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Background: Cognitive postscripts of COVID-19, codenamed as ‘cognitive COVID’ or ‘brain fog,’ characterized by multidomain cognitive impairments, are now being reckoned as the most devastating sequelae of COVID-19. However, the impact on the already demented brain has not been studied. Objective: We aimed to assess the cognitive functioning and neuroimaging following SARS-CoV-2 infection in patients with pre-existing dementia. Methods: Fourteen COVID-19 survivors with pre-existing dementia (four with Alzheimer’s disease, five with vascular dementia, three with Parkinson’s disease dementia, and two with the behavioral variant of frontotemporal dementia) were recruited. All these patients had detailed cognitive and neuroimaging evaluations within three months before suffering from COVID-19 and one year later. Results: Of the 14 patients, ten required hospitalization. All developed or increased white matter hyperintensities that mimicked multiple sclerosis and small vessel disease. There was a significant increase in fatigue (p = 0.001) and depression (p = 0.016) scores following COVID-19. The mean Frontal Assessment Battery (p

Moyamoya angiopathy (MMA) has been known to manifest with myriad of neurological manifestations, often in association with various precipitating factors. This is the first study to systematically analyze the precipitating triggers to neurological symptoms done on the largest cohort of MMA in India.A single-centered, cross-sectional observational study, recruiting 160 patients with consecutive angiographically proven MMA over a period of 5 years (2016-2021), was undertaken to evaluate the profile of immediate precipitating factors in temporal association to the neurological symptoms, along with their clinical and radiological characteristics. SPSS 25 was used for statistical analysis.Among the 160 patients (Adult-85, children-75), precipitating factors were seen in 41.3%, significantly higher in children (52%) than adults (31.8%) (p value: 0.011). The commonest triggers included fever (18.8%), emotional stress (8.1%), heavy exercise and diarrhea (6.3% each). Cold bath triggered MMA symptoms in 1.3%. Fever (p value: 0.008) and persistent crying (p value: 0.010) triggered neurological symptoms more commonly in children than in adults. Amongst MMA patients with precipitating factors, the commonest MMA presentation included cerebral infarction type (37.9%) and TIA (31.8%). The majority of precipitating factors that preceded an ischemic event were BP-lowing ones (54.7%).Neurological symptoms of MMA are commonly associated with several precipitating factors, including the lesser known triggers like cold bath. The frequency and profile precipitating factors varies with the age of presentation and type of MMA. It can serve as an early clue to the diagnosis of MMA and its careful avoidance can be largely beneficial in limiting the distressing transient neurological symptoms.

Objective: To evaluate the role of high-resolution intracranial vessel wall imaging (HR-IVWI) in differentiation of various intracranial vasculopathies in addition to luminal and clinical imaging in the largest cohort of Indian stroke patients. Methods: A single-center, cross-sectional study was undertaken recruiting consecutive stroke or TIA patients presenting within a month of onset, with luminal irregularity/narrowing upstream from the stroke territory. The patients were initially classified into TOAST and Chinese ischemic stroke sub-classification (CISS) on the basis of clinical and luminal characteristics and reclassified again following incorporation of HR-IVWI findings. Results: In our cohort of 150 patients, additional use of HR-IVWI led to a 10.7 and 14% change in initial TOAST and CISS classification respectively (p < 0.001). In TOAST classification, 12 “undetermined aetiology” were reclassified into intracranial atherosclerotic disease (ICAD), 1 “undetermined aetiology” into CNS angiitis and 1 “undetermined aetiology” into arterial dissection. Similarly, in CISS 19 “undetermined aetiology” was reclassified into 16 large artery atherosclerosis (LAA) and 3 “other aetiology” consisting of one CNS angiitis, Moyamoya disease (MMD) and arterial dissection each. Two initial classification of MMD by CISS and TOAST were changed into ICAD. The observed change in diagnosis following incorporation of HR-IVWI was proportionately highest in ICAD (LAA) subgroup (TOAST-9.3%, CISS-12%). Conclusion: Adjunctive use of HR-IVWI, to clinical and luminal assessment, can significantly improve diagnostic accuracy during evaluation of intracranial vasculopathies, with its greatest utility in diagnosing in ICAD, CNS angiitis and dissection. Advances in knowledge: HR-IVWI allows clearer etiological distinction of intracranial vasculopathies having therapeutic and prognostic implications.

