Malignant atrophic papulosis (MAP), or systemic Degos disease, is an obliterative vasculopathy of unknown origin, characterized by erythematous papules found on the skin, central nervous system (Neuro-MAP) and gastrointestinal tract. Neurological involvement occurs in approximately 20% of systemic cases, is progressive and largely fatal. It can be described in two forms: 1) the parenchymal presenting with meningoencephalitis and meningomyelitis and 2) the neurovascular presenting with large cerebral infarcts, intracranial and subarachnoid hemorrhage, subdural hematoma and venous sinus thrombosis. Predilection to subdural hematoma or hygroma is characteristic for neurological involvement in MAP in comparison to other vasculpathies and vasculitides. Peripheral nervous system manifestations are less common and include polyradiculopathy, neuropathy, and myopathy. CSF analysis usually shows mild to moderate pleocytosis, increased protein content, and normal glucose. Brain MRI may reveal cortical, subcortical and deep white matter ischemic lesions with possible nodular, leptomeningeal, dural, or ependymal enhancement. Spinal cord MRI may reveal patchy lesions from the periphery to the center or cord atrophy in progressive course. Neurological involvement in MAP has a grave prognosis. The interval from onset of papulosis to death averages two years in patients with neurological involvement. There is no confirmed treatment for MAP but there are promising reports with eculizumab and treprostinil.

Background: The literature survey shows improvement in cognitive performance following acute bouts of physical exercise and chronic exercise patterns. However, neurocognitive growth through karate, a moderate intensity physical activity, is very limited. The synchronization of the nervous and endocrine system can be best reflected through this martial art form through neurogenesis and cognitive potentiation. Numerous outstanding reviews have summarized these findings for martial arts like judo and taekwondo. This review tries to orchestrate the efficiency of karate in neurocognition. Objective of the Study: The specific aim of this review paper is to magnify the efficiency of karate training in cognitive functions, through its response to neurochemical transmissions and electrophysiological signaling Materials and Method: Numerous related literature were evaluated, screened, and selected using the Preferred Reporting Items for Systematic reviews and Meta-Analyses eligibility criteria. All appropriate publications that satisfied the primary objective of the study were scientifically and schematically presented in this review paper. Results: The psychophysiological effect of karate training and their relation with brain functions have been elaborated. This review compiles the few studies established on the cognitive benefits of karate through the electrical stimuli and neurochemical release. Conclusion: Karate may be effective in advancement of particular brain functions and neurocognitive actions through a life time. Electrophysiological studies have unraveled improved neural efficiency, stress tolerance, working and muscle memory but need further exploration. Similarly, to better understand the effects of karate on neurochemical secretions, further research involvement is required.

Background: Rapid eye movement (REM) sleep behavior disorder (RBD), a parasomnia, after being diagnosed, can predict the emergence of an alpha-synuclein-associated neurodegenerative disease (NDD) in 20-45% and 92% of patients within 5 and 14 years, respectively. RBD is less common in tauopathies, and the studies to evaluate its association with polyglutamine diseases have been very few. Objective: To revisit our knowledge on the significance of RBD in the emergence of NDDs and to review the recent updates in the potential biomarkers, which can help predict the risk of phenconversion into NDDs in idiopathic RBD (iRBD) patients. We also aimed to look at the potential neuroprotective therapies that can potentially be used earlier in iRBD patients. Methods: We conducted a review of the papers, after selecting them from the PubMed database. After a thorough screening, 51 articles were chosen to be included in this review. Results and Conclusion: The prospective studies showed that the risk of phenoconversion of iRBD into overt NDDs increased over the longer duration of follow up. Magnetic resonance imaging (MRI) findings, Electroencephalographic findings along with subtle motor signs, autonomic dysfunction, impaired olfaction, and color vision, among others, can be used to predict the onset of an NDD in iRBD. Phytocannabinoids showed a possible neuroprotective effect in animal studies. Considering how RBD is the antecedent of NDDs, there is a need for additional studies to better understand the utility of the aforementioned biomarkers and institute potential neuroprotective therapies early in the process.

Background: Neuroethics is a subsection of ethics which allows us to express our concerns with advances in neurotechnology, threat to human race, our freedom of expression, autonomy, and justice. Objectives: Aim of the review is to familiarize readers about the concept of neuroethics and alarming threats with newer neurotechnology and to sensitize them about our responsibilities as neuroscientists. Materials and Methods: Systematic literature search was conducted from 2010 to 2021 in PubMed to look at previously published review articles related to neuroethics. Relevant filters were added addressing autonomy and consent. Of the 426 articles, only 12 addressed the issue of autonomy and neuroethics and two on consent and neuroethics. Results and Conclusions: International literature has expressed limited concern on the scope of neuroethics. Newer threats are getting added on with advancing technology, and as neuroscientists, we all need to understand and educate neuroscientists about our social responsibilities in curtailing an out-of-reach situation for the mankind.

Meningiomas are benign intracranial neoplasms arising from arachnoid cap cells. High grade meningiomas are uncommon and metastasis from these is an extremely rare event. Commonest sites of metastasis from high grade meningiomas include lung, liver, lymph nodes and bone. It is unusual for meningiomas to recur in the surgical track following excision. More so, it is even the rarest phenomenon for a meningioma to implant in subgaleal location. Various mechanisms have been proposed for the scalp implantation vis-à-vis CSF dissemination, direct surgical implantation etc., It may apply to all histological grades of meningiomas. Even the benign tumors have been shown to seed at postoperative scar. This seems to have provoked our interest to review the literature regarding this scalp implantation. We have reviewed all the cases where surgical excision of intracranial meningiomas has led to seeding of surgical track as well as scalp. We have discussed the various genetic aberrations that can guide us regarding the progression of the tumor and prognosis. We also report a case of surgical track and scalp implantation of an atypical intraventricular meningioma following excision.

Background: In recent years, noninvasive brain stimulation (NIBS) has shown promise for stroke rehabilitation as a novel nonpharmaceutical neuromodulatory intervention with attractive neurophysiological theories backing it up. Objective: To find out the short-term effects of NIBS techniques on motor impairment in chronic ischemic stroke. Materials and Methods: A systematic review with meta-analysis was performed separately for transcranial direct current stimulation (tDCS), transcranial magnetic stimulation (TMS), and studies that combined both, utilizing various databases for a period spanning from 2001 to 2019. Good-quality randomized controlled trials (RCTs) on chronic ischemic stroke cases with homogeneous clinical upper motor short-term outcome measures were considered for the meta-analysis. RevMan 5.1 software was used for the meta-analysis. Meta-analysis registration: CRD42021196299; https://www.crd.york.ac.uk/PROSPERO Results: A total of 319 studies were identified initially. After necessary filters to comply with the strict recruitment criteria, only four studies qualified, two each for tDCS and TMS and none qualified for analysis under the combined category. tDCS showed a nonsignificant effect on the upper limb motor function improvement (−0.10 [95% confidence interval {CI}: −0.84 to 0.64; I² 0%; P = 0.8]), whereas the repetitive TMS showed a significant effect (0.75 [95% CI: 0.03–1.48; I² 0%; P = 0.04]). The safety analysis did not reveal any major concerns for several published protocols. Conclusions: tDCS alone did not significantly benefit motor recovery; rTMS was effective in providing immediate functional benefits in chronic ischemic stroke. While the current stroke rehabilitation protocols with NIBS appear safe, more good-quality stratified RCTs with more innovative experimental protocols are needed to analyze and quantify the efficacy of these techniques in stroke rehabilitation.

Background and Introduction: In the modern era of spine surgery for subaxial cervical spine, transfacetal screw fixation has evolved enormously. Transfacetal screw fixation for subaxial cervical spine is a biomechanically effective technique. In this fixation, four cortical surfaces of the facets are purchased by the transfacetal screws. Objectives: In this video, we demonstrated the surgical technique of posterior transfacetal screw fixation. Surgical Technique: Transfacetal screw fixation of subaxial cervical spine was done along with posterior decompression. The entry point of transfacetal screw was defined as 1 mm caudal to mid-point of lateral mass, and screws were directed perpendicular to facet joint in the sagittal plane and straight in the coronal plane. Bone chips were placed over decorticated lateral mass after decompression. Result: Patient had uneventful recovery and maintained good status at follow up. Conclusion: In subaxial cervical spine, transfacetal screw fixation is a biomechanically effective, rigid, and an inexpensive technique to obtain immediate rigid fixation.

Background and Introduction: Spinal dural arteriovenous fistula (SDAVF) is a rare but curable condition. Microsurgery is a highly effective and readily affordable treatment modality. Objective: We present a surgical video of SDAVF to demonstrate the operative nuances involved. Surgical Technique: A 53-year-old wheelchair-bound man with spastic paraparesis for 1.5 years was found to have a SDAVF at L1/2 level with a single fistula point. During surgery, a L1-L2 laminectomy and durotomy revealed a dilated vein accompanying the nerve root exiting L1/2 foramen that showed early filling on indocyanine green (ICG) video angiography. This vein was occluded, and a segment of this vein was removed during surgery, which led to resumption of normal spinal cord perfusion. Results: The patient showed gradual recovery of lower limb motor power and improved to assisted ambulation after 3 months. Conclusions: Surgery is a simple, effective, and cost-effective treatment option in SDAVF.

Background: Complex craniopharyngiomas pose a significant surgical challenge owing to its proximity to critical structures and its intrinsic nature to resist radical excision. Objective: To show that endoscopic endonasal approach (EEA) is potentially a better alternative to transcranial approach in tumors that have been operated multiple times by transcranial route for achieving radical excision with minimal morbidity. Materials and Methods: A 32-year-old male previously operated twice through interhemispheric approach for craniopharyngioma presented with blurring of vision accompanied by headache and intermittent diplopia. Imaging revealed a large lobulated suprasellar recurrence with a large calcified part adjacent to left internal carotid artery. The tumor was resected by an extended endonasal approach. The patient experienced improvement in his vision with no significant endocrine complication. Results and Conclusion: This case demonstrates the surgical technique and various operative nuances of endoscopic endonasal resection of a complex craniopharyngioma.

