Pure autonomic failure (PAF) is an alpha synucleinopathy with predominant involvement of the autonomic ganglia and peripheral nerves. The hallmark clinical feature is orthostatic hypotension. However, genitourinary, sudomotor, and cardiac involvement is also common. Many patients also develop supine hypertension. Almost a quarter of patients can phenoconvert or evolve into Parkinson's disease, multiple system atrophy, and Lewy body dementia in the future. Early severe bladder involvement, higher supine noradrenaline level, early motor involvement, and dream enactment behavior increase the risk of phenoconversion. The diagnosis is confirmed via autonomic function testing and serum noradrenaline measurement. The treatment is mainly supportive. The non-pharmacological treatment includes adequate fluid, dietary salt, compression stockings, and abdominal binders. The drug therapies to improve blood pressure include midodrine, fludrocortisone, pyridostigmine, and droxidopa. The diagnostic criteria need to be updated to incorporate the recent understandings. The treatment of orthostatic hypotension and supine hypertension is mainly based on case series and anecdotal reports. Randomized control trials are needed to ascertain the best treatment strategies for PAF.

In countries like India, many migraine patients presenting to primary care clinics fail to fulfill standard (ICHD 3) migraine diagnostic criteria. Since they do not present with typical ICHD 3 migraine diagnostic symptoms, it is necessary to define the criteria for atypical migraine. This would ensure that the patients receive the right treatment approach, both non-pharmacological and pharmacological. Looking for triggers, family history, activity affected and absolute normality in between attacks, past episodes of episodic syndromes, prodromal and oculonasal autonomic symptoms will help in identifying the migraine origin of these headaches.

Approximately 80% genes have an active expression in the brain and 40% of known genetic disorders affect the central nervous system. Revolutionary techniques like next-genome sequencing have solved the problem of the diagnostic odyssey in neurological genetic diseases. As several studies have shown the cost-effectiveness of next-genome sequencing compared to the older tests available in our diagnostic armamentarium, it becomes imperative to know about these genetic mutations and the tests available to diagnose them. We have tried to explain the basic concepts of genetics, the selection of available tests and their interpretation for neurophysicians.

Acute encephalitis syndrome (AES) refers to an acute onset of fever and clinical neurological manifestation that includes mental confusion, disorientation, delirium, or coma, which may occur because of infectious or non-infectious causes. Cerebrospinal fluid (CSF) pleocytosis generally favors infectious etiology, and a normal CSF favors an encephalopathy or non-infectious AES. Among the infectious AES, viral, bacterial, rickettsial, fungal, and parasitic causes are the commonest. Geographical and seasonal clustering and other epidemiological characteristics are important in clinical decision making. Clinical markers like eschar, skin rash, myalgia, hepatosplenomegaly, thrombocytopenia, liver and kidney dysfunction, elevated serum CK, fronto-temporal or thalamic involvement on MRI, and anterior horn cell involvement are invaluable clues for the etiological diagnosis. Categorizing the AES cases into neurologic [Herpes simplex encephalitis (HSE), Japanese encephalitis (JE), and West Nile encephalitis (WNE)] and systemic (scrub typhus, malaria, dengue, and Chikungunya) helps in rational utilization of diagnostic and management resources. In neurological AES, cranial CT/MRI revealing frontotemporal lesion is consistent with HSE, and thalamic and basal ganglia lesions are consistent with JE. Cerebrospinal fluid nucleic acid detection test or IgM antibody for JE and HSE are confirmatory. Presence of frontotemporal involvement on MRI indicates acyclovir treatment pending virological confirmation. In systemic AES, CT/MRI, PCR for HSE and JE, and acyclovir therapy may not be useful, rather treatable etiologies such as malaria, scrub typhus, and leptospirosis should be looked for. If smear or antigen for malaria is positive, should receive antimalarial, if negative doxycycline and ceftriaxone should be started pending serological confirmation of scrub typhus, leptospira, or dengue. A syndromic approach of AES based on the prevalent infection in a geographical region may be developed, which may be cost-effective.