Dystonic storm (also called status dystonicus) is a neurological emergency characterized by sustained/intermittent involuntary generalized muscle contractions resulting in repetitive painful twisting movements and abnormal postures. It is commonly documented in patients with diagnosed primary dystonic syndromes or secondary dystonic states (i.e., patients with inborn errors of metabolism, dystonic cerebral palsy, Wilson’s disease, pantothenate kinase-associated neurodegeneration, and exposure to drugs, among others). However, viral-induced dystonic storm cases have rarely been reported. We describe the case of an 11-year-old girl from rural West Bengal (India) with a dystonic storm after Japanese encephalitis. Generalized dystonic spasms lasted for about 10–20 minutes and occurred 20–30 times/day. They were associated with extreme pain, fever, exhaustion, sweating, tachycardia, tachypnea, pupillary dilatation, arterial hypertension, and mutism and were precipitated by a full bladder and relieved somewhat during sleep. When dystonic spasms abated, she had high-grade generalized rigidity of all four limbs and fixed cervical and truncal dystonia. She was put on invasive ventilation and deep intravenous sedation with continuous midazolam infusion and other supportive measures and had a good clinical recovery. During the 12 months of follow-up, she did not have any other episode of a dystonic storm. However, axial rigidity and intermittent appendicular (upper limb) dystonic posturing were observed. The authors also have briefly discussed the differential diagnoses and treatment plans for such a neurological emergency.

Background and purpose: No previous study has assessed the frequency and clinical– radiological characteristics of patients with diabetes mellitus (DM) and acute onset non-choreic and nonballistic movements. We conducted a prospective study to investigate the spectrum of acute onset movement disorders in DM.Methods: We recruited all the patients with acute onset movement disorders and hyper-glycemia who attended the wards of three hospitals in West Bengal, India from August 2014 to July 2021.Results: Among the 59 patients (mean age = 55.4± 14.3 years, 52.5% men) who were included, 41 (69.5%) had choreic or ballistic movements, and 18 (30.5%) had nonchoreic and nonballistic movements. Ballism was the most common movement disorder (n= 18, 30.5%), followed by pure chorea (n= 15, 25.4%), choreoathetosis (n= 8, 13.6%), tremor (n= 5, 8.5%), hemifacial spasm (n= 3, 5.1%), parkinsonism (n= 3, 5.1%), myoclonus (n= 3, 5.1%), dystonia (n= 2, 3.4%), and restless leg syndrome (n= 2, 3.4%). The mean duration of DM was 9.8 ± 11.4 years (89.8% of the patients had type 2 DM). Nonketotic hypergly-cemia was frequently (76.3%) detected. The majority (55.9%) had no magnetic resonance imaging (MRI) changes; the remaining showed striatal hyperintensity. Eight patients with MRI changes exhibited discordance with sidedness of movements. Most of the patients (76.3%) recovered completely.Conclusions: This is the largest clinical series depicting the clinical–radiological spectrum of acute onset movement disorders in DM. Of note was that almost one third of patients had nonchoreic and nonballistic movements. Our findings highlight the importance of a capillary blood glucose measurement in patients with acute or subacute onset movement disorders, irrespective of their past glycemic status.

Neuroparalytic snakebite envenomation is common in tropics and sub-tropics. The clinical history is sometimes unclear and misleading, leading to delay in diagnosis and initiation of life-saving treatments. It often gets so delayed that the patient may end up in neuromuscular respiratory failure. If sustained hypoxia occurs, a permanent neurological squeal may be the endpoint. We report a novel case of a 21-year-old previously healthy young Indian man who had a rapidly progressive neuroparalytic respiratory failure following a cobra bite for which he required mechanical ventilation for four days. After weaning successfully from the ventilator, he developed Lance-Adams syndrome, which persisted for over a month and eventually responded well to medical treatment.

A 47-year-old woman with history of seizure disorder (semiology of seizure unknown), not well controlled with antiepileptic drugs since last 30 years presented with 1-year history of intermittent throbbing headache. On the day prior to admission, she experienced worst headache, followed by loss of consciousness. On regaining consciousness, she had neck pain without any focal neurological deficit, but examination was marked by positive meningeal signs. She had history of oral ulceration, photosensitivity and small joints pain for which no medical consultancy was sought until. Following relevant investigations, this case came out to be moyamoya angiopathy secondary to underlying systemic lupus erythematosus. She was put on immunosuppressive and immunomodulator as per recommendations. Among neurological symptoms, headache improved dramatically without any further seizure recurrence till the 6 months of follow-up.