Background: Corpus callosotomy (CC) is a major disconnection procedure that functionally isolates the cerebral hemispheres, thereby interrupting the spread of epileptic activity from one hemisphere to the other. It is extremely useful in children suffering from non-localized drug refractory epilepsy, especially drop attacks. The technique has evolved from microscopic to minimally invasive endoscopic surgery. The extent of callosotomy also varies based on the institutional practices ranging from anterior 1/3^rd to total corpus callosotomies (TCC). The performance of TCC in conjunction with anterior, posterior, and hippocampal commissurotomies was described for the first time by the senior author from our institution. Objective: To describe the technique of performing endoscopic total corpus callosotomy, and pan commissurotomy using the interhemispheric corridor. Methods: A seven-year-old right-handed male child with seizure onset at the age of six months presented with three types of semiologies consisting of myoclonic jerks, frequent head drops and tonic posturing involving right upper and lower limbs with secondary generalization. Results: Video electroencephalography (VEEG) revealed diffuse slowing of the background, and slow spike and wave pattern. Generalized paroxysmal fast activity (GPFA) was noted in the VEEG, suggestive of LGS. MRI brain revealed bilateral parieto-occipital gliosis and gross brain atrophy. Ictal SPECT localized to left temporo-occipital area, while magnetoencephalography revealed bilateral temporal localization. Patient underwent TCC with pancommissurotomy. The patient was seizure-free (ILAE Class 3) at one-year follow up with no drop attacks, and significant reduction noted in other seizure types. Conclusion: Endoscopic corpus callosotomy and pan commissurotomy using the interhemispheric corridor is an elegant and minimally invasive technique best suited for appropriately selected children with refractory epilepsy.

Background: Tumors of the axis (C2) are rare, which represents unique surgical challenges. Aim: To describe clinical outcomes and efficacy of transoral curettage and posterior instrumentation for treating C2 tumors, with a minimum 2-year follow-up. Setting and Design: This was a retrospective study conducted at a grade 3A hospital in China. Materials and Methods: From August 2015 to May 2017, patients diagnosed with benign C2 tumors were collected in the retrospective study. The Weinstein-Boriani-Biagini anatomic zone classification was used to characterize the tumors. The Japanese Orthopedic Association (JOA) score and the visual analog scale (VAS) pain scores were recorded pre and postoperatively. All patients were treated with posterior instrumentation and transoral curettage for tumor excision and iliac crest graft for arthrodesis. Results: Five patients were included in this report. The tumors involving C2 were all benign tumors. The JOA and VAS scores improved significantly at a 3-month follow-up, and the clinical symptoms were stable at the 12-month follow-up. Computed tomography (CT) scans at a 6-month follow-up showed solid arthrodesis in all patients. With a minimum 2-year follow-up, there was no implant loosening or tumor recurrence. Conclusion: The transoral curettage coupled with posterior instrumentation with iliac crest graft provides a reasonable option for the treatment of benign C2 tumors.

Background: Endovascular treatment is the preferred treatment for acute ischemic stroke (AIS) due to main artery steno-occlusive disease, but it has temporal and technical limitations. Moreover, there is no established treatment for progressive stroke. Superficial temporal artery (STA)-middle cerebral artery (MCA) bypass is sometimes considered as a treatment option. Objective: The objective of this paper is to review the treatment outcomes of patients with AIS undergoing urgent STA-MCA bypass. Methods and Material: This was a retrospective study including 32 patients diagnosed with AIS treated with urgent STA-MCA bypass at our facility. The patients had small infarct volumes and a large diffusion-perfusion mismatch. Results: New ischemic lesions in postoperative diffusion-weighted images were detected in 15 patients (46.9%), but only four (12.5%) developed paralysis. Hyperperfusion occurred in nine patients (28.1%), and five (15.6%) had bypass occlusion at 1 week. Delayed wound healing were found in four patients (12.5%). Neurological outcome was measured 3 months after onset: Manual Muscle Testing (MMT), 3–5 in 27 patients (84.4%); modified Rankin scale (mRS), 0–2 in 17 patients (53.1%); and 0–3 in 26 patients (81.3%). Prognosis was better in patients who underwent surgery after 24 h of stroke onset (mRS, 0–2 in 56.0% cases and 0–3 in 88.0% at 3 months). Statistical analyses revealed that MMT before surgery had a significant association with favorable outcomes (P = 0.041). Conclusions: Urgent STA-MCA bypass for progressive stroke may result in a good prognosis if the right patients are selected and may play an important role in cases treated 24 h after onset in whom endovascular treatment is ineffective.

Background and Objective: The aim of this study was to evaluate the contribution of extraocular muscle function testing with video-oculography (VOG), which is a noninvasive and easily applicable method of recording eye movement with digital cameras, to the diagnosis of myasthenia gravis (MG) in patients without any clinical eye movement abnormalities. Methods and Materials: The study included 18 patients prediagnosed with ocular MG: MG Group (N = 7) with abnormal, and non-MG Group (N = 11) with normal single-fiber electromyography. Control group included 50 healthy volunteers. Ocular movements were recorded with the EyeSeeCam VOG device. Results: The inward latency of the 10° horizontal saccade and the downward latency of the 10° vertical saccade were significantly delayed; and the downward amplitude of the 10° vertical saccade was significantly lower in the MG group. Receiver operating characteristic curve analyses showed high specificity values for the discrimination of MG patients. Conclusions: This study supports the usefulness of the VOG device in revealing subclinical extraocular muscle involvement in MG.

Background: Selenium is a trace element that protects against cellular damage by oxygen radicals through selenoproteins. Ischemic stroke is associated with the generation of oxygen free radicals resulting in a condition of oxidative stress. Objectives: The present study aimed to evaluate the effect of selenium supplementation on short-term and long-term acute ischemic stroke outcomes. Methods: This was a randomized, parallel, outcome assessor blind, placebo-controlled feasibility study on ischemic stroke patients admitted in Bou-Ali Sina Hospital, Sari, Iran (2015–2017). Inclusion criteria were adults with accepted ischemic stroke by neuroimaging during the last 72h with a volume of at least one-third of MCA territory. The primary outcome was the short-term outcome measuring with the National Institutes of Health Stroke Scale (NIHSS) and the modified Rankin Scale (mRS) on day 7. The participants (44 patients) were randomized into two groups (22 in each group), one receiving intravenous selenium selenite for 5 days, and the other 40 cc normal saline as a placebo. Results: A total of 40 ischemic stroke patients (18 females, 22 males) with mean age of 68.2 ± 10 years were investigated. Selenium supplementation improved short-term outcome, 15.7% by using NIHSS (66% vs 42%, RR = 0.85 with CI = 0.54-1.35; NNT = 10; 95% CI = 5.15- 2.53, P = 0.51) and 46.3% by using mRS (57% vs 12%, RR = 0.52 with CI = 0.31-0.88; NNT = 3; 95% CI = 1.49 -7.59, P = 0.01). The long-term outcome did not change significantly by considering Barthel index >75 after 3 months in comparison to comparator group (33.3% vs 29.4%, RR = 1.13 with CI = 0.40-3.16; NNT = 26; 95% CI = 2.77 -3.54, P = 0.81]. Conclusions: Selenium selenite supplementation in acute ischemic stroke can improve short-term outcome but cannot influence the long-term outcome.

Background: Stenosis of the ICA is an important cause of ischemic stroke and associated morbidity and mortality. Carotid artery stenting (CAS) and carotid endarterectomy (CEA) help to prevent impending or subsequent ischemic stroke in such patients. Aim and Objective:

• To study the outcome and adverse events associated with CEA and CAS.
• To determine the generalization of results obtained with multicentric trials such as CREST, etc., by comparing the results obtained by a single neurosurgeon in a community setting.

Material and Methods: From Jan 2014–Dec 2017, 80 patients presented with symptomatic carotid stenosis. Out of these 80 patients, 65 underwent intervention; 34 patients underwent CEA and 31 patients underwent CAS. Pre-defined variables like age, sex, and degree of stenosis were assessed as potential risk factors, and the patients' clinical features, radiological imaging, and procedural complications were documented. Results: The primary outcome of procedure-related stroke, major adverse events (MAEs), and death at 30 days follow-up and long-term outcomes of restenosis at 1 year were analyzed. Peri-procedural stroke occurred in 2 cases (6.4%) of CAS; one suffered an ischemic stroke and other suffered a hemorrhagic stroke. Three cases of CEA suffered procedure-related events; one (2.9%) suffered TIA while the other two developed postoperative local hematoma without neurological deficit; one was treated conservatively while the other required re-exploration due to pressure symptoms. Restenosis occurred in one case that underwent CAS. Conclusion: CAS and CEA are complementary approaches in treating symptomatic carotid stenosis even when performed by a single hybrid neurosurgeon as results obtained are commensurable to major studies like CREST.

Objectives: The aim of the study was to classify patients of Pseudotumor Cerebri Syndrome (PTCS) on the basis of newer classification along with its clinico-radiological correlations. Patients and Methods: A prospective study was conducted at a tertiary care teaching hospital. A total of 25 consecutive patients who fulfilled the new diagnostic criteria for PTCS were enrolled after taking informed written consent. A thorough clinical history, neurological examination and neuroimaging was carried out. Patients were classified into Primary and Secondary PTCS followed by Definite, Probable and Suggested as per new nomenclature. Results: Out of 25 patients, there were 9 patients in the primary PTCS group and 16 in the secondary PTCS group. Mean duration of symptoms was 40.84 ± 45.68 days. Primary PTCS group patients were significantly overweight and had higher BMI as compared to the secondary group with significant number of patients reporting recent rapid weight gain. The CSF pressure and protein were non-significantly higher in the primary PTCS group. We could classify 17 (68%) patients in our study as definite PTCS, 7 (28%) as probable PTCS and suggested PTCS in one patient. In secondary PTCS group there were 16 (64%) patients. APLA positivity was found to be the most common etiology for secondary PTCS with statistical significance (P = 0.025). Conclusions: Early recognition along with appropriate classification and prompt treatment can prevent vision loss in PTCS. Apart from obesity, recent weight gain is also closely related with primary PTCS.

