome. Repeat NCS at one month helps in categorizing GBS and also in prognostication.

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Background: Despite the evolution of endoscopic techniques, large pituitary adenomas with unfavorable characteristics and irregular anatomical configurations continue to pose a challenge for the dexterity, skills, and patience of endoscopic surgeons. Transcranial surgery retains a significant role in these situations where the tumor access, hemostasis, and dissection around adjoining neurovascular tissues can be controlled efficaciously. Objective: In this report, we describe our experience with transcranial surgery for pituitary adenomas highlighting its safety and versatility in peripheral centers. Methods: We accessed the case files and imaging records of pituitary tumors operated between 2001 and 2019 at a private hospital in a major Indian city. The records were analyzed with emphasis on postoperative clinical course, visual, and endocrinological outcomes. The data was analyzed with respect to differences between transcranial and transsphenoidal procedures. Categorical variables were compared with Chi-square test/Fischer's exact test and difference in means evaluated with Welch's t-test. Results: A total of 178 procedures were performed in 173 patients with pituitary adenoma, who were the subjects of this study. Ninety-eight (56.7%) patients were treated by transsphenoidal excision whereas 80 (46.2%) underwent transcranial procedures (75 primary and five secondary). In the patients operated transcranially, we observed three deaths and nine patients suffered from significant morbidity. Visual outcomes were similar to the group operated transsphenoidally. However, incidence of panhypopituitarism was significantly higher in transcranial procedures; the extent of resection was poorer than transsphenoidal surgeries owing to more extensive nature of tumors. Conclusions: In low-volume centers, the endoscopic skills required for transsphenoidal resection of large and complex pituitary adenomas may be scarce. Transcranial surgery, dependent on familiar microsurgical techniques and equipment, may still be viable, safe, and an effective option.

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Acute-onset ophthalmoplegia is a perplexing diagnosis in a young child. When the full-blown picture of ophthalmoplegia, ataxia, and areflexia is evident, the diagnosis of Miller–Fisher syndrome (MFS), a variant of Guillain–Barre syndrome (GBS), is almost certain. However, the same is not true for isolated external ophthalmoplegia as it is etiologically heterogeneous. Only anecdotal case reports of childhood-onset acute ophthalmoplegia exist in the literature. Adult series suggest that acute onset external ophthalmoplegia is often immune-mediated and is secondary to anti-GQ1b antibodies. We present a 30-month-old boy with acute-onset bilateral external ophthalmoplegia with highly elevated serum anti-GQ1b antibodies. The child had a rapid and complete recovery with intravenous immunoglobulin. A review of all published cases of childhood anti-GQ1b antibody syndrome was performed. The case highlights that anti-GQ1b antibody syndrome should be considered even in young children with acute-onset external ophthalmoplegia. The disease has a favorable prognosis. The majority improve on conservative management. Treatment with steroids or IVIG may be considered in some after weighing the risks and benefits.

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Background: Longitudinally extensive transverse myelitis (LETM) is an immune-mediated neurological disorder affecting the spinal cord. It may be associated with the demyelination of the brain and optic nerves. Clinical features, radiological picture, and treatment outcomes vary depending on the etiology. Objectives: To assess different etiologies of LETM and analyze their differences in clinicoradiological features and treatment outcome. Materials and Methods: It is a single-center retrospective cross-sectional observational study. A total of 42 patients presented with LETM to our center for over 10 years (2010–2019) were included in this study. Their clinical, radiological, CSF findings, treatment, and neurological status (at nadir and 6 months) assessed by the EDMUS scoring system were compared and presented in this study. Results: In this study, 80% of patients were females, with the highest female predominance noted in NMOSD (87%). Among various etiologies, 16 patients had NMOSD, seven were idiopathic, five had connective tissue disease, and four were due to infective etiology. Bladder involvement was seen predominantly in patients with infective and systemic causes, whereas respiratory muscle involvement was seen predominantly with infective etiology. More than six-segment involvement was seen predominantly in idiopathic cases. All Ro-52 positive patients had relapses. NMOSD had a better neurological outcome than other etiology. Conclusions: NMOSD is the most common cause of LETM, with a good neurological outcome at 6 months, while infective etiology had a poor neurological recovery. NMOSD with Ro-52 antibody positivity had relapses despite being on immunosuppressant therapy.

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Background: Outcome of Isolated ventriculomegaly diagnosed antenatally depends on size of ventricles and associated malformations. There is scarcity of literature on the guidelines for prognostication based on outcomes as per the ventricle size. Aim: The aim of this work was to study outcome of antenatally detected isolated ventriculomegaly in terms of medical termination, postnatal neuro-developmental milestones, and mortality; and also to propose a new prognostication classification. Methods: Prospective and retrospective observational study on antenatal mothers with isolated ventriculomegaly diagnosed in fetus. Outcomes in terms of termination of pregnancy, postnatal mortality, need of surgery, and morbidity were recorded. Patients were categorized into four groups: Group 1––ventricle size <10 mm, II––11–15 mm, III––16–20 mm, and IV > 20 mm and neuro-developmental milestones were co-related. Association with chromosomal anomalies, congenital heart disease, and maternal infection were also analyzed. Results: 521 antenatal females were referred with fetal anomalies with 163 having CNS malformations. Isolated ventriculomegaly was seen in only 44. Patients of groups 1 and 2 had 100% normal neuro-developmental milestones without any intervention. Group 3 patients had normal neurodevelopmental milestones in 60% only while shunt surgery was required in 40% of patients. All patients of group 4 had poor outcome with only 50% survival. No association with chromosomal anomalies and heart disease was found. Conclusions: Prognosis of isolated ventriculomegaly depends upon size of ventricles and its progressive increase on serial ultrasounds. New proposed classification is simple and would be useful for the treating surgeons to explain the prognosis to parents so as to relieve them of anxiety.

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Background: Yoga is increasingly being used as a complementary mode of treatment for epilepsy along with pharmacotherapy; however, the quality of reporting and bias of studies on yoga in epilepsy is uncertain. Objective: This study was performed to systematically evaluate the reporting quality and bias of the published studies on yoga in epilepsy. Materials and methods: PubMed and Cochrane databases were searched for studies investigating the efficacy of yoga in epilepsy or describing the patients' or caregivers' knowledge, attitude, influence, and practice of yoga in epilepsy. The reporting qualities of the studies were appraised by standard reporting checklists (CONSORT, STROBE, COREQ, and modified CONSORT) and the risks of bias were assessed using standard tools (Cochrane Collaboration's risk of bias tool, Newcastle-Ottawa scale, and ROBINS-I tool) according to the study designs. Results: Fifteen studies (four randomized control trials, seven observational studies, three non-randomized interventional studies, and one was a mixed-method study) were included. The reporting qualities of the studies which evaluated the role of yoga in epilepsy were comparatively poor from the methodological perspective and the risks of bias were comparatively high than those which described the knowledge, attitude, influence, and practice of yoga in epilepsy. Conclusion: The reporting qualities of studies in relation to yoga and epilepsy were not satisfactory and the risks of bias were high. Hence, it is imperative to be cautious before widely recommending yoga in epilepsy.

