Movement disorder rounds: A 32-year old lady who developed a complex generalized movement disorder in all four limbs after plasma exchange for myelitis.

A 32-year old lady with AQP4-Antibody positive neuromyelitis optica developed a new-onset complex movement disorder after therapeutic plasma exchange, which was initially suspected to be hypocalcemic carpo-pedal spasm. However, when her bilateral, distal predominant, paroxysmal, stereotypic, wrist and finger flexor tonic contractions did not respond to serum calcium correction, other diagnoses were considered. The patient had a dramatic response to oral carbamazepine suggesting that the tonic spasms were likely a spinal movement disorder due to the primary demyelinating pathology.

Therapeutic plasma exchange in autoimmune neurological disorders: A comprehensive evaluation of clinical outcomes, safety, and long-term disability using the modified Rankin scale.

INTRODUCTION: Autoimmune neurological diseases (ANDs) involve the immune system attacking the nervous system, leading to various symptoms. Therapeutic plasma exchange (TPE) is used to remove pathogenic autoantibodies, aiming to improve clinical outcomes. METHODS: This ambispective observational study included 99 patients with ANDs who underwent TPE from January 2018 to June 2022 at a tertiary care center in India. Clinical outcomes were measured using the modified Rankin Scale (mRS) scores at admission, post-TPE, at 3-months, 6-months, and 1-year follow-up post-discharge. Data were analyzed using Epi Info version 7.0. RESULTS: The median mRS score improved significantly from 5 (IQR 4-5) before TPE to 3 (IQR 2-4) post-TPE (p < 0.001). Complications occurred in 5.95% of procedures, with allergic reactions being the most common. The in-hospital mortality rate was 9%. CONCLUSION: TPE is a safe and effective treatment modality for autoimmune neurological diseases, especially in resource-constrained settings. It aids in both symptomatic relief and reducing long-term functional disability.

Sensory ataxic polyneuropathy unmasking late-onset urea cycle defect.

A 63-year-old man with type 2 diabetes mellitus, alcohol consumption in moderation, and three episodes of hepatic encephalopathy presented with symmetrical lower limb distal weakness, sensory ataxia, thickened palpable nerves, mood disturbances for seven years, and a family history of schizophreniform disorders. Nerve conduction studies showed demyelinating sensorimotor polyradiculoneuropathy. CSF analysis showed mild albumino-cytological dissociation. MRI brain and lumbosacral plexus showed thickened fifth cranial nerves and lumbosacral roots. He was treated with steroids for a provisional diagnosis of chronic inflammatory polyneuropathy and became encephalopathic. EEG showed triphasic waves. Serum ammonia was 201 micrograms/dL. Further evaluation suggested ornithine transcarbamylase (OTC) deficiency. The patient underwent hemodialysis with a low protein diet, rifaximin, and sodium benzoate, with subsequent recovery.

Evaluation of Utility of Invasive Electroencephalography for Definitive Surgery in Patients with Drug-Resistant Epilepsy: A Systematic Review and Meta-Analysis.

When noninvasive tests are unable to define the epileptogenic zone in patients, intracranial electroencephalography (iEEG) is a method of localizing the epileptogenic zone. Compared with noninvasive evaluations, it offers more precise information about patterns of epileptiform activity, which results in useful diagnostic information that supports surgical decision-making. The primary aim of the present study was to assess the utility of iEEG for definitive surgery for patients with drug-resistant epilepsy. Online databases such as PubMed, Medline, Embase, Scopus, Cochrane Library, Web of Science, and IEEE Xplore were searched for MeSH terms and free-text keywords. The ROBINS I (risk of bias in non-randomized studies - of interventions) critical appraisal tool was used for quality assessment. The prevalence from different studies was pooled together using the inverse variance heterogeneity method. Egger's regression analysis and funnel plot were used to evaluate publication bias. The systematic review included 18 studies, and the meta-analysis included 10 studies to estimate the prevalence of seizure freedom (Engel class I) in patients undergoing surgery after iEEG. A total of 526 patients were included in the meta-analysis. The follow-up period ranged from 1 to 10 years. The overall pooled estimate of the prevalence of seizure freedom (Engel class I) for patients undergoing surgery after iEEG was 53% (95% confidence interval, 44%-62%). The results additionally demonstrated that 12 studies had a moderate risk of bias and 6 had a low risk. Future studies are crucial to enhance our understanding of iEEG to guide patient choices and unravel their implications.

