8/8/2006 Case 2: CADASIL versus Binswanger's Disease

Clinical History:

A 43 year old woman with a history of ulcerative colitis, hypertension, and diabetes mellitus, who presented 3 months prior to admission with unresponsiveness, and left
hemiparesis for one week prior to that. Imaging showed bilateral white matter infarcts in the cerebral hemispheres and low density regions suggestive of recent cortical infarction. The patient was severely thrombocytopenic and TTP was entertained as one diagnosis. The thrombocytopenia was refractory to steroids and plasmapheresis. She remained minimally responsive up to her death, which was attributed to sepsis. At autopsy she had bronchopneumonia, left ventricular cardiac hypertrophy, and coronary atherosclerosis.

Diagnostic Notes:

There were severe bilateral white matter lesions at autopsy neuropathological examination. The submitted slide showed encephalomalacic cavities filled with
macrophages, with highly sclerotic vessels in adjacent tissues. The vessels were markedly positive with PAS in a diffuse pattern, not in nodular masses. Congo Red and Beta-Amyloid immunostains were negative in the vessel walls. The differential diagnosis was thus one of CADASIL versus Binswanger's Disease , and there was considerable discussion. It was commented upon that the white matter changes were typical of both, that the history of hypertension and diabetes favored a diagnosis of Binswanger's, and that the difuse rather than nodular pattern of PAS-positivity was against CADASIL . There was no known family history of cerebrovascular disease . There was no final resolution of this debate, although some felt that EM might hold the answer .

Slide Image:

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