An epidemiological survey of hereditary ataxias and paraplegias was conducted in Molise, a region of Italy (335, 211 inhabitants on 1 January 1989). Total prevalence was 7.5 x 10(-5) inhabitants (95% confidence limits 4.8-11.1). There were 7 patients with Friedreich's disease, 5 with early onset cerebellar ataxia with retained tendon reflexes, 4 with ataxia-telangiectasia, 9 with hereditary spastic paraplegias (2 autosomal dominant and 7 autosomal recessive cases). There was no patient with autosomal dominant cerebellar ataxia.
Hemi-infarction of the medulla causes the clinical constellation of symptoms and signs of both the lateral and medial medullary syndromes and nearly always results from occlusion of an intracranial vertebral artery. In the case reported here, with a clinical diagnosis of hemimedullary syndrome, the expected infarction was imaged by magnetic resonance. A review of the literature confirms that the hemimedullary syndrome, in which both medial and lateral syndromes occur simultaneously, is extremely rare, since it yielded only two previous cases with adequate anatomical confirmation.
We report a further case of retarded contralateral hemiplegia syndrome after herpes zoster ophthalmicus in which the motor deficit was caused by an ischemic infarction in the territory supplied by the anterior choroidal artery. We discuss the clinical and physiopathogenetic features of the case and consider the computed tomographic and neurological patterns of anterior choroidal infarction.
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