Frontotemporal dementia (FTD) is an entity that includes a group of neurodegenerative disease with symptoms predominantly pertaining to deficits in behavior, executive function (or) language. FTD is one of the most common type of dementia before 65 years of age and is one of the most underdiagnosed dementia as most often the symptoms overlap with psychiatric manifestations. Based on the clinical features, FTD is further subdivided into behavioral variant FTD (Bv-FTD) and primary progressive dementia (PPA). We searched PubMed, MEDLINE, and Google Scholar for articles about FTD disease published in English between January 1, 1975 till 2018. We used the search terms “frontotemporal dementia,” “Fronto temporal dementia-motor neuron disease,” “dementia,” “cognition,” “behavioral variant,” and “primary progressive aphasia.

Background: Pineal parenchymal tumors account for less than 0.3% of all CNS tumors and “Pineal parenchymal tumor of intermediate differentiation” (PPTID; World Health Organization (WHO) grades II and III) exhibit intermediary differentiation and prognosis. However “Papillary tumor of the pineal region” (PTPR; WHO grades II and III) is a distinct entity. Objectives: This combination of rarity and apparent similarity often leads to perplexity regarding the treatment and prognosis among neurosurgeons. In this review, we have tried to elucidate the differences in clinical as well as treatment modalities and outcomes of these two entities. Methods: We used the PubMed Database to search for all relevant articles using the keywords “pineal parenchymal tumor of intermediate differentiation” and “Papillary tumor of the pineal region.” Articles having details regarding demographic and clinical variables along with treatment and outcomes were chosen for this study. Full text of these articles was analyzed, and data tabulated. Results: A total of 25 articles for PPTID and 45 for PTPR were found suitable for inclusion in this study. The studies were either case reports or small retrospective series with only one systemic review for each pathology. Despite the poor quality of data, some trends were apparent. Surgical resection offered a survival benefit in both pathologies. Radiotherapy was effective in increasing the survival in PPTID, while there was little to no effect in PPTR. Chemotherapy was not found to be beneficial in either. Conclusion: Both of these tumors have moderate growth rate and potential for malignant behavior. This continuum of characteristics makes their optimal treatment strategy difficult and confusing. The discussion on comprehensive literature review should give information for neurosurgeons to decide on optimal treatment strategies.

Background: Patients with Parkinson's disease (PD) commonly use complementary and alternative medications. Yoga is a mind-body intervention that is being increasingly explored as a tool in the therapeutic armamentarium of PD. Objective: To critically evaluate the studies and summarize the utility of Yoga in PD. Material and Methods: We performed a systematic literature search in the Medline and Cochrane databases and included randomized controlled trials (RCT) of Yoga in PD. The studies were evaluated for internal validity and the relevant data were extracted. Results: A total of seven studies were included in the analysis. We collated the data on the changes in motor function, gait and balance parameters, anxiety, depression and quality of life scores observed after intervention (Yoga) in patients with PD and highlighted the limitations of these studies. Conclusion: Anxiety, depression, and balance issues in PD may benefit from Yoga. Yoga has potential as an add-on therapy in PD.

Background: IgG4-related disease (IgG4RD) is a multisystemic progressive fibroinflammatory and lymphoproliferative autoimmune disorder of unknown etiopathogenesis; and its occurrence in intradural extramedullary (IDEM) location is extremely rare. Aim and Objective: The aim of this study was to review all IDEM IgG4RD cases described in English literature and to signify the role of surgery in its management at this rare location along with the surgical technique and intraoperative findings. Methods: An Internet-based search (PubMed) for the published reports describing spinal cases of IgG4RD with IDEM involvement was done using the keyword: spinal IgG4-related disease. An illustration of a case of cranial IgG4-related disease in a young man who developed metachronous cervical involvement in intradural extramedullary (IDEM) location is also presented. Results: Amongst 45 spinal IgG4RD cases reported in English Literature, only 3 cases were IDEM IgG4RD. In the illustrated case, portion of the cervical lesion causing cervical cord encasement was intradural but extraarachnoidal- located between arachnoid and dura, without any pial involvement. All these 4 cases improved following surgery. Conclusion: Timely meticulous resection of mass lesion in situations of medical treatment failure or progressive neurological decline can lead to reversibility of mass effect-associated neurological manifestations of IgG4RD.

Background: Paraclinoid segment aneurysms arise from the internal carotid artery (ICA) between the distal dural ring and the origin of the posterior communicating (PComm) artery. Objective: This pictorial study presents videos showing clipping of paraclinoid segment aneurysms. Materials and Methods: The various subtypes of these aneurysms, the nuances in the technique of clinoidectomy, and methods of proximal control are presented. Results: Cavernous ICA is designated as C4, clinoidal segment (between the proximal and distal dural rings) as C5, and supraclinoid segment (between the distal dural ring up to the PComm artery as C6 segment. The techniques used for clipping various aneurysms are based upon their subtypes and location. In the first case, in a giant superior hypophyseal artery aneurysm directed toward the suprasellar region, an intradural clinoidectomy helped in accessing the neck of the aneurysm encroaching into the clinoidal segment of ICA. In the second case, concurrent bilateral “kissing” paraclinoid segment aneurysms were clipped using a unilateral approach. In the third case, clipping of a dissecting paraclinoid segment aneurysm is demonstrated. Conclusions: Surgery still represents the most definitive form of treatment. It may also be used to evacuate an intracerebral hematoma, with an extremely tortuous proximal vessel or in an aneurysm with complex anatomy, with blister aneurysms, or following aneurysm regrowth following a failed endovascular procedure. Assessing the three-dimensional anatomy of various segments of ICA is an important step.

Background: MITLIF is popular among minimally invasive lumbar interbody fusion procedures. The common indications of MITLIF are Grade I and selected cases of Grade II spondylolisthesis, Failed Back Surgery Syndrome, spondylodiscitis and deformity correction. Objective: To describe the technical nuances of MITLIF along with an operative video of a case of L4-L5 Grade I Spondylolisthesis. Surgical Procedure: Two incisions of 2.5 cm length are made on either side, at the level to be operated and further access is created by muscle splitting. Screws, rods and cage(s) are placed through this corridor under fluoroscopic guidance. Results: This procedure has the advantages of minimal blood loss, less muscular trauma, retention of intact posterior ligamentous anatomy, shorter hospital stay, lesser CSF leak and infection rates when compared to the open approaches. Conclusions: MITLIF has emerged as the standard procedure replacing open approaches. Mastery of this approach is strongly recommended to spine surgeons.

Introduction: Hemangioblastomas (HMB) are extensively vascularized and benign neoplasms that are diagnosed predominantly in adults. The solid type of HMB is technically challenging to operate. The piecemeal resection of the tumor has been shown to have devastating intraoperative complications. Therefore, en-bloc tumor resection was shown to be the safest procedure to reduce the risk of intraoperative bleeding and facilitate the removal of large solid tumors. Unfortunately, most of these tumours are also not amenable for embolization, as they are fed by multiple pial vessels. However, the large arterial feeders may be embolized. Objective: This video abstract presents a case of en-bloc resection of a giant solid type of Vermian HMB. Surgical Technique: A 38-year-old male patient presented with headache and vomiting for 1 year, imbalance during walking for 6 months, and hoarseness of voice for 2 months. He underwent a CSF diversion procedure in another hospital and was referred to our center for definitive surgery. Contrast-enhanced MRI showed a large heterogeneously enhancing solid lesion of size 46 × 33 × 40 mm³ with central necrotic area in the posterior fossa with perilesional edema and several large flow voids on T2-MR sequence. Preoperative embolization was performed; however, there was no significant reduction in the vascularity of the lesion. The patient underwent a wide midline suboccipital craniotomy with C1 laminectomy and gross total en-bloc resection of HMB. Results: The patient had an uneventful recovery in the postoperative period. Conclusions: The safest approach in the resection of giant solid HMB involves proper preoperative planning and understanding the vascular pattern of the lesion, wide exposure, circumferential dissection, and the en-bloc delivery of the tumor.

