Analysis of Semiology, Lesion Topography and Treatment Outcomes: A Prospective Study on Post Thalamic Stroke Holmes Tremor.

OBJECTIVE: Holmes tremor (HT) comprises rest, postural and intention tremor subtypes, usually involving both proximal and distal musculature. Perturbations of nigro-striatal pathways might be fundamental in the pathogenesis of HT along with cerebello-thalamic connections. METHODS: Nine patients with an HT phenotype secondary to thalamic stroke were included. Epidemiological and clinical records were obtained. Structural and functional brain imaging were performed with magnetic resonance imaging (MRI) or computed tomography (CT) and positron emission tomography (PET), respectively. Levodopa was administered in sequentially increasing dosage, with various other drugs in case of inadequate response. Longitudinal follow-up was performed for at least three months. The essential tremor rating assessment scale (TETRAS) was used for assessment. RESULTS: The mean latency from stroke to tremor onset was 50.4 ± 30.60 days (range 21-90 days). Dystonia was the most frequently associated hyperkinetic movement (88.8%). Tremor was bilateral in 22.2% of participants. Clinical response was judged based on a reduction in the TETRAS score by a prefixed value (≥ 30%), pertaining to which 55.5% (n = 5) of subjects were classified as responders and the rest as non-responders. The responders showed improvement with significantly lower doses of levodopa than the remaining nonresponders (240 ± 54.7 mg vs. 400 ± 40.8 mg; p = 0.012). CONCLUSION: Although levodopa is useful in HT, augmenting the dosage of levodopa beyond a certain point might not benefit patients clinically. Topography of vascular lesions within the thalamus might additionally influence the phenomenology of HT.

Gastrointestinal, Respiratory, and Olfactory Neurotropism of Sars-Cov2 as a Possible Trigger of Parkinson's Disease: Is a Multi-Hit Multi-Step Process on the Cards.

Since the first emergence of COVID-19 on the global stage, there has been a wealth of evidence to suggest that SARS-Cov2 is not merely a pulmonary pathogen. This virus is unique in its ability to disrupt cellular pathways related to protein homeostasis, mitochondrial function, stress response, and aging. Such effects raise concerns about the long-term fate of survivors of COVID-19 infection, particularly regarding neurodegenerative diseases. The concept of interaction between environmental factors and alpha-synuclein formation in the olfactory bulb and vagal autonomic terminals with subsequent caudo-cranial migration has received much attention in the context of PD pathogenesis. Anosmia and gastrointestinal symptoms are two well-known symptoms of COVID-19, with evidence of an olfactory bulb and vagal infiltration by SARS-CoV2. This raises the possibility of the spread of the viral particles to the brain along multiple cranial nerve routes. Neurotropism, coupled with the ability of the SARS-Cov2 virion to induce abnormal protein folding and stress responses in the central nervous system, in presence of an inflammatory milieu, reinforced by hypoxia, coagulopathy, and endothelial dysfunction, reverberates the intriguing possibility of activation of a neurodegenerative cascade leading to the development of pathological alpha-synuclein aggregates and thus, triggering the development of PD in survivors of COVID19. This review attempts to summarize and critically appraise existing evidence from basic science research and clinical reports of links between COVID-19 and PD and explores the prospect of a multi-hit pathophysiological process, induced by SARS-Cov2 infection, ultimately converging on perturbed cellular protein homeostasis, which although is intriguing, presently lacks robust evidence for confirmation.

Bickerstaff encephalitis: An uncommon sequalae of a bacterial infection.

Bickerstaff brainstem encephalitis (BBE) is a rare neurological disorder characterised by the presence of the triad of ophthalmoplegia, ataxia and altered consciousness. It is thought to be associated to an autoimmune condition triggered by an antecedent infection. Scrub typhus is a zoonotic disease caused by Orientia tsutsugamushi, and it is associated with myriads of neurological complications. We hereby present an unusual case of BBE in a young lady, who was probably a sequalae of scrub typhus infection. Bickerstaff brainstem encephalitis in association with scrub typhus has not been reported till date.

