Background: Discovery of serum myelin oligodendrocyte glycoprotein (MOG) antibody testing in demyelination segregated MOG-IgG disease from AQ-4-IgG positive NMOSD. Aims: To study clinico-radiological manifestations, pattern of laboratory and electrophysiological investigations and response to treatment through follow up in MOG-IgG positive patients. Method: Retrospective data of MOG-IgG positive patients was collected. Demographics, clinical manifestations at onset and at follow up and relapses, anti AQ-4-IgG status, imaging and all investigations were performed, treatment of relapses and further immunomodulatory therapy were captured. Results: In our 30 patients, F: M ratio was 2.75:1 and adult: child ratio 4:1. Relapses at presentation were optic neuritis {ON}(60%), longitudinally extensive transverse myelitis {LETM}(20%), acute disseminated encephalomyelitis {ADEM}(13.4%), simultaneous ON with myelitis (3.3%) and diencephalic Syndrome (3.3%). Salient MRI features were ADEM-like lesions, middle cerebellar peduncle fluffy infiltrates, thalamic and pontine lesions and longitudinally extensive ON {LEON} as well as non-LEON. Totally, 50% patients had a relapsing course. Plasma exchange and intravenous immunoglobulin worked in patients who showed a poor response to intravenous methylprednisolone. Prednisolone, Azathioprine, Mycophenolate and Rituximab were effective attack preventing agents. Conclusions: MOG-IgG related manifestations in our cohort were monophasic/recurrent/simultaneous ON, myelitis, recurrent ADEM, brainstem encephalitis and diencephalic Syndrome. MRI features suggestive of MOG-IgG disease were confluent ADEM-like lesions, middle cerebellar peduncle fluffy lesions, LETM, LEON and non-LEON. Where indicated, patients need to go on immunomodulation as it has a relapsing course and can accumulate significant disability. Because of its unique manifestations, it needs to be considered as a distinct entity. To the best of our knowledge, this is the largest series of MOG-IgG disease reported from India.
Background: Neurosyphilis (NS) is a rarely encountered scenario today. Manifestations are heterogeneous, and their characteristics have changed in the antibiotic era. A differential diagnosis of NS is not commonly thought of even with relevant clinical-radiological features, as it mimics many common neurological syndromes. Objectives: To study the manifestations of NS in the present era and the process of diagnosis. Method: The data of ten patients with NS was collected and analyzed. Their background data, clinical features, investigations, the process of reaching the diagnosis, management and outcomes were recorded. Observations and Results: The manifestations of NS in our cohort included six patients with cognitive decline/encephalopathy and one each with meningitis with cranial nerve palsies, cerebellar ataxia, myelitis and asymptomatic NS. The presence of Argyll Robertson pupil helped to clinch diagnosis in one patient. Treponemal tests were ordered in two patients only after alternative etiologies were looked at, to begin with, whereas in six patients treponemal test was requested as a part of standard workup for dementia/ataxia. Conclusions: NS dementia and behavior changes are mistaken for degenerative, vascular, nutritional causes, autoimmune encephalitis or prion disease. Meningitis has similarities with infective (tubercular), granulomatous (sarcoidosis, Wegener's), collagen vascular disease and neoplastic meningitis, and myelitis simulates demyelination or nutritional myelopathy (B 12 deficiency). Rarely, NS can also present with cerebellar ataxia. Contemplate NS as one of the rare causes for such syndromes, and its early treatment produces good outcomes.
Background and Purpose: Severe acute respiratory syndrome coronavirus 2 (SARS-COV2) infection induces a prothrombotic state frequently associated with arterial ischemic strokes. Cerebral venous sinus thrombosis (CVST) is also reported with corona virus disease-19 (COVID-19) but a large cohort study is lacking. Our aim was to study the characteristics, treatment response, and outcomes of CVST occurring in association with COVID-19 (COVID-CVST) and the causal relationship with COVID-19. Methods: Data of 34 patients admitted in COVID facility and suffering from CVST and SARS-COV2 infection was studied with respect to their clinic-radiological and lab features, predisposing factors, treatment, and outcome. Observations and Results: 15 patients with CVST were detected positive for COVID but remained asymptomatic for the same. 14 patients had CVST along with symptoms of COVID, whereas 5 had CVST after recovery from COVID, at an average of 18 days after COVID-19. 4 patients were on aspirin as prophylaxis against thrombo-embolic events. The number of males exceeded females (22:12), conventional risk factors were seen in only 8 patients (postpartum state-3, alcohol abuse-2 and anemia-3), whereas the majority (26/34) showed none of them. The mean serum homocysteine level was normal and antiphospholipid antibody was tested normal in the assessed subjects. D-dimer and C reactive protein were elevated in all. 4 symptomatic patients who suffered from severe pneumonia died because of systemic complications. Conclusion: COVID-19 predisposes to CVST and the outcome is related to the severity of COVID pneumonia. CVST related to COVID occurs during or after a few weeks of COVID pneumonia and can also be seen in asymptomatic SARS-COV2 infection. COVID-19 can occur independently or in association with traditional thrombotic risk factors which increase the risk and severity of CVST in COVID. If recognized early, CVST associated with COVID can usually be treated effectively to achieve a very good outcome.
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