Non-motor symptoms (NMS) are common among Parkinson's disease (PD) patients and have a significant impact on quality of life. NMS such as deficits in emotion perception are gaining due focus in the recent times. As emotion perception and cognitive functions share certain common neural substrates, it becomes pertinent to evaluate existing emotion perception deficits in view of underlying cognitive deficits. The current systematic review aimed at examining studies on emotion perception PD in the last decade. We carried out a systematic review of 44 studies from the PubMed database. We reviewed studies examining emotion perception and associated cognitive deficits, especially executive function and visuospatial function in PD. This review also examines how early and advanced PD differ in emotion perception deficits and how the presence of common neuropsychiatric conditions such as anxiety, apathy, and depression as well as neurosurgical procedure such as deep brain stimulation affect emotion perception. The need for future research employing a comprehensive evaluation of neurocognitive functions and emotion perception is underscored as it has a significant bearing on planning holistic intervention strategies.

Background: The significant burden of stroke on the mortality rates of developing countries, including India, is well-documented in the literature. However, robust data regarding the aggregates of evidence on the quality of life (QOL) of stroke survivors is limited. Objective: To gather relevant information for policymakers on the QOL of stroke survivors based on observational studies conducted in the Indian setting. Methods: We searched PubMed, Scopus, and Google Scholar for studies conducted in the Indian setting. The methodological quality of each study was scored, and data were extracted from the published reports. The risk of bias assessment was conducted based on the JBI Critical Appraisal Checklist criteria. The relevant data regarding QOL were analyzed by a random effects model using R software. Results: 16 studies were included in the systematic review in which the majority of the studies recruited study participants in the hospital-based setting with an average duration of 3-6 months following the stroke episode. Our findings suggest that the pooled mean quality of life in the four dimensions of the World Health Organization Quality of Life instrument (WHOQOL-BREF) were ranged from 46.86 to 61.37 and the overall Stroke Specific Quality of Life scale (SS-QOL) mean score was 157.16. There was a significant inconsistency among the included studies as heterogeneity was high (I² >97%). Conclusion: Assessment of the quality of life among stroke survivors is a crucial step to predict the illness' imposed burden and ascertain the effectiveness of the treatment. The present meta-analysis elucidates the aggregate estimates of quality of life and contributes to the research on the quality of life following a stroke in an Indian context.

Perimenopause is linked to increased migraine (Mg), especially menstrual Mg (MMg), influenced by hormonal changes. Compared to nonmenstrual attacks, menstrual attacks are more disabling and less responsive to treatment. Women with perimenstrual estrogen withdrawal have been linked to Mg during menstruation, whereas Mg during perimenopause has been linked to unpredictable fluctuations in estrogen levels. It has been widely established that female sex hormones play a role in Mg, but how it occurs remains unclear. This narrative review was identified using Medline and PubMed searches between 1946 and 2021. Search terms included “headache,” “migraine,” “menstrual migraine,” “menstruation,” “menopause,” “perimenopause,” “estrogen,” and “progesterone.” This article focuses on the candidate genes and female hormones that play a role in MMg. More study is necessary to understand better the environmental components that play a critical role in disease development. Currently, there is insufficient clinical evidence to support the function of menstrual Mg. The specific research facts examined MMg unique candidate genes and female hormonal factors that support their association and found MMg etiologic processes for generating an early diagnostic marker.

