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.

Quantification of Epistemic Capacity and Physical Frailty in Chronic Kidney Disease: Koch's Disease Co-infection.

Background Chronic kidney disease (CKD) and tuberculosis (TB) co-infection devastates the affected individual physically and psychologically. Moreover, poor immune status and mental turmoil worsen cognition and quality of life. Hence, studying the cognitive function and quality of life among such patients is necessary. This study aimed to determine the changes in mini-mental state examination (MMSE) score and general health questionnaire (GHQ-12) score at six months from baseline. Methodology This prospective, observational study was conducted at Sriram Chandra Bhanja Medical College and Hospital, India, from February 2020 to December 2021. A total of 40 patients with stage 3-4 CKD and pulmonary TB were assessed with MMSE and GHQ-12 scales at baseline, two, and six months. The study population was grouped as ≤50 and >50 years of age. We used R software (version 4.1.1) for data analysis. Results In total, 40 (69%) of the 58 enrolled participants completed this study. The mean age of the study population was 50.93 ± 9.83 years. The baseline MMSE scores (≤50 years: 20.8 ± 2.1, >50 years: 20.1 ± 1.7, p = 0.17) were increased (≤50 years: 25.4 ± 1.8, >50 years: 22.4 ± 1.6, p = 0.08) at six months. The baseline GHQ-12 scores (≤50 years: 22.8 ± 2.6, >50 years: 23.1 ± 2.8, p = 0.56) were reduced (≤50 years: 17.9 ± 1.9, >50 years: 20.3 ± 2.3, p = 0.14) at six months. Conclusions The study participants' cognitive function and quality of life improved after six months of modified antitubercular drugs. Nevertheless, the intergroup differences were not statistically significant.

Monomelic amyotrophy with clinico-radiological and electrophysiological evaluation: A study from Eastern India.

Background: Monomelic amyotrophy (MMA) is a benign, rare, sporadic disorder of adolescent and young adults with male predominance, where neurogenic amyotrophy is restricted to an upper or lower limb. It is a variant of lower motor neuron disorder with insidious onset and slow progression for 2-4 years. Paucity of cohort studies as well as relative unawareness among physicians in eastern India stimulated us to do this work. Material and Methods: Prospective observational study involving 140 cases of MMA from 2012 to 2016, conducted at S.C.B.Medical College, cuttack, Odisha to evaluate clinical profile, electrophysiology and radiological features. All the data were analysed & subjected to statistical analysis through SPSS software version 24. Results: Mean age at onset and presentation were 19.6yrs and 21.7yrs respectively and the average duration 3.2yrs. Upper limb involvement was more common (91.4%) with distal affection (83%) more than proximal (7%). Isolated Leg amyotrophy found in 12 cases (~9%) and 10 cases were having thigh weakness & atrophy. B/L Upper Limb distal involvement was present in 18.5% cases asymmetrically. Family history found in~2.8%. Autonomic symptoms were present in affected and bilateral homologous Limb in 21%, 5.8%(~6%) patients. Changes in electromyography (EMG) were present in affected limb (100%) and clinically unaffected limb (15%). Conclusion: MMA is a benign disease of young males with weakness and atrophy confined to unilateral limb or asymmetrical homologous limb and areflexia without sensory loss. It progresses variably for 2-3 years followed by stabilization without progression to Motor neuron disease.

Study of Clinicoradiological Profile and Prognosis of Longitudinally Extensive Transverse Myelitis from a Single Tertiary Center in Eastern India.

CONTEXT: Longitudinally extensive transverse myelitis (LETM) is defined as a hyperintense spinal cord lesion extending over three or more vertebral levels on sagittal T2-weighted spinal magnetic resonance imaging (MRI). Among the different causes of LETM, neuromyelitis optica (NMO) is the most common and associated with bad prognosis. AIMS: We aimed to study the clinicoradiological profile, etiology, and prognosis of LETM. SETTINGS AND DESIGN: This is a hospital-based cross-sectional prospective study. SUBJECTS AND METHODS: The study included 37 patients who presented with paraplegia/paresis or quadriplegia/paresis with MRI spine showing LETM. They were analyzed for their clinical features, routine blood parameters, MRI findings, serum aquaporin 4 (AQP4) antibodies. All patients were followed up for 1 year for prognosis. STATISTICAL ANALYSIS USED: Data analysis was performed using SPSS software (version 17, SPSS Inc.; Chicago, IL, USA). The different groups were compared with each other using Chi-square test. RESULTS: The mean age in our series was 35.97 ± 13.2 years. There was a slight female predominance of LETM in our series with a female: male ratio of 1.05:1. Thoracic spinal cord segments were the most commonly (56.76%) affected. The tendency to involve three to five segments is more common (40.54%). Serum AQP4 antibody (Ab) test was done in 26 patients with LETM. Among them, AQP4 Ab was found positive in nine patients (34.62%). A total of 22 patients (59.46%) were clinically diagnosed as NMO, 4 patients as postinfectious myelitis, 1 each in tuberculous myelitis and subacute combined degeneration, and 9 were idiopathic LETM. Prognosis of LETM in the form of Expanded Disability Status Scale at 1 year was poor in patients with NMO particularly those with positive AQP4 Ab. CONCLUSION: NMO is the most common cause of LETM. Because of poor prognosis of LETM in patients with NMO, particularly those with positive AQP4 Ab, it is very essential for early and accurate diagnosis of etiology of LETM, so that appropriate treatment can be provided to the patient.