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Coffin-Siris syndrome (CSS) (OMIM #135900) involves multiple congenital malformations, including hypotonia, short stature, sparse scalp hair, a coarse face, prominent eyebrows, a wide mouth, delayed bone age, and hypoplastic or absent fifth fingers/toes or nails, together with developmental delay. The cause of CSS is suggested to be related to alterations in the BRG- or HRBM-associated factor (BAF) pathway in humans. In this gene family, pathogenic variations in the AT-rich interactive domain-containing protein 1B (ARID1B) gene are revealed to be a significant element causing neurodevelopmental disability in patients with CSS. Herein, we describe the clinical features and gene variations in four Chinese patients with CSS. All the patients shared common features of short fifth fingers/toes or hypoplastic nails, coarse facial features, thick eyebrows, long cilia, a flat nasal bridge, a broad nose, a wide mouth, a high palate, and hypotonia. Besides, they had an intellectual disability, language, and motor developmental delay. Candidate genes were screened for variations using polymerase chain reaction (PCR) and sequencing. The variations were sequenced by next-generation sequencing and confirmed by first-generation sequencing. Exome sequencing suggested four de novo variations in the ARID1B gene in four unrelated patients. These included two frameshift variations (c.3581delC, c.6661_6662insG) and two nonsense variations (c.1936C>T, c.2248C>T). Of the four variations, three variations were novel. The results in our present study broaden the understanding of the disease and further interpret the molecular genetic mechanism of these rare variations in CSS.

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Background: Migraine is one of the primary headaches having a global prevalence of 15%. It is characterized by neurovascular dysfunction and recurrent episodes of headache. The hyperexcitability of the cerebral cortex has been recognized as an important factor in the pathogenesis of migraine, and magnesium (Mg) being a regulator of neuronal excitability is thought to participate in migraine pathogenesis. Objectives: To determine the serum levels of Mg in patients of migraine during the attack and in between attacks as compared to healthy controls. Methods: A total of 50 patients of migraine who fulfilled inclusion criteria were enrolled in the study along with the same number of healthy controls. International Classification of Headache Disorders 3^rd Edition, 2013 (ICHD-III) criteria was used for the diagnosis of migraine. Results: The mean serum Mg in migraine cases during the interictal phase was lower than healthy controls (1.849 ± 0.135 vs 2.090 ± 0.205, P < 0.001), which was statistically significant. It was also found that mean serum Mg during attacks was significantly lower than in between attacks (1.822 ± 0.149 vs 1.849 ± 0.135, P = 0.003). Serum Mg levels in migraine cases showed an inverse linear relationship with the frequency of attacks. Conclusion: Relatively low serum Mg in migraine cases when compared with healthy controls and inverse relation of serum Mg levels with the frequency of migraine attacks suggests that Mg is significantly involved in mechanisms underlying migraine pathogenesis, which can be explored as a therapeutic option.

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Background: Increased incidence of cardiovascular disease, including stroke, has been consistently observed in patients with chronic inflammatory diseases, although data on antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are still limited due to the relative rarity of the disease. Methods: Two investigators independently searched published studies indexed in MEDLINE and EMBASE database from inception to June 2019 using the search terms related to AAV and stroke. The eligible study must be cohort study that consisted of cohort of patients with AAV and cohort of patients without AAV. The study must follow the participants for incident stroke. The magnitude of difference in the incidence of stoke between the cohorts must be reported. Pooled effect estimates were calculated by combining the effect estimate of each eligible study using generic inverse variance method. Statistical heterogeneity was assessed using the Cochran's Q test and I² statistics. All analyses were conducted using RevMan 5.3 software from the Cochrane Collaboration. Results: A total of six cohort studies fulfilled the eligibility criteria and were included into the meta-analysis. Patients with AAV had a higher risk of developing incident stroke than individuals without AAV with the a pooled risk ratio of 2.02 (95% CI, 1.02–4.00; I² of 89%). Funnel revealed no suggestive evidence of publication bias. Conclusion: A significantly higher risk of incident stroke among patients with AAV than individuals without AAV was demonstrated by this meta-analysis.

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Background: Telemedicine (TM) consultations have shown to be feasible for the management of neurological conditions including movement disorders. In contrast, satisfaction with such consultations have been less studied. Objective: To assess the satisfaction of persons with a movement disorder with a TM consultation in comparison to previous experiences in face-to-face visits. Subjects and Methods: A cross-sectional multicenter study was carried out. Persons with a diagnosis of a movement disorder underwent a TM consultation. After the consultation concluded, a satisfaction survey was sent for the subject to fill out anonymously. The survey included ease of use-related items, setup-related items, and quality-of-service-related items. Results: A total of 175 survey responses were received (response rate of 71.4%), all of which were included for analysis. A total of 102 subjects considered that the TM consultation involved much less time in comparison to their previous experience with face-to-face visits. Overall, 96% reported to be satisfied with the consultation. In addition, 92% were satisfied or very satisfied with the neurologist ability to communicate recommendations. Furthermore, 93.7% indicated that the consultation was valuable, and 90.9% considered that they would recommend teleconsultation to another patient. Conclusion: Patients with a diagnosis of a movement disorder consider TM as a convenient and potential tool for health services with a high level of satisfaction.

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Background: Trace elements have been implicated in pathogenesis of epilepsy. Studies till date have shown altered levels of serum trace elements in children with epilepsy. Objective: The objective of the current was to estimate serum levels of trace elements in children with well-controlled and drug refractory epilepsy and compare it with controls. Methodology: In a tertiary care teaching hospital of North India, serum selenium, copper, zinc, and iron were estimated in well-controlled and drug refractory epileptic children aged 2–12 years and compared with age and gender matched controls. Results: A total of 106 children with epilepsy (55 drug refractory and 51 well controlled) and 52 age and gender matched controls were included in the study. Serum selenium and copper were significantly decreased in cases compared to controls. After classifying epilepsy into well-controlled and drug refractory cases, only in the latter the significant difference for serum selenium and copper levels remained compared to controls. Additionally, in the drug refractory cases, serum iron levels were significantly reduced compared to controls. Conclusions: Serum trace elements are altered in children with epilepsy (more so in the drug refractory group) compared to controls. Monitoring of serum trace elements in children with epilepsy should be considered. Up to one-third of epilepsy is drug refractory of which only another third are amenable to surgery. It is worth investigating the therapeutic potential of altered micronutrient status in these patients.

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Ventriculoperitoneal shunt insertion is one of the most common pediatric neurosurgical procedures performed. Shunt migration is one of the infrequent complications of shunt insertion and often requires a change of the shunt system. The objective of the study is to systematically review the sites of shunt migration and factors associated with mortality in children. Comprehensive search and review of the literature were done according to the PRISMA guidelines. Citations were selected using the following inclusion criteria: 1. Shunt migration in ventriculoperitoneal shunts was the primary inclusion criteria, 2. Age of the study participants <18 years, and 3. Patient-level data available in the study. Exclusion criteria were 1. Age >18 years, 2. Patient-level data not available, 3. Full text of the article not available, and 4. Article not in the English language. Sites of migration and risk factors for mortality were assessed. In total, 111 studies out of 161 studies were included in the final analysis. The scrotum was the most common site of shunt migration (30.67%), followed by anal migration, migration into the bowel, chest wall/thoracic migration, and intracranial/subgaleal migration. Univariate analysis showed the presence of infection and site of migration to be significantly associated with mortality. Multivariate analysis showed the presence of infection and age at presentation to be significant predictors of mortality. This study highlights that presence of infection is a significant predictor of mortality in cases of shunt migration. Infection should be managed expeditiously for optimum management of shunt migration.

