The Spectrum of Neurological Manifestations in Scrub Typhus.

BACKGROUND: Scrub typhus is a mite-borne zoonotic disease caused by Orientia tsutsugamushi and commonly presents with fever, rash, and eschar. Systemic complications develop later in the illness including, meningoencephalitis, pericardial effusion, myocarditis, and pneumonitis. In this article, we will be presenting different neurological manifestations of scrub typhus along with functional outcomes studied at a tertiary care center in New Delhi. METHODS: This ambispective observational study was conducted at Army Hospital Research and Referral, New Delhi, during January 2018- January 2020. Febrile illness, serologically confirmed as scrub typhus and developing neurological complications were included. A predesigned clinical proforma was recorded for demographics, clinical features, neurological examination, supported with laboratory and/or radiology evaluation, and functional outcomes using the modified Rankin Scale (mRS). RESULTS: In our cohort of 7 patients' majority were male (71%) with mean age at presentation being 42.5 years. Eschar was present in only 2 cases (28%) and a syndromic clinical diagnosis of meningoencephalitis was made in 3 (43%), acute flaccid quadriparesis in 2 (28%); and symptomatic seizure and parkinsonism in 1 patient each (14%). CSF showed lymphocytic pleocytosis with protein elevation in 57% cases. Systemic dysfunction was noted in the form of thrombocytopenia (57%), hyponatremia (42%), elevated transaminases (57%). Symptoms resolved with Doxycycline ± Rifampicin therapy in all cases, with good functional outcomes in majority of (89%) cases. CONCLUSION: Neurological complications in scrub typhus have a wide spectrum involving meninges, encephalon, basal ganglia, cranial, and peripheral nerves. High index of suspicion with early serological testing (ELISA) is a must in undifferentiated fevers. Timely initiation of appropriate therapy leads to good clinical outcomes, in majority of cases with neurological involvement.

A Comparative Analysis of Clinical and Imaging Features of Aquaporin 4 (AQP4) Antibody Positive, Myelin Oligodendrocyte Glycoprotein (MOG) Antibody Positive and Double Seronegative Subtypes of Neuro Myelitis Optica Spectrum Disorder (NMOSD).

Objectives: Study was conducted with aim of comparing subtypes types of NMOSD based on serology. Methods: In this retrospective study, patients ≥18 years were included satisfying IPND 2015 criteria. Three groups were created based on seropositivity for AQP4 antibody, MOG antibody or double seronegative. Demographic, clinical and imaging were compared using regression analysis. Results: Forty-six patients, 28 (60.9%) AQP4+, 11 (23.9%) MOG + and remaining 7 (15.2%) double seronegative were included. Thirty-seven patients (80.4%) had presenting symptoms localized to optic nerve and/or cord [AQP4 + 22 (78.5%), MOG + 9 (81.8%) and double seronegative 6 (85.7%)]. Presentation with bilateral optic neuritis was more common in AQP4- patients. Twenty (86.8%) out of the 23 patients who had relapsing disease localized to optic nerve and/or spinal cord [AQP4 + 13/14 (92.8%), MOG + 3/5 (60%) and double seronegative 4/4 (100%)]. Relapses were more common in AQP4+ (77% vs 12% vs10%). In AQP4 negative group disability (EDSS 4.2 vs 3.3) and progression index was relatively less (1.6 vs 1.1). CSF pleocytosis (38.8% vs 17.9%) and raised proteins (66.6% vs 32.1%) were also more common. Optic nerve MRI (>50% optic nerve and chiasma involvement) was more commonly abnormal in AQP4 negative (52.9% vs 31.2%). Regression analysis revealed females to be significantly higher in AQP4 positive NMOSD (89.3%) when compared to MOG positive (36.4%) and double seronegative (42.9%). Conclusion: Gender was the only significant difference between the three groups. There was trend towards greater disability and more relapses in AQP4 + groups.