Background: Peripheral neuropathy in chronic kidney disease (CKD) is the most common neurological complication. We aimed to look at the prevalence and patterns of neuropathy in children with CKD. Methods: This cross-sectional study was conducted over 1 year in children with CKD, stage III and above. Nerve conduction studies (NCS) were performed as per standard protocols using surface electrodes on the muscles and by supramaximal stimulation of the corresponding nerves. Presence of electrophysiological abnormalities in the absence of clinical symptoms or signs was considered as subclinical neuropathy. Results: Nearly 45 children were evaluated. The majority were males (n = 39, 86.7%). The mean age was 7.9 ± 3 years (range 2–14). The mean estimated glomerular filtration rate (GFR) at enrolment was 23.3 ± 14.6 mL/min/1.73 m² (range 5–67). The majority of children were in stage III (n = 19, 42%), followed by stages V (n = 15, 33%) and IV (n = 11, 25%). There was no evidence of clinical neuropathy; 13 children (29%) showed subclinical neuropathy. All the nerves had an axonal pattern of involvement. Motor polyneuropathy was most common type of peripheral neuropathy. The commonest nerves involved were tibial and common peroneal nerves. There were no biochemical or clinical predictors of neuropathy in our cohort. Conclusion: The prevalence of subclinical neuropathy is high in children with CKD, stage III and above. Axonal motor polyneuropathy is the predominant pattern. Electrophysiological assessment of nerve function should be routinely done in children with advanced stages of CKD to prevent chronic complications.

Objective: To assess the impact of coronavirus disease 2019 (COVID-19) pandemic on sleep disorders among Parkinson's disease (PD) patients using validated questionnaires. Materials and Methods: This prospective study involved 50 PD patients and 50 age, gender, and body mass index-matched controls. All participants underwent assessment of cognition using Montreal Cognitive Assessment scale, sleep quality using Parkinson's disease sleep scale-2 (PDSS-2; for PD patients) and Pittsburgh Sleep Quality Index (PSQI; for PD patients and healthy controls), excessive daytime sleepiness (EDS) using Epworth sleepiness scale (ESS), insomnia symptoms and severity using insomnia severity index (ISI), restless legs syndrome (RLS) using International RLS Study Group criteria, rapid eye movement sleep behavior disorder (RBD) using RBD Single-Question Screen (RBD1Q), and depression using Patient Health Questionnaire-9 scale. Results: Eighty-eight percent of PD patients reported one or more sleep disorders, compared to 28% controls. While 72% of PD patients reported poor sleep quality (PDSS-2 ≥15, PSQI >5), 60% had insomnia, 58% reported RBD, 50% had EDS, and 36% reported RLS. Depressive symptoms were reported by 70% patients. PD patients with and without poor sleep quality were comparable with regards to demographic and clinical variables, except for depressive symptoms (P < 0.001). Depressive symptoms showed a significant association with EDS (P = 0.008), RBD (P < 0.001), and insomnia (P = 0.001). Conclusion: Prevalence of sleep disorders increased in PD patients during the COVID-19 pandemic. Prevalence of EDS, RBD, and RLS in PD patients was higher compared to that reported in studies during the pre-COVID-19 times. Presence of depressive symptoms was a significant correlate of presence of sleep disorders in PD patients.

Background and Aims: Guillain-Barré Syndrome (GBS), an immune-mediated neuropathy, is characterized by antibodies against gangliosides/ganglioside complexes (GSCs) of peripheral nerves. Antecedent infections have been reported to induce antibodies that cross-react with the host gangliosides and thereby have a pivotal role in conferring an increased risk for developing GBS. Data pertaining to the impact of various antecedent infections, particularly those prevalent in tropical countries like India on the ganglioside/GSC antibodies is sparse. We aimed at exploring the association between six antecedent infections and the profile of ganglioside/GSC antibodies in GBS. Methods: Patients with GBS (n = 150) and healthy controls (n = 50) were examined for the serum profile of antibodies against GM1, GM2, GD1a, GD1b, GT1b, and GQ1b and their GSCs by ELISA. These antibodies were correlated with immunoreactivities against Campylobacter jejuni, Japanese encephalitis (JE), dengue, influenza, zika, and chikungunya infections. Results: The frequencies of antibodies against six single gangliosides (P < 0.001) and their GSCs (P = 0.039) were significantly higher in patients as compared to controls. Except for GT1b-antibody which was more frequent in axonal GBS, none of the other ganglioside/GSC antibodies correlated with the electrophysiological subtypes of GBS. Antecedent JE infection was significantly associated with increased frequency of antibodies against GD1a, GD1b, GT1b, and GQ1b. Antibodies against GSCs were not influenced by the antecedent infections. Interpretation: This study for the first time shows an association between antecedent JE infection and ganglioside antibodies in GBS. This finding reinforces the determining role of antecedent infections on ganglioside antibody responses and the subsequent immunological processes in GBS.

