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Degos'disease,a rare multisystem vasculopathy of unknown etiology,only occasionally involves the nervous system.We report the Mayo Clinic experience of the neurologic features of Degos'disease in a series of 15 patients.All 15 patients had the typical skin lesions of Degos'disease, confirmed by skin biopsy.Ten patients developed neurologic manifestations including fatal hemorrhagic or ischemic strokes(n=5),disabling polyradiculoneuropathy(n=1),and nonspecific neurologic symptoms without objective findings(n=4).Results of laboratory tests varied but none were pathognomonic of the disease.Long-term follow-up revealed death in six patients;nine patients were nearly asymptomatic.Immunosuppressive and antiplatelet agents were not of benefit.CNS infarcts and hemorrhages with intravascular thrombi,but without evidence of vasculitis,were characteristic features at autopsy. |
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