Moyamoya angiopathy, a rare cerebrovascular condition, can be primary (moyamoya disease) or secondary (moyamoya syndrome). Genetic factors, such as the ring finger protein 213 (RNF213), have been associated with moyamoya disease. However, X-linked moyamoya angiopathy/moyamoya syndrome and hypergonadotropic hypogonadism associated with moyamoya syndrome are rare. We report a case of a 14-year-old boy who presented with transient bilateral hemiparesis, recurrent seizures and cognitive decline. He previously had surgery for left-sided cryptorchidism and had been diagnosed with "epileptic attacks" or "functional movement disorders" in previous hospital admissions. Magnetic resonance angiography of the brain showed narrowing of supraclinoid portion of internal carotid arteries, as well as of middle and anterior cerebral arteries, and the presence of multiple collaterals. These findings were suggestive of moyamoya angiopathy. Laboratory investigations and karyotyping revealed a diagnosis of Klinefelter syndrome. This case presents a unique association of moyamoya angiopathy and Klinefelter syndrome in a boy from a poor socio-economic background, where the diagnosis and adequate treatment were delayed due to a lack of awareness and expertise.

Systemic lupus erythematosus is a chronic autoimmune connective tissue disorder that can affect all the neuroaxes in the central and peripheral nervous systems. Myelopathy in systemic lupus erythematosus is one of the least common neuropsychiatric syndromes accounting for 1%-2% of cases. Myelopathy has long been diagnosed based on clinical findings, laboratory tests, and gold-standard gadolinium-enhanced magnetic resonance imaging (MRI). MRI-negative myelopathy is a recently described subset of myelopathies. Here, we report the case of a young woman from rural West Bengal, India, who presented with overlapping features of white-matter and gray-matter myelopathy associated with peripheral neuropathy and bilateral asymmetric lower motor neuron-type facial paresis. The historical analysis yielded clues toward an etiological diagnosis of systemic lupus erythematosus, further substantiated by seropositivity of lupus-specific autoantibodies. Her neurological disabilities responded poorly to oral administration of hydroxychloroquine, bolus intravenous administration of methylprednisolone, and high-dose cyclophosphamide therapy but eventually responded remarkably well to cyclical rituximab therapy. This case adds to the tally of cases of MRI-negative lupus myelopathy. MRI-negative myelopathy in systemic lupus erythematosus can be easily missed if not meticulous attention is paid during clinical history taking and examinations.

Scrub typhus is a potentially life-threatening but curable disease that can produce multi-organ failure. Neurological manifestations in scrub typhus have gained attention recently, where the entire neural axis except the myoneural junction can be involved. Although the pathogenesis of neurological involvement has not been established, immune-mediated mechanisms are suspected. This article reports the clinicopathological features of scrub typhus cases presenting several rare neurological and neuropsychiatric manifestations.Three hundred fifty-four serologically confirmed scrub typhus cases were admitted to the Department of General Medicine of Burdwan Medical College and Hospital (West Bengal, India) between May 2018 and May 2022. There were 50 patients who had predominantly neurological manifestations. Of these 50 cases, ten patients presented with extremely rare neurological manifestations.We report 10 cases of scrub typhus (four men and six women) who presented with complex neurological pictures (posterior reversible encephalopathy syndrome, Opalski syndrome, parkinsonism, cerebellitis, isolated opsoclonus, acute transverse myelitis, myositis, polyradiculoneuropathy with cranial neuropathy, acute transient behavioral changes, and fibromyalgia). Immune-mediated mechanisms might have mediated the pathogenesis of most cases following scrub typhus infection.From a clinicopathological point of view, each case was unique in its presentation and treatment response. In any acute onset neurological disorders associated with febrile illness in the tropics or subtropics, scrub typhus infection should be included in the differential diagnosis, despite the absence of eschar and unremarkable neuroimaging findings. This otherwise curable disease may result in multi-organ dysfunction syndrome and death if the diagnosis is delayed.