Multimodality imaging in cerebral venous thrombosis: a synopsis for emergency radiologist.

Cerebral venous thrombosis (CVT) is an uncommon but potentially fatal condition which presents with a wide range of symptoms. Some of these presenting features are vague thus contributing to the delay in diagnosis. A prompt diagnosis and initiation of appropriate therapy are therefore of paramount importance. In this pictorial, we have tried to illustrate the direct and indirect imaging features of CVT in detail on multiple imaging modalities, along with the potential pitfalls of imaging.

IMPETUS Stroke: Assessment of hospital infrastructure and workflow for implementation of uniform stroke care pathway in India.

BACKGROUND: India accounts for 13.3% of global disability-adjusted life years (DALYs) lost due to stroke with a relatively younger age of onset compared to the Western population. In India's public healthcare system, many stroke patients seek care at tertiary-level government-funded medical colleges where an optimal level of stroke care is expected. However, there are no studies from India that have assessed the quality of stroke care, including infrastructure, imaging facilities, or the availability of stroke care units in medical colleges. AIM: This study aimed to understand the existing protocols and management of acute stroke care across 22 medical colleges in India, as part of the baseline assessment of the ongoing IMPETUS stroke study. METHODS: A semi-structured quantitative pre-tested questionnaire, developed based on review of literature and expert discussion, was mailed to 22 participating sites of the IMPETUS stroke study. The questionnaire assessed comprehensively all components of stroke care, including human resources, emergency system, in-hospital care, and secondary prevention. A descriptive analysis of their status was undertaken. RESULTS: In the emergency services, limited stroke helpline numbers, 3/22 (14%); prenotification system, 5/22 (23%); and stroke-trained physicians were available, 6/22 (27%). One-third of hospitals did not have on-call neurologists. Although non-contrast computed tomography (NCCT) was always available, 39% of hospitals were not doing computed tomography (CT) angiography and 13/22 (59%) were not doing magnetic resonance imaging (MRI) after routine working hours. Intravenous thrombolysis was being done in 20/22 (91%) hospitals, but 36% of hospitals did not provide it free of cost. Endovascular therapy was available only in 6/22 (27%) hospitals. The study highlighted the scarcity of multidisciplinary stroke teams, 8/22 (36%), and stroke units, 7/22 (32%). Lifesaving surgeries like hematoma evacuation, 11/22 (50%), and decompressive craniectomy, 9/22 (41%), were performed in limited numbers. The availability of occupational therapists, speech therapists, and cognitive rehabilitation was minimal. CONCLUSION: This study highlighted the current status of acute stroke management in publicly funded tertiary care hospitals. Lack of prenotification, limited number of stroke-trained physicians and neurosurgeons, relatively lesser provision of free thrombolytic agents, limited stroke units, and lack of rehabilitation services are areas needing urgent attention by policymakers and creation of sustainable education models for uniform stroke care by medical professionals across the country.

Sociodemographic determinants of epilepsy and treatment-seeking behavior among patients with epilepsy - A hospital-based study from western India.