Background: Although leptomeningeal melanin pigmentation is well-known, it is not described in the neurosurgical literature. Dark pigmentation native to these transparent membranes might have microsurgical relevance in identifying a plane of dissection. Objective: To describe the prevalence of leptomeningeal pigmentation and determine whether its recognition helps during microsurgical excision of extra-axial brain tumors. Materials and Methods: This was a prospective cross-sectional observational study in cadavers and neurosurgical patients. Eight adult cadaveric brains were examined for leptomeningeal pigmentation and biopsies taken for histological studies. A total of 126 patients undergoing surgery for cerebellopontine angle and suprasellar lesions were included in the clinical study. The surgeon determined whether the recognition of pigmentation was useful during microneurosurgical arachnoid dissection. Results: Seven of 8 cadavers (87.5%) had leptomeningeal pigmentation on the ventral pons, optic chiasm, lamina terminalis, olfactory tract, and gyrus rectus and infrequently on the cerebral convexities. The prevalence of pigmentation was 76.9% in the clinical study, was seen in all pediatric patients, and was significantly higher in males (P = 0.009), with no significant association with skin color. Immunochemistry identified the pigmentation as melanin. Recognition of this pigmentation was deemed to be useful during tumor excision in 78% of the cases. Conclusions: Leptomeningeal melanin pigmentation has a prevalence of 76% to 87% in the Indian population and is more often seen in younger males. It is most commonly identified overlying the ventral brain stem and optic chiasm. Recognition of its presence is helpful during arachnoid dissection during most cerebellopontine angle and suprasellar tumor resections.

Introduction: Corticospinal tracts (CST) forms the basis of motor neurophysiology after stroke. Motor skill recovery has been correlated well to the microstructural properties of CST in both hemispheres. Functional imaging has opened up new possibilities of imaging functionality of cortex and fiber tracts in the brain. We studied therapy-induced changes in blood oxygenation level-dependent (BOLD) and DTI imaging on 20 chronic stroke patients at baseline, 8, and 24 weeks. Settings and Design: All the patients were subjected to MR imaging on a 1.5 T MR scanner. We used block design for BOLD with alternate baseline and activation cycles (repetition time (TR) =4520 ms, echo time (TE) = 44 ms, slices = 31, slice thickness = 4 mm). DTI parameters were as follows: TE = 76 ms, TR = 10,726 ms, EPI factor = 127, resolution = 128 × 128 matrix, field of view = 230 mm and a slice thickness of 4.0 mm. Statistical Analysis Used: The data was analyzed on SPSS software and tractography/DTI processing software (M/s. Siemens Medical Solutions, Erlangen Germany. Results: The mean axial diffusivity (λ[INSIDE:1]) and radial diffusivity (λ[INSIDE:2]) in the affected hemisphere were 0. 30 and 0.18, respectively. The mean number (FN) ratio (± SD) was 0.27 ± 0.14 at baseline, 0.33 ± 0.19 at 8 weeks, and 0.41 ± 0.23 at 24 weeks. Multivariate regression analysis at baseline showed that rFA was well-correlated to the Fugl-Meyer score (regression coefficient: 0.198, F = 10.382, P = 0.001), MI followed by signal intensity. Discussion: All patients had high % signal intensity after 8 weeks of physiotherapy regime with a greater percentage change in rFA as compared at follow-up suggesting that a focused exercise regime in stroke patients helps in the reconnection of neural and myelin networks. Conclusion: Clinical and functional recovery after stroke is well-correlated with the DTI and BOLD parameters i.e., rFA ratios, CST involvement fiber numbers, and % signal intensity of the ipsilesional cortex.

Background: Stroke is one of the leading causes of death and disability in India. Angiotensin-converting enzyme (ACE) is involved in the development of hypertension, atherosclerosis, cardio, and cerebrovascular disease and is a plausible genetic candidate for stroke. The role of ACE polymorphism is still uncertain and requires further investigation. Objective: The aim of this study was to study the role of ACE insertion/deletion polymorphism in acute ischemic stroke (AIS). Materials and Methods: One hundred thirty AIS cases and 130 age and sex matched healthy controls were recruited. Ten milliliters venous blood was drawn and 6 mL of blood was used for routine hematological, biochemical tests. Remaining 4 mL of EDTA blood was used for DNA extraction, PCR amplification, and restriction digestion. Three genotypes (II, ID, and DD) were visualized on 3% agarose gel. Association between genotypes among stroke case was done by Chi-square test with P value <0.05 taken as significant. Results: DD genotype was significantly associated with the risk of stroke with P value of 0.0001. Both the dominant and recessive models showed that the DD genotype was independently associated with an increased risk of ischemic stroke (OR = 20.732; 95% CI: 2.7241–157.7864; P value = 0.003 for the recessive model and OR = 2.848; 95% CI: 1.5127–5.3649; P value = 0.001 for dominant model). Conclusions: Our study showed a strong association between ACE polymorphism and the risk of AIS. This study paves the way for further studies to confirm the role of ACE polymorphism as genetic risk factors for AIS.

Background: Laminectomy with lateral mass and transfacet fixation are widely accepted surgical techniques for cervical compressive myelopathy (CCM). Objective: To evaluate multilevel fixation with additional fixation of C7-T1 transfacet junction may help achieve better surgical outcome both in short- and long-term follow-up. Material and Methods: Based on utilizing C7-T1 transfacet junction fixation, 102 consecutive patients undergoing surgery for CCM were divided into Group A: cervical laminectomy with lateral mass fixation only, and Group B: cervical laminectomy and lateral mass fixation including C7-T1 transfacet junction in fixation. The groups were compared for at 3 months and 2 years postsurgery for persistence of preoperative symptoms, neurological outcome, and sagittal balance (T1 slope) of cervical spine. Results: The average age of the study population was 59.11 ± 12.05 years with 71 (69.6%) men. There were no significant differences between the groups neither for presenting complaints nor for postoperative complications. Patients in group B had lesser length of postsurgical hospital stay (7.57 ± 6.61 vs 5.55 ± 1.81; P = 0.018). At 3 months follow-up, patients in group B had higher motor power of upper limb (3.64 ± 1.91 vs 4.47 ± 0.57; P < 0.001), lower limbs (5.07 ± 1.72 vs 5.92 ± 1.13; P = 0.005), and total modified Japanese orthopedic association score (MJOS) score (13.68 ± 3.42 vs 15.51 ± 1.87; P = 0.001). Patients in groups B had lower postoperative T1 slope scores (26.93 ± 8.73 vs 17.60 ± 4.97; P = <0.001). At 2 years follow-up of 53 patients, patients in group B had a better upper limb motor function (3.77 ± 1.14 vs 4.44 ± 0.50; P = 0.021) and total MJOS score (13.85 ± 3.49 vs. 15.37 ± 1.86; P < 0.052). Conclusion: Normalizing sagittal balance in patients with CCM by cervical laminectomy and posterior fixation including cervical thoracic junction (C7-T1 transfacet junctional fixation) may significantly improve neurological outcome both in short-term and long-term follow-up.

