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1/2. Neurological involvement as a poor prognostic factor in catastrophic antiphospholipid syndrome: autopsy findings in 12 cases.

    CAPS is an uncommon disease, characterized by clinical evidence of multiple organ involvement and histopathological evidence of multiple vessel occlusions, in patients with either primary or secondary antiphospholipid syndrome. The present series describes the clinical manifestations and autopsy findings of 12 patients with CAPS. Neurological involvement was considered the main cause of death in all of them. CNS pathology revealed thrombotic microangiopathy as well as small and large vessel occlusions in several brain areas. Neurological involvement in CAPS is strongly associated with thrombotic microangiopathy and should be considered a potential cause of death in these patients.
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2/2. Intracranial giant cell arteritis with fatal middle cerebral artery territory infarct.

    A 37 year-old man who developed a fatal middle cerebral territory infarct was found at autopsy, to have widespread granulomatous angiitis involving meningeal and intracranial--extracerebral vessels but not intracerebral vessels or other extra-cranial vessels. The findings are unique and overlap with those of granulomatous angiitis of the nervous system (GANS) and classic giant cell arteritis (GCA). A possible precipitant for this devastating illness was a recent chlamydia infection. The salient clinical and pathologic differences between GANS and GCA of the nervous system are discussed.
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