BACKGROUND: Epilepsy ranks among the major noncommunicable diseases that confer significant morbidity and mortality. A lack of knowledge about epilepsy, coupled with negative attitude and false practices, has been shown to be influenced by sociodemographic parameters and in turn impacts healthcare-seeking behavior. METHODS: An observational single-center study was conducted at a tertiary care center in western India. Data on sociodemographic parameters, clinical and healthcare-seeking patterns were recorded from all patients above 18 years with a diagnosis of epilepsy. A pre-validated questionnaire was then administered to assess the knowledge, attitude, and practices related to epilepsy. Data collected were evaluated. RESULTS: A total of 320 patients with epilepsy were recruited for the study. Majority of the study subjects were young Hindu males from urban and semi-urban areas. Idiopathic generalized epilepsy was the most common diagnosis with significant number of patients showing poor seizure control. The knowledge, attitude, and practice (KAP) responses showed serious gaps in various aspects. Prevalent misconceptions were that epilepsy is a mental problem (40%), is a hereditary disorder (24.1%), is contagious (13.4%), and is a result of previous life sin (38.8%). With respect to discrimination in epilepsy using KAP questionnaire, most of the respondents (>80%) did not object to sitting or playing with a child with epilepsy. A significant proportion of patients (78.8%) feared from the side effects of long-term antiepileptic drug treatment. Almost one-third persons (31.6%) did not know the correct response in terms of first aid measures. The mean KAP score was 14.33 (±3.017 SD), significantly higher in better educated people from urban areas (p < 0.001 for both). Healthcare-seeking behavior with preference for early allopathic care had a positive correlation with various sociodemographic characteristics as well as with higher mean KAP scores. CONCLUSION: Despite improved literacy and urbanization, knowledge regarding epilepsy still remains poor with wide prevalence of traditional wisdom and practices. Though better education, employment, and awareness may partially overcome the impediments resulting in delay in seeking early appropriate healthcare after first seizure, the issue is more complex and multifactorial, and a multipronged approach is need of the hour.

Clinical Profile, Follow-up, and Role of Neuroimaging in Pediatric Guillain-Barré Syndrome in the COVID Era: An Ambispective Study.

Background: To define the varied presentations of Guillain-Barré syndrome in children in the COVID era and 6 months' follow-up outcome. Methods: Ambispective study of 15 months' duration involving children with Guillain-Barré syndrome aged 1 month to 18 years at a tertiary care pediatric hospital. They were categorized into groups A and B based on COVID-19 serology testing. Hughes Disability Scale was used for disability assessment. Modified Rankin scale was used for improvement assessment in follow-up. Results: Of 19 children with Guillain-Barré syndrome, 9 (47%) were females and 10 (53%) were males. Groups A and B had children with negative (8) and positive serology (11), respectively. The most common presentation in both groups was motor weakness. Post-COVID pediatric Guillain-Barré syndrome presented with variants of Guillain-Barré syndrome rather than the classical form (P = .03). In group B, patients with elevated inflammatory markers had poor response to intravenous immunoglobulin, and 5 of 11 patients had good response to pulse steroids, probably depicting an inflammation-predominant pathology. Conclusion: Post-COVID Guillain-Barré syndrome in children presented with Guillain-Barré syndrome variants rather than the classic form. Neuroimaging is of great value in both confirming Guillain-Barré syndrome diagnosis and excluding differentials. Patients with elevated inflammatory markers and residual weakness may be given a pulse steroid trial.

Neuroimaging Spectrum in COVID-19 Infection: A Single-Center Experience.

Background and Purpose The ongoing coronavirus disease 2019 (COVID-19) pandemic is a multisystemic disease and involvement of the nervous system is well established. The neurological and neuroimaging features of the disease have been extensively evaluated. Our study aimed to elucidate the neuroradiological findings in COVID-19 infected patients admitted to our institute during the first and second waves of the pandemic in India. Methods This was a single-center retrospective study of all COVID-19 positive patients who underwent neuroimaging between March 2020 and May 2021. The presenting neurological complaints, the imaging findings in computed tomography (CT) imaging, and/or magnetic resonance imaging (MRI) were recorded. They recorded the findings in the subheadings of ischemic stroke, hemorrhagic stroke, parainfectious demyelination, acute encephalitis syndrome, and changes of global hypoxic changes. Patients with age-related, chronic, and incidental findings were excluded. Results The study comprised of 180 COVID-19 positive patients who underwent neuroimaging. CT scan was performed for 169 patients, MRI for 28, and a combination of both CT and MRI was performed for 17 patients. Seventy percent of patients were males, and median age was 61.5 years (interquartile range: 48.25-70.75). Out of the 180 patients, 66 patients had nonspecific findings that could not be attributed to COVID-19 infection. In the remaining 114 patients, 77 (42.7%) had ischemic findings, while 22 (12.2%) had hemorrhagic stroke. Hypoxic ischemic changes were noted in five patients. The rest of the patients had a spectrum of changes including, cerebellitis (3), tumefactive demyelination (1), COVID-19-associated encephalitis (1), hemorrhagic acute demyelinating encephalomyelitis (1), transverse myelitis (1), cytotoxic lesions of corpus callosum (1), Guillain-Barre syndrome (1), and COVID-19-associated microhemorrhages (1). Conclusion Neurological manifestations of COVID-19 infection are not uncommon, and our understanding of this topic is expanding. A complex interplay of neurotropism and direct central nervous system invasion, immune activation and cytokine storm, vasculitis, and parainfectious processes are implicated in the pathophysiology. While the most common imaging finding was ischemic stroke, followed by hemorrhagic stroke, a diverse range of parainfectious findings was also noted in our study.

