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Background and Aims: To study the clinical profile of genetically proven Huntington's disease (HD) patients from eastern India. Methods: This cross sectional study selected patients of HD after genetic confirmation of expanded CAG repeats in Huntingtin (HTT) gene. We performed detail clinical evaluation including cognitive and neuropsychological assessment, and imaging of brain. Results: This study included 75 patients (male: 57.3%; female: 42.7%). Mean age at onset was 37.12 (range 16-62) years; juvenile variety (onset below 20 years) was detected in 5.3%. Paternal transmission was commoner. Manifestations at onset were motor in 81.3% patients, behavioral in 10.7% and cognitive impairment in 8%. After chorea, next common movement disorder was dystonia. Frontal lobe dysfunction was found in 77.3% patients. Behavioral disturbances were observed in 77.3% patients and commonly manifested as depression, irritable behavior and anxiety. Among the three onset groups (motor/behavioral/cognitive), there was no significant difference regarding age at onset, gender distribution, pattern of inheritance (paternal/maternal), and at the time of evaluation, all groups had essentially similar pattern of clinical features. Mean CAG repeat of the patients was 48.25 (range 40-79). Our study showed some differing clinical characteristics compared to previous studies from the Indian subcontinent. Conclusion: Clinical features in our study showed differences from previous studies from the Indian subcontinent. We had more cognitive-onset patients. However, behavioral onset was lower in our study. Motor, behavioral and cognitive onset groups of HD were comparable regarding demographics, family history, CAG repeat lengths and major clinical features at the time of evaluation.

Abstract Background Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes, the most common maternally inherited mitochondrial disease, can present with a wide range of neurological manifestations including both central and peripheral nervous system involvement. The most frequent genetic mutation reported in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome is A3243G in MT-TL1 gene. Stroke-like episodes, dementia, epilepsy, lactic acidemia, myopathy, recurrent headaches, hearing impairment, diabetes, and short stature constitute the known presentations in this syndrome. Among the abnormal involuntary movements in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome, myoclonus is the commonest. Other movement disorders, including chorea, are rarely reported in this disorder. Case presentation A 14-year-old South Asian boy from rural Bengal (India), born of a second degree consanguineous marriage, with normal birth and development history, presented with abnormal brief jerky movements involving his trunk and limbs, with recurrent falls for 10 months. We present here a case of heteroplasmic mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome with A3251G mutation, in which the clinical picture was dominated by a host of involuntary abnormal movements including chorea-ballism, myoclonus, and oromandibular dystonia in a backdrop of cognitive decline, seizure, and stroke-like episode. A final diagnosis was established by muscle biopsy and genetic study. Haloperidol was administered to control the involuntary movements along with introduction of co-enzyme Q, besides symptomatic management for his focal seizures. Six months into follow-up his seizures and abnormal movements were controlled significantly with slight improvement of cognitive abilities. Conclusion The dominance of hyperkinetic movements in the clinical scenario and the finding of a point mutation A3251G in MT-TL1 gene make this a rare presentation.

Aims: To evaluate the behavioural and psychological symptoms of dementia (BPSD), to determine their correlation with types and stages of dementia and patient demographics, and to assess the impact on caregiver distress. Methods: This cross-sectional study recruited consecutive dementia patients and caregivers who attended our cognitive clinic. Standard criteria were used to classify types of dementia. BPSD were assessed with the Neuropsychiatric Inventory, and its distress scale was used for caregiver distress. Results: Of a total 107 patients, nearly all (99.1%) had at least one BPSD; 71% had ≥4 symptoms. Most frequent were apathy and agitation, followed by irritability, sleep and appetite disorders, and mood disorders; disinhibition and euphoria were least frequent. BPSD were less prominent with increasing age; males showed more agitation. Apathy and eating disorders were more prevalent in the rural community. BPSD were highest in frontotemporal dementia (FTD), followed by dementia with Lewy bodies (DLB), and least in vascular dementia. Hallucinations were more common in DLB, aberrant motor behaviour in FTD. All domains of BPSD, except for anxiety and euphoria, were more prominent with increasing severity of dementia. Increasing BPSD (except for euphoria) caused higher caregiver distress. Conclusion: BPSD are universally present, bear correlates with dementia type and severity, and cause significant caregiver distress.

