Phenotypic spectrum of probable and genetically-confirmed idiopathic basal ganglia calcification.

Idiopathic basal ganglia calcification is characterized by mineral deposits in the brain, an autosomal dominant pattern of inheritance in most cases and genetic heterogeneity. The first causal genes, SLC20A2 and PDGFRB, have recently been reported. Diagnosing idiopathic basal ganglia calcification necessitates the exclusion of other causes, including calcification related to normal ageing, for which no normative data exist.

Clinical outcome in 287 consecutive young adults (15 to 45 years) with ischemic stroke.

Objective: To determine the 3-year outcome in 287 young adults (15 to 45 years old) consecutively admitted between 1992 and 1996 for an ischemic stroke.

Methods: Follow-up was obtained with clinical examinations or telephone interviews, and data were recorded about risk factors, associated disorders, causes of stroke, and current treatments. Functional outcomes were classified with the modified Rankin Scale (mRS).

Nerve granulomas and vasculitis in sarcoid peripheral neuropathy: a clinicopathological study of 11 patients.

Peripheral neuropathy is a rare, yet treatable manifestation of sarcoidosis, a multisystem disorder characterized by the presence of non-caseating granulomas that are seldom found in nerve biopsy specimens. In order to learn more about the subject, we reviewed our clinical and pathological findings in a series of 11 patients (six men and five women aged 26-83 years) with symptomatic neuropathy associated with characteristic granulomas in nerve biopsy specimens. Only two patients were known to have sarcoidosis before the occurrence of the neuropathy.

Early predictors of death and disability after acute cerebral ischemic event.

Background And Purpose: Many clinical trials are currently being conducted to evaluate the ability of neuroprotectors and thrombolytic agents to improve survival and functional outcome after ischemic stroke. Such trials require early predictors of survival and disability for ethical and methodological reasons. The aim of the study was to determine which variables, of those easily assessable during the first 24 hours after stroke onset, would be predictors of 8-day mortality rate and 3-month clinical outcome.

Psychoacoustical deficits related to bilateral subcortical hemorrhages. A case with apperceptive auditory agnosia.

We report a case of acute deafness secondary to bilateral hemorrhages involving the external capsule and extending to both temporal isthmi. The lesions probably disrupted both auditory radiations. Deafness disappeared within 2 weeks leading to a transient auditory agnosia for environmental and verbal sounds.