Background and Objectives: Charcot-Marie-Tooth (CMT) disease is the commonest inherited neuromuscular disorder and has heterogeneous manifestations. Data regarding genetic basis of CMT from India is limited. This study aims to report the variations by using high throughput sequencing in Indian CMT cohort. Methods: Fifty-five probands (M:F 29:26) with suspected inherited neuropathy underwent genetic testing (whole exome: 31, clinical exome: 17 and targeted panel: 7). Their clinical and genetic data were analysed. Results: Age at onset ranged from infancy to 54 years. Clinical features included early-onset neuropathy (n=23), skeletal deformities (n=45), impaired vision (n=8), impaired hearing (n=6), facial palsy (n=8), thickened nerves (n=4), impaired cognition (n=5), seizures (n=5), pyramidal signs (n=7), ataxia (n=8) and vocal cord palsy, slow tongue movements and psychosis in one patient each. Twenty-eight patients had demyelinating electrophysiology. Abnormal visual and auditory evoked potentials were noted in 60.60% and 37.5% respectively. Sixty two variants were identified in 37 genes including variants of uncertain significance (n=34) and novel variants (n=45). Eleven patients had additional variations in genes implicated in CMTs/ other neurological disorders. Ten patients did not have variations in neuropathy associated genes, but had variations in genes implicated in other neurological disorders. In seven patients, no variations were detected. Conclusion: In this single centre cohort study from India, genetic diagnosis could be established in 87% of patients with inherited neuropathy. The identified spectrum of genetic variations adds to the pool of existing data and provides a platform for validation studies in cell culture or animal model systems.

Objective: 1. To study the clinical profile, progression and outcome of GBS during peripartum period in a tertiary care hospital. 2. To identify the determinants of outcome in GBS during peripartum period. Background: Guillain-Barré Syndrome (GBS) is an acute, autoimmune disorder of the peripheral nervous system triggered by a bacterial or viral infection or other antecedent events. Modern day critical care has dramatically improved the outcome of GBS. GBS during pregnancy is rare with an annual incidence of 2.8/100,000 population. Pregnancy itself is a life-threatening condition and full of complications. GBS during pregnancy makes its more complicated in terms of both maternal and fetal care during peripartum period. Methods: This was a prospective study conducted by the Department of Neurology, Gauhati Medical College including 36 patients of GBS presenting during peripartum period in between December 2019 and November 2021. Their clinical and electrophysiological features were analyzed. Hughes grading, EGRIS, mEGOS, MRC sum score were used. The fetal outcome was observed and patients were followed up for GBS outcome at 3 months and 6 months. Results: The mean age of patients was 25.97 years. AIDP was the most common subtype found in 21 (58.33%) patients followed by AMAN in 7 (19.4%), AMSAN in 3 (8.33%). In 3 cases NCS was equivocal and in 3 cases inexcitable. Respiratory distress was found in 13 (36.1%) cases, out of which 4 (11.1%) required mechanical ventilation and 1 (2.8%) died. The pre-term birth rate and stillbirth rate were 35.7% (n = 10) and 8.33% (n = 3), respectively with 66.7% (n = 24) spontaneous vaginal delivery(SVD). At 3 month 26 (72.2%) had complete recovery. 5 (13.9%) and 4 (11.1%) had partial and poor recovery, respectively. 78.9% of primigravidae had complete recovery in comparison to 64.7% in multigravidae indicating better outcome in primigravidae in this study. Conclusion: The outcome of GBS during peripartum period is favorable. Primigravidae are more commonly affected but have better outcome than the multigravidae. The risk of developing GBS in pregnancy decreases significantly after delivery and is minimal after 2 weeks. GBS is not an indication for LSCS. Stillbirth rate and preterm birth rate is higher in pregnancy associated with GBS without fearsome neonatal complications.

