An Observational Study of Multi-Faceted Demyelinating Disorders.

BACKGROUND AND OBJECTIVES: Idiopathic inflammatory demyelinating diseases of the central nervous system (IIDCDs) are wide-ranging disorders due to their similarities and differences. In order to address these conditions, studying their characteristics is essential. The endpoints of our study were to assess the incidence, presenting features, MRI findings, and predictors of disease progression of prevalent demyelinating disorders. MATERIAL AND METHODS: This prospective, observational study was conducted at Srirama Chandra Bhanja (SCB) Medical College and Hospital, India, from August 2018 to November 2021. Individuals of 18-65 years of age with common demyelinating disorders were assessed at baseline, six, 12, and 24 months. Univariate and multivariate analyses were performed for the assessment of predictors. We used R software (version 4.2.1; R Foundation for Statistical Computing, Vienna, Austria) for data analysis. RESULTS: Two hundred twenty (79%) of 278 enrolled participants completed this study. The mean age of the study population was 52.3±11.4 years. One hundred thirty-eight (63%) of them were males. The most common IIDCD in our study was neuromyelitis optica spectrum disorder (NMOSD: 87, 39.5%), followed by multiple sclerosis (MS: 72, 32.7%), acute transverse myelitis (ATM: 35, 15.9%), and acute disseminated encephalomyelitis (ADEM: 26, 11.8%). The univariate analysis revealed that male gender, diabetes mellitus, and history of smoking or alcoholism were significant predictors of the disease progression. CONCLUSION: The IIDCDs were polysymptomatic at the initial presentation. Male diabetics are more prone to progressive disorders. However, multivariate analysis did not provide statistically significant results.

Series of Dopa Responsive Dystonia Masquerading as Other Diseases with Short Review.

Dopa-responsive dystonia (DRD) encompasses a group of clinically and genetically heterogeneous disorders that typically manifest as limb-onset, diurnally fluctuating dystonia presenting in early life and exhibits a robust and sustained response to levodopa treatment. DRD is one of the treatable dystonia syndromes of childhood. It starts with the involvement of lower limb and associated with characteristic diurnal variation. Many times it is misdiagnosed as cerebral palsy due to selective lower limb preference. We report a series of three cases of DRD which were previously misdiagnosed. The first case presented as myelopathy and other two were diagnosed as cerebral palsy. It is a treatable condition with very good response to drugs. Early diagnosis and adequate therapy can prevent from catastrophic complications.