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Sixteen syndrome is rare variant of one and a half syndrome resulting from lesion affecting bilateral dorsal pontine structures. This report describes a case of 16 syndrome in systemic lupus erythematosus (SLE). A 28 year old woman with underlying lupus was presented with sudden left side body weakness and diplopia. Examination showed features of 16 syndrome with one and a half syndrome, facial diplegia and left hemiparesis. Neuroimaging revealed an acute infarct of bilateral dorsal pons. The patient was placed on antiplatelet therapy. However, she developed left leg deep vein thrombosis and pulmonary embolism after one week. She was then given subcutaneous low molecular weight heparin, followed by warfarin and cycles of cyclophosphamide. Her hemiparesis, ocular symptoms, and facial diplegia improved after one year. SLE increases the risk of stroke and thrombotic events in a young patient. Achieving disease remission is important to prevent stroke in SLE patients.

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Background: Severe dysautonomia is typically seen during acute phase of Guillain–Barré syndrome (GBS). Objective: To investigate the relationship of cardiovascular autonomic dysfunction with motor recovery in GBS. Materials and Methods: Consecutive GBS patients presented to our hospital were recruited. Clinical assessment was evaluated with the Medical Research Council (MRC) sum score and GBS disability score (GDS). All patients had series of autonomic testing on admission and after treatment at 6 and 24 weeks. Both computation-dependent tests (heart rate variability [HRV] and baroreflex sensitivity [BRS]) and autonomic maneuvers were performed. Age- and gender-matched healthy controls (HC) were recruited. The data obtained at admission, 6 weeks and 24 weeks were compared within groups for statistical difference. Results: Six patients (4 men; mean age 39.5 ± 14.3 years) were recruited over one year. Five had GBS and one Miller Fisher syndrome. The mean MRC sum score and GDS on admission were 52.3 ± 4.3 and 3.5 ± 0.8 respectively. During admission, time-domain average RR interval (AVNN) and BRS were significantly poorer among cases compared to HC. Active standing 30:15 ratio and cold pressor test at admission were also significantly abnormal when compared with HC. All the autonomic parameters had normalized by 6 weeks and these were significant for the high frequency-HRV, BRS, and active standing 30:15 ratio. For MRC and GDS, there were significant improvements in the scoring over a period of 24 weeks. Conclusions: Dysautonomia in GBS improved gradually and in keeping with motor and disability recovery.

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Background: Guillain Barre syndrome (GBS) is an immune-mediated peripheral neuropathy characterized by the demyelination and axonal damage of the peripheral neurons. The pathogenesis of GBS involves the breakdown of the blood-brain barrier after which pro inflammatory cytokines attack the neurons in the peripheral nervous system. Aims: This study aims to evaluate five markers, namely matrix metalloproteinase (MMP)-2 and MMP-9, vascular endothelial growth factor (VEGF)-A, basic fibroblast growth factor (bFGF), and SFLT-1, which could have a role in the inflammatory response in patients with GBS and healthy controls. Settings and Design: In this prospective study, patients diagnosed with GBS at the department of neurology, Kasturba Medical College, Manipal, Karnataka were enrolled. Methods and Material: The markers selected for this study were analyzed using the ELISA method and expressed as given in the kit provided by the company. Ethical clearance was obtained from the Institutional Ethical Committee. Statistical Analysis: Results were evaluated using SPSS version 17.0 and expressed as mean ± SD. Error bars for each were drawn. Results: The levels of all five parameters showed a significant increase in patients as compared to controls. Conclusions: Disruption of the basement membrane of endoneurium by MMP-2 and MMP-9, recruitment and migration of macrophages and other cytokines by VEGF-A, bFGF, and soluble fms-like tyrosine kinase-1 (SFLT-1) are plausible, which leads to inflammation process and thus neuronal damage leading to the development of GBS.

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Anoctaminopathies are a group of autosomal recessive skeletal muscle disorders with various clinical phenotypes, caused by anoctamin 5 (ANO5) gene mutations and the abnormal expression of ANO5 protein. Patients with recessive mutations in ANO5 present with variable symptoms ranging from asymptomatic hyperCKemia and exercise-induced myalgia to proximal and/or distal muscle weakness. Here, we describe the clinical, pathological, and molecular findings of two unrelated patients with ANO5-related muscular dystrophy (MD). Ninety-six histologically identified MD cases were subjected to next-generation sequencing using a customized panel of 54 genes (IIlumina Design Studio). Two patients were diagnosed with ANO5-related MD. One patient had a pathogenic homozygous mutation of c.1406G>A in exon 14, while the other patient had a novel heterozygous mutation of c.2141C>G in exon 19 of ANO5 gene. Both showed two different phenotypes (limb girdle MD and Miyoshi myopathy) and histomorphological patterns. Muscle biopsy of one patient in addition showed amyloid deposit in the walls of interstitial blood vessels. ANO5-related MD is a heterogeneous disease with different clinical phenotypes as well as genotypes. All muscle biopsies with unclassified muscular dystrophies should be subjected to Congo red stain. The results of this study suggest that screening for ANO5 gene should represent an early step in the diagnostic work-up of the patients with undiagnosed MD and persistent asymptomatic hyperCKemia, even when muscle biopsy histomorphology is normal.

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Objectives: Different variant of GBM has been reported viz. Epithelioid Glioblastoma (GBM-E), Rhabdoid GBM (GBM-R), Small cell GBM (GBM-SC), Giant cell GBM (GBM-GC), GBM with neuro ectodermal differentiation (GBM-PNET) with unknown behavior. Materials: We conducted a systematic review and individual patient data analysis of these rare GBM variants. We searched PubMed, google search, and Cochrane library for eligible studies till July 1^st 2016 published in English language and collected data regarding age, sex, subtype and treatment received, Progression Free Survival (PFS), Overall Survival (OS). Statistical Package for social sciences (SPSS) v16 software was used for all statistical analysis. Results: We retrieved data of 196 patients with rare GBM subtypes. Among these GBM-GC is commonest (51%), followed by GBM-R (19%), GBM-PNET (13%), GBM-SC (9%) and GBM-E (8%). Median age at diagnosis was 38, 40, 43.5, 69.5 and 18 years, respectively. Male: female ratio was 2:1 for GBM-E, and 1:3 for GBM-SC. Maximal safe resection followed by adjuvant local radiation was used for most of the patients. However, 6 patients with GBM-PNET, 3 each of GBM-E, GBM-SC received adjuvant craniospinal radiation. Out of 88 patients who received chemotherapy, 64 received Temozolomide alone or combination chemotherapy containing Temozolomide. Median PFS and OS for the entire cohort were 9 and 16 months. In univariate analysis, patient with a Gross Total Resection had significantly better PFS and OS compared to those with a Sub Total Resection [23 vs. 13 months (p-0.01)]. Median OS for GBM PNET, GBM-GC, GBM-SC, GBM-R and GBM-E were 32, 18.3, 11, 12 and 7.7 months, respectively (P = 0.001). Interestingly, 31.3%, 37.8% of patients with GBM-E, GBM-R had CSF dissemination. Conclusion: Overall cohort of rarer GBM variant has equivalent survival compared to GBM not otherwise specified. However, epithelioid and Rhabdoid GBM has worst survival and one third shows CSF dissemination.

