Cerebellar ataxia due to isolated vitamin E deficiency.

Ataxia is a common and important neurological finding in medical practice. Severe deficiency of Vitamin E can profoundly affect the central nervous system and can cause ataxia and peripheral neuropathy resembling Friedreich's ataxia. Vitamin E deficiency can occur with abetalipoproteinemia, cholestatic liver disease or fat malabsorption. Ataxia with isolated Vit E deficiency (AVED) is an Autosomal Recessive genetic disorder with a mutation in the alpha tocopherol transfer protein gene (TTPA). This condition responds to high dose of Vit E and is one of the important causes of treatable ataxia. We report a young patient with Ataxia with isolated Vit E deficiency (AVED) who responded partially to replacement of Vitamin E.

Greek stroke score, Siriraj score and Allen score in clinical diagnosis of intracerebral hemorrhage and infarct: validation and comparison study.

AIM: To compare Greek stroke score with available previous two stroke scores for the diagnosis of cerebral ischemia and hemorrhage in acute stroke patients, and validate the Greek stroke score. SETTING: A tertiary hospital in India. MATERIALS AND METHODS: In a prospective study acute stroke patients were evaluated with Greek stroke score, Allen score and Siriraj stroke score. Comparability (Kappa Statistics) and validity (sensitivity, specificity, negative predictive value and positive predictive value) of the Greek stroke score and previous scores were tested. RESULT: Out of the 91 patients enrolled in the study, 47 patients had cerebral infarction and 44 patients had hemorrhage by CT scan. Allen score was uncertain/equivocal in 39 patients, Siriraj Stroke score in 22 and Greek stroke score in 47 patients. Sensitivity, Specificity, positive predictive value, negative predictive value for Allen score were 0.5(95% CI:0.34,0.58), 0.94(95% CI:0.86,0.98), 0.81(95% CI:0.56,0.95), 0.78(95% CI: 0.71,0.81) for Siriraj score were 0.75(95% CI: 0.63,0.84), 0.81(95% CI: 0.71,0.89), 0.77(95% CI: 0.65,0.86), 0.78(95% CI 0.69,0.86) and for Greek Score were 0.42(95% CI: 0.23,0.53), 0.93(95% CI: 0.87,0.98), 0.71(95% CI:0.39,0.91), 0.81(95% CI:0.75,0.85) respectively. Greek stroke score was compared with previous scores using kappa statistics which revealed substantial strength of agreement between the Allen Score for certain results. CONCLUSION: The overall comparability of Greek stroke score and Allen score was better as compared to Greek stroke score and Siriraj stroke score. Greek Stroke score was more specific in diagnosing hemorrhage as compared to Siriraj score. However, all these stroke scores lack accuracy hence could not be applied safely to guide the physician in management of stroke.