Background: Alzheimer's disease (AD) is the most common cause of dementia. Although genetic mutations are known in rare familial form, exact cause of neurodegeneration in sporadic AD is still unknown. While ApoE ε4 and IL-6 C-174G/C patterns have been found to increase the risk of AD in Caucasians, the results are inconsistent in other ethnic groups. Objective: The aim of this study was to evaluate the effect of ApoE and IL-6-174G/C polymorphisms among patients of AD in the Eastern part of India. Materials and Methods: Consecutive patients of probable AD diagnosed as per National Institute on Aging-Alzheimer's Association (NIA-AA) criteria with age, gender, and education-matched healthy controls were recruited between December 2015 and September 2018. Patients were clinically evaluated and along with controls were genotyped for ApoE and IL-6-174G/C polymorphisms by the polymerase chain reaction method. Results: A total 115 patients and 162 controls showed a similar pattern of ApoE and IL-6-174G/C polymorphism pattern. While ε3ε3 and GG patterns were the commonest, followed by ε3ε4 and GC pattern in ApoE and IL-6 respectively, the effect of ApoE ε4 and IL-6-174 C allele on AD symptoms could not be established. However, patients with onset before 50 years were found to have significantly higher proportion of ApoE ε4 and C allele of IL-6-174 in comparison to patients with onset above 50. These young patients were also having more atypical presentation than their older counterpart. Conclusion: Our study revealed a novel role of both ApoE ε4 and C allele of IL-6-174 together in developing early onset AD with more atypical clinical features.

Background: Globally, social distancing has been practiced during the ongoing coronavirus disease 2019 (COVID-19) pandemic to prevent the transmission of the virus. One of the measures to ensure social distancing and restricting the movements has been national lockdown, to break the chain of transmission. Telemedicine is a cost-effective measure to provide medical services to remote underserved areas. Objective: The present study aimed to evaluate the efficacy and acceptability of teleconsultation as an alternative option to in-person consultation in providing continued medical care for neurology patients during the national lockdown period of the COVID-19 pandemic. Materials and Methods: The clinical demographic profile, frequency of different neurological disorders, and treatment details of the patients attending the teleneurology consultation (TNCO) outpatient department (OPD) at Sir Sunderlal Hospital (S.S.H.), Institute of Medical Sciences (I.M.S.), BHU, Varanasi, India, were recorded in a prespecified pro forma. Results: A total of 1,567 patients attended the TNCO OPD over 90 days. The average patient attendance was 35 per day, and 72% were males. Out of these, 77% of patients were from the same district, and the majority of patients (68%) were regularly followed up in-person by the neurology OPD. The most common illness for consultation was epilepsy (19%) followed by low backache and stroke (18% each). The satisfaction rate among the patients with respect to teleservices was high (90%). Conclusion: TNCO seems to be as effective as in-person OPD in the management of neurological disorders. During the lockdown due to the COVID-19 pandemic, avoiding physical visits through TNCO may reduce the spread of the virus. Parallel tele-OPD with routine OPD is a good option in the future.

Background: Dizziness is a typical manifestation of vestibular pathologies. Clinical studies have shown that it affects 1.82% of young adults to more than 30% older adults. Habituation and compensation are some traditional rehabilitation protocols. Objective: Yoga is also known to have a significant effect on vestibulopathy. Hence, a need arises to compare the above two maneuvers. Materials and Methods: Participants were recruited (n = 32) after screening using the Dix–Hallpike and head impulse test. They were then divided into two groups (n = 16). The first one, Group A, received yogasanas, and the second one, Group B, received gaze stabilization and habituation exercises for 4 days a week for 3 weeks. Pre- and post intervention outcome measures were taken using the Motion Sensitivity Quotient (MSQ) score and Dizziness Handicap Inventory (DHI) scale. Results: In reducing symptoms of dizziness, the between-group comparison shows that Group A has shown greater improvement (12.37% ±1.43%) in MSQ and Group B has shown greater improvement (16.12 ± 3.56) in DHI. Within-group comparison shows that both the interventions are effective in reducing symptoms of dizziness (P < 0.05). Conclusion: Both yogasanas and gaze stabilization along with habituation exercises are effective in improving the symptoms in patients with peripheral vestibular dysfunction. When compared between the groups, yogasanas had a superior hand in the MSQ score, whereas gaze stabilization and habituation exercises had a superior hand in the DHI scale.

Background: Huntington's disease (HD) is a progressive neurodegenerative disorder characterized by motor, cognitive, and psychiatric abnormalities. Currently, matched analyses of structural and functional differences in the brain from the same study cohort and, specifically, in HD patients from an ethnically diverse Indian population are lacking. Such findings aid in identifying noninvasive and sensitive imaging biomarkers. Objective: The aim of the study was to understand the structural and functional differences between HD and control brain, and presymptomatic and symptomatic HD brain in the Indian population. Materials and Methods: Seventeen HD (11 symptomatic HD [S-HD] and six presymptomatic HD [P-HD], with comparable CAG repeats), and 12 healthy controls were examined. Macrostructural (volume), microstructural (diffusivity), and functional (neurochemical levels and glucose metabolism) imaging of the brain was done along with the determination of visual latencies. Results: HD brain showed increased intercaudate distance; significant subcortical volumetric loss; reduced fractional anisotropy; increased mean, axial, and radial diffusivity; lower levels of total N-acetyl aspartate; elevated total choline levels; and reduced glucose metabolism compared with control brain. Interestingly, compared with P-HD, S-HD patients demonstrated a strong inverse correlation between age at onset and CAG repeat length, and prolonged P100 latency. In addition, caudate and putamen in S-HD brain showed significant volumetric loss and increased diffusivity compared with P-HD brain. Conclusions: HD brain showed distinct macrostructural, microstructural, and functional differences compared with control brain in the Indian population. Interestingly, patients with S-HD had a significant volumetric loss, increased diffusivity, altered neurochemical profile, and delayed P100 latency compared with P-HD patients. Examining these alterations clinically could aid in monitoring the progression of HD.

Objective: Indirect bypass surgeries for moyamoya disease have included modifications of procedures involving placement of the superficial temporal artery on the brain pial surface. We evaluate the functional and angiographic outcomes of patients treated with encephaloduroarteriomyosynangiosis (indirect) revascularization and examine the outcome in relation to demographic and radiological factors. Materials and Methods: Patients treated surgically for moyamoya disease over a 14-year period were identified. Demographics, clinical presentation, and radiology were analyzed to assign a stage for the disease (Suzuki staging) and the extent of revascularization (Matsushima grade) at the last follow-up. A modified Rankin score was used to assess the clinical status at presentation and the functional outcome at follow-up. Results: There were 46 patients operated on by a single surgeon over a 14-year period. A higher incidence of motor deficits, seizures, and speech deficits was seen in the pediatric population. Age, sex, preoperative Suzuki disease stage, and hemispheric involvement had no bearing on angiographic outcome at last follow-up. Three of 46 patients (6.5%) developed immediate postoperative complications. Among 43 patients on follow-up, 39 had stable disease or showed improvement in clinical symptoms with 90% event-free status at last follow-up. Conclusions: Indirect revascularization procedures are an effective alternative to direct cerebral revascularizations in the early or advanced stages of moyamoya disease. This is effective in a predominant ischemic presentation as noted in our series.

