Multiple spontane Dissektionen der hirnversorgenden Arterien bei Ehlers-Danlos-artiger Bindegewebserkrankung Typ III mit atypischer klinischer Manifestation

We report on a patient with spontaneous bilateral dissections of the carotid arteries and dissections of the right vertebral and basilar artery who developed cerebral infarction in the bilateral capsula interna, basal ganglia and border zones following global hypoperfusion. The patient presented atypically with psychomotoric slowing. Headache was the only symptom leading to the diagnosis of spontaneous carotid dissections. Electron microscopy of a skin biopsy sample revealed an Ehlers-Danlos-like connective tissue disease Type III, although the patient showed no typical habitus with hyperextensible skin and hypermobile joints. Spontaneous dissection of cerebral arteries is a leading cause for cerebral infarction in young patients. They can present without ischaemic symptoms and should therefore be considered in patients with atypical symptoms. Even in patients without typical habitus, connective tissue disease can be the underlying aetiology. In our case, neurological symptoms progressed in spite of sufficient anticoagulation. Stent-assisted angioplasty could not be performed due to the anatomical situation of the carotid arteries. In general, however, stent-assisted angioplasty might be an option, if anticoagulation therapy fails to prevent progression of the disease.