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Background: Though frontal lobe contusion is a major cause for morbidity and prolonged hospitalization following excision of anterior skull base meningiomas, there is only limited literature on this complication. This study aimed to find out the incidence of postoperative frontal lobe contusion, identify the risk factors for its development and its impact on early postoperative outcome. Methods: Data from 110 patients who underwent excision of anterior skull base meningiomas through a unilateral supraorbital craniotomy from 2001 to 2018 were retrospectively analyzed. The risk factors analyzed for development of postoperative contusion were tumor location, size, volume, peritumoral edema, tumor consistency, extent of resection, tumor grade and type of retraction used. Results: Simpson grade II excision was achieved in ninety-two patients (83.6%). There was no frontal lobe contusion in eighty-two patients (74.5%). Frontal lobe contusion was noted in twenty-eight patients (25.5%), but was severe in only four patients (3.6%). On multivariate analysis, fixed retractor use (OR 11.56 [1.21–110.03]; P =0.03) and WHO grade II tumor (OR 3.29 [1.11–9.77]; P =0.03) were independently associated with postoperative frontal lobe contusion. Patients with higher contusion grade had significantly longer postoperative hospitalization (P =.02) and lower KPS score at discharge (P =.01). Conclusions: Unilateral supraorbital craniotomy and lateral subfrontal approach is an excellent procedure for excision of anterior skull base meningiomas with minimal postoperative complications related to frontal lobe retraction. Frontal lobe contusion should be avoided with the use of dynamic retraction, since postoperative contusion prolongs hospitalization and worsens the functional outcome at discharge.

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Background and Aim: Basilar artery occlusion (BAO) is known for its catastrophic outcomes, whether death or disability, in approximately 70% of patients. Mechanical thrombectomy (MT) has been approved as an intervention in large vessel occlusion of anterior circulation, based on multiple randomized controlled trials (RCTs) and meta-analyses. Even though two RCTs appeared recently, there is still uncertainty about the effect of MT in BAO. Our study aims to report the outcome of MT in BAO and the variables affecting good outcomes and mortality rate. Materials and Methods: We retrospectively collected the clinical and radiological data of 30 BAO patients treated in our center by MT between July 2016 and July 2021. A favorable clinical outcome was considered if mRS was ≤2. A favorable radiological result was considered if modified Thrombolysis in Cerebral Infarction (mTICI) was ≥2b at the end of the intervention. Multiple variables were tested for their effects on favorable clinical outcomes and mortality. Results: The mean age of the 30 patients was 61.23 ± 16.81 years; 20/30 (66.7%) were male. A favorable functional outcome was achieved in 40.7%. Successful revascularization was achieved in 26 patients (86.7%). Mortality at 90 days was observed in 11 patients (36.7%). The presenting National Institute of Health and Stroke Scale (NIHSS) was the only predictor of mortality, and the optimal cut-off value for death was 15 with area under the curve (AUC) = 0.758 (sensitivity 91% and specificity 59%) and P value = 0.02. Conclusion: Thrombectomy is an effective procedure in BAO which has naturally a bad outcome. The presenting NIHSS might be the only predictor of mortality in our study.

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Chordoid meningioma, classified as WHO grade II, are rare tumors comprising only 0.5% of all meningiomas. Chordoid meningioma is an aggressive tumor with high local recurrence. Orbital chordoid meningioma is a much rare entity with very few cases reported in the literature. We report a case of a 77-year-old male who presented with a painless progressive swelling over the right lateral orbital wall.

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Introduction: Surgical management of craniopharyngioma is debatable and still lacks clear guidelines. Long-term complications are attributed to radical resection of the tumor. Extent of resection may not be the only factor which determines the functional outcome, because studies have reported hypothalamic and visual morbidities even with conservative resection. In this article, we analyze the extent of resection, long-term outcome, and various prognostic factors in adults and children. Materials and Methods: Newly diagnosed cases of craniopharyngioma operated between 2001 and 2013 were reviewed retrospectively. PFS and OS were calculated. Predictors of various outcome parameters were analyzed. Results: Of 140 patients, 41% were children and 59% were adults. Postoperatively, vision has improved in 63% and worsened in 12%. GTR was achieved in 66%. The median follow-up was 67 months. PFS at 5 and 10 years was 95% and 41.5%, respectively. OS at 5 and 10 years was 92.6% and 89.5%, respectively. Symptoms of raised ICP and hypothalamic involvement, extensive calcifications, tumor size >5 cm, and multi-compartmental tumors were associated with presence of residual tumor. Regression analysis showed symptoms of hypothalamic involvement, size of the tumor > 5 cm, and hydrocephalus predicted postoperative hypothalamic morbidities. No significant difference was found in postoperative visual, endocrinological, hypothalamic, and functional outcomes between GTR and STR. Conclusion: Conservative resection should be carried out in patients with preoperative hypothalamic symptoms and imaging evidence of extensive hypothalamic involvement. Size of the tumor and invasiveness are the other factors should be considered before radical excision of craniopharyngiomas.

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Background: Hypertensive intracerebral hemorrhage (HICH) seriously endangers the quality of life of patients and can even lead to death. Craniotomy is a common treatment method for HICH. Objective: The aim of this study was to investigate the efficacy of two different sizes of craniotomy in patients with HICH, as well as to evaluate their effects on C-reactive protein (CRP) and blood lactate levels. Materials and Methods: A total of 72 patients with HICH in the basal ganglia were operated on in our hospital from February 2017 to March 2019 and randomly divided into two groups: the small bone window (SBW) group (n = 37) and the large bone flap group (n = 35). The curative effects of the two kinds of operations were evaluated by the length of operation, the days of hospitalization, the rate of hematoma clearance, the rate of rebleeding, and the incidence of complications. Additionally, the levels of CRP and lactate were compared between the two groups. Results: The results showed that the average intraoperative time, hospital stay, rebleeding rate, and postoperative complications of patients in the SBW group were less than those in the large bone flap group. Moreover, the number of patients in the SBW group with good postoperative recovery, including class V and class IV, was higher than that in the large bone flap group. Minimally invasive craniotomy with SBW reduced the lactic acid and CRP levels more quickly than the large bone flap group. Conclusions: An SBW was superior to a large bone flap in terms of the operative effect and lactate and CRP levels. It is concluded that an SBW has significant advantages over a large bone flap.