Background: Major neurocognitive disorders (major NCD) predominantly affect the elderly. Major NCD results in significant morbidity and socioeconomic burden. Objectives: To estimate the individual cost of care of a person with major NCD according to disease severity and component costs. Methods and Material: Fifty patients of major NCD with primary caregivers attending memory clinic of tertiary care center were included. A detailed questionnaire administered after inclusion provided demographic and clinical information. Caregivers were interviewed about details of care provided. Cognitive function was assessed by Hindi mental state examination (HMSE), and major NCD severity was determined by clinical dementia rating scale (CDR). Results: The annual cost of care per patient with mild to moderate and severe major MCD was INR 78288 and INR 167808, respectively. Costs increased with increasing severity of the disease. Direct nonmedical costs were significantly higher than direct medical costs in severe major NCD group and vice versa was found in mild to moderate major NCD group. Conclusion: Increasing population of elderly and prevalence of major NCD suggest an economic burden on caring families and hence the government. Comprehensive health policy toward providing affordable care to people with major NCD is the need of the hour.

Context: Hypnic headache (HH) is a rare primary headache syndrome first described by Raskin in 1988. Aim: To describe the occurrence of HH in Indian patients and compare its clinicoepidemiological features to those published in the literature and attempt to trace some of the evolving concepts regarding its etiology and clinical features since it was first described. Materials and Methods: Patients attending the neurology outpatient department of a tertiary referral teaching hospital from 01-05-2011 to 30-04-2016 who were identified to have HH as per ICHD 3 beta criteria were included in the study. A meticulous history of the headache and comorbidities, clinical examination, Epworth Sleepiness Scale, blood counts, blood biochemistry, magnetic resonance imaging (MRI) scans of the brain and polysomnography (in selected patients) were done and the results were compared to selected international literature. Results: A total of 11 patients with HH were identified during the study period, of which 8 (72.72%) were males and 3 (27.27%) were females. The age of the patients varied from a minimum of 53 years to a maximum of 78 years (Mean: 63.36, SD: 8.09). The frequency of attacks per month ranged from 5 to 46(Mean: 20.36, SD: 11.67). The duration of each headache episode ranged from a minimum of 30 minutes to a maximum of 4 hours (Mean: 1.93, SD: 1.23). The occurrence of the headaches was maximum during the time periods of 0.00–2.00am and 2.00–4.00am (38% and 36%, respectively). The pain was dull in a majority of patients, 7 (63.63%). Trigeminal autonomic features such as lacrimation, ptosis, or rhinorrhea were not recorded from our cohort. Motor activity was noted in 7 (63.63%) cases. Two (18.18%) patients had associated migraine headaches whereas 3 (27.27%) had associated tension-type headaches. None of the patients in our series had chronic obstructive pulmonary disease. Four (36.36%) patients had systemic hypertension and 1 patient (9.09%) had diabetes mellitus. Two (18.18%) patients had symptoms of obstructive sleep apnea syndrome. Three (27.27%) patients had symptoms of excessive daytime sleepiness according to the Epworth Sleepiness Scale. Blood examinations and MRI were normal in all patients, except for the findings of a few lacunar infarcts and nonspecific T2 weighted hyperintensities in 3 patients (27.27%). Conclusion: Our study proves the existence of the newly described primary headache syndrome called HH in the Indian population. On comparing our results with the international literature, the similarities are much greater than the differences. MRI voxel-based morphometry to demonstrate the loss of gray matter in the posterior hypothalamus may prove to be a reliable test to diagnose primary HH in the future.

Background: Intracranial large artery disease (ICLAD) in ischemic stroke patients is associated with an increased risk for recurrent stroke; however, it is not known if ICLAD influences functional status following stroke. We studied the 6-month functional outcome in south Asian ischemic stroke patients and compared those with and without ICLAD. Materials and Methods: This is a prospective cohort study of consecutive south Asian ischemic stroke patients. ICLAD was assessed with transcranial color-coded Doppler ultrasound or magnetic resonance angiography. Functional outcomes were obtained via telephone interviews with poor outcome defined as modified Rankin scale of 3–6. Results: Of 216 ischemic stroke patients studied, 203 (93.9%) had follow-up data, of whom 50.7% (103) had ICLAD. Patients with ICLAD had a higher prevalence of hypertension (P < 0.001), hyperlipidemia (P = 0.047), ischemic heart disease (P = 0.030), and extracranial carotid disease (P = 0.005). A higher proportion of patients with ICLAD had poor functional outcome at 6 months (30.1%) versus those without ICLAD (13.0%) (P = 0.004). After adjusting for age, sex, hypertension, hyperlipidemia, diabetes, ischemic heart disease, atrial fibrillation, extracranial carotid stenosis, and recurrent vascular events, patients with ICLAD were 3.01 (95% confidence interval: 1.35–7.10) times more likely than those without ICLAD to have poor functional outcome. Conclusions: The presence of ICLAD rendered poorer functional prognosis after stroke. These findings support the specific evaluation of the benefits of known acute stroke treatments such as thrombolysis, as well as investigation of potential novel strategies such as acute stenting.

Introduction: By 6 months following a stroke, approximately 65% of stroke survivors cannot incorporate into daily activities, resulting completely dependent. The aim of this study is to assess the overall outcome in psychological and physical well-being, increase in residual abilities, reintegration and social inclusion for people with chronic disabilities resulting from stroke, after home rehabilitation. Materials and Methods: We assessed the functional outcome of a group of 600 patients with disability related to several pathological conditions and undergoing home rehabilitation therapy (twice a week for 40 sessions per year) as granted by the Public Health. We evaluated the outcome with Barthel and Functional Independence Measure (FIM) scales. Furthermore, in a group of 73 persons with chronic stroke we also carried out an overall evaluation, by using specific rating scales (FIM, Stroke Impact Scale, Motricity Index, Tinetti Balance Scale, 10-m Walking Test, 6-min Walking Test, 5 Repetitions Sit-to-Stand Test, and Hamilton Depression Scale) after 120 days (114 ± 6) from the end of the home rehabilitation program. Results: The results highlighted a statistically significant improvement between T0 and T1 and a significant worsening between T1 and T2 (follow-up at 3 months). Hamilton Depression Scale is the only parameter that significantly improves both at the time T1 and T2. Conclusions: Territorial rehabilitation system should consider some fundamental aspects: centrality of the person and the individual health project; identification of specific and personalized rehabilitation plan and prognosis based on the results of a follow-up; ultra-specialization of interventions; multidisciplinary team; highlight of the emerging needs; and coordination of the different care pathways.

Purpose: The health-related quality of life (HRQoL) is an important endpoint in modern clinical practice with improved survival of pediatric posterior fossa malignant brain tumors (PFMBTs). We evaluated the effect of environmental and psychosocial milieu on QoL and cognitive functioning (CF) of Indian children with PFMBT. Methods: In a cross-sectional study, 47 children <18 years of age with medulloblastoma or anaplastic ependymoma were evaluated ≥6 months after completion of adjuvant therapy. All clinical and socioeconomic details, educational status of child and family members, socioeconomic status, environmental factors affecting QoL were documented. Children underwent HRQoL evaluation using Pediatric quality of life Inventory (PedsQL) questionnaire and neuropsychological evaluation. Results: The median age of the cohort at presentation was 7 years (1–18) and median duration of evaluation after adjuvant therapy was 16 months. In 47 families, 72.34% had low monthly income and 76.6% of mothers took formal education. QoL scores were above median values. Parents reported scores highlighted that Lansky performance score (P = 0.001) and maternal education (P = 0.043) significantly influenced the cognitive component of QoL. Twenty-seven children had below-average IQ. Young age at presentation (P = 0.020), maternal education (P = 0.032), high socioeconomic status (P = 0.001) influenced the IQ score. Even though the majority of children (57.44%) had below-average IQ, they had a score of more than 50 on the cognitive functioning scale. A total of 72.5% of the eligible children in our cohort went back to school following therapy, though often with a delay of one academic year. Conclusions: Overall cognitive functioning scores of these children are good, but they are not representative of actual neurocognitive tasks based performance or IQ scores. Children should remain under regular follow-up with a neurocognitive assessment and psychological counseling at regular intervals.