Background: One of the major challenges is to deliver adequate health care in rural India, where more than two-thirds of India's population lives. There is a severe shortage of specialists in rural areas with one of the world's lowest physician/population ratios. There is only one neurologist per 1.25 million population. Stroke rehabilitation is virtually nonexistent in most district hospitals. Two innovative solutions include training physicians in district hospitals to diagnose and manage acute stroke ('Stroke physician model') and using a low-cost Telestroke model. We will be assessing the efficacy of these models through a cluster-randomized trial with a standard of care database maintained simultaneously in tertiary nodal centers with neurologists. Methods: SMART INDIA is a multicenter, open-label cluster-randomized trial with the hospital as a unit of randomization. The study will include district hospitals from the different states of India. We plan to enroll 22 district hospitals where a general physician manages the emergency without the services of a neurologist. These units (hospitals) will be randomized into either of two interventions using computer-generated random sequences with allocation concealment. Blinding of patients and clinicians will not be possible. The outcome assessment will be conducted by the blinded central adjudication team. The study includes 12 expert centers involved in the Telestroke arm by providing neurologists and telerehabilitation round the clock for attending calls. These centers will also be the training hub for “stroke physicians” where they will be given intensive short-term training for the management of acute stroke. There will be a preintervention data collection (1 month), followed by the intervention model implementation (3 months). Outcomes: The primary outcome will be the composite score (percentage) of performance of acute stroke care bundle assessed at 1 and 3 months after the intervention. The highest score (100%) will be achieved if all the eligible patients receive the standard stroke care bundle. The study will have an open-label extension for 3 more months. Conclusion: SMART INDIA assesses whether the low-cost Telestroke model is superior to the stroke physician model in achieving acute stroke care delivery. The results of this study can be utilized in national programs for stroke and can be a role model for stroke care delivery in low- and middle-Income countries. (CTRI/2021/11/038196)

Neuropathy has been described in significantly higher proportion of PD patients than in control subjects. This study ascertains the prevelance of neuropathy and its determinants in PD patients, in particular relation with nutritional status, along with impact of neuropathy on Quality of life. Methods: This was a hospital based observational cross-sectional study of PD patients attending the Neurology OPD of a tertiary care hospital. The prevalence and type of neuropathy was determined using the validated MNSI scale. The nutritional status was assessed using MNA score and PDQ-39 was used for assessing quality of life. Patients with and without neuropathy were compared to ascertain risk factors for neuropathy. Results: Twenty-four out of 93 PD patients (26%) had neuropathy and 12 (50%) out of them had painful neuropathy. Older patents and those who had longer duration of disease had higher prevalence of neuropathy. In addition there was significant correlation with malnutrition and neuropathy. 79% of patients with neuropathy had abnormal nutritional status. On comparison of patients with painful neuropathy as compared to those without pain, Vitamin B 12 levels were found to be low only in the former group. Conclusions: Our study shows that there is significant prevalence of neuropathy in PD patents that affects the quality of life of PD patients. Neuropathy in PD is disease dependent and is precipitated by malnutrition. Hence, neuropathy must be timely diagnosed and effective nutritional management may help to improve the patient's quality of life.