Utility of optical coherence tomography in patients of central immune mediated demyelinating diseases - A prospective study.

Optical coherence tomography (OCT) is a non-invasive tool to measure thickness of various layers of retina. Recently, retinal nerve fibre layer (RNFL) and ganglion cell and inner plexiform layer (GCIP) thinning has been observed in OCT in patients with multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD), This study compared OCT profile, along with visual acuity (VA), color vision (CV), contrast saturation (CS) and visual evoked potentials (VEP) in two main cohorts of MS and NMOSD and with controls, during acute episode of optic neuritis (ON), at 3 and 6 months. We found that changes of ON were present in 75% of MS eyes and in 45% of NMOSD patients. Of these, subclinical involvement was present in 56.25% of MS eyes and only in 5% of NMOSD eyes suggesting frequent subclinical involvement in the former. Mean RNFL was 95.23 ± 15.53 in MS and 66.14 ± 43.73 in NMOSD after 6 months of ON episode. Thinning of NQ and IQ was observed in NMOSD eyes in the immediate period after ON attack. At 6 months relative sparing of RNFL in TQ was observed in NMOSD ON eyes and MS ON showed predilection for involvement of TQ.

Determination of sensitivities and specificities of cerebrospinal fluid free light chains to diagnose multiple sclerosis- a multicentric case-control study.

BACKGROUND: CSF free light chains help diagnose multiple sclerosis, but no data is available on the Asian population. Our objective was to study the diagnostic utility of CSF free light chains for diagnosing multiple sclerosis in Indian patients. METHODS: Prospective multicentric case-control study. Cases included those who were tested for oligoclonal bands and fulfilled the modified McDonald criteria 2017 for multiple sclerosis and clinically isolated syndromes. Those tested for oligoclonal bands (OCB) but with other diagnoses- inflammatory and non-inflammatory were included as controls. Clinical details were collected from electronic medical records. CSF and serum kappa and lambda free light chains were measured, apart from oligoclonal bands, immunoglobulin, and albumin in paired serum and CSF samples. RESULTS: There were 70 patients (31 cases and 39 controls). The mean age was 43.41(SD 16.073) years, and 43(61.4%) were females. CSF kappa showed highest specificity 97.4%, at a cut off 2.06 mg/L (sensitivity 71%) and highest sensitivity 90.3%, at a cut off 0.47 mg/L (specificity 79.5%). Best balance of sensitivity and specificity for CSF kappa was seen at a cut-off of ≥ 0.63 mg/L {sensitivity 87·1 (CI - 70.17-96.37), and specificity 87·18 (CI -72.57-95.70)}. The ratio of Kappa/lambda showed highest specificity of 100%(similar to OCB) with a sensitivity of 71% at a cut off of 1.72. The ratio of sum of kappa and lambda light chains, and Qalb (∑CSF FLC/Qalb), showed the highest specificity (94.87%)among the blood brain barrier corrected ratios. CONCLUSION: This study showed that the diagnostic utility of CSF kappa was comparable to OCB to diagnose multiple sclerosis in sensitivity, but not specificity, so can be a screening test before testing for OCB in our population.