Parakinesia Brachialis Oscitans - a Rare Post-Stroke Phenomenon.

Background: Abnormal involuntary movement of paralyzed upper limb during yawning is a rare phenomenon termed as parakinesia brachialis oscitans. Case Report: We describe a 59-year-old gentleman with abnormal involuntary movement of paralyzed right upper limb during yawning 2 weeks following ischemic stroke of left middle cerebral artery territory. Discussion: This is a rare post-stroke phenomenon and its pathophysiological mechanism is poorly understood but this entity highlights possible preserved extrapyramidal pathway which might help in rehabilitating stroke survivors.

A video-based discussion of movement disorders in paediatric anti NMDAR encephalitis: A case series from Eastern India.

PURPOSE: The spectrum of movement disorders associated with anti N-Methyl-d-Aspartate-Receptor (NMDAR) encephalitis is myriad, particularly in children, possibilities of which were investigated from two tertiary care centres. METHODS: A retrospective study was conducted in two tertiary referral centres in Eastern India, analysing data of 8 paediatric patients diagnosed as anti NMDAR encephalitis, presenting with one or more movement disorders (MDs). RESULTS: All the patients were of Bengali ethnicity with a median age of 9 years (3-16 years) and with female predilection (62.5%). CSF pleocytosis was a common feature in all. Seizures were described in 62.5%% of patients with a solitary patient exhibiting abnormalities on brain imaging. 3 out of 8 (37.5%) of patients presented with a single MD while the remaining had more than one type. Oro-linguo-facial dyskinesias and dystonia (37.5% each) were the most common movement type followed by chorea (12.5%). Complex stereotypies, myoclonus and facial tics were noted in one patient each. All patients received pulse methyl prednisolone. Escalation to second line therapy in form of rituximab was done for 5 patients (62.5%). Following immunotherapy, hyperkinetic movements resolved in 50% of patients, with persistence of movements in one (12.5%). A mortality of 37.5% was noted. Median duration of follow up was 26 months, during which none of the patients had evidence of systemic neoplasm. CONCLUSION: MDs are a core feature of anti NMDAR encephalitis, particularly in the paediatric age group, understanding and characterization of which, is the key to early diagnosis and effective therapy.

Correlation of ATP7B gene mutations with clinical phenotype and radiological features in Indian Wilson disease patients.

INTRODUCTION: Wilson disease (WD) is characterized by a wide variety of clinical manifestations. Our study aimed to correlate genotype with clinical and radiological features in Indian WD patients. METHODS: We conducted a descriptive observational study in a tertiary care neurology referral center of eastern India over a period of 2 years. Demographic data collection, clinical examination and relevant investigations were done for all WD patients meeting the inclusion criteria. Based on previous reports of mutation hotspots for WD in Eastern India, we performed PCR-Sanger sequencing of selected exons of ATP7B gene. To understand the role of each of these covariates on the occurrence of common mutation, we applied a logistic regression as well as random forest in a supervised learning framework. RESULTS: Fifty-two WD patients were included in the study. c.813C > A (p.C271X) was the commonest identified mutation. The statistical methods applied to our data-set reveal the most important features for predicting common mutation or its absence. We also found that the state-of-the-art classification algorithms are good at predicting the absence of common mutation (with true positive rates being 0.7647 and 0.8823 for logistic classifier and random forest, respectively), but predicting the occurrence remains a harder modeling challenge. CONCLUSIONS: WD patients in eastern India have significant genotypic and phenotypic diversity. Statistical methods for binary classification show some early promise of detecting common mutations and suggest important covariates, but further studies with larger samples and screening of remaining exons are warranted for understanding the full genetic landscape of Wilson disease.

Recurrent ataxia and dysarthria in myelin oligodendrocyte glycoprotein antibody-associated disorder.