Background: EMPOwER, a 12-week, double-blind (DB), randomized, placebo-controlled study evaluated the efficacy and safety of erenumab in adult patients with episodic migraine (EM) from Asia, the Middle East, and Latin America. This study analyzes the Indian experience for the use of erunumab for prevention of episodic migraine. Objective: The study aimed to evaluate the efficacy and tolerability of erenumab (70 mg and 140 mg) in EM patients from India. Methods: Randomized patients received monthly subcutaneous injections of placebo and erenumab 70 mg or 140 mg for 3 months. The primary endpoint was a change from the baseline in monthly migraine days (MMDs) at month 3. Other endpoints included achievement of ≥50%, ≥75%, and 100% reduction in MMD; a change in monthly acute migraine-specific medication treatment days; a change in patient-reported outcomes; and safety assessment. Results: Of the 539 patients screened, 351 patients were randomized (erenumab, 70 mg: n = 133 and 140 mg: n = 94; placebo: n = 124). The mean (±SD) age, disease duration, and MMD were 35.1 (±8.6) years, 6.77 (±6.01) years, and 7.82 (±2.89) days, respectively. The placebo-adjusted difference in mean MMD for erenumab 70 mg was -0.88 (95% CI, -2.16, 0.39; P = 0.174) days, and that for erenumab 140 mg was -1.01 (-2.42, 0.41; P = 0.164) days versus placebo. Secondary and exploratory endpoints demonstrated consistently better results in both erenumab dosage groups versus placebo. Treatment-emergent adverse events were comparable across groups (erenumab, 70 mg: 22.7% and 140 mg: 24.5%; placebo: 25.2%). Conclusion: Both doses of erenumab showed numerical improvement for efficacy endpoints and were well-tolerated in the Indian population. No new safety signals were reported.

Background: Mucormycosis (MCR) has been increasingly described in patients with coronavirus disease 2019 (COVID-19), but the epidemiological factors, neurological presentation, and outcome of such patients are not well described. Aims: To study the patient demographics, presenting symptoms and signs, the role of co-morbidities, medications used to treat COVID-19, and the outcomes of management and to study the spectrum of neuraxis involvement and its outcome. Methods: It was a prospective, observational, cross-sectional hospital-based single center cohort study. Confirmed MCR cases with and without COVID-19 were collected. The study was carried out over a period of 3 months from May to July 2021, followed by 3-month follow-up. Information on epidemiological factors, neurological findings, treatment (including medical and surgical treatment), and outcome was recorded. Results: A total of 141 patients were diagnosed with MCR, out of which 98 were COVID-associated MCR (CAM). The CAM incidence was 0.39% among COVID-19-positive patients. The MCR case fatality rate at 90 days was 43.9% but was higher for CAM than for non-CAM patients. Older ages (>50 years), diabetes mellitus, multiple risk factors, diabetic ketoacidosis on admission, brain involvement, and history of COVID-19 pneumonitis were associated with a higher risk for death. Conclusions: Possibly because of improper usage of corticosteroids, zinc, oxygen, and tocilizumab, there was sudden surge of cases of MCR in the COVID-19 pandemic. Therefore, treating physicians should use the COVID-19 pneumonia regimen judiciously. Neurological involvement itself is a poor prognostic sign, but combined surgical and medical management exhibited better outcome.

Background and Purpose: Cross-sectional area (CSA) is the most important parameter to study peripheral nerves by high-resolution ultrasonography. The aim was to acquire normative data of CSA of the main upper and lower limb nerves in the Indian population. Methods: CSA of nerves was determined in 100 healthy volunteers at 11 predetermined sites: median and ulnar at the wrist, mid-forearm, elbow; radial (spiral groove); tibial (popliteal fossa, medial malleolus); common peroneal (CPN, fibular head) and sural (lateral malleolus). Results: The mean age of participants was 40.7 ± 13.0 years (range: 18-79). Fifty were < 40 years of age. The mean height, weight and BMI were 161.5 ± 8.3 centimeters (range: 145—179), 58.6 ± 10.1 kilograms (range: 32-90) and 22.4 ± 3.2 kilogram/square meter (range: 14.03-30.44), respectively. The median and ulnar nerve measurements were non-uniform throughout its course, and the CSA was largest at the elbow and ulnar groove, respectively. With advancing age, there was a significant difference for median and ulnar nerves at the wrist (median P = 0.002, ulnar P = 0.009) and tibial nerve (popliteal fossa, P = 0.045, medial malleolus, P = 0.005), CPN (P = 0.047). Men had greater CSA of upper limb nerves and tibial nerves at popliteal fossa (P < 0.05) as compared to women. A positive correlation was noted with weight. Conclusion: It is apt for every defined population to have its own set of normative data of CSA as it varies with ethnicity, age, and the built of individuals. We provide a valuable set of CSA data for nerves in the Indian population, which can be used for comparison while investigating peripheral nerve disorders.