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An intimate understanding of dynamic angioarchitectural development of vascular malformations involving the intracranium may provide mechanistic insight into the incipient pathogenesis of these lesions. The authors report two patients harboring cerebral developmental venous anomaly (DVA) and three patients harboring sporadically developing cerebral arteriovenous malformations (AVM) in whom the internal cerebral vein (ICV) represented the chief venous egress route. Onyx embolization successfully achieved complete obliteration in all patients harboring cerebral AVMs. Two female patients presenting with a chief complaint of chronic headaches was found to harbor deeply situated DVA draining via the lateral group of direct lateral vein (DLV) egress via the ICV. Three female patients presenting with chronic headaches or intraparenchymal hemorrhage were found to possess cerebral AVMs deriving arterial feeders from the anterior cerebral arteries, with major venous drainage into the ICV via the direct lateral veins or terminal vein. Common drainage of DVAs and AVMs may indicate a common originate genesis. This pattern of venous drainage in the context of seeking to develop a cohesive and coherent model illumining our understanding of the mechanistic incipient pathogenesis of AVMs.

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Background: Severe peri-ictal respiratory dysfunction is a potential biomarker for high SUDEP risk and correlates with an attenuated hypercapnic ventilatory response (HCVR). Prior studies suggest a potential role for selective serotonergic reuptake inhibitors in modifying the HCVR, but this approach has not been studied in the epilepsy population. Objectives: To assess the feasibility of using fluoxetine to augment HCVR in epilepsy patients. Methods and Material: An inter-ictal HCVR was measured using a CO₂ rebreathing technique in patients with epilepsy aged 18–75 years. Eligible participants were randomized to fluoxetine or placebo, and the HCVR was repeated at the end of week 4. Primary outcomes were recruitment and retention rate. Results: Of the 30 subjects enrolled, 22 were randomized (mean: 3.8 subjects/3 months), with a retention rate of 100% in fluoxetine and 95% in placebo. Conclusions: Our results demonstrate feasibility for a larger definitive future study to assess the efficacy of fluoxetine in augmenting HCVR.

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Background: Neural tube defect (NTD) is a multifactorial disorder. Decrease transfer of folate to the developing embryo is one of the etiologies. It could be due to decrease folate receptors resulting in NTD in fetus. Objective: To analyze serum folate receptor alpha (FOLR1) concentration in women having fetus with NTD and compare it with women having normal fetus during and after pregnancy. Material and Methods: This was a prospective case control study conducted in a tertiary care hospital. Pregnant women with detected isolated NTD in fetus were enrolled as cases and equal number of matched pregnant women without any fetal congenital malformation were recruited as controls. Serum FOLR1 levels were analyzed in cases and controls during pregnancy and 6 weeks after delivery. Results: Mean serum FOLR1 concentration during pregnancy was 70.5 pg/mL (range: 23.8–98.5 pg/mL) and 103.9 pg/mL (range: 70-110 pg/mL) in cases and controls, respectively. Serum level of FOLR1 was 448.9 pg/mL (range: 133.5–475) and 414.5 pg/mL (range: 269.7–412.5) in cases and controls at 6 weeks postpartum, respectively. There was statistically significant difference (P < 0.001) between cases and control during pregnancy but not in postpartum (P = 0.092). There was significant increase in level of FOLR1 in both cases and control at 6 weeks postpartum as compared to antenatal period. Conclusions: Maternal serum FOLR1 is significantly reduced in pregnancy with fetal NTD as compared to normal pregnancy. The level is significantly increased in postpartum period in both groups. FOLR1 level being similar in both groups in postpartum indicates that it is not influenced by the history of fetal NTD.

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Background: Human leukocyte antigen (HLA) is associated with drug-induced cutaneous adverse reactions, including antiepileptic drugs (AEDs). HLA gene polymorphism has a regional discrepancy, and it is therefore important to study it in different populations. Objective: To investigate the role of HLA in AED-induced mild cutaneous adverse drug reactions (cADRs) in a Northeast Han Chinese population. Methods: A case-control study was performed in the First Hospital of Jilin University between August 2016 and March 2017. In total, 26 patients with mild cADRs induced by AEDs and 23 AED-tolerant control patients were included. Sequence-based typing (SBT) was used to detect HLA-A and HLA-B genotypes. Differences in genotype frequencies between groups were assessed using Fisher's exact test. Results: In the mild cADRs group, 22 patients (84.6%) presented with maculopapular exanthema (MPE) and four patients (15.4%) presented with an isolated itch. The median duration between the AED exposure and cADRs was 7.5 days (IQR, 3 − 14 days). We failed to find statistically significant differences in HLA alleles between the cADRs group and the control group when considering all the drugs included in our study together or when considering oxcarbazepine (OXC), carbamazepine (CBZ), and levetiracetam (LEV) alone (P > 0.05). Conclusions: Our findings indicated that there was no correlation between HLA alleles and AED-induced mild cADRs in the Northeast Han Chinese population.

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Background: This pilot survey aims to provide an insight into the variations of corticosteroid prescription among health care professionals across the Indian subcontinent and serve as a prerequisite for the future development of corticosteroid therapy guidelines in brain tumor patients. Materials and Methods: Participants of this anonymized online questionnaire-based survey included health care professionals involved in treating brain tumor patients. Unique links were electronically mailed to health care professionals from a database populated from professional associations. Descriptive statistical analysis, Chi-square test, and/or exact test were used for data analysis. Results: Seventy-three percent of the respondents were radiation oncologists followed by neurosurgeons (23%), medical oncologists (2%), and other specialties (2%). Raised intracranial pressure (90%) was the commonest indication for prescribing corticosteroids. Fifty percent of neurosurgeons preferred corticosteroids to be given routinely for all patients undergoing surgery for brain tumors while 64% of the radiation oncologists would prescribe based on imaging findings and/or on appearance of neurologic symptoms. Most of the respondents (90%) used a flat dosage pattern for determining the starting dose of corticosteroids. Emerging data about the long-term use of corticosteroids having a negative impact on the survival of brain tumor patients were not known by 52% of the respondents. The majority of the practitioners (94%) agreed regarding the formulation of evidence-based guidelines for prescribing corticosteroids in brain tumor patients. Conclusion: In view of the wide variations of corticosteroid therapy practices among health care professionals across various parts of the world, our pilot survey provides significant information which can act as a suitable benchmark to form uniform practice guidelines.

