Abstract

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary neurological disease accompanied by recurrent ischemic events, characterized by the presence of psychiatric disorders. The aim of this study was to examine the occurrence of depression and its severity among patients with CADASIL. Sixteen patients with diffuse white matter changes on MRI and clinical signs suggesting CADASIL were included in the study. Definitive diagnosis of CADASIL was obtained by electron microscopic analysis of skin biopsies. Testing of the patients? affective status was primarily devoted to detecting depression. Electron microscopic examinations of all skin biopsies revealed numerous granular osmiophilic material (GOM) deposits embedded into the basal lamina around altered or degenerated vascular smooth muscle cells (VSMCs). Clinical symptoms of depression were present in a great number of examined CADASIL patients. The frequency of depression was higher than previously reported. Psychiatric disturbances might also represent the onset of CADASIL, especially in young patients, and should be evaluated by differential diagnosis.

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