Cardiac Abnormalities in Refractory Status Epilepticus-an Exploratory Study.

Background and Purpose: Cardiac abnormalities have been reported during ongoing seizures and refractory status epilepticus (RSE). Reduced heart rate variability (HRV) and cardiac arrhythmias may contribute to sudden unexpected death in epilepsy. We sought to explore the utility of electrocardiographic and echocardiographic changes in patients with RSE prognosis and functional outcome. Methods: Patients of RSE underwent electrocardiogram (ECG), holter, troponin-I (Trop I), N-terminal prohormone of brain natriuretic peptide (NT-proBNP), and 2-dimensional echocardiogram (2D Echo) along with continuous electroencephalogram in first 24 hours and admission. Heart rate changes/arrhythmias, corrected QT interval (QTc) and HRV, ventricular dysfunction or regional motion wall abnormality were studied on 2D Echo. These parameters were also at baseline, at discharge or death and 30 days post discharge. Results: This prospective observational study conducted over 18 months enrolled 20 patients with RSE, fulfilling the inclusion criteria. Mean age was 47.75±17.2 years with male: female ratio of 1:1. Mean time to presentation from seizure onset was 8.80±7.024 hours. Central nervous system infection (35.0%), autoimmune encephalitis (20.0%) and cerebrovascular disease (20.0%) were the common etiologies. Amongst cardiac injury markers, cardiac enzymes and QTc prolongation were the commonest abnormalities in RSE. Both reduced HRV and presence of cardiac injury markers had significant correlation with poor outcome along with poor Glasgow coma scale (GCS) and modified Rankin scale (mRS) at presentation, and presence of non convulsive status epilepticus (NCSE). Conclusions: Presence of poor GCS, poor mRS, markers of cardiac injury, reduced HRV and occurrence of NCSE have a consistent correlation with mortality and poor clinical outcome. Therefore, routine assessment of cardiac abnormalities using affordable, easily accessible and non-invasive tools such as ECG, 2D Echo, holter NT-proBNP and Trop I is recommended in RSE patients.

Resting-state Quantitative EEG Spectral Patterns in Migraine During Ictal Phase Reveal Deviant Brain Oscillations: Potential Role of Density Spectral Array.

Background. Migraine headache may have a substantial bearing on the brain functions and rhythms. Electrophysiological methods can detect changes in brain oscillation. The present work examined the frequency band power through quantitative electroencephalogram (qEEG) and density spectral array (DSA) to elucidate the resting state neuronal oscillations in migraine. Methods. Clinical details were inquired, and EEG was recorded in migraineurs and healthy controls. The acquired data were analyzed to determine power spectral density values and obtain DSA graphs. The absolute and relative powers for the alpha, theta, and delta frequencies in frontocentral, parieto-occipital, and temporal regions were determined. A correlation of significant EEG findings with clinical features of migraine was sought. Results. Forty-five participants were enrolled in the study. The spectrum analysis revealed an increase in the relative theta power (P < .001) and a reduction in relative alpha power (P < .001) in the observed cortical areas among the migraineurs as compared to the healthy controls. Relative delta power was increased over the frontocentral region (P = .001), slightly more on the symptomatic side of the head. In addition, frontocentral delta power had a moderate positive correlation (r = .697, n = 22, P = .000) with migraine severity. Conclusion. The study supports the evidence of a neuronal dysfunction existing in the resting state during the ictal phase of migraine. qEEG can reveal these aberrant oscillations. Utility of DSA to depict the changes in brain activity in migraine is a potential area for research. The information can help formulate new therapeutic strategies towards alteration in cortical excitability using brain stimulation techniques.

Study Protocol: IMPETUS: Implementing a Uniform Stroke Care Pathway in Medical Colleges of India: IMPETUS Stroke.