Background: Optimal fluid management during neurosurgery is controversial. Evidences suggest that goal-directed fluid therapy (GDFT) can improve postoperative outcome. This study aimed to assess the intraoperative use of GDFT on the duration of hospital stay and postoperative complications in patients undergoing craniotomy for large supratentorial tumors. Materials and Methods: Forty patients of 18–65 years age undergoing large supratentorial tumor surgery were prospectively randomized into two groups. Control-group received fluid regimen based on routine hemodynamic monitoring, whereas patients belonging to GDFT group received fluid based on stroke volume variation (SVV)-guided therapy. A colloid bolus of 250 ml 6% hydroxyl ethyl starch was given, if the SVV was more than 12% in the GDFT group. Hemodynamic parameters, such as blood pressure and heart rate, and dynamic parameters, such as cardiac index, stroke volume index, and SVV, were recorded at different time intervals. Results: The total amount of fluid required was significantly lower in GDFT (P = 0.003) group as compared to the Control group. Intraoperative complications were significantly lower in GDFT group (P = 0.005), but the incidence of tight brain was significantly higher in the control group. The duration of hospital stay (P = 0.07) and incidence of postoperative complications (P = 0.32) were lower in GDFT group. Neurological outcomes at-discharge were similar in both the groups. Conclusions: This study did not show any benefit of GDFT over conventional intraoperative fluid therapy in terms of incidence of postoperative complications, hospital and ICU stay, and Glasgow outcome scores at-discharge in patients undergoing craniotomy for excision of large supratentorial tumors. However, the use of GDFT leads to better perioperative fluid management and brain relaxation scores. Clinical Trial Registry: CTRI/2016/10/007350.

Background: Surgical excision of giant (>4 cm size) vestibular schwannomas (VS) with preservation of facial nerve (FN) function remains a challenge. Objective: Our surgical technique using an extra-arachnoid plane of dissection and limited meatal drilling is described with the goal of improving FN preservation. Methods: Surgical results with respect to FN preservation were analyzed for two groups of giant VS patients: Group A–operated between 2002 and 2009 using “standard” surgical technique, group B—operated between 2009 and 2016 using extra-arachnoidal dissection and limited meatal drilling. All patients had a minimum follow-up of 1 year. Results: Group A: Of the 115 patients, total excision was possible in 103 (89.5%), near-total in 7 (6%), and subtotal in 5 (4.3%) patients. At a >6-month follow-up, 68 (59.1%) patients had good FN function (House-Brackmann [H&B] grades 1–2), while 21 (18.3%) patients had poor function (H&B grade 3–5). Grade 6 involvement was seen in 26 (22.6%). Five patients had lower cranial nerve impairment necessitating tracheostomy. Group B: Of the 98 patients, total excision was achieved in 70 (71.4%) patients, near-total in 9 (9.2%), and subtotal in 19 (19.4%). Four patients had repeat surgery; 14 underwent gamma-knife radiosurgery. At >6-month follow-up, 78 (79.5%) patients had good FN function (H&B grades 1–2), while 20 (20.4%) had poor function (H&B grade 3–5). Conclusions: With our 'modified' surgical technique of extra-arachnoidal dissection of VS throughout surgery and limited meatal drilling, an improved rate of functional FN preservation was observed.

Background: The COVID-19 pandemic has put the entire medical fraternity into a very challenging and demanding situation. Along with always being at the risk of COVID infection, healthcare workers (HCWs) are also facing neurological problems due to long working hours in personal protective equipment (PPE). These symptoms and their characteristics need to be observed and studied in-depth to understand the problems experienced by HCWs and to design new solutions to overcome such problems. Objectives: This study intends to evaluate the various neurological manifestations among the HCWs wearing PPE for prolonged periods. Materials and Methods: We conducted a questionnaire-based cross-sectional study at a Covid care center from western India from April 20 to June 01, 2021 by using a self-administered web-based questionnaire. A total of 256 HCWs were surveyed. The de-identified data were analyzed using JMP 15.0.0. Results: Among a total of 256 HCWs surveyed for this study, the majority (58.6%) were aged 24–35 years, with a male preponderance (65.62%, n = 168). Participants included doctors (41%), nurses (35%), paramedical staff (22%), and housekeeping staff (1%). The symptoms encountered among the HCWs wearing the PPE were headache, classified further as donning headache in 112 (44.98%), doffing headache in 56 (26.24%), slowed mentation in 48 (21.05%), and excessive sleepiness in 86 (38.74%), which affected their work performance. The age of the HCWs had a significant correlation with all the symptoms. Conclusion: Headache, slowed mentation, and excessive sleepiness was encountered among the HCWs wearing PPE, which depended upon the duration of PPE usage. The most common symptom was headache, which was of moderate to severe intensity.

Background: Lumbar interbody fusion has gained popularity for the treatment of spondylolisthesis in recent years. Both procedures have their own advantages and disadvantages. Objective: The aim of this study was to compare the clinical and radiological outcomes of MIS TLIF and OLIF in terms of visual analog score, Oswestry Disability Index, Intraoperative blood loss, and various other parameters. Material and Methods: This is a retrospective matched-pair comparative analysis between two groups who underwent Single level either OLIF or MISTLIF for spondylolisthesis operated from January 2017 to January 2020 at a Single institute by a Single surgeon. Results: Statistically there is no difference in various preoperative parameters between the two groups. The intraoperative blood loss is 112.5 mL in OLIF, and 144.6 mL is MISTLIF. The duration of surgery in OLIF is 4.41 h and 3.16 h in MISTLIF. The mean duration of the requirement of postoperative analgesia is 3 months after OLIF and 5 months after MISTLIF. The time taken for returning to regular activities is less in OLIF than MISTLIF. Conclusions: Although both OLIF and MISTLIF have almost equivalent outcomes, OLIF is better than MISTLIF in having lesser intraoperative blood loss and achieving better lordotic correction.

Background: Quality of health care is a major issue. Providing care that accommodates individual patient preferences and values is termed patient-centered care. Objectives: In this study, we assessed family satisfaction (FS) with intensive care unit (ICU) care and family satisfaction with decision making in the care of critically ill neurological/neurosurgical patients. Materials and Methods: The FS-ICU questionnaire was used to assess family satisfaction. Data were analyzed using frequency tables and rates. Results: Of the 154 FS-ICU questionnaires analyzed, the overall satisfaction rate with care was 59.97, with information needs was 56.52, and with decision making was 59.46. Lower satisfaction rates among families may be due to the highly morbid nature of neurological illness in their kin or due to differences in socioeconomic factors. Conclusions: Periodic audit of the FS questionnaire is useful in assessing the quality of health care in the neuro-ICU. Efforts to incorporate suggestions of study subjects may improve FS with patient care and decision making.

Background: Cytogenetic microarray (CMA) has brought a revolution in the field of cytogenetics by improving resolution by 500 times that of traditional karyotyping. Analysis and interpretation of whole genome copy number variations (CNVs) is quite a challenging task for clinicians. Some software packages and databases are available which are based on algorithm. However, there is no clear rule to decide the pathogenicity. Objective: To formulate a step-wise approach to evaluate the interpretation of a CNV and to help the clinicians to interpret the CNV reported by a laboratory with help of four representative cases of different phenotypes. Methods and Material: CMA was done using AffymetrixCytoscan 750K array in four cases from different families. Analysis was done based on the proposed approach. Results: The prediction of the effect of these CNVs depends on multiple factors and can change over time as the databases are expanded. CMA in four cases from different families revealed, a rare co-occurrence of 22q13.3 duplication and 22q13.3 deletion in a proband, a deletion along with mosaicism on 10q21.2 and 10p15.3, respectively, in the second case. Third case resulted in one variant of unknown significance of 1.2 Kb deletion on 10q11.22 and the fourth case showed a benign CNV. All CNVs were analyzed based on the proposed approach and helped in subsequent management and counselling of the families. The results indicate the effectiveness of a principled, feature-based, statistical framework for uncharacterized CNV interpretation, which future studies can expand upon to construct more reliable classifiers.

Background: Infectious spondylodiscitis of the lumbar spine is a common serious disease for which evidence-based therapeutic concepts are still lacking. Objective: This retrospective study compared the impact of the health status of patients on the length of hospital stay with regard to the treatment concept, i.e., antibiotic therapy or antibiotic therapy in combination with fixation surgery. Patients and Methods: The study included 54 consecutive patients with infectious spondylodiscitis of the lumbar spine who had been treated at our clinic between 2004 and 2013. Records included patient demographics, concomitant diseases, the neurological status and treatment modality, and the length of hospital stay. Results: 40 men and 14 women with a mean age of 64.2 (30-89) years were included. 13 patients were only treated with antibiotics (group A), 7 patients with abscess decompression (group B), 18 patients with early dorsal fusion (<10 days after admission) (group C), and 16 patients with late dorsal fusion (≥10 days after admission; group D). Patients undergoing early dorsal fusion had a significantly shorter hospital stay (33.2 days) than patients undergoing late dorsal fusion (57.0 days), P = 0.016. Mean hospital stay of patients treated with antibiotics was 30.3 days, that of patients receiving abscess decompression 57.8 days. Patients receiving only antibiotics had a significantly lower CRP level at admission than patients undergoing early fusion, P < 0.05. Conclusion: Patients with one or more relevant chronic concomitant diseases showed faster recovery, shorter hospital stays, and earlier return to daily routine after early dorsal fusion than after late dorsal fusion or abscess evacuation alone.

Syphilis is a reemergent infection worldwide. There has been a steady increase in incidence across many population groups in the last decades, requiring public health authorities' attention. We report a case of a 67-year-old man with neurosyphilis who presented with abrupt clinical ophthalmic changes and sensorineural hearing loss. The neuroradiological investigation demonstrated cranial nerves and vascular involvement, detected on high-resolution vessel wall imaging in brain magnetic resonance imaging. CSF and blood VDRL test were positive, as well as blood serum fluorescent treponemal antibody absorption test (FTA-ABS) and chemiluminescent magnetic microparticle immunoassay (CMIA). A test for the human immunodeficiency virus was negative. The patient was administered intravenous penicillin G for 21 days and was discharged well, with no neurologic signs.

