Presentation
Several episodes of juvenile ischemic stroke, positive family history of juvenile stroke and a violaceous skin discoloration.
Patient Data
Chronic ischemic infarctions in several locations: a cystic right posterior borderzone infarction, two small cystic left cerebellar infarctions, and a small chronic left thalamic ischemia.
No signs of acute/subacute ischemia on DWI.
TOF-MRA shows no occlusions or stenoses of the proximal intracranial circulation.
Multifocal occlusions of the peripheral arteries in the anterior and posterior circulation. There is a network of very thin collateral vessels in the periphery. These findings are best seen in the lateral projection of the ICA.
However, the proximal arteries show no stenosis or occlusion, no proximal moyamoya-like collaterals.
In the lateral projections of the external carotid artery there are small transdural anastomoses connecting the ECA to the posterior ICA territory.
Case Discussion
A patient with juvenile ischemic stroke was referred to our neurovascular center for diagnostic workup.
The patient reported a family history of juvenile ischemic stroke having two siblings who suffered from stroke at age 5 and 30 years respectively.
The patient showed a violaceous skin discoloration, net-like but discontinuous spread pattern, persistent even in warm conditions, which is called livedo racemosa.
The MRI shown above shows post-ischemic lesions in multiple territories including a borderzone infarction. The cerebral angiogram shows peripheral, but no proximal, occlusions of the intracranial arteries. There is peripheral collateralization and neoangiogenesis giving the picture of peripheral angiomatosis. There is little additional collateralization via transdural anastomoses.
There were no signs of vasculitis. Causes for repeat embolism were excluded. Thrombophilia screening was negative. Skin biopsy showed no signs of Sneddon syndrome.
The patient met the clinical and angiographic criteria for diagnosis of Divry van Bogaert syndrome.
Case imaging courtesy of Prof. R. Chapot (Alfried Krupp Krankenhaus Essen, Germany).