Introduction: In India, a national program for stroke (national programme for the control of cardiovascular diseases, diabetes, cancer, and stroke) and stroke management guidelines exist. Its successful implementation would need an organized system of stroke care in practice. However, many challenges exist including lack of awareness, prehospital notification systems, stroke ready hospitals, infrastructural weaknesses, and rehabilitation. We present here a protocol to investigate the feasibility and fidelity of implementing a uniform stroke care pathway in medical colleges of India. Methods and Analysis: This is a multicentric, prospective, multiphase, mixed-method, quasi-experimental implementation study intended to examine the changes in a select set of stroke care-related indicators over time within the sites exposed to the same implementation strategy. We shall conduct process evaluation of the implementation process as well as evaluate the effect of the implementation strategy using the interrupted time series design. During implementation phase, education and training about standard stroke care pathway will be provided to all stakeholders of implementing sites. Patient-level outcomes in the form of modified Rankin Scale score will be collected for all consecutive patients throughout the study. Process evaluation outcomes will be collected and reported in the form of various stroke care indicators. We will report level and trend changes in various indicators during the three study phases. Discussion: Acute stroke requires timely detection, management, and secondary prevention. Implementation of the uniform stroke care pathway is a unique opportunity to promote the requirements of homogenous stroke care in medical colleges of India.

The Yin and Yang of Operating on a Posterior Fossa Meningioma: The Schmahmann Syndrome.

The cerebellum is classically linked with control of motor function, such as coordination, balance, and regulation of movement. There is an increasing awareness, now, of the non-motor functions of the cerebellum, and the occurrence of behavioral anomalies with cerebellar disorders. We present the first report of Schmahmann syndrome (cerebellar cognitive affective syndrome [CCAS]) occurring secondary to posterior fossa meningioma excision. A 35-year-old lady with a posterior fossa meningioma developed an infarct of the right posterosuperior cerebellar hemisphere and ipsilateral superior vermis, following suboccipital craniotomy and tumor resection. Few days after the surgery, she presented with aggressive and emotional behavior, irrelevant talk, and emotional lability. The CCAS scale was administered, and she scored poorly on almost all parameters. A neuropsychological evaluation was also done. The occurrence of CCAS, posterior fossa syndrome (PFS), and behavioral abnormalities like abnormal pathological laughter/crying provides further clinical evidence of the "affective" functions of the cerebellum, modulated mainly by the posterior lobe and vermis of the cerebellum.

Multiphase computed tomography angiography (mCTA) derived source images in acute ischemic stroke: Beyond collaterals. Can it obviate the need for computed tomography perfusion (CTP)?

BACKGROUND AND PURPOSE: To compare Multiphase CT Angiography derived source images (mCTA-SI) in acute ischemic stroke (AIS) with CT Perfusion (CTP) derived automated color maps of cerebral blood flow (CBF) and cerebral blood volume (CBV) and to assess the comparability of mCTA-SI with CTP in the prediction of final radiological and clinical outcome. METHODS: This prospective single-centre observational study comprised of patients with AIS of the anterior circulation, presenting within 24 h and undergoing neuroimaging under stroke protocol with follow-up. Non-contrast computed tomography (NCCT), mCTA, and CTP were acquired with follow-up NCCT at 24 h and modified Rankin score (mRS) at 3 months. mCTA-SI and CTP color maps were scored by the ASPECTS (Alberta Stroke program early CT score) method and compared amongst each other and with the outcome. ROC (Receiver operating characteristic) curves were plotted considering mRS 0-2 and FIV≤ 28 ml as favourable clinical and radiological outcomes respectively. RESULTS: The study included 55 patients. The 1st and 2nd phase of mCTA-SI correlated significantly with CBF maps (r = 0.845, p < 0.01, r = 0.842, p < 0.01 respectively). 3rd phase of mCTA-SI correlated significantly with CBV maps (r = 0.904, p < 0.01). A favourable functional and radiological outcome was best predicted by the 1st (AUC 0.8, 95%CI 0.671-0.896) and 2nd ( AUC 0.895, 95% CI 0.783-0.962) phase of mCTA-SI respectively. CONCLUSIONS: The 1st and 2nd phases of mCTA-SI produces results congruent to CBF color maps and the 3rd phase of mCTA-SI simulate CBV color maps. In addition to predicting radiological and functional outcomes, mCTA can predict the salvageable and non-salvageable tissue in AIS and is non-inferior to CTP.