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Background: Intracerebral hemorrhage is a leading cause of death and disability worldwide. After intracerebral hemorrhage, cerebral blood flow (CBF) becomes extremely low approaching ischemic thresholds. Concurrently, CBF velocities become strongly correlated to CBF itself post-injury. Identification of such hemodynamic disturbances can be used to predict outcome immediately post-injury when indices are measured using transcranial doppler ultrasonography (TCD). TCD permits non-invasive assessment of different CBF velocities as well as pulsatility index (PI). Abnormal measurement of such indices is believed to correlate to poor outcome. Aim: To investigate the effect of cerebral hemodynamics after cranioplasty in decompressive craniectomy patients using pre and postoperative TCD. Materials and Methods: This study is a prospective study of 3 years duration undertaken on 64 patients. All the patients were evaluated by TCDbefore and after decompressive craniectomy.All patients were evaluated by transcranial Doppler (TCD) 1 week before and 7–15 days after cranioplasty. TCD results were obtained though trans-temporal approach. Results: Statistically significant differences between the values before and after craniectomy were detected in Peak Systolic Volume (PSV) for the Anterior Cerebral Artery (ACA) (P = 0.001), in PSV for the Middle Cerebral Artery (MCA) (P < 0.004), in Mean Bloodflow Velocity (MV) for the MCA (P < 0.003), and in PSV for the Posterior Cerebral artery (PCA) (P = 0.001) on the ipsilateral side. There were statistically significant differences between the values before and after cranioplasty in PSV for the PCA (P = 0.004), on contralateral side.After decompressive craniectomy, the PI values for the MCA decreased, on average, to 31+/- 33% of the pre-surgical value in the treated side and to 28+/- 31% on the opposite side. On the other hand, the mean PI values for the extracranial ICA reduced to 34+/- 21% of the initial values in the treated side, and to 21+/- 31% on the opposite side.Cranioplasty improved CBF velocities in all major intracrainal arteries, not only on the side of the lesion adjacent to the cranioplasty, but also in distant regions, such as in the contralateral hemisphere. Conclusion: Decompressive craniectomy significantly improves cerebral hemodynamics both on ipsilateral and contralateral cerebral hemispheres.Concomitantly, PI values on TCD decrease significantly postoperatively, mainly in the decompressed cerebral hemisphere, indicating reduction in cerebrovascular resistance. We conclude that cranioplasty improves neurological status and the mechanism of postoperative improvement of neurological status may be due to increased CBF velocity.

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Conventionally, the working projection for aneurysm coiling demonstrates the inflow artery, the aneurysm dome, the neck, and bifurcation branches in a two-dimensional (2D) image. We report two cases of ruptured Anterior communicating artery aneurysms, in whom a conventional working projection could not be obtained in a single plane angiography system. We used a down-the-barrel working projection created by aligning the origins of the A2 segments of bilateral anterior cerebral arteries to complete the coiling procedure. The translucent volume-rendered image obtained using three-dimensional rotational angiography was utilized to create a Down-the-barrel view. This strategy may be employed by operators performing neuroendovascular procedures in single plane angiosuites when a conventional working projection strategy fails.

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Background: Coronavirus is a novel virus which has disrupted life in the past year. While it involves the lungs in the majority and this has been extensively studied, it involves other organ systems. More number of studies need to be focused on the extrapulmonary manifestations of the disease. Objective: To delineate the clinical manifestations of coronavirus disease 2019 (COVID-19) virus on the central and peripheral nervous systems and to assess the risk factors and the outcome of COVID-19 patients with neurological manifestations. Materials and Methods: All patients who were SARS-CoV-2 RNA polymerase chain reaction (PCR) positive were assessed, and detailed clinical history and laboratory findings were collected. Data was analyzed using percentage, mean, and frequency. Results: Out of 864 patients, 17 (N = 17, 1.96%) had neurological manifestations. Twelve out of 17 had comorbid conditions. Patients had diverse presentations ranging from acute cerebrovascular accident to paraplegia and encephalopathy. Ten (58.8%) patients presented with acute cerebrovascular accidents. Of the patients who developed stroke, five (50%) died. Conclusions: COVID-19 usually presents as a respiratory disease. The neurological manifestations of COVID-19 are not uncommon. One should be aware of a wide spectrum of neurological signs and symptoms of COVID-19 for early diagnosis and treatment for preventing mortality and morbidity.

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Background: Microvascular decompression (MVD) has become widely accepted for hemifacial spasm (HFS). The significance of facial nerve notch (FNN) for the outcome after MVD is still unknown. Objective: To investigate the correlative analysis between the FNN at the root exit zone (REZ) and the curative effect of MVD for HFS. Method: A prospective study was performed, which included 232 patients with Cohen score ≥2 who had undergone MVD. The patients were divided into two groups: the group with facial nerve notch (FNN) and the group without FNN, according to whether there is FNN caused by the offending vessels during the MVD operation. All the patients were followed up for 24 months. Results: The group with FNN caused by offending vessels at the REZ area was confirmed in 119 patients, of which 117 patients were immediately cured and two patients received partial relief on the 1^st day of postoperation. The group without FNN at the REZ area was confirmed in 113 patients, of which 43 patients were immediately completely cured while 70 patients were not immediately completely cured after MVD operation. After 24 months of follow-up, 119 patients with FNN were all completely cured, 71 patients without FNN were completely cured, and 42 patients without FNN were not completely cured. Multivariate logistic regression analysis shows that compared with without FNN group, the FNN group had statistical significance for the clinical results both on the-1^st -day (P < 0.01) and 2 years of follow-up (P < 0.05) after MVD operation. Conclusions: FNN is an important reference factor to evaluate both the short-term and long-term curative effect of microvascular decompression for hemifacial spasm. The postoperative effect of patients with FNN is much better than that of patients without FNN.

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Background: Among screening instruments used to diagnose dementia by interviewing the caregivers rather than the patients, Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE) is widely used. Objectives: To adapt and validate IQCODE in a Malayalam-speaking population and to study the utility of IQCODE in diagnosing dementia. Materials and Methods: Cross-sectional study conducted as a two-phase survey on individuals aged ≥55 years availing dementia care services at three tertiary care hospitals and a dementia clinic in Kerala, India. In the first phase, modifications were made in the questionnaire (IQCODE) in accordance with local social and cultural practices. The questionnaire was then translated to Malayalam and back-translated to English. In the second phase, this was administered to healthy elderly subjects and close caretakers of dementia patients. These patients underwent neuropsychology evaluation consisting of mini-mental state examination (MMSE) and Addenbrooke's cognition examination-Malayalam (ACE-M), and the statistical correlation between mean IQCODE scores and ACE-M scores was assessed using ANOVA test. Results: A total of 396 patients with dementia were included in the study. The mean age group was 72.54 ± 7.11 years. The mean IQCODE score was 3.93 ± 0.68, and the mean ACE-M score was 39.96 ± 23.41. It has a statistically significant correlation with the total score of MMSE and ACE-M scales, confirming its utility as a good screening instrument. Conclusion: The IQCODE could be adapted and translated to Malayalam without making any major changes in its content and has good acceptance among this cohort of hospital-based subjects.

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Background: Although the asterion has long been used as a skeletal surface marker of the transverse-sigmoid sinuses junction (TSSJ) point in the retrosigmoid approach, abundant evidence shows that the relationship between asterion and TSSJ point varies greatly. In recent years, new technologies have been developed, such as neuronavigation and three-dimensional volume rendering imaging, that can guide in exposing the TSSJ point individually. However, they are not only expensive but also difficult to apply in emergency surgery. Objective: To introduce a quick, practical, and low-cost new method for locating the TSSJ point precisely. Methods: In this retrospective before-after study, the test group located the TSSJ point with our new method during a 6-month period, while the control group used asterion as a surface landmark to estimate the TSSJ during the preceding 6 months. The primary outcome is the immediate exposure rate of the TSSJ point by the initial burr hole. Results: There were 60 patients in both control and test groups as no significant difference in the general clinical characteristics of both groups were observed. The new three-step method significantly increased the TSSJ exposure rate by initial burr hole compared with the control group (96.67% vs. 53.33%, P = 0.0002). Moreover, the total bone loss and craniotomy duration were significantly reduced by the new method. Incidence of sinus injury (10% vs. 6.6%), post-operation infection (3.33% vs. 3.33%), and CSF leakage (3.33% vs. 0%) were similar. Conclusions: The novel three-step approach accurately locates TSSJ points in retrosigmoid craniotomy, reduces bone defects, saves time, and does not increase the risk of sinus injury, infection, and CSF leakage.