Background and Purpose: Limited data is available regarding the prevalence of aortic plaques in cryptogenic ischemic strokes and its correlation with risk factors. Aim: To determine the prevalence of aortic plaques in patients with cryptogenic ischemic stroke and its association with vascular risk factors and future vascular events. Methodology: Patients with cryptogenic ischemic stroke/TIA evaluated with CT angiogram (CTA) were recruited. Aortic plaque thickness ≥4 mm, plaque ulceration, protruding components, and complex plaques were considered significant. Results: Of the 1,767 patients, 229 (12.9%) had cryptogenic ischemic stroke/transient ischemic attack (TIA). Of them, 36 (15.7%) patients were detected to have significant aortic plaques. The significant plaque group had a higher mean age (68.3 ± 8.3 vs 54.4 ± 13.2, P = 0.0005) and were more likely to have risk factors like hypertension (P = 0.025), coronary artery disease (CAD)(P = 0.015), and peripheral vascular disease (POVD) (P = 0.029). Plaque morphology showed plaques of ≥4 mm, ulcerated plaques, protruding components, and complex plaques in 14.8%, 5.6%, 3.1%, and 6.5% patients, respectively, which are predominantly located in the aortic arch (44%). At 1 year combined endpoint of stroke, TIA, and vascular death was significantly higher in the significant plaque group (19.4% vs 6.2%, P = 0.016). Advanced age (adjusted OR-1.11, 95% CI-1.07–1.17, P = <0.001) was identified as an independent risk factor for significant aortic plaques on multivariate analysis. Conclusions: Aortic plaques are an under-recognized cause of cryptogenic stroke. The significant risk factors in aortic plaque group highlight the importance of aggressive risk factor control for secondary stroke prevention.

Background: Prediction of outcome of West syndrome (WS) in relation to etiology and electrophysiology remain pertinent challenges. Objective: This study aimed to compare electro-clinical and imaging characteristics between WS of “unknown-etiology”; “symptomatic”WS; to gauge the evolution and impact of electroencephalographic (EEG) patterns on seizure outcomes. Materials and Methods: Electro-clinico-radiological data of 76 children with WS who were followed up for atleast 1 year was collected for reviewing clinical, therapeutic and EEG profiles (sub-typed as typical and modified hypsarrhythmia [HA]). Quantified seizure scores were assessed. Results: Among 76 children included in this retrospective analysis, 31 (40.8%) were of unknown-etiology and 45 (59.2%) were “symptomatic” (structural cause/developmental-encephalopathy). Children with symptomatic WS (p = 0.037), specifically with gliosis on imaging (p = 0.05) and typical HA (including the multifocal subtype; P = 0.023) were more likely to have other seizure types before onset of spasms and exhibit prior delay or regression in milestones (p = 0.017). There was negative correlation between time to diagnosis and reduction in seizure scores (r = -0.32; p = 0.005).Significant reduction was noted in seizure scores with pharmacotherapy, irrespective of etiology (P < 0.001 in unknown-etiology and symptomatic subgroups). Seizure freedom rates did not differ between typical and modified HA groups (p = 0.215) with a higher proportion of children with meaningful reduction in seizure scores in the former sub-group (p = 0.030). Children who failed to achieve seizure remission were more likely to exhibit developmental impairment (p = 0.019). Conclusions: Early diagnosis and initiation of optimal therapy is crucial towards improving outcome, irrespective of etiology (which impacts pre-spasm development) and HA subtypes.

Background: Subacute Sclerosing Pan Encephalitis (SSPE) may present with atypical clinical features and lead to diagnostic dilemma. Conventional magnetic resonance imaging (MRI) may be normal in early stage of SSPE. Objective: The aim of this work was to study the demography, clinical profile including atypical features of SSPE patients, and the utility of diffusion tensor imaging (DTI) as an adjunctive diagnostic tool to the anti-measles antibody and conventional MRI. Material and Methods: Consecutive 25 patients of SSPE were included. Clinical details were recorded at baseline and 6 months follow-up. Anti-measles antibody in serum and CSF, CSF/Serum Quotient reference (CSQ ref), and radiological details including comparison of DTI between SSPE patients and controls were also noted. Results: Of 25 patients, 17 (68%) were male. The most common presenting feature at onset was myoclonus with or without falls (13, 52%). Atypical features such as seizure, hemiparesis, and visual problems were present in 28% patients. At 6 months, 9 patients had progressive course (6 expired), 10 were static, and 6 lost to follow-up. MRI was normal in 8 (32%) patients (stage 2/3- 7/1). On comparison between SSPE patients (N = 10) and control (N = 10) groups, fractional anisotropy (FA), and apparent diffusion coefficient (ADC) values were reduced and elevated, respectively, at most of the regions of interest with significant difference at many sites. Conclusions: A significant number of patients (28%) had atypical features at onset. DTI is an adjunctive tool which supplements the conventional MRI and increase diagnostic yield. It may be a future option to assess disease progression and treatment response.

Background: Autonomic dysfunction can precede the onset of motor symptoms in Parkinson's disease and are very disabling, but often overlooked. Objective: This study was done to assess the presence of autonomic symptoms in PD; evaluate any gender differences and association of autonomic dysfunction with fatigue and quality of life. Methods: Demographic profile and disease characteristics of PD patients were assessed. Autonomic symptoms were evaluated by SCOPA-AUT, fatigue by FSS, and QOL with PDQ-39 in patient's vernacular language. Patients with SCOPA-AUT score ≥9 were considered as having autonomic impairment. Gender variation in the involvement of different domains were analyzed along with the risk factors for autonomic dysfunction. Results: Among 94 patients, 59 (63%) had autonomic dysfunction. All SCOPA domains showed significant impairment in PD. Nocturia (69.14%) and constipation (52.12%) constituted most frequent symptoms in our cohort. Mean urinary (7.89 vs. 6.05) and sexual (1.47 vs. 0.42) domain scores were significantly (P < 0.05) higher in males while cardiovascular dysfunction (0.81 vs. 1.64) was predominant in females. Patients with autonomic dysfunction had longer duration of disease (P = 0.033), severe disease (P = 0.0001) with higher UPDRS (P = 0.001). Autonomic symptoms correlated significantly (P < 0.0001) with Fatigue (r = 0.60) also. Patients with autonomic dysfunction had poor QOL (r = 0.67) and mobility, activities of daily living, emotional well-being, stigma, cognition, communication, and bodily-discomfort dimensions were worst affected (P < 0.05). Conclusions: There was a significant variation of autonomic symptoms with gender. Disease severity and duration were significant risk factors for autonomic dysfunction. Also there was a strong co-relation of fatigue and poor QOL with autonomic dysfunction.

Background: Combination fractures of the C1–C2 complex especially atlas and hangman are relatively uncommon and management usually compromises C1–C2 mobility. Objective: To evaluate the treatment of combined C1- hangman's fracture with and without intraoperative O- arm based navigation system, and its outcome in terms of preserving C1-C2 mobility. Methods: This was a case series of patients with combined C1 and hangman's fracture, managed at a tertiary care hospital during February 2009 to December 2016. Neurological function assessed with American Spine Injury Association (ASIA) impairment scale. Radiological fusion of the operated segment assessed with computed tomographic scan, criteria used for successful fusion included formation of callus across the fracture. Preservation of rotational motion between C1 and C2 was assessed by cervical flexion rotation (CFR) test. Results: We included 10 patients (male/female: 9/1; mean 47.7 ± 17.5 years) in our study. Operative intervention was performed in 9 patients. We used intraoperative computed tomogram (CT) scan with navigation in 5 patients. The mean follow-up period was 28.7 months (range 6 to 70 months). Neurological recovery occurred in all 4 patients with preoperative neurological deficits. Radiological fusion occurred in all cases. Rotation at C1-2 was preserved in all 5 cases operated under O-arm guidance and in one patient with type 1 fracture who was managed conservatively. Conclusions: The goals in treating these complex fractures are to achieve early maximum stability and preserving maximum range of motion. These are often competing phenomena, which can be achieved by using intra operative CT scan and navigation system.

Background: Liquid biopsies have emerged as convenient alternative diagnostic methods to invasive biopsies, by evaluating disease-specific biomarkers and monitoring the disease risk noninvasively. Phosphatase and tensin homolog deleted in chromosome 10 (PTEN) is a potent tumor suppressor, and its deletion/mutations are common in gliomas. Objective: Evaluate the feasibility of non-invasive detection of PTEN and its downstream genes in serum exosomes of glioma patients. Materials and methods: PTEN, Yes-associated-protein 1 (YAP1), and lysyl oxidase (LOX) transcript expression were monitored through polymerase chain reaction (PCR) in serum exosomes and their paired tumor tissues. The impact of PTEN and its axis genes expression on the overall survival (OS) was monitored. Results: Out of the 106 glioma serum samples evaluated, PTEN was retained/lost in 65.4%/34.6% of the tumor samples while it was retained/lost in 67.1%/32.9% of their paired exosomal fractions. PTEN expression in both tissue and paired exosomal fractions was observed in 48.11% of the samples. Sanger sequencing detected three mutations (Chr10: 89720791(A>G), Chr10:89720749(C>T), and Chr10:89720850(A>G). Both PTEN-responsive downstream genes (YAP1) and LOX axis were upregulated in the PTEN-deficient samples. PTEN loss was associated with poor survival in the glioma patients (hazard ratio (HR) 0.68, confidence interval (CI): 0.35–1.31, P = 0.28). The OS of the exosomal PTEN cohort coincided with the tumor-tissue PTEN devoid group (HR 1.08, CI: 0.49–2.36, P = 0.85). While, old age yielded the worst prognosis; gender, location, and grade were not prognostic of OS in the multivariate analysis. Conclusions: PTEN and its responsive genes YAP1 and LOX can be detected in serum exosomes and can serve as essential tools for the non-invasive evaluation/identification of aggressive gliomas.

Background: Systemic inflammatory markers have been found to be of value in predicting the prognosis in renal cell and gastrointestinal cancers. Recent studies in gliomas correlating with Systemic inflammatory markers (SIMS) have shown promise in predicting survival. Objective: Meningiomas are the commonest intracranial primary brain tumors in adults. There is a need to distinguish low and high grade meningiomas preoperatively as they have crucial implications for the radicality of surgical excision and prognostication. Methods: A retrospective search was conducted, and the newly diagnosed meningioma cases were studied. The preoperative hematological parameters of the patients and pathological tumor grades were noted. The grade 1 meningioma was defined as low-grade meningioma while grade 2 and 3 meningiomas were defined as high-grade meningioma. Statistical analysis was performed to determine the association between the hematological parameters and tumor grade. Results: Seven hundred and eighty meningioma cases with a mean age of 43.5 years were included. The ANC, NLR, and dNLR were found to be significantly elevated in high-grade meningioma (P = 0.03). Other inflammatory parameters including TLC failed to show a statistically significant difference when compared between the grades of meningioma. ROC analysis further showed limited value of these markers in predicting meningioma grade. However, an elevated ANC (OR = 1.08, 95% CI = 1.02–1.14), male sex (OR = 1.71, 95% CI = 1.13–2.58), and tumor origin from sites other than the skull base or thecal sac (OR = 3.33, 95% CI = 1.93–6.12) were predictive of high-grade meningioma in regression analysis. Conclusion: Preoperative hematological inflammatory parameters have limited value in predicting the grade of tumor in meningiomas. An elevated preoperative ANC, NLR, and dNLR were found to be more frequently associated with high grade of meningiomas.