Background: Normative data for autonomic function tests (AFT) is not available for Indian population. Objective: The aim of the study was to establish normative data in AFT and its correlation with age, gender, and body mass index. Material and Methods: The study was done on 254 healthy subjects of age ≥18 years. All AFTs were done in autonomic laboratory at the Department of Neurology, Christian Medical College and Hospital, Ludhiana. Cardiovascular tests (heart rate response to deep breathing, HR changes in Valsalva maneuver and head-up tilt test (HUT)) and quantitative sudomotor axon reflex testing (QSART) were performed in all the subjects. Fifty subjects underwent thermoregulatory sweat test (TST). Results: The mean age (SD) of study participants was 43 (16.0) years (range 20–84), and 129 (50.8%) were men. The normative value range (2.5–97.5 percentile) for HR difference, E: I ratio, and Valsalva ratio (VR) was 3.5–47.0, 1.05–1.93, and 1.11–2.64, respectively, for all the subjects. HR difference and E: I ratio showed an significant inverse relation with age (r = -0.623 and r = -0.584, respectively). VR also showed an inverse relation with age (r = -0.575, P =< 0.001), and female had a lower value than male (1.63 vs 1.78, P =< 0.001). In QSART, mean (SD) sweat volume was higher in males 0.630 (0.230) compared to females 0.513 (0.132) for all sites, P < 0.001, and similar trend was noticed for sweat area in TST. Discussion and Conclusion: Normative AFT data has been established for Indian population for the first time. The values are comparable to previously published studies.

Background and Objective: Most of the existing qualitative facial nerve grading systems are very subjective while the quantitative grading systems are more complex, require longer data input time and specific software. There is a need for having a scoring system with graphic criteria to improve the subjectivity, reliability and convenience. We aimed to develop and validate such a reliable graphic scale for use in Bell's palsy. Methods: Face videos of patients with unilateral facial paralysis were recorded using smartphones and analyzed for six items including five voluntary facial movements apart from complications of facial palsy (synkinesis, hyperkinesis, and contracture). 15 videos were used for pilot study, 75 for the development of scale and 110 for its validation. Each video was rated on two separate occasions by 3 independent raters, a score of 0-4 was assigned to each item using the graphic scoring criteria, and a composite score was obtained (range 0–24). Five disease severity categories: normal (score 0), mild (score 1–6), moderate (score 7-12), severe (score: 13–18) and profound facial weakness (score: 19-24). Results: The proposed scale and its component items had high inter-rater and intra-rater reliability (Kappa >0.7). Good correlation (Pearson co-efficient >0.7) was seen among the voluntary movements. The proposed scale is a valid tool to score motor deficits and complications of facial palsy. Conclusions: The proposed scale is a valid and reliable graphic scale to describe facial motor dysfunction and its secondary defects.