Multiple sclerosis (MS), a subset of chronic primary inflammatory demyelinating disorders of the central nervous system, is closely associated with various movement disorders. These disorders may be due to MS pathophysiology or be coincidental. This review describes the full spectrum of movement disorders in MS with their possible mechanistic pathways and therapeutic modalities. The authors conducted a narrative literature review by searching for 'multiple sclerosis' and the specific movement disorder on PubMed until October 2021. Relevant articles were screened, selected, and included in the review according to groups of movement disorders. The most prevalent movement disorders described in MS include restless leg syndrome, tremor, ataxia, parkinsonism, paroxysmal dyskinesias, chorea and ballism, facial myokymia, including hemifacial spasm and spastic paretic hemifacial contracture, tics, and tourettism. The anatomical basis of some of these disorders is poorly understood; however, the link between them and MS is supported by clinical and neuroimaging evidence. Treatment options are disorder-specific and often multidisciplinary, including pharmacological, surgical, and physical therapies. Movements disorders in MS involve multiple pathophysiological processes and anatomical pathways. Since these disorders can be the presenting symptoms, they may aid in early diagnosis and managing the patient, including monitoring disease progression. Treatment of these disorders is a challenge. Further work needs to be done to understand the prevalence and the pathophysiological mechanisms responsible for movement disorders in MS.

Goodpasture Syndrome with a rare incidence of one case per million accounts for 20% of RPGN cases. It occurs due to antibodies against the NC1 domain of the alpha3 chain of type IV collagen. It usually presents with glomerulonephritispulmonary hemorrhage. But 35% may present with glomerulonephritis alone. Circulating anti-GBM antibodies may not be present always. Herein we present a 24-year-old female from our IPGMERSSKM Hospital, Kolkata with complaints of 30 days fever, 25 days of erythematous Skin lesions, 7 days of cola colored frothy urine, 3 days of anasarcaOliguria, 3 days of shortness of breathcough. Chest-X-ray revealed Pulmonary vascular congestion. Yet no history of pulmonary hemorrhage. ABG revealed Type 1 respiratory failure. ST-T changes in ECGDilated left Atrium and ventricle with severe Global HypokinesiaEF 44% in Echocardiography. Very high Troponin-I (247.62). Plenty of Dysmorphic RBCRBC Cast in Urine study. Urea 62 Creatinine 2.62.8gm protein per 24-hour urine. Evidence of CardiomyopathyRPGN prompted us to start Pulse methylprednisolone. Hemodialysis was also initiated, which improved dyspneaanasarca. ANA dsDNA ANCA MPO PR3 antiGBM came negative repeatedly. Renal Biopsy revealed NECROTISING CRESCENTIC GLOMERULONEPHRITIS WITH LINEAR Ig-G Staining consistent with Goodpasture Syndrome. The patient underwent 9 rounds of Plasmapheresis and subsequently being followed up on monthly daycare basis cyclophosphamide immunosuppression (EUVASCYCLOPS protocol) and Oral Prednisolone regimen. ESRD was successfully averted. Erythematous Skin lesions Biopsy confirmed perforating dermatoses. Discussion: Possibly cardiomyopathy could have resulted from oliguria due to RPGN in this case Conclusion: To our knowledge, this is a unique and rare case of co-existence of DCM with anti-GBM negative Renal involvement only Goodpasture disease along with perforating dermatoses.

Moyamoya Angiopathy (MMA) is frequently not considered in differential diagnosis of Multiple Sclerosis (MS). This is the first study to prospectively analyze rate of misdiagnosis of MMA as MS and its clinical implications. Of the 160 angiographically proven MMA, 5 patients had an initial misdiagnosis of MS (3.13%). These 5 cases had female-predominance (80%).Out of the 5 cases, 4 cases (80%) presented with hemiparesis; 3 cases (60%) had an immediate precipitating factor. Radiologically, presence of both periventricular and juxtacortical white-matter-lesions was seen in 4 out of 5 cases (80%);none had infratentorial/spinal lesion, while all 5 cases had presence of "Ivy" sign and abnormal flow voids. Differentiation relies on careful evaluation of clinico-radiological features. MMA should be considered as a rare but important differential to MS.

Background To compare the frequency and pattern of cognitive impairment in neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) patients. Results Twenty NMOSD and forty MS patients were included. Clinical and detailed neuropsychological assessment was done using frontal assessment battery and Kolkata cognitive battery supplemented with additional standard tests for different domains of cognitive functions. Domain wise tests were performed and compared. 15/20 (75%) NMOSD and 32/40 (80%) MS patients had cognitive impairment (p = 0.65). Executive function, verbal fluency, information processing speed, visuo-constructional ability, attention, complex calculation, and memory were more commonly involved in NMOSD in decreasing order. Compared to MS, the pattern was similar except that verbal fluency was more impaired in NMOSD. Expanded Disability Status scale (EDSS) correlated with cognitive involvement in NMOSD (p = 0.02) as against MS. Conclusions Executive function, verbal fluencies, and information processing speed were more affected compared to visual and verbal memory in NMOSD patients. The pattern of cognitive performance was similar in the MS group, even though clinical and radiological characteristics and pathophysiology is different, suggesting similar brain involvement.