Objectives: Hereditary sensory and autonomic neuropathy (HSAN) is a group of rare disorders affecting the sensory and autonomic neurons. Herein, we describe the clinical and genetic profile of six children with HSAN. Methods: Hospital records of six children diagnosed with HSAN over 7 years (2011–2018) were retrieved. Clinical features, electrophysiological studies, and genetic reports were collected from the case files. Results: The presenting clinical features in these six cases were developmental delay, recurrent febrile episodes, rhinitis, recurrent nonhealing ulcers, burns, self-mutilations, chronic osteomyelitis, and corneal ulcers. Electrophysiology studies showed predominant sensory axonal neuropathy. Autonomic features noted were recurrent fever, constipation, abdominal distension, hypertension, and vasomotor rhinitis. Genetic testing was done with next-generation sequencing in all six children. Causative genetic variants were identified in the NTRK1, PRDM12, DST gene, and a novel compound heterozygous variant in the FLVCR1 gene. The diagnosis of HSAN was delayed in most of our children due to variable presentation and lack of awareness among the treating paediatricians. Conclusions: Although the clinical presentation of HASN is highly variable, it is dominated by pain and temperature insensitivity and self-mutilation. Our report of six children with HSAN expands the existing knowledge on phenotype and genotype spectrum of HSAN.

Background: There is a strong need to identify simple and cost-effective biomarkers for multiple sclerosis (MS). Objectives: To evaluate the serum levels of receptor for advanced glycation end products (RAGE) ligand, the high-mobility group box (HMGB) 1 and its correlation with changes in the physical and psychological indicators in MS patients. Methods: During the 12-month follow-up, the serum level of HMGB1, expanded disability status scale (EDSS) score, rate of clinical relapse, quality of life, and other psychological indicators were assessed at baseline, after 6 months, and after 12 months and compared between 60 newly diagnosed MS patients with 60 healthy controls (HCs). Data were analyzed using t-test and Mann–Whitney U test, two-way repeated measures analysis of variance (ANOVA) and Spearman's rank correlation coefficient. Results: A significant decrease was observed in the EDSS score (P < 0.001) and a significant increase in the serum level of HMGB1 in all MS patients (P = 0.009). The serum level of HMGB1 was higher in MS patients, compared with HCs (baseline: 65.8%, P = 0.007; six-month follow-up: 73.9%, P = 0.004; and 12-month follow-up: 77.6%, P = 0.021). There were significant positive correlations between the serum level of HMGB1 and scores of MS impact scale-psychological subscale (MSIS-PS) (r = 0.59, P < 0.001), Beck depression inventory (BDI) (r = 0.491, P = 0.031), and Pittsburgh sleep quality index (PSQI) (r = 0.471, P = 0.035). Conclusion: The serum level of HMGB1 could predict the patients' psychiatric status better than their physical status.

Background: Rett syndrome (RTT) is a rare neurological disorder that primarily affects the females. Most cases of RTT are caused by a de novo mutation in the MECP2 gene located on the X chromosome. About 1000 MECP2 mutations have been found to be associated with RTT. Objective: The present study is aimed at the mutation screening of MECP2 gene in the RTT patients belonging to the south Indian state of Kerala. Materials and Methods: In total 22 girls with a clinical suspicion of RTT were recruited for the study. Exons 2, 3, and 4 of MECP2 were amplified and sequenced. Results: MECP2 mutations were observed in 12 patients. While 7 mutations were pathogenic, 4 were benign. All of the mutations were located in exons 3 and 4 of MECP2, spanning the methyl-CpG DNA binding domain (MBD), transcription repression domain (TRD), and C-terminal domain (CTD) domains of the MECP2 protein. Four novel mutations were identified. There were no mutations in the MECP2 gene of 10 patients with a clinical suspicion of RTT. Conclusions: A recommended screening strategy for RTT is to first look for mutations in exons 3 and 4 of MECP2, followed by exons 1 and 2, testing for large deletions in MECP2, and screening for mutations in genes, such as CDKL5 and FOXG1 that are reported to cause a Rett-like phenotype.

Background: Behçet's disease (BD) is a multisystem vasculitis, which can involve the central nervous system, the gastrointestinal tract, the blood vessels, the joints, and the lungs. Neurological involvement is about 5%, but the headache is common in these patients. Objective: This study aimed to investigate whether differences exist in the onset and types of primary headaches between patients with BD without neurological involvement (BWoNI) and patients with neurological involvement, that is, neuro-Behçet's disease (NBD). Materials and Methods: A total of 136 consecutive BD patients (98 BWoNI and 38 NBD) were evaluated and compared. Results: Primary headaches beginning before and after the onset of BD were found in 27.6 and 48.1% of BWoNI patients, respectively, and in 10.5 and 34.2% of NBD patients, respectively. The start of primary headaches was more frequent after then before the onset of BD in each group and overall. The primary headache types were similar in both BWoNI and NBD patients. Conclusions: The primary headache starts more frequently after then before the onset of BD, and characteristics of primary headaches were similar in both BWoNI and NBD patients.

Background: Vascular dementia (VaD) is a clinically heterogeneous entity. There is a dearth of studies for comparison of the cognitive profile of cerebral small-vessel disease (SVD) with large-vessel disease. Objective: We planned to evaluate and compare the cognitive profile of SVD and large-vessel VaD and evaluate various risk factors associated with them. Materials and Methods: Patients of VaD were recruited after excluding mixed and ambiguous cases. Patients were classified into SVD and large-vessel VaD and analyzed for their clinic-epidemiological and cognitive profiles. Results: Among 76 patients, 48 (62.5%) have SVD and 28 (37.5%) have large-vessel disease. Hypertension (93.4%) was the commonest risk factor, followed by smoking (34.21%), hyperlipidemia (26.31%), and diabetes mellitus (DM, 22.36%). Hypertension (P < 0.05) and DM were common in SVD, whereas smoking, hyperlipidaemia, and cardiac diseases were common in large-vessel disease. Attention (77.1% vs 25%), executive function (68.8% vs 28.6%), and calculation (58.3% vs 32.1%) were significantly more impaired in SVD compared to large-vessel disease, whereas visuoperceptual (21.4% vs 6.3%), praxis (28.6% vs 4.2%), and gnosis (14.3% vs 2.1%) were significantly more impaired in large-vessel disease than in SVD. Disruption of frontal-subcortical connection was responsible for the cognitive profile in SVD, but in large-vessel disease, it resulted from the cumulative loss of function from different lesions. Conclusions: Despite having common vascular risk factors, few are more common in SVD than in large-vessel disease. The different clinical and cognitive profile is due to the diverse anatomical lesions in these two subclasses of VaD.

Background: Despite enormous advances in the diagnosis and treatment of ischemic stroke over the past decades, the extent of “standard” investigation to define its causes is heterogeneous. Young patients often undergo a myriad of diagnostic tests in developed countries, but the cost-effectiveness of this approach is uncertain. Objectives: Our main goal was to compare the frequencies of ischemic stroke of undetermined and determined etiologies in young patients with “complete” or “incomplete” investigation according to either a stepwise or an extensive protocol. Methods: Data from 143 young patients with ischemic stroke were reviewed. For each patient, available data were assessed by means of a stepwise and an extensive protocol of investigation. We compared the frequencies of ischemic stroke of undetermined and determined etiology according to “complete” or “incomplete” investigation according to each protocol. Results: Completeness of investigation led to a significant increase in determination of stroke etiology when a stepwise approach but not an extensive protocol was applied. Conclusions: These results suggest that ordering an extensive workup to all young patients does not enhance the capability of determining causes of ischemic stroke. Evidence-based guidelines to define pathways of investigation and consensus about the interpretation of tests are deeply needed.

Aims: To study the trigeminal nerve tractography and assessment of diffusion tensor imaging (DTI) parameters by comparing the values of fractional anisotropy (FA) of trigeminal (V) nerve on the affected side with that of the unaffected opposite side. Materials and Methods: Prospective comparative study done for one year included 30 patients who presented with trigeminal neuralgia and 30 controls who did not have any present or past history of neurological or neurosurgical symptoms were enrolled in the study. Results: Most common age of presentation of TN is >50 years, and the commonly involved branch is V2 (maxillary branch of the trigeminal nerve). Most of the patients were having symptoms for more than 1 year. The superior cerebellar artery was the most common vessel compressing the V nerve at the root entry zone (REZ), followed in the order by anterior inferior cerebellar artery and petrosal vein. Neurovascular compression (NVC) was more commonly seen in the study group when compared with the control group. In all the patients in the study group, fractional anisotropy (FA) was decreased on the affected side as compared to the unaffected side. In the study group, 10 patients were having NVC on both sides, but FA was significantly decreased only on the affected side. No significant difference in FA values at the REZ of bilateral V nerves in the control group. Conclusion: DTI metrics of the trigeminal nerve is a very helpful imaging technique in patients with trigeminal neuralgia. It not only helps in anatomical imaging but also reinforces the association between NVC and TN.