Background: Prevalence of antibody-mediated autoimmune encephalitis (AE) is reported to be comparable to infectious encephalitis in Western populations. We evaluated the frequency and significance of AE and neuronal autoantibodies in comparison to infectious etiologies among patients presenting with encephalitis in a South Asian population. Methods: Ninety-nine consecutive patients with a clinical diagnosis of encephalitis/meningoencephalitis admitted to two of the largest tertiary-care hospitals in Sri Lanka were studied. PCR and ELISA were used to screen viruses while Gram stain and culture were used to screen bacteria. Sera were tested for antibodies binding to primary embryonic rat hippocampal neuronal cultures and cell-based assays for antibodies to NMDAR, LGI1, CASPR2, Contactin2, AMPAR, GABA_AR, GABA_BR, aquaporin-4 and MOG. Results: Patient ages ranged from 1 month to 73 years (mean = 24.91; SD = 21.33) with a male: female ratio of 1.75:1. A viral etiology was identified in 27.3% and bacterial meningoencephalitis was diagnosed in 17.1%. Sera of nine patients had antibodies binding to live primary neurons, but only five had specific antibodies to CASPR2 (n = 1), NMDAR (n = 2) or GABA_BR-antibodies (n = 2). Moreover, the patients with CASPR2 antibodies and NMDAR-antibodies were also positive for dengue antibodies. Only the two patients with NMDAR-antibodies had features and responses to immunotherapy consistent with AE. Conclusions: Identified infectious forms of meningoencephalitis (44.4%) greatly exceeded the occurrence of neuronal autoantibodies (9.1%) and AE (2%) in Sri Lanka, and this may be common in those regions where infections are prevalent.

Context: The medial plantar nerve (MP) sensory nerve action potential (SNAP) has been shown to be a sensitive indicator for detecting a length-dependent axonal peripheral neuropathy. However, literature survey shows paucity of age stratified data. This study was undertaken to obtain age stratified reference data for MP SNAP amplitude and latency. Aim: To establish age-stratified reference data in Indian subjects for the MP SNAP. Study Setting and Design: The study was conducted in the electrodiagnostic laboratory of a tertiary city hospital and is retrospective study. Materials and Methods: A retrospective study was conducted using the nerve conduction study reports of 173 patients with only upper limb symptoms and findings. Patients were between the ages of 18 and 86 years, stratified into six groups, a = 18-30 years, b = 31- 40 years, c = 41-50 years, d = 51- 60 years, e = 61-70 years, f ≥70 years. Statistical Methods: Stata 12.1 statistical program was used. Lower limit of the SNAP amplitude was obtained using mean-2SD of transformed data. Analysis of variance defined the intergroup variability, linear regression and Pearson's correlation assessed the statistical significance. Results: The lower limit of normal MP SNAP amplitude for each age group is as follows: a: 8.7uv b: 7.5uv c: 3.7 uv d: 2.9uv e: 2.0 uv f: 1.4uv. The amplitude difference between the groups b & c, c & d and e and f using analysis of variance with Bonferroni correction and Tukey post-hoc test was not significant, but the other groups showed statistically significant variance. The equation of regression for the predicted amplitude value with age was defined as Y^ = {3.5 + age (-.0233) – 2 (0.389)}³. Conclusion: This study provides age stratified reference data for MP SNAP. There is evidence to suggest that MP SNAP amplitude varies with age hence age stratified data should be used to define abnormality.

Chronic encephalitis manifesting as an epilepsy syndrome most commonly presents as Rasmussen's syndrome, usually characterized by epilepsia partialis continua, hemiparesis, and progressive cortical deficits such as aphasia, hemianopia, and cognitive decline. It is characterized by progressive hemispheric cortical atrophy on imaging and is usually seen in childhood. Adult-onset of the syndrome is rare, and only a few cases have been reported with bilateral symptoms. We present a patient with pseudobulbar affect and frontal lobe dysfunction who developed multifocal myoclonic jerks, right hemibody focal motor seizures, and right hemiparesis with bilateral cerebellar signs. Magnetic resonance imaging showed progressive hemispheric atrophy and bilateral features in Positron emission tomography–computed tomography (PET CT). Brain biopsy revealed chronic T-cell infiltrate. We discuss this case as the patient had several features that were atypical for Rasmussen's encephalitis (or syndrome).