Introduction: Early emergence from anesthesia is valuable, especially among neurosurgical patients for postoperative neurological evaluation and appropriate interventions. However, the factors affecting the emergence in patients undergoing clipping of ruptured aneurysms have not been studied. Materials and Methods: This was a prospective observational study on patients of aneurysmal subarachnoid hemorrhage with World Federation of Neurological Surgeons (WFNS) Grades I to III, undergoing surgical clipping. All relevant preoperative and intraoperative details were collected and analyzed to assess the factors affecting emergence time. Results: A total of 67 patients with a median age of 46 years were included in the study. The number of patients with Fisher Grades I, II, III, and IV was 6, 20, 25, and 16, respectively. The median time to emergence was 17 minutes (interquartile range 10–240 minutes). On univariate analysis, the factors that were found to have a significant relationship with time to emergence were preoperative Glasgow Coma Score (GCS; P = 0.02), WFNS grade (P = 0.005, temporary clipping time (P = 0.03), and the temperature at the end of surgery (P < 0.001) In the multivariate analysis using generalized linear model, preinduction GCS (P < 0.001), patient's temperature at the end of surgery (P < 0.001), and temporary clipping time (P = 0.01) had a significant impact on the emergence time, independent of age, American Society of Anesthesiologists grade, Fisher grade, duration of anesthesia and of each other, with GCS and temperature having the maximum impact. ROC curve for temperature had a cutoff value at 35.3°C with an 83% probability of awakening beyond 15 minutes if the temperature decreased below 35.3°C. Conclusion: The preinduction GCS, the temperature of patients at the end of surgery, and the duration of temporary clipping have a significant independent impact on the time to emergence from neurosurgical anesthesia, in the order of the strength of the association.

Background: Up to three-fourths of the poststroke subjects may experience shoulder subluxation as a challenging complication. The existing rehabilitation management is based on the clinical assessment. Ultrasonographic evaluation demonstrates findings, which cannot be discerned by the usual methods. Objectives: To determine the effect of rehabilitation protocol based on the sonographic findings of the subluxed shoulder on reduction of the subluxation and upper limb motor recovery. Materials and Methods: Setting: Department of Occupational therapy of a Rehabilitation Institute. Study Design: A prospective case series. Subjects: 08 Poststroke hemiparetic patients with subluxed shoulder. Outcome measure: Ultrasongraphy of the bilateral shoulder joints, Fingerbreadth palpation method, Visual analog scale (VAS), Fugl-Meyer assessment of upper extremity (FMA-UE). Intervention: As per the findings of the sonography, management in the form of shoulder support, physical agent modalities, motor therapy, and precautions and positioning was provided to the subjects for the period of 3 months. Results: Post intervention, the participants showed 1 to 6 mm of reduction of acromion-greater tuberosity distance in addition to the reduction of atrophy and soft tissue or joint effusion. Furthermore, the participants also exhibited FMA-UE change ranging from 5 to 21. Conclusion: The ultrasonographic evaluation explores objective measurement and involvement of specific soft tissues among poststroke subject with the shoulder subluxation. The management based on the sonographic findings is an objective and valid approach.

Introduction: Genetically defined spinocerebellar ataxia (SCA) type 1 and 2 patients have differential clinical profile along with probable distinctive cortical and subcortical neurodegeneration. We compared the degree of brain atrophy in the two subtypes with their phenotypic and genotypic parameters. Methods: MRI was performed using a 3T scanner (Philips, Achieva) to obtain 3D T1-weighted scans of the whole brain and analyzed by FreeSurfer (version 5.3 and 6 dev.) software. Genetically proven SCA1 (n = 18) and SCA2 (n = 25) patients with age-matched healthy controls (n = 8) were recruited. Clinical severity was assessed by the International Cooperative Ataxia Rating Scale (ICARS). To know the differential pattern of atrophy, the groups were compared using ANOVA/Kruskal-Wallis test and followed by correlation analysis with multiple corrections. Further, machine learning-based classification of SCA subtypes was carried out. Result: We found (i) bilateral frontal, parietal, temporal, and occipital atrophy in SCA1 and SCA2 patients; (ii) reduced volume of cerebellum, regions of brain stem, basal ganglia along with the certain subcortical areas such as hippocampus, amygdala, thalamus, diencephalon, and corpus callosum in SCA1 and SCA2 subtypes; (iii) higher subcortical atrophy SCA2 than SCA1 (iv) correlation between brain atrophy and disease attributes; (v) differential predictive pattern of two SCA subtypes using machine learning approach. Conclusion: The present study suggests that SCA1 and SCA2 do not differ in cortical thinning while a characteristic pattern of subcortical atrophy SCA2 > SCA1 is observed along with correlation of brain atrophy and disease attributes. This may provide the diagnostic guidance of MRI to SCA subtypes and differential therapies.

Background: Chronic stress is known to be associated with dementia. Aim: This study looked for major stressors and their associations with dementia in the elderly demented population. Methods: Case group consisted of 60 subjects (age group 55-75 years; M:F = 7:3) with postscreening for mild cognitive impairment (MCI) and mild Alzheimer's type dementia. Control group consisted of two subgroups: related (N = 60) and not-related (N = 60) subjects screened and confirmed not to be suffering from cognitive impairment. Sociodemographic variables were collected using a personal data sheet. The severity of cognitive impairment was assessed using the Clinical Dementia Rating (CDR) Scale. Subjects with a CDR score of range 0.5-1 were included in mild cognitive impairment and mild Alzheimer's type dementia. Stressful life events were evaluated using the Presumptive Stressful Life Event Scale (PSLES). A present stress level of caregivers was evaluated using Depression Anxiety Stress Scales (DASS). Assessed stress level scores were compared and validated with serum and plasma levels of cortisol and ACTH respectively. Results: Different groups exhibited distinct PSLES and DASS scores with statistical significance. It was also observed that Cortisol and ACTH levels were elevated in case group as compared with that of both sub-group controls. Conclusions: The outcome measures of this study projected that neural degeneration leading to mild cognitive impairment and dementia may be due to accumulative cortisol hormone associated with stressful life events.

Background: Monochromatic infrared energy (MIRE) has evoked mixed results for symptomatic relief of painful diabetic peripheral neuropathy (DPN). However, intraepidermal nerve-fiber density (IENFD) the gold standard for small-fiber neuropathy has not been evaluated. Objective: We assessed the IENFD, pain symptoms and quality of life (QoL) with MIRE therapy compared to placebo in painful DPN. Material and Methods: Participants with type 2 diabetes and painful DPN were randomized to receive MIRE or sham therapy dosed thrice a week for 12 weeks. Quantitative assessment of IENFD was performed from 3 mm skin punch-biopsy specimens at baseline and after 12 weeks. We also assessed the QoL with Norfolk QOL, symptom severity with visual analogue scale (VAS), and neuropathy assessment with Michigan neuropathy severity instrument and neuropathy disability score. Results: Thirty-eight participants were enrolled and 30 completed the study protocol. The mean age of participants in MIRE cohort was 59.1 ± 9.2 years, duration of diabetes 12.9 ± 3.1 years, and symptom duration of 3.9 ± 3.7 months. The mean IENFD was 0.90 ± 0.73/mm² (P < 0.01) and 1.71 ± 1.11/mm² in the MIRE cohort and 0.60 ± 0.89/mm² and 2.17 ± 0.98/mm² (P < 0.01) in sham cohort at baseline and after 3 months. The median decline in VAS was 5.1 (4.0-7.6) and 3.0 (0.4-5.6) points (intergroup difference, P = 0.01); and an increase in Norfolk QoL-DN by 15 (11-18) and 4 (4-14.2) points (intergroup difference, P = 0.021) in MIRE and sham cohort, respectively after 3 months. Conclusions: MIRE therapy does not increase IENFD over short-term usage. However, MIRE therapy provides symptomatic benefit and improves QoL in patients with painful DPN.

