Individuals with a variety of congenital heart disorders may be at an increased risk of intracranial aneurysm development and cervicocephalic arterial dissection, particularly in adolescence. The muscular arteries of the head and neck are derived from neural crest cells and the neural crest is also of major importance in early cardiac development, suggesting that an abnormality of the neural crest may be the common pathogenetic factor explaining this association.

A 30 year old woman was admitted to hospital with a rapidly progressive decline in level of consciousness and seizures. Neuroimaging studies disclosed thrombus in the superior sagittal sinus, bilateral cerebral venous infarctions, and oedema. She was treated with intravenous heparin and propofol for control of agitation and increased intracranial pressure. She made an excellent recovery. Three weeks after admission she alerted us to a painless brownish discolouration of many of her fingernails. Bilateral subungual haematomas in diVerent stages of resolution were noted (figure). These lesions had been created by frequent nail bed compression with a pencil to monitor motor response, a common practice of applying noxious pain stimuli in comatose patients admitted to neurological intensive care units.

The screening of asymptomatic individuals in families with intracranial aneurysms has been advocated to detect unruptured aneurysms before a major hemorrhage occurs. We report a 39-year-old male member of a large Dutch family, with a documented history of intracranial aneurysms, who suffered a subarachnoid hemorrhage 2 years after cerebral digital subtraction angiography using intravenously administered contrast medium showed no abnormalities. Conventional arteriography demonstrated three intracranial aneurysms measuring 3 x 3 mm. Potential alternative screening procedures are discussed.

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