Background: The rising incidence of stroke in India indicates the importance of evaluating the existing knowledge, attitude, and practice (KAP) in the community, which is essential for stroke control. Objective: To explore and compare stroke-related KAP among participants from stroke-affected families (SAFs) and nonstroke-affected families (NSFs). Design: Using stratified random sampling, a three-phase house-to-house survey was conducted in Kolkata, West Bengal, India. First, field investigators screened subjects of stroke; second, the neurologist confirmed positive cases; and third, under supervision of the neuropsychologist, a validated questionnaire on KAP was administered to participants from SAFs and age-matched NSAFs from the same neighborhood. Results: The KAP questionnaire was administered to 282 participants each from both groups. Knowledge about stroke prevailed in 97% participants and was significantly higher in the SAF group. Both SAF and NSAF groups had better knowledge about prominent symptoms of stroke (loss of consciousness and paralysis) and admitted it as emergency situation requiring hospitalization and that it was potentially preventable. Those persons belonging to the SAF group, however, had lesser knowledge of the risk factors such as diabetes (P < 0.001), smoking (P < 0.014), alcoholism (P < .0.0001), family history (P < .0.0001) and mild stroke symptoms such as headache, (P < 0.001), vomiting (P < 0.001), and fits (P 0.003) as compared to the NSAF group. Conclusions: Persons from both SAF and non-SAF groups are aware about stroke but possess lesser knowledge about the many symptoms of stroke and risk factors, indicating the necessity of enhancement of existence knowledge on symptoms for better diagnosis and of risk factors for better prevention.

Currently, the stroke incidence in India is much higher than Western industrialized countries. Large vessel intracranial atherosclerosis is the commonest cause of ischemic stroke in India. The common risk factors, that is, hypertension, diabetes, smoking, and dyslipidemia are quite prevalent and inadequately controlled; mainly because of poor public awareness and inadequate infrastructure. Only a small number of ischemic stroke cases are able to have the benefit of thrombolytic therapy. Benefits from stem cell therapy in established stroke cases are under evaluation. Presently, prevention of stroke is the best option considering the Indian scenario through control and/or avoiding risk factors of stroke. Interventional studies are an important need for this scenario.

Rising stroke and higher mortality among Indian population needs focused attention for prevention and early management of stroke. In India, very few studies have been carried out to determine the causes of deficiencies in knowledge, attitude and practice (KAP) of stroke among Indians. Study on KAP is essential to improve the awareness about stroke, early diagnosis and institution of appropriate management. In this article, we have reviewed the existing literature on this issue and tried to compare it with those of developed countries and suggested the measures we need to adopt in India to improve awareness and knowledge base.

Background: Parkinsonism is known to be associated with clinically significant impairments on an array of cognitive deficits. The degree of impairment is dependent not only on the course of the disease, but also on other bio-social factors. The objective of the present study was to examine the cognitive dysfunction in non-demented idiopathic Parkinson′s disease (IPD) in relation to age, stage of disease, and educational level in a sample in Kolkata, India. Materials and Methods: The sample consisted of 51 (42 males, 9 females) right-handed patients suffering from non-demented IPD, of age between 40 and 82 years. Data were collected during on-phase medication by using the Kolkata Cognitive Screening Battery. Data were analyzed using means, standard deviations, and multivariate analyses of variance (MANOVA). Results and Conclusion: The patients with IPD were impaired in comparison to the available normative data in almost all aspects of cognitive functioning and higher order mental processes. With increasing age, the patients showed greater impairment in delayed memory and recognition task. Patients of more severe stage showed greater impairment in MMSE, delayed recall, and information. Those with lower education had more impaired visuoconstructional ability, information, comprehension, similarities, and arithmetic.

Cognitive learning complexity identification of assessment questions is an essential task in the domain of education, as it helps both the teacher and the learner to discover the thinking process required to answer a given question. Bloom's Taxonomy cognitive levels are considered as a benchmark standard for the classification of cognitive thinking (learning complexity) in an educational environment. However, it was observed that some of the action verbs of Bloom's Taxonomy are overlapping in multiple levels of the hierarchy, causing ambiguity about the real sense of cognition required. The paper describes two methodologies to automatically identify the cognitive learning complexity of given questions. The first methodology uses labelled Latent Dirichlet Allocation (LDA) as a machine learning approach. The second methodology uses the BERT framework for multi-class text classification for deep learning. The experiments were performed on an ensemble of 3000+ educational questions, which were based on previously published datasets along with the TREC question corpus and AI2 Biology How/Why question corpus datasets. The labelled LDA reached an accuracy of 83% while BERT based approach reached 89% accuracy. An analysis of both the results is shown, evaluating the significant factors responsible for determining cognitive knowledge.