The spectrum of central nervous system demyelinating disorders is vast and heterogeneous and, often, with overlapping clinical presentations. Misdiagnosis might occur in some cases with serious therapeutic repercussions. However, introduction of several new biomarkers such as aquaporin-4 IgG and myelin oligodendrocyte glycoprotein IgG has made distinction between diseases such as multiple sclerosis and myelin oligodendrocyte glycoprotein antibody-associated disorder easier. Here, we report a case of a 15-year-old male patient with subacute multifocal neurological presentation without encephalopathy, eventually diagnosed as myelin oligodendrocyte glycoprotein antibody-associated disorder.

2D perovskite-based high spatial resolution X-ray detectors.

X-ray radiography is the most widely used imaging technique with applications encompassing medical and industrial imaging, homeland security, and materials research. Although a significant amount of research and development has gone into improving the spatial resolution of the current state-of-the-art indirect X-ray detectors, it is still limited by the detector thickness and microcolumnar structure quality. This paper demonstrates high spatial resolution X-ray imaging with solution-processable two-dimensional hybrid perovskite single-crystal scintillators grown inside microcapillary channels as small as 20 µm. These highly scalable non-hygroscopic detectors demonstrate excellent spatial resolution similar to the direct X-ray detectors. X-ray imaging results of a camera constructed using this scintillator show Modulation Transfer Function values significantly better than the current state-of-the-art X-ray detectors. These structured detectors open up a new era of low-cost large-area ultrahigh spatial resolution high frame rate X-ray imaging with numerous applications.

Not the Stereotypical Wilson Disease: A Case Report.

Background: Wilson disease (WD), a potentially treatable genetic disorder with perturbations in copper metabolism, presents with hepatic and neuropsychiatric manifestations. Both hyper and hypokinetic movements predominate the latter spectrum. Motor stereotypies, however, are exceedingly rare. Case Report: We present a case of a 12-year-old girl, with progressive behavioural alterations and cognitive impairment, with motor stereotypies involving the upper limbs, as the dominant movement semiology. She was diagnosed as WD with evidence of striatal involvement on brain imaging. Her motor symptoms partially responded to chelation therapy. Discussion: There are about five documented cases of motor stereotypies in WD worldwide, with only one being previously reported from India.

Spectrum of Anti-NMDA Receptor Antibody Encephalitis: Clinical Profile, Management and Outcomes.

BACKGROUND: Anti-N-methyl D-aspartate receptor (anti NMDAR) antibody encephalitis is an immune-mediated entity characterised by a constellation of neuro-psychiatric symptoms. OBJECTIVE: To describe clinical profile and treatment outcomes of patients with anti NMDAR antibody encephalitis. SETTINGS AND DESIGN: Subjects were selected by screening for all patients satisfying Graus et al.'s criteria for probable anti NMDAR antibody encephalitis, admitted in neurology department of a tertiary care centre in Eastern India. MATERIALS AND METHODS: A prospective, longitudinal study was conducted by identifying 25 patients with anti NMDAR antibodies in CSF and or serum, between September 2018 to February 2020. STATISTICAL ANALYSIS: Chi square test was used to compare variables. RESULTS: Out of 98 patients screened, 25 subjects (14 females: 11 male) were positive for anti NMDAR autoantibodies, with a mean age of 17 years. 13 subjects belonged to paediatric age group. Most common presenting feature was memory/learning deficit (88%) followed by behavioural abnormalities (84%) and seizures (68%). 11 patients (44%) patients needed escalation to second line therapy, rituximab. Seven (28%) and twelve (48%) patients underwent complete (mRS 0-1) and partial recovery (mRS 2-3) respectively, while 4 (16%) became disabled (mRS 4-5). Mortality was 8%. Paediatric population had a better outcome in terms of disability (p = 0.043). CONCLUSION: Anti NMDAR-Ab encephalitis is the most common cause of antibody positive autoimmune encephalitis worldwide. There are important clinical markers and investigational profiles which carry prognostic significance.