Hemichorea Secondary to Non-Ketotic Hyperglycemia as the Presenting Manifestation of Diabetes Mellitus.

Nonketotic hyperglycemia is an unusual and rare cause of hemichorea. Hemichorea though rare may be the initial manifestation of diabetes mellitus. Correction of the hyperglycemia usually results in total resolution of the signs and symptoms. We present the case of a 71 yr old female, who presented with subacute onset of choreiform movement of left upper and lower extremities over 8 days. Her serum glucose level was 416 mg/dl and urine ketone bodies were absent. Computed tomography of brain showed right caudate nucleus and right lentiform nucleus hyperdensity suggesting hyperglycemia related hemichorea syndrome. Restoration of euglycemia along with treatment with haloperidol and tetrabenazine led to eventual resolution of all symptoms. So, nonketotic hyperglycemia should be kept as a differential diagnosis in a patient with hemichorea.

Concurrent intramedullary spinal cord and multiple intracranial tuberculomas with tuberculous optic neuritis: A rare case report.

Tuberculosis (TB) remains a worldwide burden, with a large majority of new active TB cases occurring in underdeveloped and developing countries. Tuberculous meningitis (TBM) is one of the common infections of central nervous system. Other manifestations include intracranial tuberculoma, tubercular brain abscess, spinal tuberculoma, and granulomatous arachnoiditis. Visual impairment in TBM may be due to optic neuritis, optochiasmatic arachnoiditis (OCA), tuberculoma in the chiasmatic region or in the optic pathways, chorioretinitis, secondary to hydrocephalus and increased intracranial pressure, and finally due to ethambutol toxicity. We report a case of young girl with concurrent spinal cord intramedullary tuberculoma and multiple intracranial tuberculomas with TBM and bilateral visual impairment due to tuberculous optic neuritis.

Chorea and Orofaciolingual Dystonia in a 40 Year Old Male.

Neuroacanthocytosis is a heterogeneous group of disorders which result in progressive neurodegeneration, predominantly of the basal ganglia, and erythrocyte acanthocytosis. We report a case of neuroacanthocytosis with typical phenotype of choreoacanthocytosis. A 40 year male presented with features of chorea with orofaciolingual dystonia producing eating and speech difficulties. There were features of self mutilation in form of lip and tongue biting. Peripheral blood smear examination revealed acanthocytes in our patient. Neuroimaging showed bilateral caudate atrophy and nerve conduction study showed motor axonal neuropathy. This case report describes the typical features and investigations to diagnose this rare disorder which is usually underdiagnosed.

High dose Intravenous Anti-D Immune Globulin is More Effective and Safe in Indian Paediatric Patients of Immune Thrombocytopenic Purpura.

INTRODUCTION: Immune Thrombocytopenia (ITP) is characterised by an autoimmune antibody-mediated destruction of platelets and impaired platelet production. Few controlled trials exist to guide management of patients with ITP in Indian scenario for which patients require an individualized approach. Anti-D (Rho (D) immune globulin) at a higher dose can prove to be a cost effective and safe alternative for Indian patients with ITP. AIM: To compare the safety and efficacy of higher dose (75µg/kg) intravenous Anti-D immune globulin against the standard dose of 50µg/kg for the management of ITP in Indian patients. MATERIALS AND METHODS: One hundred and sixty four children with newly diagnosed ITP between 4-14 years were randomly selected for inclusion and were treated with 50µg/kg (standard dose) or 75µg /kg (higher dose) of Anti-D to compare the efficacy and safety of higher dose intravenous anti-D immune globulin. Efficacy of Anti-D was measured in terms of rate of response and median time to response for increase in platelet counts. Any adverse event was noted. A decrease in haemoglobin concentration suggested accompanying haemolysis. RESULTS: Seventy one out of 84 patients treated with Anti-D at 75µg/kg produced complete response (85%) with median time of response being 2.5 days. On the contrary, 45 patients (70%) patients treated with 50µg/kg had complete response. However, there was no significant increase in haemolysis with higher dose. A significant correlation was found between dose and peak increase in platelet count measured at 7th day following administration. However, there was no relationship between the decrease in haemoglobin and the dose given, or between the increase in platelet count and fall in haemoglobin. CONCLUSION: A 75µg/kg dose of Anti-D is more effective with acceptable side effect in comparison to 50µg dose for treatment of newly diagnosed Indian patients of ITP.