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.

Cerebral Venous Sinus Thrombosis in Children: A Study from a Tertiary Care Hospital of Eastern India.

CONTEXT: Cerebral venous sinus (sinovenous) thrombosis (CVST) in childhood is a rare, but under recognized, disorder, typically of multifactorial etiology, with neurologic sequelae apparent in up to 40% of survivors and mortality approaching 10%. AIM: The aim of this study was to enlist the patients diagnosed as CVST younger than 14 years of age and to diagnose the etiology along with radiological correlation. SETTINGS AND DESIGN: This prospective clinical study was conducted for 2 years in the Department of Neurology, Srirama Chandra Bhanja Medical College & Hospital (SCBMCH), Cuttack, Odisha, India. MATERIALS AND METHODS: All the patients were enlisted in a prestructured format with detailed clinico-radiological evaluation. Treatment was performed according to recent guidelines. Outcome after 3 months was analyzed. Ethical clearance was obtained from institutional ethics committee. STATISTICAL ANALYSIS: Data were statistically analyzed using IBM SPSS Statistics for Windows, version 20 (IBM Corp., Armonk, N.Y., USA). RESULTS: The total number of patients included in the study was 30. Of them, six were neonates. The most common provocative factor was tuberculous meningitis. Phototherapy after neonatal hyperbilirubinemia was prominent cause in neonatal age group. Multiple sinus involvement was seen in most of the patients. Transverse sinus was the most common sinus to be involved. CONCLUSION: CVST is an underdiagnosed but important cause of stroke in childhood, occurring most often in the neonatal period. Mortality and morbidity are significant. Infections hyper coagulative disorders are the two primary associations. Magnetic resonance venography is the investigation of choice. Early diagnosis with management along with plan for secondary prevention can save from catastrophic consequences.

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.