An ambiguous definition of "asymptomatic" Moyamoya Angiopathy(aMMA) of absence of ischemic/hemorrhagic episodes in MMA patients, has led to its variable adaptation in the limited past-studies.To observe the clinic-radiological characteristics and prospective follow-up of apparently "asymptomatic" MMA, and to determine if it is truly asymptomatic or not.An observation, cohort study of 122 angiographically proven MMA over 6 years was undertaken from a single, tertiary-care-center to observe the clinico-radiological characteristics, prospective follow-up of apparently aMMA. Amongst them, 6 had an initial diagnosis of aMMA following evaluation by atleast one post-graduate doctor, which were further scrutinized by 3 different neurologists for epidemiological, clinical, radiological characteristics and subsequent follow-up. Data were analyzed using descriptive statistics.Mean age was 23.7 ± 13.14 years. 3 of 6 underwent brain-imaging for evaluation of non-migraine-like headache, 1 for dizziness, 2 as part of familial screening for MMA. 4 of 6 patients had specific-triggers for aggravation of symptoms. Brain-imaging revealed old vascular insults and ivy sign in 5 of 6 each (83.3%), mean suzuki staging was 3.6±0.82. 4 of 6 underwent cerebral perfusion study, all had hypoperfusion. Revascularization surgery was done in 2 of 6, rest were managed conservatively. None had any new-onset neurological deficit or radiological progression over a mean follow-up period of 22.3 ± 20.22 months.Apparently aMMA may not be truly asymptomatic and often have subtle "paroxysmal events" precipitated by specific-triggers, indicative of transient ischemic symptoms. Thus, warrants for a more precise definition to avoid misclassification of aMMA.

Moyamoya angiopathy, a rare cerebrovascular condition, can be primary (moyamoya disease) or secondary (moyamoya syndrome). Genetic factors, such as the ring finger protein 213 (RNF213), have been associated with moyamoya disease. However, X-linked moyamoya angiopathy/moyamoya syndrome and hypergonadotropic hypogonadism associated with moyamoya syndrome are rare. We report a case of a 14-year-old boy who presented with transient bilateral hemiparesis, recurrent seizures and cognitive decline. He previously had surgery for left-sided cryptorchidism and had been diagnosed with "epileptic attacks" or "functional movement disorders" in previous hospital admissions. Magnetic resonance angiography of the brain showed narrowing of supraclinoid portion of internal carotid arteries, as well as of middle and anterior cerebral arteries, and the presence of multiple collaterals. These findings were suggestive of moyamoya angiopathy. Laboratory investigations and karyotyping revealed a diagnosis of Klinefelter syndrome. This case presents a unique association of moyamoya angiopathy and Klinefelter syndrome in a boy from a poor socio-economic background, where the diagnosis and adequate treatment were delayed due to a lack of awareness and expertise.

A 42-year-old man from rural India presented with asymmetric progressive paraparesis mimicking compressive dorsal myelopathy, followed by distal upper limb, truncal and neck-flexor weakness, further complicated by acute urinary retention. His sensory deficits were marked by loss of joint position sense (JPS) and graded loss of vibration sense, along with a definite sensory level. Deep tendon jerks were hypo-to-areflexic, plantar was bilaterally extensor. He had become less attentive and occasionally failed to keep track with conversations. A syndromic diagnosis of myeloradiculoneuropathy with cognitive impairments was made. Further tailored investigations revealed vitamin B12deficiency with positive anti-parietal cell antibody. Diagnosis of subacute combined cord degeneration (SACD) was confirmed. Neuro-imaging revealed intramedullary intensity changes only along lateral aspect of spinal cord instead of characteristic posterior involvement. Following parenteral vitamin B12supplementation, patient started showing improvement in motor power and subjective sensory symptoms. His bladder symptoms persisted initially, however recovered finally after 6 months.

INTRODUCTION Clinical spectrum of Moyamoya angiopathy (MMA) differs across populations with different ethnicity. This study, the largest one done among Indian population was undertaken to assess clinico-radiological profile of MMA patients in eastern India. METHODS A single centre cross-sectional study was undertaken among 76 MMA cases. Each patient was evaluated for epidemiological, clinical and radiological characteristics. SPSS 25 was used for statistical analysis. P