Multicentric clinical and epidemiological comparison of neuromyelitis optica spectrum disorder with multiple sclerosis from India.

BACKGROUND: In India, Neuromyelitis optica spectrum disorders (NMOSD) can often be misdiagnosed as multiple sclerosis (MS) leading to wrong or delayed treatment. Although diagnostic criteria exist it is important to flag certain highlights in the phenotype by direct comparison which will prompt investigation in the right direction. The aim was to identify distinguishing features, especially differences in disability status and frequency of the optico-spinal syndrome. METHODS: This study was designed as a multicentric, hospital based, ambispective, observational study of patients with primary demyelination due to either NMOSD or MS. Various variables were collected using a data extraction proforma and were compared using statistical means. RESULTS: A total of 212 patients, 166 (78.3%) with MS and 46 (21.7%) with NMOSD, were included from six different cities across India. The male to female ratio was 1:1.3 in MS group and 1:2.3 in NMOSD group. Significant differences on logistic regression included: patients with NMOSD were more disabled despite having a shorter duration of illness with a high progression index (EDSS/ duration of disease in years) of 5.99 vs 0.74 respectively (p = 0.02); in subset of relapsing patients relapsing optico-spinal syndrome (optic neuritis with myelitis) was more common in NMOSD (39.1% vs 0.8%); presence of at least one T2 lesion in the last available MRI brain (78.6% vs 39.1%) and presence of at least one gadolinium enhancing lesion in brain MRI documented during course of illness (30.2% vs 8.7%) was more in MS patients. If the patient with demyelination had a progression index of ≥ 0.39, the Likelihood Ratio (LR) of having NMOSD was 1.32 (95% CI 1.06-1.64), the sensitivity was 0.74 and specificity 0.44. Other notable variables significant on univariate but not on multivariate analysis were: other autoimmune diseases were present more in the NMOSD group (13% vs 2.4%); proportion of patients who had only school education (up to class 12) but not higher were more in NMOSD (67.4% vs 38.5%); the most common clinical presentation in MS patients was either a brainstem or cerebral syndrome (41% vs 21.8%) while it was isolated myelitis in NMOSD patients (37% vs 19.3%). Other findings included: optic neuritis as a presenting feature was common and present in similar proportions in both the groups (around 37%); 50% (23/46) of NMOSD and around 30% (50/166) of MS patients had a single clinical episode during the course of their illness and in the relapsing patients, mean no of relapses (around 2.7) and ARR (MS 0.38, NMOSD 0.54) were similar. Secondary progressive MS was diagnosed in 4.8% (8/166) and primary progressive MS was diagnosed in 3.7% (6/166). CONCLUSION: Index of suspicion for NMOSD should be high in a patient if: the course is relatively short; disability is out of proportion and progression index is ≥0.39 or the patient has had recurrent optico-spinal relapses. It is important to distinguish early in the course NMOSD from MS as timely specific treatment may prevent future disability.

Post-mumps acute disseminated encephalomyelitis in an adolescent.

Mumps is an acute communicable self-limiting swelling of the parotid or other salivary glands. Various organs can be involved including the testes, central nervous system, mammary glands, ovary, pancreas, kidneys, and heart. We hereby present a rare case of an 18-year-old unvaccinated male with acute disseminated encephalomyelitis following mumps without parotitis.

Protean Neurological Manifestations in Chikungunya.

Unplanned urbanization and secondary migration has caused increased spurt in arboviral diseases especially Dengue and Chikungunya. With this exponential rise in these illness, now we are beginning to notice uncommon presentations of these common illnesses. Here we present two interesting cases: one of paraparesis and another of quadriparesis with respiratory involvement secondary to Chikungunya, although the mechanism in one is hypokalemia and the other is GBS secondary to Chikungunya. Just the magnitude of cases presenting in metros and major cities of our country warrant sensitizing the physicians about these uncommon manifestations..