Catastrophic health expenditure in patients with Guillain Barre syndrome: Retrospective study.

INTRODUCTION: Catastrophic health expenditure (CHE) is a reliable measure of the financial unpreparedness of the studied population to meet unexpected health issues. The alarming proportion of patients who incur CHE in the wake of an acute neurological illness like Guillain Barre Syndrome (GBS) is of serious concern in a country like India where a large majority of households are uninsured. METHODOLOGY: Medical records of patients diagnosed with at a tertiary care centre in Delhi were analysed retrospectively to determine the rate of CHE. Clinical details and other contributory variables were also recorded. RESULTS: 53 patients with a median age of 29 years (10.5-46.5) were included in the study. Tow- third of patients were less than 40 years of age and 58.5% were male. 90.6% of patients incurred CHE with a median amount INR 273 300 spent out of pocket. CONCLUSION: The enormous magnitude of financial distress and crisis emerging out of an acute neurological illness needs to be addressed with urgency to prevent impoverishment of already weakened households.

Knowledge, Attitude, and Practice of epilepsy in Wayanad, Kerala.

OBJECTIVE: Epilepsy is a common neurological condition characterized by recurrent seizures. The aim of the study was to assess the level of Knowledge, Attitude, and Practices (KAP) in epilepsy in the district of Wayanad. METHODS: This study was conducted among adults residing in Wayanad. This was a community-based door-to-door survey conducted among adults residing in Wayanad District between August 2021 and November 2021. A semi-structured questionnaire was used for data collection. The questionnaire included 23 items that could define the information on the level of knowledge and attitude toward epilepsy. RESULT: A total of 394 subjects without epilepsy were included in this study. According to the results of this study, 68.8% of respondents were female and 85.3% of them had completed at least primary school education. 93.1% of participants had heard about epilepsy and 64.5% of participants were aware that epilepsy was a brain disorder. 30.5% of participants believed that a person with epilepsy could not get married. 73.9% of participants recommended allopathic treatment for epilepsy and only 9.9% of participants knew about surgical treatment option for epilepsy. CONCLUSION: Despite having a good understanding of the disease, there is a general tendency in the population to consider patients with epilepsy as incapable of leading a normal life. The choice of modern treatment methods over traditional is also a positive aspect.

INSTRuCT: Protocol, Infrastructure, and Governance.

Background and Purpose: Very few large scale multicentric stroke clinical trials have been done in India. The Indian Council of Medical Research funded INSTRuCT (Indian Stroke Clinical Trial Network) as a task force project with the objectives to establish a state-of-the-art stroke clinical trial network and to conduct pharmacological and nonpharmacological stroke clinical trials relevant to the nation and globally. The purpose of the article is to enumerate the structure of multicentric stroke network, with emphasis on its scope, challenges and expectations in India. Methods: Multiple expert group meetings were conducted by Indian Council of Medical Research to understand the scope of network to perform stroke clinical trials in the country. Established stroke centers with annual volume of 200 patients with stroke with prior experience of conducting clinical trials were included. Central coordinating center, standard operating procedures, data and safety monitoring board were formed. Discussion: In first phase, 2 trials were initiated namely, SPRINT (Secondary Prevention by Structured Semi-Interactive Stroke Prevention Package in India) and Ayurveda treatment in the rehabilitation of patients with ischemic stroke in India (RESTORE [Rehabilitation of Ischemic stroke Patients in India: A Randomized controlled trial]). In second phase, 4 trials have been approved. SPRINT trial was the first to be initiated. SPRINT trial randomized first patient on April 28, 2018; recruited 3048 patients with an average of 128.5 per month so far. The first follow-up was completed on May 27, 2019. RESTORE trial randomized first patient on May 22, 2019; recruited 49 patients with an average of 3.7 per month so far. The first follow-up was completed on August 30, 2019. Conclusions: In next 5 years, INSTRuCT will be able to complete high-quality large scale stroke trials which are relevant globally. REGISTRATION: URL: http://www.ctri.nic.in/; Unique Identifier: CTRI/2017/05/008507.