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Aims: To study the latency, amplitude, and source localization of magnetic evoked field (MEF) responses to visual, auditory, and somatosensory stimuli in Wilson's disease (WD) using magnetoencephalography (MEG) and compare it with “healthy” controls, and correlate the observations with disease severity and brain MRI. Methods: MEF of 28 patients with neurological WD (age: 22.82 ± 5.8 years; M:F = 12:16) and 21 matched controls (age: 25.0 ± 4.6 years; M:F = 10:11) were recorded using MEG. Source localization was performed using standard models on the components of M100, M20, and M100 for visual, somatosensory, and auditory evoked fields, respectively and its latency/amplitude was correlated with disease severity. Results: There were significant differences in source location between control and WD during visual evoked field (VEF) and auditory evoked field (AEF) studies. Latencies of M20 (right-p = 0.02; left-p = 0.04) and M32 (right-p = 0.01) components of SSEF were significantly prolonged. The amplitude of M20 was significantly reduced in patients bilaterally (P = 0.001). There was a trend for the prolonged latency of M100 of VEF in patients (P = 0.09). Five patients had reduced right M145 compared to 8 controls. The left somatosensory evoked fields (SSEF) latency correlated with disease severity (P = 0.04). There was no significant correlation between major components of other MEF with disease severity or MRI score. Conclusions: This study, first of its kind to use MEF analysis in a large cohort of patients with WD, detected subclinical but a variable degree of abnormalities, most consistently of SSEF. It provides valuable insights of functioning and localization of various pathways in a disease known to have protean clinical manifestations and widespread MRI changes.

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Background: Complex angioarchitecture of brain arteriovenous malformations (BAVM) are often difficult to evaluate with standard imaging technique of digital subtraction angiography (DSA). These details are better provided by 3D rotational angiography (3D-RA). Objective: The aim of the study is to compare two-dimensional digital subtraction angiography (2D-DSA) and 3D rotational angiography in the evaluation of BAVM angiographic architecture. Materials and Methods: 2D-DSA and 3D-RA of 167 consecutive patients with BAVM were analyzed for arterial feeders, nidal patterns, and detection of flow-related aneurysms, arteriovenous fistulous components, venous drainage, and draining vein stenosis. Results: 3D-RA detected a significantly higher number of aneurysms and draining venous stenoses (P < 0.001). The detected number of true intranidal aneurysms was significantly higher with 3D-RA (n = 94) vs 2D-DSA (n = 34) (P < 0.001). 2D-DSA has low sensitivity (43.1%) and specificity (73.4%) for detecting intranidal aneurysms. 3D-RA detected a significantly higher number (12.6%) of BAVM patients with feeding artery aneurysms as compared to 2D-DSA (8.4%), P value of 0.004. 3D-RA accurately depicted the distal course of dominant arterial feeders and fistulous sites in BAVMs. Direct arteriovenous communications were evident in 31.1% with 3D-RA, as compared to 2D-DSA (15%) with P value < 0.0001. A significantly higher number of draining vein stenosis was detected with 3D-RA (21.6%) as compared to 2D-DSA (13.2%), P value < 0.001. Conclusion: 3D-RA is better than 2D-DSA for detecting BAVM-associated flow-related aneurysms, distal course of the dominant arterial feeders, direct visualization of the fistulous components, deep venous drainage, and draining venous stenosis; findings imperative for making a prudent therapeutic decision.

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Background: There is dearth of literature on impact of age – pediatric versus adult, Suzuki staging – early versus late, and revascularization type – indirect versus direct on clinical and angiographic outcome in moyamoya disease (MMD). Hence, we intend to comprehensively evaluate factors influencing outcome in MMD following surgical revascularization. Methods: MMD patients operated at our institute from January 2011 to August 2018 were followed for an average 24 months. Primary outcomes were modified Rankin score (mRS) and stroke risk reduction. Secondary outcome was angiographic outcome score (AOS). Results: A total of 133 patients including 37.6% adults (>18 years; n = 50) underwent 175 revascularizations – 89 direct (DR) and 86 indirect (IR) subgroups. Mean mRS scores improved in pediatric DR (P < 0.001), IR (P < 0.001), adult IR (P = 0.10), and DR (P = 0.25) subgroups. Recurrent stroke rate was similar in pediatric-DR (2.7%) and IR (7.6%, P = 0.61), and adult-DR (0%) and IR (4.1%) subgroups (P = 1.00). AOS scores improved in pediatric DR (P = 0.002) and IR (P = 0.01), and adult-DR (P = 0.02) and IR (P = 0.06) subgroups. Late-stage MMD (Suzuki IV–VI) showed better improvement in mRS scores than early-stage-Suzuki (I–III; P < 0.001). Recurrent stroke rates were similar (P = 0.26) and AOS scores improved significantly in early- and late-stage MMD (P < 0.001). Conclusions: In pediatrics, clinical and angiographic outcome significantly improved after revascularization procedure, and in adults, angiographic improvement was more evident than clinical recovery. Late-stage Suzuki MMD patients demonstrated significantly better clinical improvement than early stage. The angiographic improvement was equivalent to early stage, irrespective of age and type of revascularization.

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Background: Cerebral venous thrombosis (CVT) is an uncommon cause of stroke and shares common risk factors with arterial strokes such as hyperhomocysteinemia, tobacco, alcohol, drugs, and hypercoagulable state. These risk factors can alter both arterial and venous health leading to the occurrence of atherosclerosis in CVT patients. Aims: To evaluate carotid hemodynamics in CVT patients. Settings and Design: Prospective hospital-based case-control study. Methods: This study included 50 consecutive CVT patients and 50 healthy controls. The demographic data, vascular risk factors, clinical data, biochemical, and radiological parameters were recorded. Carotid sonography was performed in CVT patients within the first 24 h of admission. Statistical Analysis: MedCalc 17. Results: The age of the patients was 35.04 ± 9.48 years and the controls 38.88 ± 10.41 years with male preponderance in both groups. Risk factors for atherosclerosis among patients included hyperhomocysteinemia (40 patients), diabetes mellitus (4 patients), hypertension (9 patients), alcohol (17 patients), and tobacco (21 patients). Eight patients had abnormal carotid sonography. Six had nonflow-limiting plaques, one had carotid occlusion, two had increased intimal-medial thickness, and one had increased peak systolic velocity. Among the controls, three subjects had nonflow-limiting plaques. There was no difference in carotid hemodynamic parameters between controls and patients; and those with normal and elevated homocysteine. Conclusion: This is the first study to our knowledge looking at carotid health in venous strokes. The relative risk for carotid atherosclerosis in CVT patients is higher and requires long-term follow-up for the initiation of preventive measures.

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Segmental schwannomatosis involving a nerve in single limb is uncommon. Schwannomatosis is used to describe a distinct clinicopathological disease of multiple schwannomas without manifestations of neurofibromatosis, and it is termed segmental when a single extremity is involved. Surgery is indicated when there is progressive clinical deterioration or progressive increase in size of the tumors. We present a case series of segmental schwannomatosis involving the lower limb.