Background: Subacute sclerosing panencephalitis (SSPE) is a rare entity characterized by a protracted course and progressive neurological deterioration. Objective: We present patterns of diffusion restriction in eight cases of SSPE, a seldom described imaging attribute. Methods: A retrospective analysis was performed on the clinical and neuroimaging data obtained from records of patients with proven SSPE. Patients whose magnetic resonance imaging (MRI) showed evidence of diffusion restriction were included in the analysis. MRI was performed on 3 T and 1.5-T clinical MR systems. Imaging characteristics were reviewed and tabulated by two neuroradiologists. Results: Eight SSPE patients (seven men, one woman; age range: 5–15 years; mean age: 11 years) diagnosed and managed at our institute were included in the analysis. Restricted diffusion was evident in the basal ganglia (n = 3), corpus callosum (n = 2), white matter (n = 2) and in bilateral middle cerebellar peduncles (MCP) (n = 2). One patient had diffusion restriction in the genu of the corpus callosum and bilateral frontal cortical white matter. None of the diffusion-restricted lesions showed contrast enhancement or susceptibility. Six cases fulfilled the diagnostic criteria for fulminant SSPE (fSSPE). The extent of neuroparenchymal involvement was greater in this subset of patients. Conclusions: Restricted diffusion in SSPE, hitherto infrequently described, can indeed occur in both grey and white matter structures and in both supratentorial and infratentorial compartments. Parenchymal diffusion restriction in SSPE possibly reflects an early time point in the clinical evolution. A greater extent of parenchymal diffusion restriction may portend a rapid downhill course, possibly qualifying for fSSPE.

Wernekinck commissure syndrome is a rare midbrain infarction, it consists of several symptoms including bilateral cerebellar ataxia, ophthalmoplegia, and palatal tremor. Holmes tremor is a rare clinical syndrome characterized by a combination of resting, postural, and action tremors. We describe two cases of Wernekinck commissure syndrome with Holmes tremor. To the best of our knowledge, it has been rarely reported in the literature to date. Both of the cases were presented with acute onset of bilateral cerebellar ataxia, dysarthria, and Holmes tremor. In the treatment, one patient was given “clonazepam and benheisol,” the other was received acupuncture therapy, both of them showed a marked improvement in ataxia and tremor.

Background: Extraskeletal mesenchymal chondrosarcoma (MCS) of the central nervous system (CNS) is extremely rare. Herein, we present the clinicopathological features of five CNS extraskeletal MCS. Material and Methods: Over the past 10 years, five cases of CNS MCS have been retrieved from in the archives of histopathology department. All biopsies were stained with vimentin, S-100, CD99, desmin, GFAP, INI1, WT1, STAT6, and EMA. Results: There were four males and one female patient in the age group of 1.5–35 years. The clinical and radiological impression was meningioma in three cases, glomus jugulare and primitive neuroectodermal tumor in one case each. All showed classic biphasic morphology, areas of undifferentiated small blue round cells sharply demarcated from the island of cartilage. Three patients experienced multiple recurrences and died subsequently. Conclusion: Extraskeletal MCS of CNS is rare and favors children and young adults. They show aggressive behavior and tend to recur despite surgery and radiotherapy.

Background: Stellate ganglion block (SGB) causes blockage of sympathetic nerve activity, which may lead to intracerebral vessel dilatation and relieve cerebral vasospasm in patients of aneurysmal subarachnoid hemorrhage (aSAH). Objective: The aim of this study was to evaluate the efficacy and safety of SGB to relieve cerebral vasospasm on clinicoradiological parameters. Materials and Methods: We prospectively included 20 patients with clinical and angiographic evidence of vasospasm post aneurysmal clipping. Cerebral blood flow velocity and Lindegaard ratio were assessed using transcranial Doppler (TCD). Location of vasospasm, vessel diameter, vasospasm severity, parenchymal filling time, and venous sinus filling time were assessed on digital subtraction angiography (DSA). Patients received ultrasound-guided SGB with 10 mL of 0.5% bupivacaine on the ipsilateral side of the vasospasm. After 30 minutes, the neurological status, TCD, and DSA parameters were reevaluated. Results: After SGB, there was statistically significant reduction in the middle cerebral artery (MCA) peak systolic velocity (P = 0.005), mean flow velocity (P = 0.025), and Lindegaard ratio (P = 0.022) on TCD. We observed significant dilatation in the mean vessel diameter measured at the mid-M1 segment of MCA (P = 0.003) and mid-A1 segment of ACA (P = 0.002) on DSA. The mean parenchymal filling time and mean venous sinus filling time decreased nonsignificantly after SGB (P = 0.163/0.104). Neurological improvement was observed in five (25%) patients. Conclusion: SGB has positive clinicoradiological influence in the management of cerebral vasospasm of large vessels. However, its effect on cerebral microvasculature is limited and needs a larger database for further analysis.

Only three cases of pleomorphic adenomas (PAs) of ectopic lacrimal gland mass in deep orbital location have been reported in the literature. This report describes a case of PA of the ectopic lacrimal gland situated deep in the orbit leading to proptosis, which was managed successfully by supraorbital orbitotomy via eyebrow incision. A 60-year-old female presented with complaints of gradually progressive, painless proptosis of the right eye. Imaging revealed a well-defined circumscribed mass lesion in the right orbit located predominantly in the postero-superior aspect of the globe. The patient underwent complete excision through supraorbital orbitotomy approach, using eyebrow incision. There was no clinical evidence of recurrence at eight months follow up. In orbit, PAs of the ectopic lacrimal gland are extremely rare, and the success of the treatment depends on the removal of the tumor with its capsule.

Background: Juvenile dermatomyositis (JDM) is one of the commonest forms of inflammatory myositis in childhood. Objective: The objective study was to study the clinical characteristics and course of JDM patients. Material and Methods: Retrospective analysis of the charts of 25 JDM patients admitted to two hospitals in Bangalore from March 2011 to July 2017. Results: The mean age at onset of disease was 7.74 ± 3.74 years. The male to female ratio was 1.5:1. All patients had skin rashes typical of JDM and 24/25 had demonstrable muscle weakness. Six patients were either lost to follow-up or died. Of the remaining 19 patients, 11 (57.9%) had a monocyclic course, 5 (26.3%) patients had a chronic continuous course, and 3 (15.8%) patients had a polycyclic course. Conclusions: JDM though rare should always be considered in the differential diagnosis in any child with skin rash and muscle pains and weakness. When diagnosed early and treated appropriately, sustained remission without medications is possible in a good proportion of patients.

Context: Paediatric pituitary adenomas (PPAs) are uncommon, with evidence confined to small cohorts. Aim: We aimed to elucidate the baseline profile and outcomes of PPAs in a large, contemporary, monocentric cohort. Settings, Design: Pituitary clinic at PGIMER over 8 years (2010-2018). Subjects and Methods: PPAs in patients (≤20 years at diagnosis) were included. A retrospective review of their baseline clinico-biochemical and radiological profiles and outcomes post pituitary surgery/medical management was performed. Results: There were a total of 74 patients, of which 42 were female. The median age was 15 (IQR 13-18) years. Corticotropinomas (32.4%) and somatotropinomas (25.7%) were common, with 1 case of TSHoma and pituitary blastoma. The most common presentation was headache (57%) overall and menstrual irregularities (64.2%) in girls. Most (78%) had macroadenomas. Prolactinomas showed an excellent response to primary medical therapy (83.3%). Transsphenoidal surgery was performed in 81% of patients. Diabetes insipidus (30%) and hyponatremia (26.7%) emerged as common postoperative complications. Adjuvant medical management was required in 25%, and radiotherapy in 18%. Remission rates in Cushing's and acromegaly were 62.5% and 57.8%, respectively, with long-term hormone deficits noted in one-third of patients. Conclusion: PPAs have unique features and management challenges, including effects on growth and puberty. Functional tumours and macroadenomas are common. Remission can be achieved in more than half of the patients, with endocrine deficits persisting in about a third of cases, needing long-term surveillance.

Hyperekplexia, an underdiagnosed motor paroxysm of infancy, mimics epilepsy closely. It is hallmarked by episodic and excessive startle response, brief episodes of intense, generalized hypertonia, or stiffness in response to unexpected auditory and/or tactile stimuli right from birth. Though a seemingly benign entity with an excellent prognosis, hyperekplexia has been occasionally associated with recurrent apneas, feeding difficulties, and sudden infant death syndrome (SIDS). We describe three unrelated children with hyperekplexia (two SLC6A5; one GLRA1). All three children had the onset of motor paroxysms from the neonatal period and were initially labeled as drug-resistant epilepsy leading to a variable diagnostic delay, the longest being 2.5 years. An excellent response to oral clonazepam with a good neurodevelopmental outcome was observed. The lack of habituation on the nose-tapping test is a simple clinical clue to the diagnosis. Early differentiation from epilepsy minimizes treatment cost, allays caregiver anxiety, and empowers them with abortive measures.

Background and Objective: Temporal trends in clinico-epidemiological parameters of stroke among Indian women have not been studied. We aimed to study the changes in these parameters over the last decade. Material and Methods: 417 strokes (ischemic/hemorrhagic) were prospectively recruited in two timelines. In total, 267 strokes were recruited in 2005 while 150 strokes were recruited in 2016–17. Patients underwent stepwise evaluation via a structured proforma. Demographic factors, stroke subtypes, and risk factors were analyzed. Results: Female strokes had a higher mean age in 2017 compared to 2005 (60.90 ± 16.9 vs. 53.21 ± 16.75 years, P = 0.002). Hemorrhagic strokes among females increased over the last decade. Female strokes with dyslipidemia were significantly lower in 2017 compared to 2005 (P = 0.002). Proportion of hypertensive and diabetic strokes were not significantly different between these two periods. Conclusions: Over the past decade, the mean age of stroke onset has increased while the prevalence of dyslipidemia has reduced significantly among Indian female stroke patients.

Background: Complete cervical spinal cord injury is devastating with the currently available treatment modalities offering no hope for improvement. Intrathecal pressure is raised following spinal cord injury due to injured and edematous spinal cord. Due to constraints of the thecal sac, this sets up a vicious cascade leading to further spinal cord injury. Durotomy and expansile duraplasty could potentially prevent this secondary spinal cord injury. The aim of our study is to assess the advantage of durotomy and expansile duraplasty in addition to spinal bony decompression and fixation for traumatic cervical spine fracture. Methods: Two patients with posttraumatic complete cervical spinal cord injury (ASIA A) were managed with expansile duraplasty in addition to decompression and fixation. A thorough examination including perianal sensations and bulbocavernosus reflex was done to rule out the possibility of incomplete cord injury with spinal shock. Both the patients underwent posterior decompression and lax duraplasty. Standard protocols of spinal cord injury were followed like maintenance of MAP >85 mmHg. Results: Both the patients showed significant improvement in clinical status improving to ASIA D from ASIA A. Conclusion: Durotomy and duraplasty may be offered in all patients with complete spinal cord injury who are undergoing instrumentation.

