A cerebral arteriopathy with subcortical infarcts and leukoencephalopathy is described with a pedigree suggestive for an autosomal dominant condition. In contrast to the vasculopathy designated with the acronym CADASIL, no deposits of granular osmiophilic material were detected in the vasculature and no point mutations in the NOTCH 3 gene were found. The disease occurred in a family living near Hamburg, Germany, and affected 11 women and 11 men over the last six generations. Onset of the disease was between the age of 12 and 50. Clinical symptoms included gait disturbances, dysarthria, sensomotoric deficits and a progressive dementia. Migraine-like complaints and epileptic seizures were observed in one case each. Cranial computer tomography and magnetic resonance imaging scans showed large confluent areas with decreased density in the white matter and small necroses in the brain stem, the basal ganglia and the white matter. A correlation with factors predisposing for vascular diseases could not be demonstrated. In five cases an autopsy was performed which disclosed an angiopathy affecting predominantly the penetrating arteries with consecutive lacunar infarcts, diffuse demyelination and rarefication of the subcortical white matter and degeneration of the pyramidal tracts. Histologically, the vessels showed concentric and excentric intimal proliferation, an elastosis and hyalinosis, splitting of the lamina elastica interna and a degeneration of the tunica muscularis. Electron microscopy revealed fragmentation and thickening of the basal lamina but electron-dense granules characteristic for CADASIL were not detected.

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Hemorrhages from rupturing aneurysms in the main cerebral arteries are repeatedly the object of forensic dissection. The victims are often relatively young, death is sudden and unexpected, and there is no known history of serious illness. Of a total 62,888 dissections carried out by the Institut für Rechtsmedizin of the Free University of Berlin (1956-1984), the University of Hamburg (1970-1984), and the University of Munich (1964-1984), there were 246 such cases (0.39% of the dissected material); 122 were male and 124 female. To compare possible differences between these cases and clinically conducted autopsies with regard to localization and extent of the aneurysm, age and sex of the deceased, an evaluation of the dissected material of the Department of Neuropathology, University Hospital, Hamburg-Eppendorf (1960-1984) was carried out simultaneously in altogether 160 cases, 66 male and 94 female. The phenomena are discussed. In the morphological findings, particular attention is focused on the occasional difficulty in establishing the source of the hemorrhage, which is of crucial importance when differentiating from traumatically caused hemorrhages. Some specific, particularly problematic causes (e.g., where errors were made in medical treatment), are presented in the form of case histories.

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