Anti-Sjögren's-syndrome-related antigen A autoantibodies (Anti-SSA antibody) and meningoencephalitis: Sjögren's syndrome waiting to be unveiled? A case series and review of literature.

Aseptic meningoencephalitis (AME) constitutes a variable proportion of meningoencephalitis. Patients with AME are not routinely evaluated for autoimmune disorders. Primary Sjögren's syndrome (pSS) is a prevalent, but under suspected systemic autoimmune disease characterised by exocrinopathy, though sicca symptoms may not be the dominant or presenting feature. This study was undertaken to enumerate the clinical, radiological and laboratory features of meningoencephalitis related to pSS among the total cohort of meningoencephalitis admitted in our hospital. Retrospective patient records were screened for diagnosis of meningoencephalitis from April 2016 to March 2020. Those patients with anti-SSA positivity and clinical diagnosis of pSS were included. We have reviewed all cases of Sjögren's syndrome with meningoencephalitis available in literature. Four patients with meningoencephalitis with pSS were identified. Their clinical presentations, investigations, and good response to steroids have been described with special emphasis on evolving clinical features. In all patients, sicca features were absent. Anti-SSA was positive in all. The diagnosis of pSS was considered after ruling out all infectious and other autoimmune aetiologies. Two had extra-neurological organ manifestations and required addition of second line immunosuppressive agents for optimum disease control. Consistent with this case series, absent sicca symptoms have been described in pSS patients presenting with meningoencephalitis in literature. This case series is of special interest as it describes the initial presentation of pSS as meningoencephalitis with sicca features in absentia, thereby highlighting the need for a high index of suspicion and the need for workup for pSS in AME.

Epidemiology of Motoric Cognitive Risk Syndrome in the Kerala Einstein Study: Protocol for a Prospective Cohort Study.

BACKGROUND: The southern India state of Kerala has among the highest proportion of older adults in its population in the country. An increase in chronic age-related diseases such as dementia is expected in the older Kerala population. Identifying older individuals early in the course of cognitive decline offers the best hope of introducing preventive measures early and planning management. However, the epidemiology and pathogenesis of predementia syndromes at the early stages of cognitive decline in older adults are not well established in India. OBJECTIVE: The Kerala Einstein Study (KES) is a community-based cohort study that was established in 2008 and is based in the Kozhikode district in Kerala state. KES aims to establish risk factors and brain substrates of motoric cognitive risk syndrome (MCR), a predementia syndrome characterized by the presence of slow gait and subjective cognitive concerns in individuals without dementia or disability. This protocol describes the study design and procedures for this KES project. METHODS: KES is proposing to enroll a sample of 1000 adults ≥60 years old from urban and rural areas in the Kozhikode district of Kerala state: 200 recruited in the previous phase of KES and 800 new participants to be recruited in this project. MCR is the cognitive phenotype of primary interest. The associations between previously established risk factors for dementia as well as novel risk factors (apathy and traumatic brain injury) and MCR will be examined in KES. Risk factor profiles for MCR will be compared between urban and rural residents as well as with individuals who meet the criteria for mild cognitive impairment (MCI). Cognitive and physical function, medical history and medications, sociodemographic characteristics, lifestyle patterns, and activities of daily living will be evaluated. Participants will also undergo magnetic resonance imaging and electrocardiogram investigations. Longitudinal follow-up is planned in a subset of participants as a prelude to future longitudinal studies. RESULTS: KES (2R01AG039330-07) was funded by the US National Institutes of Health in September 2019 and received approval from the Indian Medical Council of Research to start the study in June 2021. We had recruited 433 new participants from urban and rural sites in Kozhikode as of May 2023: 41.1% (178/433) women, 67.7% (293/433) rural residents, and 13.4% (58/433) MCR cases. Enrollment is actively ongoing at all the KES recruitment sites. CONCLUSIONS: KES will provide new insights into risk factors and brain substrates associated with MCR in India and will help guide future development of regionally specific preventive interventions for dementia. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/49933.