Background: Small intracranial aneurysms have a low risk of rupture. However, ruptured anterior communicating artery (ACoA) aneurysms are usually smaller in clinical practice. The retrospective study aimed to investigate the geometrical and hemodynamic changes of small unruptured ACoA aneurysms during serial follow-ups. Materials and Methods: We retrospectively collected patients with small unruptured ACoA aneurysms that were not repaired, who had serial follow-ups from the Electronic Medical Record System in four tertiary hospitals. The geometrical parameters of ACoA aneurysms were measured using a three-dimensional reconstructed model. Intra-aneurysmal hemodynamic parameters were computed using a high-resolution computational fluid dynamics model. Geometrical and hemodynamic changes of the aneurysms were evaluated at each follow-up. Results: Five patients with small unruptured ACoA aneurysms that were not repaired were identified and included in this analysis. Aneurysms rupture occurred in two patients with aneurysm growth. The formation and enlargement of an irregular bleb at the aneurysm neck or dome were observed before the rupture. Ruptured aneurysms showed high wall shear stress (WSS) in the high inflow zone of aneurysm neck while low WSS and high oscillatory shear index (OSI) in the flow-recirculating region of aneurysm dome. Three unruptured aneurysms maintained a stable morphology and a physiological level of WSS. Conclusions: Aneurysm growth, low WSS, and high OSI at the dome and/or high WSS at the neck potentially contribute to the rupture of small ACoA aneurysms. These aneurysms should be considered for the treatment regardless of the small size.

Background and Objectives: Folate is an important vitamin with a significant role in cell metabolism processes, and its deficiency is associated with several diseases. In addition, cerebral folate deficiency is associated with neurodevelopmental disorders. Studying the association of serum and cerebral folate deficiency with childhood neurodevelopmental disorders such as refractory epilepsy, developmental delay, and regression can be an important step towards the improvement of symptoms of such disorders. Materials and Methods: In this cross-sectional study, from February to October 2018, 60 children aged 6 months to 5 years; known cases of idiopathic refractory epilepsy; were selected randomly. After recording demographic, and clinical characteristics, cerebrospinal fluid (CSF) and blood samples were taken from the patients and sent to a laboratory for measurement of 5-methyltetrahydrofolate (5MTHF), folate, and homocysteine levels. Results: Sixty patients completed the study, including 33 boys (55%) and 27 girls (45%). Mean ± SD of the studied population was 26.93 ± 19.97 months. Eighteen children (30%) had refractory epilepsy, 11 (18.3%) had developmental delay, 12 (20%) had refractory epilepsy and developmental delay, and 19 (31.7%) had refractory epilepsy and developmental regression. The results of brain magnetic resonance imaging (MRI) were normal in 47 (78.3%) children and atrophic in 13 (21.7%) children. Mean ± SD of serum level of homocysteine was 9.14 ± 8.58 μmol/L, that of folate was 11.60 ± 6.89 nmol/L, and that of 5MTHF was 69.23 ± 54.16 nmol/L. Conclusion: Measurement of serum folate, homocysteine, and CSF level of 5MTHF are of great importance in patients with developmental disabilities.

Background: Central nervous system (CNS) involvement in leprosy is sparsely documented. Neurophysiological tests and magnetic resonance imaging (MRI) helps in demonstrating CNS involvement in the patient of pure neuritic leprosy. Objectives: To demonstrate CNS involvement in pure neuritic leprosy. Methods: Detailed clinical presentation and skin lesions were evaluated. Sural nerve biopsy, MRI diffusion tensor imaging of spinal cord and optic nerve were performed. Visual evoked potential and tibial somatosensory evoked potential were done. Their clinical, electrophysiological, and MRI were done at follow-up visits. Results: We report three patients of pure neuritic leprosy with bilateral foot drop as the initial presentation. MRI T2W sequence of cervico dorsal cord showed dorsal column hyperintensity in two patients. Diffusion-weighted MR revealed decrease fractional anisotropy and an increase in the apparent diffusion coefficient. Similar findings were also noted in the optic nerves. The patients were managed with multidrug therapy multibacillary regimen and steroid in tapering dose. At follow-up, they showed clinical improvement in vision and power of ankle dorsiflexor. Conclusions: Patients of pure neuritic leprosy may manifest with bilateral foot drop with the involvement of posterior column and cranial nerves.

The use of intraoperative neurophysiological monitoring is a helpful tool during surgeries of conus medullaris tumors and helps in reducing the risk of post-operative functional compromise like paraparesis, sensory deficits, or urological dysfunctions. External anal sphincter (EAS) motor evoked potential (MEP) monitoring is usually done for monitoring sacral nerve roots and to prevent post-operative neurological deficits. Here we describe a case where unilateral motor cortical stimulation aided us to differentiate the laterality of TcMEP responses in EAS.

Background: Normal pressure hydrocephalus (NPH) is a syndrome characterized by gait disturbance, dementia, urinary incontinence, and dilation of ventricular system with normal opening cerebrospinal fluid pressure. Idiopathic NPH (i NPH) presenting as psychosis is uncommon. Objectives: The objective of this study is to describe idiopathic NPH presenting as Psychosis. Subjects and Methods: Patients availing dementia care service from three tertiary care hospitals, across Kerala, with a diagnosis of psychosis were evaluated. Results: Three patients with NPH, diagnosed as per the consensus criteria, presenting as psychosis are described. Patient 1: Fifty-five-year-old lady with psychosis was found to have iNPH. She gave history of psychosis relieved with successful shunting of the hydrocephalus. She developed recurrence of psychosis each time the hydrocephalus recurred due to shunt malfunction and was relieved with correction of hydrocephalus with shunting. Patient 2 was a 67-year-old gentleman with long history of pyschosis with paranoid ideas. Five years after onset of illness, he developed gait apraxia, and a CT scan done showed normal pressure hydrocephalus. Patient 3 had bipolar illness since age of 60 years of age. He also developed gait apraxia 5 years into the illness and was diagnosed as having iNPH following imaging. Conclusions: All the patients had psychosis much before other features of iNPH developed. One of the patient's psychosis was temporally associated with onset and offset of hydrocephalus, thereby strongly supporting the causative nature of iNPH. iNPH though rare can be one of the causes for late onset secondary psychosis.

Cerebral small vessel disease (CSVD) is a well-known cause of vascular dementia. Though a majority of these cases are sporadic, familial monogenic causes are being frequently identified as well. Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a rare autosomal recessive CSVD, caused by mutation in HTRA 1 gene on chromosome 10q (10q25.3-q26.2) in homozygous or compound heterozygous form. Indian literature has been quite scant with very few case reports of CARASIL, and only three familial cases were confirmed with mutational analysis. Testing facilities of HTRA 1 genetic mutation are now more widely available in India than before, and should be encouraged for appropriate patients. This would help in diagnosing, prognosticating and avoiding unnecessary further investigations and medications for these patients. We herein review the Indian scenario and our previously reported experiences of this disorder, while adding a case from north India with a befitting clinical history, family history, neuroimaging and documented HTRA1 genetic mutation.

