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Primary angiitis of the central nervous system presenting as chronic superior sagittal sinus thrombosis
Author(s) -
Baca Kirsten E.,
Troncoso Juan C.,
Wityk Robert J.,
Crain Barbara J.
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a396-b
Subject(s) - medicine , superior sagittal sinus , thrombosis , sss* , pathology , autopsy , radiology , surgery
We report the neuropathological findings in a man with a 2‐year history of superior sagittal sinus (SSS) thrombosis and multiple hemorrhages who was subsequently found to have angiitis. The patient presented at age 55 with right‐sided neurologic deficits. Imaging revealed hemorrhage in the superior frontal lobes and thrombosis of the anterior two‐thirds of the SSS. He then developed left‐sided deficits, headache, memory loss, confusion, personality changes, and ataxia. Further imaging revealed cortical vein thrombosis in the right frontal lobe, hemorrhage in the left frontal lobe, and persistent SSS thrombosis. His symptoms continued to worsen. He developed additional hemorrhages and died at age 57. Autopsy confirmed complete SSS thrombosis and multiple hemorrhages of different ages. Scattered clusters of enlarged vascular profiles and petechial hemorrhages were most prominent in the parasagittal white matter and suggested venous backup secondary to SSS thrombosis. Microscopic examination revealed granulomatous inflammation of small and medium‐sized vessels throughout the hemispheres, brainstem, and meninges, including the SSS. Surrounding capillaries were thickened. The findings suggest that the chronic sinus thrombosis in this patient was related in an etiologic fashion to the angiitis.