Tuberculous Otitis Media in a Patient with Multiple Cerebral Tuberculomas: Case Report.

Tuberculous otitis media is a rare entity which requires high index of suspicion in an endemic country like India. A 38-year-old female with no prior history of tuberculosis or any predisposing factor presented to us with fever and altered sensorium. She was subsequently diagnosed to have disseminated tuberculosis with multiple cerebral tuberculomas. She complained of ear discharge which on further evaluation was established as smear positive tuberculous otitis media. This rare coexistence is seldom reported in literature.

Comparison of Neurological Manifestations in the Two Waves of COVID-19 Infection: A Cross-Sectional Study.

Introduction: Coronavirus Disease-19 (COVID-19) is an ongoing pandemic caused by highly contagious virus severe acute respiratory syndrome coronavirus-2 (SARS-COV-2) that has infected millions of people across the world. Most of the countries have seen two wave patterns of the pandemic. The second wave is potentially more challenging due to high influx of cases, differing properties of the emerging mutants, and other dynamics of the evolving pandemic. Neurological manifestations are common among COVID-19 positive patients. In this context, the present study attempts to compare the neurological manifestation in the first and second waves of COVID-19. Methodology: A single-center retrospective observational study was undertaken to compare neurological manifestations in the first and second waves of COVID-19. A sample of 1500 patients in the second wave admitted with COVID-19 were included in this study and the findings were compared with 1700 patients in the first wave (data derived from a former study in the same center). A detailed questionnaire addressing co-morbidities, admission details, and clinical features was employed to collect data from the hospital records. Results: Out of 1500 COVID-19 patients in the second wave of COVID-19, 355 (23.7%) of them had one or more neurological manifestations during their in-patient stay. The most common neurological symptom in the 2nd wave of COVID-19 was headache reported in 216 (14.4%) of patients followed by fatigue in 130 (8.7%), myalgia in 120 (8.0%), smell and taste disorders (STD) in 90 (6.0%), altered sensorium in 40 (2.7%), dizziness in 24 (1.6%), seizures in 34 (2.3%), encephalopathy in 26 (1.7%), strokes in 13 (0.9%), etc., Compared to the first wave of COVID-19, dizziness (P < 0.001), myalgia (P = 0.001), headache (P < 0.001) and meningoencephalitis (P = 0.01) were more common while cerebrovascular syndromes (P = 0.001) were less common in the second wave. The mortality in the 2nd wave neurological subgroup was higher [66 (18.6%)] than 1st wave neurological subgroup [23 (10%)]. Conclusion: Meningoencephalitis, headache, and seizures were found to be more common in second wave as compared to first wave. The severity and mortality rate were higher in the second wave.

Pharmacoresistant Abdominal Seizures in Symptomatic Localization-Related Epilepsy.

Abdominal epilepsy is an uncommon cause of recurrent abdominal pain with or without other complaints seen in children and adults which often goes unnoticed. Here we are presenting a case of abdominal epilepsy in a 7-year boy who had recurrent abdominal pain since many years. He had a history of ventriculoperitoneal shunting which was performed for obstructive hydrocephalus at 1 month and left hemiparesis. He was evaluated at multiple centers for abdominal complaints before being referred here. The video electroencephalogram done showed inter ictal and ictal abnormalities which correlated with magnetic resonance imaging and positron emission tomography abnormalities on the right side. He was started on antiepileptics with a partial response and continued to get events even with four antiepileptics but the frequency and severity reduced significantly. The present case report is to highlight the drug refractoriness of abdominal seizures in a patient with symptomatic focal epilepsy.

Therapeutic Challenge in a Case of Recent Onset Refractory Cluster Seizures.

A dilemma exists in context to the timing of surgery in a case presenting with explosive onset seizures secondary to a focal cortical dysplasia (FCD). This case report highlights the challenges faced in the management of a 4-year-old child with recent onset cluster seizures refractory to anti-epileptic drugs. A 4-year-old girl presented with an acute onset of cluster seizures (up to 32 in a day), semiologically characterized by tonic upper limb extension and laughter lasting for few seconds with no response to multiple anti-epileptic drugs. The clinical, electrographic, neuroimaging and interictal positron emission tomography data were concordant and consistent with a left middle frontal gyrus dysplasia which was successfully resected under electrocorticographic guidance. Patient is seizure free at 2 months of follow up. (Engel Class 1). Surgical resection is feasible and potentially more effective in the early phase of clinical presentation of FCD.