Background: Friedreich's ataxia (FRDA) is caused by homozygous GAA repeat expansions or compound heterozygous (CH) mutations in FXN gene. Its broad clinical spectrum makes it difficult to identify, thus an accurate diagnosis can only be made by genetic testing. Objective: This study aims to present data on FXN variants observed in patients with sporadic or recessive ataxia, including detailed data of the first CH Mexican patients. Materials and Methods: One hundred and eight patients with recessive or sporadic cerebellar ataxia were referred to our institution between 2009 and 2019 for FXN molecular testing. This was achieved using a combined methodology of triplet repeat-primed PCR (polymerase chain reaction), long PCR, FXN sequencing and multiplex-ligation probe-amplification. Results: Eighteen patients had a homozygous FXN genotype; whereas five were CH patients with a slow progression and phenotypic variability, including a late-onset case with spastic paraparesis, and a Charcot-Marie-Tooth-like case. Conclusions: These first Mexican CH patients pose important implications for genetic counseling and FRDA management.

Background: High-speed drilling during neurosurgical procedures lead to aerosol generation. Contaminated particles when dispersed can lead to transmission of disease in healthcare workers via air-borne route. We designed and validated the utility of an innovative microscope drape, in mitigating the dispersion of aerosol, while drilling on an ex-vivo model. Methods: Two consecutive high-speed drilling operative simulations were performed using a dry-model under operative-microscope with and without drape, respectively, to demonstrate the degree of particulate dispersion while high-speed drilling using a 10% Povidone-Iodine solution for irrigation. Results: There was significantly lower particulate dispersion and soiling of surgeon's gown, mask, and surrounding drapes outside the immediate operative field using the customized microscope mounted drape. Conclusions: Customized microscope mounted drape is effective in containing the widespread particulate dispersion associated with high-speed drilling under a microscope. The benefits of this specialized drape can translate into a safer micro-environment for operating surgeons during drilling procedures.

The management of established pulmonary thromboembolism (PTE) coexisting with acute ischemic stroke (AIS) is quite challenging. We report the case of a 52-year-old man with concurrent massive right middle cerebral artery AIS and acute PTE, who was successfully managed despite the contradictory guidelines to manage them simultaneously. The patient underwent decompression craniotomy followed by anticoagulant therapy. The current case report demonstrates that full-dose heparin, despite being relatively contraindicated in an AIS, can achieve a good outcome when given under close monitoring. The transesophageal echo with bubble contrast during the Valsalva maneuver demonstrated patent foramen ovale with a right to left shunt as a cause of AIS and PTE in this patient.

New-onset refractory status epilepticus (NORSE) describes prolonged or recurring new onset seizures which fail to respond to antiseizure medications. NORSE poses a challenge in diagnosis and treatment, and limited high-quality evidence exists to guide management. The efficacy of Electroconvulsive therapy (ECT) in aborting refractory status epilepticus has been described in case reports, but its application remains uncommon, particularly in young children. We describe a case of NORSE in a 3-year old child in which ECT played an important role in aborting status epilepticus, facilitating the diagnosis and surgical excision of an underlying focal cortical dysplasia. Although further research is needed, our case suggests that ECT can be a valuable tool in the treatment of refractory status epilepticus in children.

A disturbed iron metabolism may damage brain and trigger disorders known as neurodegeneration with brain iron accumulation (NBIA). NBIAs are rare, inherited disorders in which responsible mutations affect the function of proteins that participate in tissue iron homeostasis. Accumulated iron, which may be recognized as a low signal intensity on T2-weighted MRI images, oftentimes points to a diagnosis. Recent genetic discoveries confirm that NBIA is not a homogenous group of diseases. Fifteen different NBIAs have been described to date; among these, autosomal recessive inheritance was reported in 13, and autosmal dominant and X-linked dominant inheritance in one disease, respectively. Among NBIAs, the most common is pantothenate kinase-associated neurodegeneration (PKAN-NBIA 1) (30%–50% of all NBIA cases), that occurrs as a consequence of the autosomal recessive mutation in PANK2 gene, followed by phospholipase 2-associated neurodegeneration (PLAN, NBIA 2), due to mutation in PLA2G6 gene, and mitochondrial membrane protein-associated neurodegeneration (MPAN) with the underlying C19orf12 mutation [Table 1]. NBIAs are characterized by complex motor presentations from early-onset degeneration and premature fatality to adult-onset parkinsonism and dystonia. Epileptic seizures, pyramidal signs, visual disorders, and cognitive deterioration can develop. NBIAs are often refractory to therapeutical strategies, although certain interventions may provide significant symptomatic relief in selected patients. In this review, we discuss the expanding clinical spectrum of these complex and rare syndromes, their genetic and imaging features, and potential therapeutical targets and strategies.{Table 1}

Rise in intracranial tension (ICT) has varied clinical presentation which can range from subtle disturbances like headache to frank neurologic impairment. An important aspect is rapidity of rise of ICT. Pseudotumor cerebri is associated with many syndromes, toxication, and drugs. Our case is a unique one given the rarity of eltroxin, which is otherwise relatively safe drug and commonly used in this part of the world, induced Pseudotumor cerebri. Our patient had dramatic response to discontinuation of levothyroxine.

Polymyositis is a group of muscle disease characterised by progressive muscle inflammation and predominantly muscle weakness. It usually presents subacutely with proximal weakness and mild diffuse muscular pain. Some patients have atypical presentation like early respiratory difficulty, Motor neuron disease (MND), or isolated dysphagia which leads to delay in diagnosis and treatment. We present one such case.

Cerebrospinal fluid shunts provide many and different complications. Only 1–3% of them are rare, related to migration, and perforation of hollow organs. Even more unusual are the complications associated with the distal catheter leaving the peritoneal cavity and the tip migrating through some of the anatomical foramens to the muscle layers on the back. We present a rare complication, migration of the distal lumboperitoneal shunt catheter through the retroperitoneum and the gluteal muscles to the hypoderma. The peritoneal catheter entered the retroperitoneal area, passed through the greater sciatic foramen below the piriformis, and through the gluteal muscles, before it reached the hypodermal area. Despite the continuous improvement of the material of flexible silicone catheters, injuries of hollow organs with subsequent migration to unusual areas still occur. Our case is the only one that has been reported, in which the catheter made its way through the retroperitoneum and the gluteal muscles.

We present a rare case of Bickerstaff's brainstem encephalitis (BBE) overlapped with Guillain-Barré syndrome (GBS), which might be triggered by the patient's hypopituitarism condition. A 36-year-old woman with a history of hypopituitarism presented with a headache on the first day. Gradually, diplopia, ataxia, dysarthria, dysphagia, hypoesthesia, limb weakness, hypersomnolence, and respiratory muscle paralysis were developed in less than ten days. Based on brain computed tomography scan, magnetic resonance imaging scan, nerve conduction studies, cerebrospinal fluids analysis, anti-ganglioside antibodies and hormones tests, and clinical investigations, we diagnosed the patient with BBE overlapped with GBS. Treatment with corticosteroids and immunoglobulin resulted in clinical improvement. To our knowledge, this is the first case report of a hypopituitarism patient with BBE overlapped by GBS in English literature. Hypopituitarism patients have immune dysfunction. Based on previously reported autoimmune diseases associated with triggering GBS and its subtypes, hypopituitarism could be considered a noninfectious trigger.