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Background: Collateral status of the circle of Willis was associated with white matter hyperintensities (WMHs) in patients with internal carotid artery (ICA) stenosis, but few have investigated the effect of leptomeningeal anastomoses. Objective: The aim of this study was to observe the association between WMHs and the laterality of the posterior cerebral artery (PCA) that presents leptomeningeal anastomoses in patients with severe ICA stenosis. Materials and Methods: WMHs and ipsilateral PCA laterality were evaluated in patients with unilateral ICA stenosis ≥70% (including occlusion) and contralateral ICA stenosis <50% or no stenosis. Ipsilateral PCA laterality was compared between two groups of no/mild and severe score of global, deep and periventricular WMHs, respectively. Results: We included 115 patients with unilateral ICA stenosis ≥70%. There were 60 patients with no/mild and 55 with severe global WMHs. The patients with severe global WMHs were older (OR = 1.849, 95% CI: 1.058–3.229, P = 0.031) and had higher incidence of negative PCA laterality (OR = 3.301, 95% CI: 1.140–9.558, P = 0.028). The patients with severe deep WMHs were also older (OR = 2.031, 95% CI: 1.130–3.651, P = 0.018) and had higher incidence of negative PCA laterality (OR = 4.250, 95% CI: 1.501–12.032, P = 0.006). There was no significant difference between the patients with no/mild and severe periventricular WMHs in the incidence of negative PCA laterality. Conclusions: The incidence of negative PCA laterality was higher in patients with severe global and deep WMHs, but not higher in patients with severe periventricular WMHs. The leptomeningeal anastomoses may affect the deep WMHs in patients with severe ICA stenosis.

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A 70-year-old man presented to the Emergency Department with left hemiparesis, slurred speech, and elevated blood pressure. A brain computed tomography scan revealed an ischemic lesion in the right frontal and parietal lobes. At clinical examination bilateral pseudo gynecomastia was detected together with the presence of multiple elastic, adipose bulging masses on the neck, trunk, and upper limbs. A type I–II Lanois–Bensaude syndrome was diagnosed. Ultrasonography confirmed their adipose nature. Multiple symmetric lipomatosis, also known as Lanois–Bensaude syndrome or Madelung disease, is a very rare condition with extreme variability in its clinical presentation. The simultaneous occurrence of ischemic stroke and lipomatosis in the same patient might be due to a mitochondrial function impairment, which could lead to abnormal fat tissue distribution and defective cellular energy production, thus resulting in neuronal sufferance and death. The possibility that, in our case, lipomatosis could have represented a further risk factor in promoting the stroke occurrence is discussed. In our opinion, multiple symmetric lipomatosis must be carefully evaluated to improve the patients' quality of life.

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Meningeal melanocytoma is a rare benign tumor, most frequently located in the posterior fossa and spinal canal. Localized tumors present as leptomeningeal masses and range from well-differentiated melanocytomas to lesions of intermediate malignancy and overtly malignant melanomas. Spinal meningeal melanocytoma has a benign course and is amenable for gross total resection. The outcome is favorable following complete resection. Meningeal melanocytoma may occasionally be associated with histological benign leptomeningeal spread and aggressive clinical course in spite of the absence of malignant transformation. We report a case of intraspinal melanocytoma in a 57-year-old female, which clinically as well as radiologically mimicked other spinal lesions. The final diagnosis was confirmed on histopathology.

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Background: Arachnoid cysts are benign extra-cerebral congenital lesions that are thought to arise from the splitting of the arachnoid membrane. Although most arachnoid cysts remain static with advancing age, occasionally they can become symptomatic due to cyst enlargement or hemorrhage. Hemorrhage into the arachnoid cysts (intra-cystic hemorrhage) with or without associated subdural hematoma is of rare occurrence. Case Discussion: A 23-year-old male presented with a history of sudden onset severe headache while sleeping. The patient also noticed double vision, especially when looking towards the left side. Non-contrast computed tomography (NCCT) scan of the head showed left temporal pole hematoma with left Sylvian fissure bleed and bilateral fronto-temporoparietal (FTP) subacute SDH. Conclusion: Every patient with incidentally detected AC, especially in the middle cranial fossa, should be counseled regarding the risk of possible complications including hemorrhage and regular follow-up.

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Background: Compression of ulnar nerve at the elbow is the second most common peripheral neuropathy of the upper extremity. Objective: Due to the lack of the gold diagnostic standard for ulnar nerve involvement at elbow level (UNE) and the lack of sufficient study in this field, we decided to evaluate patients with symptoms of this disease who have normal conventional electrodiagnostic study (EDX) in first evaluation. Materials and Methods: In this cross-sectional study, 18 persons were selected from patients who were referred to the clinic of Physical Medicine and Rehabilitation. If conventional EDX was normal, compound nerve action potential (CNAP) test (peak latency and amplitude) was carried out. Patients with normal conventional EDX but abnormal ulnar CNAP included to our study. After 3 months, if they had not been treated for ulnar neuropathy, they were reexamined by conventional EDX plus ulnar CNAP measurement. Results: In total, 18 patients (11 females, 7 males) aged 28–58 years old (mean = 40.11) were analyzed in this study. After 3 months, 14 patients (77.8%) demonstrated parameter changes consistent with UNE in conventional EDX. Conclusion: Based on the results of this study, ulnar CNAP has diagnostic value in patients with symptoms of UNE who have normal routine EDX. Therefore, ulnar CNAP should be taken into account for early diagnosis of ulnar neuropathy when routine electrodiagnostic tests are normal.

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Background: Children aged 5 to 14 years are employed in the informal units of footwear making and are exposed to a mixture of organic solvents present in the adhesives. Organic solvents are reported to be toxic to the neurobehavioral system. Objective: The present study was undertaken among the working children of informal footwear units of Agra to assess neurobehavioral functions using neurobehavioral tests. Materials and Methods: The study included 151 working children and 157 school children. We used the interview technique to collect the data regarding demographic, occupational, personal, and clinical characteristics using a pre-designed and pre-tested proforma. Each study participant was then subjected to a neurobehavioral test battery that included digit symbol test, finger dexterity, tweezer dexterity, vocabulary test, picture completion, and maze completion test. Results: The mean age of working children was 10.8 ± 1.5 years and that of school children was 11.0 ± 1.5 years. Except for the vocabulary test scores, all other test scores were significantly lower among working children as compared with the school children. Conclusion: The study revealed that the working children in informal footwear making units are exposed to a mixture of chemicals that affect their neurobehavioral function as reflected by poor neurobehavioral test battery (NBT) scores.

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Neurophysiological monitoring assesses the functional integrity of the brainstem by using monitoring and mapping techniques. We report an operated case of a pontomedullary lesion in a patient who developed central hypoventilation postoperatively. The intraoperative use of neurophysiological and cardiovascular monitoring was unable to predict/prevent this hypoventilation. We describe the inherent limitations of monitoring the respiratory system, including spontaneous respiration. Moreover, we suggest the novel application of diaphragmatic motor evoked potential for real-time monitoring of respiratory pathways during brainstem surgeries.

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