Variable treatment response in a patient with pyridoxal N phosphate oxidase (PNPO) deficiency- understanding the paradox.

A 6-year-old girl presented with history of infantile onset epileptic encephalopathy and developmental delay. She had polymorphic seizures that were refractory to regular anti-seizure medication. Incomplete control of seizures was achieved on starting pyridoxine, riboflavin and thiamine. Clinical exome sequencing done at 4 years revealed PNPO deficiency with a homozygous mutation in the highly conserved exon 3:c.352G > A p.Gly118R region of the gene. Thereafter, pyridoxine was weaned and pyridoxal phosphate was added with resultant refractory status epilepticus, which necessitated our approach to start pyridoxine and stop pyridoxal phosphate. With two antiseizure medication and three vitamins, she had improved seizure control. At 6 years of age an attempt to wean off riboflavin resulted in break through seizures. After restarting riboflavin along with pyridoxal phosphate, pyridoxine in low doses and two antiseizure medications, the child achieved good seizure control. Though partial responsiveness to pyridoxine with gene mutation in the exon 3: c.352G > A p. Gly118R is known, riboflavin dependence and transient worsening of seizures off pyridoxine has not been described to our knowledge. Our case highlights the importance of identifying the precise gene mutationsequence to properly identify variants relative to individual phenotypic expression, treatment responsivness and need for added vitamin supplementation.

Abdominal epilepsia partialis continua in neurocysticercosis.

Epilepsia partialis continua (EPC) of abdominal muscles is a rare entity with variable clinical localization and aetiology. A 25-year-old man presented with sudden onset of intermittent focal myoclonic movements involving the abdominal muscles on the right side exclusively, lasting from 20 minutes to an hour. Brain MRI revealed a ring-enhancing lesion, suggestive of cysticercal granuloma over the left precentral gyrus. The patient fulfilled the revised diagnostic criteria for definitive diagnosis of neurocysticercosis. EEG did not show focal abnormalities during the events. Episodes of EPC were controlled with difficulty using 600 mg oxcarbazepine, 200 mg lacosamide, and 2,000 mg levetiracetam. The patient received antiparasitic therapy with albendazole (15 mg/kg for two weeks) and oral dexamethasone (0.1 mg/kg) for two weeks which was then tapered. The involvement of the primary motor cortex during ictal propagation may account for this curious phenomenon. This is the first report of abdominal EPC in a patient with inflammatory granuloma as a result of neurocysticercosis.

Nine Syndrome: Case Report and Review of Clinical Signs in Internuclear Ophthalmoplegia.

The pathologic involvement of brainstem and midbrain nuclei and white matter tracts in various combinations may result in a spectrum of arithmetically derived syndromes. They include 'one and a half syndrome', 'eight and a half syndrome' and 'fifteen and a half syndrome'. We report a case of 'nine syndrome', which has been reported more recently, caused by caused by acute pontine infarcts and characterised clinically by a combination of internuclear ophthalmolplegia, ipsilateral horizontal gaze palsy, lower motor neuron type of facial palsy, contralateral hemiparesis and hemianesthesia. We highlight the genesis of this combination of clinical signs, revisit the different variants of INO and review the literature on 'Nine syndrome'.

Intractable ictal vomiting: a new form of reflex epilepsy?

In this description of the clinical course of a young female with persistent and protracted vomiting along with occasional loss of consciousness and subtle motor manifestations, the differential diagnosis is debated. The epileptic origin of her symptoms was substantiated by the presence of interictal epileptiform discharges and dramatic response to valproate monotherapy. Possible lobar localizations are discussed with the support of existing literature on this rare ictal manifestation [Published with video sequence on www.epilepticdisorders.com].