Comparison of Clinical and Radiological Features of Aquaporin4 (AQP-4) Antibody Positive Neuromyelitis Optica Spectrum Disorder (NMOSD) and Anti Myelin Oligodendrocyte Glycoprotein (Anti-MOG) Syndrome-Our Experience from Northwest India.

Background: More and more cases of myelin oligodendrocyte glycoprotein (MOG) antibody are being diagnosed with the availability of laboratory tests helping us to know the differing patterns from AQP-4 antibody disease and we need to understand the natural course, treatment, and prognosis in a better way. Objectives: Neuromyelitis optica spectrum disorder (NMOSD) and anti-MOG syndromes are immune-mediated inflammatory demyelinating conditions of the central nervous system (CNS) that mainly involve the optic nerves and the spinal cord. We conducted this study to compare demographic, clinical, laboratory, and radiological features of AQP-4 antibody and MOG antibody positive patients. Methods: A single-centre retrospective observational study from a large tertiary care university centre of Northwest India conducted during 2019--2021. We screened all patients presenting with acute CNS demyelinating attacks and recruited total 47 patients of which 25 were positive for AQP4 antibody and 22 were positive for MOG antibody. No patient tested positive for both antibodies. Data were collected using a standardized format including demographic, clinical, laboratory, and neuroimaging data. Results: In our study, total 47 patients were included, amongst which 25 patients were AQP4 antibody and 22 patients were MOG antibody positive. Though there was no gender preponderance, pediatric patients were more frequently affected in MOG antibody positive group. In AQP-4 antibody positive patients, myelitis was most common presenting clinical feature followed by optic neuritis (ON), simultaneous ON with myelitis, and brainstem syndrome. In MOG antibody positive group, myelitis was the commonest phenotype followed by ON, brainstem syndrome, and cerebral syndrome. The neuroimaging revealed involvement of medulla mainly area postrema, cervicodorsal spinal cord and extension of cervical lesion up to brainstem more commonly in AQP4 antibody group, on the other hand involvement of upper brainstem (midbrain and pons), cortex, and conus was more common in MOG antibody group. Conclusion: We have made an attempt to find differentiating features in AQP-4 vs. MOG antibody positive cases but they were of no statistically significance value as the numbers were small. Further larger studies may prove helpful in planning better strategies in two groups.

CARASIL - A Review of Patients from India.

Cerebral small vessel disease (CSVD) is a well-known cause of vascular dementia. Though a majority of these cases are sporadic, familial monogenic causes are being frequently identified as well. Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a rare autosomal recessive CSVD, caused by mutation in HTRA 1 gene on chromosome 10q (10q25.3-q26.2) in homozygous or compound heterozygous form. Indian literature has been quite scant with very few case reports of CARASIL, and only three familial cases were confirmed with mutational analysis. Testing facilities of HTRA 1 genetic mutation are now more widely available in India than before, and should be encouraged for appropriate patients. This would help in diagnosing, prognosticating and avoiding unnecessary further investigations and medications for these patients. We herein review the Indian scenario and our previously reported experiences of this disorder, while adding a case from north India with a befitting clinical history, family history, neuroimaging and documented HTRA1 genetic mutation.

Uncommon Presentation of Rasmussen's Encephalitis.

Rasmussen's encephalopathy (RE) is an uncommon neurological disease of inflammatory origin which is characterized by intractable focal epilepsy, progressive limb weakness, and cognitive deterioration. RE presenting as movement disorder like hemidystonia or hemichorea is a rare occurrence. The duration of prodromal stage of RE is usually in weeks or months. Prolonged prodromal stage like in years is rarely reported. Magnetic resonance imaging (MRI) is a good biomarker in RE and it also suggests the sequential progression of disease. Here we report two cases of RE, one presenting with hemidystonia and other case with unusually prolonged prodromal stage duration of 7 years. In spite of severe hemi-atrophy of brain in second case response to immunomodulators was dramatic.

Isolated Acute Cerebrovascular Involvement in COVID-19 without Fever and Respiratory Symptoms: An Indian Perspective.

BACKGROUND: Stroke in COVID-19 has been reported in critically ill patients globally. Stroke as a singular manifestation of COVID-19 in absence of typical symptoms (fever, cough and dyspnea) is under- recognized. OBJECTIVE: Comparative study of clinical and laboratory parameters of COVID-19 stroke patients without typical symptoms at onset with stroke cases without COVID-19 infection. METHODS: 28consecutive stroke patients, eight with coronavirus infection and twenty without COVID-19 admitted to neurology department of a tertiary care centre of North West India between 20 June,2020 and 19 July,2020 were enrolled in this retrospective study. RESULTS: COVID-19 patients had higher frequency of seizures (4[50%]) vs 2[10%];p= 0.03)and altered mental status(6[75%] vs 6[30%] p= 0.04). Severity of ischemic stroke(NIHSS >20, 3[75 %] vs 2[18%])and mortality(p=0.04)despite comparable vascular risk factors for stroke between the two groups was higher in COVID-19 patients. Three out of four COVID-19 young strokes died. Two females with COVID-19 did not develop any typical symptoms, six males(75%) developed fever with dyspnea after a mean delay of 2.7 days(Standard deviation 1.7) from stroke onset. All six patients who developed fever subsequently expired. Inflammatory markers (neutrophil to lymphocyte ratio;p<0.001and ESR: p<0.001), transaminases(p=0.038) and creatinine (p=0.009) were significantly elevated in COVID-19 patients. CONCLUSION: Isolated cerebrovascular involvement can be a presentation of COVID-19.Stroke severity and mortality is higher in COVID-19 with young strokes being no exemption. Development of fever was associated with clinical worsening. COVID-19 pandemic is far from over in India, such atypical presentations need to be recognized early and warrant stringent diagnostic protocols.

Cysticercotic encephalitis: a life threatening form of neurocysicercosis.

Neurocysticercosis is the most frequent neuroparasitosis and is caused by Taenia solium larvae (cysticerci). Its most common presenting feature is seizure, although it may present as headache,focal deficits, hydrocephalous, or as features of raised intracranial pressure. We herein report a case of 40-year-old male who presented with features of acute encephalitis and raised intracranial pressure with magnetic resonance imaging suggestive of multiple neurocysticerci with diffuse cerebral edema. A diagnosis of cysticercotic encephalitis was made, which is a syndrome of encephalitis with clinical and radiologic evidences of diffuse cerebral edema caused by parenchymal cysticercosis. It is important for the clinicians to be aware of this medical emergency requiring urgent attention as delay may lead to fatal outcome.

Acute intermittent porphyria presenting with neurological emergency: review of six cases.

Acute intermittent porphyria presenting with short duration of gastrointestinal symptoms followed by rapidly progressive fulminant neurological syndrome during first attack is relatively uncommon. It is a neurological emergency and mimics many other psychiatric and medical disorders and can be fatal if it remains undiagnosed and untreated. Further, specific treatment in the form of Heme arginate is not universally available and very costly, so high clinical suspicion and early diagnosis and management of acute attack and prevention of further attacks are very important. We report a series of six cases who presented with convulsion and/or polyneuropathy early in the course of disease to highlight this fact.