Background: In skull base surgery, zygomaticectomy is an effective method to increase surgical exposure and reduce brain retraction. However, the traditional zygomaticectomy methods are complicated and more invasive. Objective: To improve the procedure of zygomaticectomy, we introduced a modified technique to harvest integrated zygomatic arch-temporal bone flap. Subjects and Methods: A modified technique to section the zygomatic arch integrated with the temporal bone flap was described in the present work. This technique was applied in eight skull base lesion patients. The improved surgical angle was measured using Osirix software. Results: The surgical exposure is satisfied and no temporal lobe contusion or severe complications occurred in the patients. An increased surgical angle was obtained by zygomatic arch removing, with a mean value of 13.31°. Conclusions: This integrated zygomatic arch-temporal bone flap technique achieved increased exposure, decreased temporal lobe retraction, and minimal bone loss, leading to better cosmetics and functional reconstructions.

Hydatid disease is caused by Echinococcus granulosus. The liver is the first organ of entry, while the central nervous system is affected in about 2% of cases. Primary brain hydatid cyst is extremely rare. The cysts are generally located in cerebral lobes. Here, we reported a 68-year-old Syrian immigrant woman with hydatid cyst in the left cerebellopontine angle. The patient presented gait disturbance, asymmetrical palatal arcs, negative gag reflex, and disturbed cerebellar tests on the left side. The patient was operated via suboccipital craniotomy. The cyst ruptured during manipulation, but it was totally removed.

Oculomotor nerve palsy following traumatic brain injury is a rare entity. A head injury can result in diffuse neuronal axonal injury with subsequent microbleed within the nerve tissue. We report an unusual case of a patient who presented with complete isolated right third nerve palsy following a road traffic accident. In this patient, magnetic resonance imaging (MRI) revealed swelling and edema of the right oculomotor nerve along its cisternal segment with contrast enhancement. The acquisition of susceptibility-weighted images on MRI helped to clinch the diagnosis of intraneural hemorrhage as a cause of post-traumatic oculomotor nerve palsy.

Background: Hydatid cyst is a parasitic infection caused by Echinococcus granulosus and human is an accidental host. Liver and lungs are the most widely recognized organ of association but relatively low in other organs. Orbital involvement of the hydatid cyst is rare. Case Description: The case was a 47-year-old lady presented in the late stage of disease when corneal ulcer had developed and with complete vision loss along with headache, proptosis, and pain in the left eye. Cystic lesion in the retro-orbital region of the left eye was revealed by Computed tomography scan and magnetic resonance imaging. The patient underwent surgery. Hundreds of daughter cysts were seen intraoperatively and cyst wall sent for histopathological examination to confirm the diagnosis. The patient was further treated with albendazole for 3 months. Conclusion: Although hydatid cyst is uncommon, it ought to be considered in the differential diagnosis of cystic lesion of orbital locale, more so in the endemic territories.

Background: Primary intracranial malignant melanomas (PIMMs) are uncommon lesions of the central nervous system. Brainstem involvement is of rare occurrence. Methods and Material: A 33-year-old male presented with the chief complaints of progressively increasing headache, visual disturbances, and diplopia from the last 6 months. Magnetic resonance imaging (MRI) showed a well-defined lesion in the midbrain appearing heterogeneously hyperintense on T1WI and containing mixed hyperintense and hypointense areas on T2WI. Multiple areas of SWI blooming, suggestive of hemorrhage, were seen within the lesion. Conclusion: Brainstem malignant melanoma can masquerade cavernoma as seen in our case. Therefore, malignant melanomas should be considered in the differential diagnosis of brainstem lesions that presents with bleed.

Traumatic Pseudoaneurysm of Superficial temporal artery (STA) is an uncommon entity. Surgical management is the treatment of choice, and endovascular management is also equally feasible particularly for the proximal STA aneurysm. The manual compression for the treatment of STA aneurysm is also described, but it has a high chance of failure. We report a case of traumatic pseudoaneurysm of STA in a pregnant female, successfully treated with a modified manual compression technique.

Introduction: Hydatid cyst usually involves liver and lung. But it has been reported in uncommon locations like the spine and brain. There are few reports of cervical spine to brachial plexus. This report is about such extensively involved hydatid cyst management. Case Report: A 32-year-old male had complaints of neck pain, left-hand weakness, and atrophy. MRI of cervical spine and thorax revealed a multicystic lesion involving the cervical spine, vertebrae, brachial plexus, and lung apex. The lesion was removed in two stages. Discussion: Due to scarcity of literature, dense adhesions with dura and surroundings, risk of rupture, anaphylactic shock, and its management require proper planning. Excision of cervical, followed by plexus lesion, might help. Long-term use of albendazole prevents recurrence as suggested by other reports. Conclusion: Extensive spinal hydatid cyst is exceptionally challenging. These behave like malignant lesions. To get the best results, surgical planning is as essential as medical management.

Spontaneous (nontraumatic, nonsurgical) cerebrospinal fluid (CSF) rhinorrhea constitute only 3–4% of all cases. Here, we present a case of a 66-year-old male who presented with spontaneous CSF rhinorrhea, where intraoperative findings revealed an anterior clival defect as the cause. Such cases are extremely rare, and to the best of our knowledge only five of such cases are described in the literature. In this report, we discuss the possible etiology and management of this rare condition.

Background: “Thirteen-and-a-half” is a newly described clinical syndrome characterized by the combination of the one-and-a-half syndrome with fifth and seventh cranial nerve nuclei involvement (1^1/2 + 5 + 7 = 13^1/2). To the authors' knowledge, this is the first report of the thirteen-and-a-half syndrome secondary to pontine cavernoma and, overall, only the second reported case of this syndrome in the literature till date. Case Report: A 20-year-old man presented with the clinical features suggestive of the thirteen-and-a-half syndrome, explained radiologically by pontine cavernoma. We operated him using a suboccipital transvermian approach and he is doing well at 2.5 years follow-up. Interestingly, his one-and-a-half syndrome has partially improved to left horizontal gaze palsy. Conclusion: The clinical appreciation of the thirteen-and-a-half syndrome precisely localizes the lesion to ipsilateral dorsal pontine tegmentum. Neurosurgeons must be aware of the newly described “one-and-a-half- plus” syndromes as they help in a better understanding of pathoanatomy caused by different disease processes in the brainstem.

Background: Cavernous sinus hemangiomas are benign vascular tumors of the cavernous sinus. Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's angioma is a rare, aberrant thrombus organization occurring independently or in pre-existent lesions, and information on its occurrence in the intracranial region, or its management is sparse. Objective: We aim to describe a case of IPEH occurring in a pre-existing cavernous sinus hemangioma, treated with surgical excision and radiation therapy, providing insight into the appropriate treatment of these tumors and their response to therapy. Materials and Methods: We describe a right cavernous sinus hemangioma, recurring after initial radiation therapy and requiring surgical excision, at which point the presence of IPEH was noted. A sellar recurrence following partial excision was treated with hypofractionated Stereotactic radiotherapy (SRT), and this provided an excellent long-term outcome. Conclusions: Cavernous sinus hemangiomas are treated with surgical excision or primary radiation therapy. IPEH must be looked for in all excised specimens, as there are no radiological features that indicate its presence, and it may resemble the original lesion histologically. When detected, adjuvant radiation must be administered as the IPEH responds well to the use of radiation therapy.

Somatosensory-evoked potential and motor-evoked potential (MEP) are used during complex spinal surgeries to ensure the neurological integrity of the patient. Here, we report a case of a patient with type 3 thoracolumbar scoliosis whose surgery was stopped due to loss of signal in the neuromonitoring over the right lower limb in the exposure stage, before instrumentation and manipulation. The patient had right lower limb weakness and hence the surgery was abandoned. The same abnormality was present when we tried surgery for the second time. Surgery was aborted and the potentials recovered and the patient woke up without disability. In summary, this case illustrates that changes in MEPs can occur even at early stages in more vulnerable cases and is to be looked for to avoid disability.

Background: Mechanical stimulation may lead to internal carotid artery (ICA) dissection and aneurysm. Case Description: We encountered a rare case of ICA dissection and aneurysm with prolonged styloid process (SP) fracture. A 37-year-old sales worker presented with right-sided amaurosis fugax. After admission to a nearby optical clinic, he was admitted to our hospital. Computed tomography angiography (CTA) and digital subtraction angiography showed dissection and apparent aneurysmal change in the right cervical portion of the ICA. CTA also showed elongated SPs, so we diagnosed Eagle's syndrome, and fracture of the right-side process. After 2 weeks of antiplatelet therapy, the aneurysm enlarged and dissection remained, so we treated the patient with coil embolization and stenting. Conclusion: We encountered a rare case of ICA dissection and aneurysm with Eagle's syndrome. Endovascular treatment was performed because the SP was fractured.

A case of a patient admitted to the hospital for preterm labor, who was diagnosed with and treated for posterior reversible encephalopathy (PRES) is presented in detail. During labor, the patient experienced a sudden increase in blood pressure with disturbance of consciousness and visual disturbances. Medical imaging examination revealed vasogenic edema in multiple locations in the brain. After administering treatment neurological symptoms receded. The presented case proves that PRES should always be considered in similar cases.

Rosette forming glioneural tumors (RGNT) are a rare type of low-grade brain tumor included in 2007 in WHO classification. Given the benign nature of the disease, a complete surgical excision has been considered optimum. However, a handful of cases have reported the locally aggressive nature of RGNT. In addition, radiation may also be considered for a tumor located in areas where surgical excision is difficult. We present a similar case, where surgical risk was weighed against resection and we treated the patient with conformal radiation.

Schizophrenia is very rare before the age of 13, which is known as very early onset schizophrenia. There are few reports which document cases of schizophrenia before 5 years of age. Childhood onset schizophrenia has more chronic course characterized by sever social and cognitive consequences and increased negative and disorganization symptoms. Although diagnostic criteria of schizophrenia according to DSM5 are same as of adult onset but it's very difficult to explore psychopathology such as delusion and hallucination which depend upon cognitive development of the child. Further, the diagnosis of early onset schizophrenia is difficult because of similarity with neurological and metabolic disorder of childhood. This warrants comprehensive evaluation of such cases by multidisciplinary team consisting pediatrician, neurologist and psychiatrist.