Mutations in KMT2B (lysine-specific methyltransferase 2B) gene, which is primarily involved in methylation of Histone3lys4 (H3K4), has been recently described to cause early-onset generalized progressive dystonia (DYT28) by two independent researchers. Unlike other primary dystonias, mutations in KMT2B gene is associated with additional features like dysmorphism (elongated face, bulbous nose), microcephaly, short stature, and multisystemic involvement. Herein, we describe a 13-year-old boy with early-onset, generalized, progressive complex severe dystonia, along with mild intellectual disability, dysmorphism, and dermatological manifestations associated with a novel missense variation in KMT2B gene and also expand the phenotypic spectrum of the same.

Wernicke's encephalopathy (WE) is an acute neuropsychiatric disorder that results from thiamine (vitamin B1) deficiency. The typical clinical manifestations, which occur as triads in 20% of patients with the disorder, are acute mental status changes, ophthalmoplegia, and ataxia. Brain magnetic resonance imaging (MRI) has important value in diagnosis as it can reveal abnormalities in the thalamus, mammillary body, third and fourth ventricles, and periaqueductal area. Here we describe a 44-year-old female patient with WE, in the context of fasting following bowel surgery. The unique neuroimaging findings were symmetrical mammillary body and dorsal midbrain abnormalities, only evident on contrast-enhanced brain MRI.

Extraosseous primary spinal intradural Ewing's sarcoma (ES) is an unusual entity. Bleed within such tumors with acute neurological worsening is extremely rare, especially in the pediatric age. In this article, we present two children with intradural-extramedullary ES who had sudden decline in their neurological status consequent to an intratumoral hemorrhage. We discuss their clinical course and also briefly review the pertinent literature. Spinal intradural Ewing's sarcomas possibly have a tendency to bleed. A short clinical symptomatology along with an acute neurological deterioration and radiologic evidence of intratumoral hemorrhage in a spinal intradural tumor should raise the suspicion of an ES.

Hashimoto's encephalopathy (HE) is a rare neuroendocrine disorder, and there are no reports regarding anesthetic implications in a patient diagnosed with HE. These patients can have a plethora of systemic manifestations involving cardiovascular, respiratory, renal, gastrointestinal, hematologic, and central nervous systems. Once diagnosed, meticulous preoperative assessment is essential to rule out systemic involvement and to reduce the perioperative morbidity. Perioperative steroid therapy, neuromonitoring in the perioperative period, opioid free analgesia, and avoidance of nephrotoxic drugs are of paramount importance in the management of such patients. Awareness of the clinical condition and extreme vigilance can detect relapses in the perioperative period. Thus, a meticulous preoperative assessment, balanced anesthesia, perioperative neuromonitoring, and steroid therapy are essential to reduce the perioperative morbidity.

Transverse myelitis in multiple sclerosis is typically a short cord lesion with patchy distribution. Rarely, longitudinally extensive transverse myelitis can be seen in those with highly active disease or frequent relapses. The recognition of this uncommon phenotype in multiple sclerosis is important as the treatment is largely different from other demyelinating diseases. We describe a patient with highly active relapsing-remitting multiple sclerosis on interferon beta-1a who developed LETM after multiple relapses.

Primary pituitary tuberculosis (PTA) is a very rare disease. The clinical diagnosis is difficult as it is clinically radiologically indistinguishable from other sellar lesions. We present a case of PTA without any predisposing etiology and radiologically mimicking a pituitary macroadenoma. The patient underwent endoscopic transsphenoidal resection where pus admixed with mucoid was seen coming out of the lesion intraoperatively. Histology of the cyst wall was suspective of tuberculous etiology and QuantiFERON Tb Gold done for confirmation was positive. Postoperatively patient showed obvious improvement in visual symptoms. Patient developed diabetes insipidus and was put on desmopressin and was started with anti-tuberculosis medication for 18 months. Timely surgical intervention followed by anti-tubercular therapy and hormone replacement is the mainstay of treatment in these cases. For intraoperative management, we advise thorough wash with antibiotic and saline rather than curetting the walls of the abscess to decrease the postoperative incidence of endocrine abnormalities.

Ayurvedic medicine is an ancient and traditional system of health care. It is safe but inadvert and unsupervised use can lead to serious health complications. Lead is a common constituent of these medicines. Here authors describe two cases of lead encephalopathy as a result of long-term ayurvedic medication intake. First case was a 54-year-old female taking ayurvedic medications since long time presented with acute confusional state and memory disturbances with abdominal pain. MRI brain showed symmetric basal ganglia and cortical signal changes and edema with significantly elevated lead levels in blood. She responded to chelation therapy with oral penicillamine with complete clinical and radiological resolution. Second case presented was a 45-year female taking ayurvedic medications for hypertension presented with headaches and rapid deterioration in sensorium leading to coma and death. MRI brain showed diffuse cerebral edema with basal ganglia signal changes with elevated lead levels in blood. These two cases highlight the need for increased awareness that some Ayurvedic medicines may contain potentially harmful levels of lead and people who use them are at risk of developing associated toxicity which can even be fatal.

Lead poisoning is a multisystem disorder, more commonly affecting children. Occupational exposure, traditional medicines, and contaminated alcohol have been associated with lead encephalopathy in adults. Herein, we report a patient of lead toxicity presenting to the emergency services as acute encephalopathy with symptomatic hyponatremia and chronic recurrent abdominal colic and vomiting. This 50-year-old battery mechanic had multisystem involvement with anemia, basophilic stippling, lead line on the gums, and chronic hypertension. The blood lead level was more than 65 mcg/dL. Computed tomography of the brain showed intracranial calcifications and the MRI brain showed bilateral symmetric involvement of the thalamus, basal ganglia, brainstem, and external capsule. His sensorium improved rapidly after the correction of hyponatremia, however, apathy and psychomotor slowing persisted. This case highlights the importance of recognizing clinical markers and characteristic imaging findings, which can provide clues to an early diagnosis of this otherwise rare clinical condition, and prompt chelation therapy and avoid further lead exposure.

Background: The ongoing Coronavirus disease-19 (COVID-19) pandemic has revealed a plethora of extrapulmonary manifestations including neurological presentations. To date, nervous system demyelination has been relatively infrequently reported in this setting. Also, while most data point toward immune activation as a causative process, few studies propound a direct effect. Case Description: A 35-year-old man presented with severe new-onset headache, hemiparesis, and focal seizures culminating in deeply altered sensorium. Radiological evaluation showed a large expansile demyelinating lesion in the right cerebral hemisphere. Nasopharyngeal swab COVID reverse transcription–polymerase chain reaction (RT-PCR) was positive. After initial non-response to steroids, the patient responded well to plasma exchange leading to complete recovery. Conclusions: This report highlights a case of active severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection presenting with tumefactive demyelination and subsequent response to therapy. It is important to recognize atypical presentations at this juncture as it may be crucial for planning treatment strategies.

Behçet's disease is a multisystem inflammatory disease that manifests with oral–genital ulcers, skin lesions, arthritis, and ophthalmologic and neurovascular findings. Neurological involvement of Behçet's disease is called neuro-Behçet's disease, and it is a difficult entity to diagnose because of insufficient symptoms. We present a 26-year-old male patient with complaints of headache, dizziness, and drop foot in the right lower extremity. He underwent gross total tumor resection with the preliminary diagnosis of low-grade glioma, according to the preoperative magnetic resonance imaging. Histopathological examination and further investigation revealed an interesting neuro-Behçet's disease case because of the localization and clinical occurrence of the lesion.