MRI changes were universal but diverse and involved almost all the structures of the brain in symptomatic patients. A fair correlation between MRI observations and various clinical features provides an explanation for the protean manifestations of the disease.

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SRDs are widely prevalent in India. Considering the health implications and poor awareness, there is a need to sensitize physicians and increase awareness among the public.

This study shows some distinctive characteristics of JMG, such as higher frequency of ocular myasthenia, benign course, better long-term outcome and lack of association of thymoma and other autoimmune disorders.

The clinical manifestations of Wilson disease (WD) are varied and challenging. We conducted the current study to present the phenotypic characteristics and follow-up for a large cohort of patients with WD. We reviewed the medical records of 282 cases of WD (male:female ratio, 196:86) for clinical features, investigations, treatment, and outcome data. The clinical presentations were as follows: hepatic, 42 (14.9%); hepato-neurologic, 10 (3.5%); neurologic, 195 (69.1%); pure psychiatric, 7 (2.4%); osseomuscular, 6 (2.1%); and "presymptomatic," 15 (5.3%). Mean age was 15.9 years. Presymptomatic patients and those with the hepatic form of WD were younger and patients with osseomuscular and psychiatric forms were older than neurologic patients. The mean duration of illness at the time of diagnosis was 28 months. Predominant neurologic features were as follows: parkinsonism, 62.3%; dystonia, 35.4%; cerebellar, 28%; pyramidal signs, 16%; chorea, 9%; athetosis, 2.2%; myoclonus, 3.4%; and behavioral abnormalities, 16%. Kayser-Fleischer (KF) rings were seen as follows: neurologic patients, 100%; hepatic patients, 86%; and presymptomatic patients, 59%. Positive family history was noted in 47% and consanguinity in 54%. Patients born of consanguineous parents had an earlier age of onset and shorter duration of illness before presentation. Serum ceruloplasmin was decreased in 93% and 24-hour urinary copper excretion was increased in 70% of patients. Neuroimaging (computed tomography/magnetic resonance imaging) and electrophysiologic abnormalities were seen in many patients. Overall, 195 patients were on D-penicillamine therapy and 182 on zinc sulphate. Follow-up data, available for 225 patients, for a mean duration of 46 months, revealed improvement in 176, no change in 20, and deterioration in 6. Twenty-three patients died. To conclude, despite increased awareness and recognition and significant inroads into therapeutic frontiers, follow-up remains poor in developing countries and a return to previous level of functioning is not universal.

Study design: Prospective cross-sectional study. Objective: To study epidemiology, complication, neurological and functional outcome in nontraumatic spinal cord lesions (NTSCL) after inpatient rehabilitation. Setting: Neurological rehabilitation unit of a tertiary research hospital. Methods: Sixty-four patients (M/F ¼ 28:36) with NTSCL admitted from June 2005 to January 2008 for multidisciplinary rehabilitation. Epidemiology, medical complications during stay in rehabilitation, admission and dischargeFBarthel Index (BI) and American Spinal Injury Association (ASIA) impairment scale for functional and neurological recovery, respectively, were recorded and analyzed. Results: NTSCL constituted 60% (64 of 106) of the total SCL patients admitted for rehabilitation during the same period. Female patients outnumbered males (56.25%) in the study. Mean age, duration of illness and duration of stay in rehabilitation were 30.64±13.67 years (6-57), 7.09±9.15 months (1-48) and 55.75 ± 40.91 days (14-193), respectively. The ratio of paraplegia and quadriplegia was 2:1. Forty-four patients (68.75%) had incomplete cord lesion according to the ASIA impairment scale. Spinal tumors (26.6%) were found to be the most common etiology, followed by Pott's spine (25%) and transverse myelitis (22%). Urinary tract infection was found to be the most common complication (50%), followed by spasticity (35.93%) and urinary incontinence (31.25%). The mean BI scores showed significant (P ¼ 0.000) functional recovery during rehabilitation using paired Student's t-test. The ASIA impairment scale showed significant neurological recovery (P ¼ 0.001) using the Wilcoxon non-parametric test. Conclusions: NTSCL constitute a significant proportion of overall SCL. Female population, paraplegia and incomplete cord lesions are more common among NTSCL in this study. Patients with NTSCL recover significantly both neurologically and functionally with rehabilitation intervention.

Objectives:To evaluate (1) the prevalence of operationally defined depressive disorder (ICD-10) in chronic stroke subjects and (2) the relationship of post-stroke depression (PSD) with disability.Design:Cross-sectional, descriptive study.Setting:Neurological rehabilitation unit of a tertiary care university research center.Materials and Methods:Participants were those with first episode of supratentorial stroke of more than 3 months' duration with impaired balance and gait who had been referred for rehabilitation. Data were collected on demographic data, stroke data (side and type of lesion and post-stroke duration), cognition (mini mental state examination), depressive ideation (Hamilton Depression Rating Scale - HRDS), impairment (Scandinavian Stroke Scale), balance (Berg Balance Scale), ambulatory status (Functional Ambulation Category), walking ability (speed), and independence in activities of daily living (Barthel Index). Statistical analysis was done using SPSS 13.0. We carried out the chi-square test for ordinal variables and the independent t test for continuous variables.Results:Fifty-one patients (M:F: 41:10) of mean age 46.06 ± 11.19 years and mean post-stroke duration of 467.33 ± 436.39 days) were included in the study. Eighteen of the 51 participants (35.29%) met the criteria for depression. Demographic variables like male gender, being married, living in a nuclear family, urban background, and higher HRDS score were significantly correlated with PSD (P < 0.05). Depression was related to functional disability after stroke but to a statistically insignificant level (P > 0.05) and was unrelated to lesion-related parameters.Conclusion:Depression occurs in one-third of chronic stroke survivors and is prevalent in subjects referred for rehabilitation. PSD is related primarily to demographic variables and only to a lesser extent to functional disability following stroke.