Neurosyphilis, a chronic infection of the nervous system by Treponema pallidum can present in all stages of syphilis. Recently, it is found that neurosyphilis presents with novel manifestations. Here, we report a young patient who had neurosyphilis presenting as Normal Pressure Hydrocephalus (NPH) with bilateral optic atrophy. The patient showed improvement with treatment for neurosyphilis. Hence, it is evident that in young patients presenting with NPH and associated features, secondary etiologies should be investigated.

Background: Cardiac fibroelastomas are exceedingly rare etiologies of stroke, increasingly recognized with better diagnostic modalities. Surgical excision is the only curative option; however, timing of heparinization for cardiac bypass remains a challenging decision. Objective: We present a case of a 56-year-old female, diagnosed with multiple posterior circulation strokes and a left atrial mass, who successfully underwent anticoagulation and resection in 1 week. Material and Methods: MRI of the brain demonstrated numerous embolic-looking posterior circulation strokes. Transthoracic echo was negative for atrial pathology; a transesophageal echo revealed a left atrial mass, histologically consistent with papillary fibroelastoma. Results: Resection was delayed for up to 1 week to allow safe heparinization. We present a unique case of embolic stroke of undetermined source (ESUS) arising from a histologically rare tumor. Conclusion: We make the case for extensive workup for evaluation of posterior circulation strokes, including TEE, as well as timely resection of cardiac tumors.

Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis is a rare type of non-paraneoplastic limbic encephalitis (LE) mainly characterized by seizures, cognitive disorder, faciobrachial dystonic seizures (FBDS), hyponatremia, insomnia, and autonomic dysfunction. Here, we report the case of an elderly female patient who tested positive for antibodies against LGI1 and was initially thought to have Hashimoto encephalopathy (HE) due to its similar clinical features and the patient's high titers of antithyroid antibody. Interestingly, during the course of the disease, the patient exhibited typical FBDS and brain magnetic resonance imaging (MRI) showed a hyperintense signal evolution from T2/Fluid attenuated inversion recovery (FLAIR) to T1-weighted image in the bilateral basal ganglia (BG), which have rarely been reported previously.

Identification of the precise cause is fundamental to the secondary prevention of acute ischemic stroke. Several uncommon causes of stroke have been identified as a result of the attempts made to identify the cause of a cryptogenic stroke. However, unless proper protocol is followed each time, without missing any steps, it might not be possible to accurately determine the cause of strokes. Herein, we report a case wherein the cause of stroke was not detected in time because a fundamental step was missed while attempting to determine the cause. The 44-year-old male patient in our case developed recurrent ischemic stroke due to vertebral artery dissection. Although vertebral artery dissection is not a rare cause of stroke, it was missed while focusing on the rare embolic condition, Valsalva sinus aneurysm. Although a cardiac embolism is a potential cause of stroke in the present case, it is critical to examine all parts from the ostium to the end of the relevant artery (vertebral arteries) when checking for the possible causes of stroke. The ideal protocol to be followed when determining stroke etiology would be as follows: 1) identifying anatomical location and vascular territory of the lesion, 2) evaluating relevant arterial pathology or testing potential embolic source according to the presence of one relevant artery for ischemic lesion, and 3) performing a detailed evaluation of the rare causes of stroke.

Movement disorders are common presentations to psychiatry and neurology clinics in general hospitals. Many a times, liaison between psychiatry and neurology helps in determining the exact etiology and diagnosis of rare movement disorders. Paroxysmal dyskinesia is a group of disorders caused by a genetic mutation. It is one of the rare presentations among dyskinetic disorders but often encountered by psychiatrists due to the psychogenic nature of episodes and apparently normal neurological findings in-between the episodes. Liaison work with a neurologist is of great importance rather than misdiagnosing them as a dissociative or psychogenic movement disorder. Unawareness of their presentation can lead to repeated consultations, unwarranted psychotherapies, and can create a sense of therapeutic nihilism among the treating psychiatrists. Predominantly four different variants are described in the literature under this group and new classification has been proposed recently. We encountered a case of paroxysmal kinesigenic dyskinesia with obstructive sleep apnea masquerading as a dissociative disorder.

Background: There is an intriguing relationship between familial Mediterranean fever (FMF) and multiple sclerosis (MS). While FMF is a hereditary autosomal recessive disease characterized by recurrent, acute, self-limited attacks of fever and polyserositis, MS is a chronic, inflammatory demyelinating disease of the central nervous system, characterized by autoreactive lymphocytes, microglial activation, and chronic neurodegeneration. In patients suffering from both FMF and MS, it would be interesting to test whether treatments for MS affect the course of FMF, or vice versa. However, this interaction has not been studied yet. Case Presentation: Herein is reported a case with FMF and relapsing-remitting MS in whom fingolimod, an immunomodulatory oral MS therapy, led to near-complete resolution of FMF symptoms. Conclusion: This report demonstrates an interesting clinical observation which may have promise for patients suffering both from MS and FMF. The drug's effect on the course of FMF needs further research.

Eosinophilic myelitis is an important cause of transverse myelopathy and has to be considered in an appropriate clinical setting. Eosinophilic myelitis due to parasitic infection should be suspected in cases with cerebrospinal fluid (CSF) eosinophilia along with migratory serpiginous skin lesions and recent travel to endemic areas. We report a case with a 1-month history of fever followed by truncal paresthesias, erythematous creeping skin eruptions, and paraparesis with blood and CSF eosinophilia on a background history of consuming undercooked fish. Magnetic resonance imaging (MRI) spine showed long segment T2 hyperintensities with contrast enhancement. He was tested positive for 24kDa antigenic component of Gnathostoma spinigerum in CSF and serum by immunoblot testing. The patient showed significant improvement with parenteral steroids.

Obsessive compulsive disorder (OCD) is a common neuropsychiatric disorder involving predominantly the cortico-striato-thalamo-cortical loop. Although it is usually associated with various disorders of basal ganglia and thalamus, it is difficult to say what kind of impairment causes this situation exactly. Structural brain lesions may be one of the rare causes of refractory psychiatric symptoms. Analysis of such type of cases gives an idea about the neurobiology of psychiatric diseases. In this manuscript, we presented a case of refractory OCD with symptoms regressing after thalamic infarction and discussed with relevant literature.

Myoclonus–dystonia syndrome (MDS) is an autosomal dominant disorder due to a mutated epsilon–sarcoglycan gene (SGCE) at the dystonia 11 (DYT11) locus on chromosome 7q21-31. ε-sarcoglycan has been identified in vascular smooth muscle and has been suggested to stabilize the capillary system. This report describes two siblings with MDS treated with bilateral globus pallidus interna deep brain stimulation. One patient had a history of bleeding following dental procedures, menorrhagia, and DBS placement complicated by intraoperative bleeding during cannula insertion. The other sibling endorsed frequent epistaxis. Subsequent procedures were typically treated perioperatively with platelet or tranexamic acid transfusion. Hematologic workup showed chronic borderline thrombocytopenia but did not elucidate a cause-specific platelet dysfunction or underlying coagulopathy. The bleeding history and thrombocytopenia observed suggest a potential link between MDS and platelet dysfunction. Mutated ε-sarcoglycan may destabilize the capillary system, thus impairing vasoconstriction and leading to suboptimal platelet aggregation.

CLIPPERS is a rare subacute inflammatory disorder of the CNS, with a poorly understood pathogenesis and with heterogeneous clinical manifestations. Reports of extra-pontine involvement in this syndrome are rare, and as far as we know there are only two cases reported with simultaneous involvement of the CNS and pulmonary parenchyma. We report the case of a young patient with a subacute condition of cognitive dysfunction, as well as motor, sensory and balance changes. Imaging showed pontine and extrapontine brain involvement in association with multiple pulmonary nodules, characterizing a very unusual presentation of this syndrome. It is important for radiologists to be aware of that rare presentation in order to make a prompt diagnosis of this condition, enabling early treatment and avoiding permanent neurological sequelae.

Background: Postmarketing surveillance of COVID-19 vaccination reveals that the COVID-19 vaccine administration is associated with several rare but serious neurological complications. Case Report: We report a case of new-onset tumefactive demyelinating brain lesion that developed after administration of an adenovector-based COVID-19 vaccine. A middle-aged female presented with recent right hemiparesis, which was noticed 2 days after she received the first dose of the vaccine. Magnetic resonance imaging (MRI) revealed a large subcortical T2/FLAIR hyperintensities involving corpus callosum as well. The patient responded to oral methylprednisolone. At 4 weeks, a follow-up MRI revealed a reduction in size of the lesion. Conclusion: To conclude, adenovector-based COVID-19 vaccination may be associated with a tumefactive demyelinating lesion.

Spotted fever group (SFG) Rickettsial infection has rarely been reported to develop severe central nervous system involvement. We report a case of meningoencephalitis presenting with fever, headache, drowsiness, seizures, and a skin rash, showing ELISA IgM positivity to SFG Rickettsia group. MRI brain showed multifocal bilateral asymmetric patchy areas of T2W/FLAIR hyperintensity with significant diffusion restriction. Treatment with doxycycline resulted in clinical & radiological improvement. Rickettsial meningoencephalitis is a relatively under-diagnosed entity, probably due to the low index of suspicion and the lack of definitive diagnostic facilities in developing countries. This case was presented to highlight the importance of clinical suspicion mainly in endemic areas, the potential severity of the disease, and the need of early initiation of therapy to prevent mortality and long term neurological morbidity.

Background: We report a 74-year-old lady with hemichorea of 6 months duration due to severe carotid stenosis without cerebral infarction. Electroencephalography and single-photon emission computed tomography studies suggested hemispheric abnormality. Hemichorea resolved following carotid endarterectomy. Discussion: Hemichorea is the commonest movement disorder following acute stroke. It is rarely reported in association with severe carotid stenosis without evident infarction. In the absence of corresponding structural abnormalities on magnetic resonance imaging in hemichorea, vascular imaging is critical in establishing the diagnosis. Hemichorea in setting of carotid or vascular stenosis is often resistant to medical treatment and best responds to reperfusion. Conclusion: Hemichorea due to critical hemispheric ischemia is a well-described rare entity that best responds to reperfusion strategies. Awareness is essential to identify this entity and manage appropriately.