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Divry van Bogaert syndrome

Case contributed by Yves Leonard Voss
Diagnosis almost certain

Presentation

Episodes of anomic aphasia. Attention deficits. Mild cognitive impairment. Severe violaceous skin discolouration since adolescence.

Patient Data

Age: 40 years
Gender: Female
mri

There is severe cortical atrophy, multiple disseminated cortical lesions and some subcortical white matter lesions.

dsa

Peripheral angiomatosis.

Case Discussion

Young patient (40yrs) with cognitive impairment and episodes of anomic aphasia. 

The MRI shows multiple peripherally located small ischemic lesions predominantly cortical. 

The cathether angiogram shows severe peripheral angiomatosis, which means there are several peripheral occlusions and signs of extensive peripheral thin neovascularizations. 

Several other tests were performed to exclude other causes like vasculitis, repeat embolism and thrombophilia. 

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