[Giant cell angiitis of the brain]

A report is given on a 25-year-old man who was admitted to the hospital because of severe headache (CSF: 280 cells per mm3, 90% lymphocytes, later normal). Headache persisted for one year when acute deterioration occurred. The CSF showed 140 cells per mm3 (100% lymphocytes), 1 g% protein. Improvement of the patient's state after corticosteroid therapy. 9 months later he interrupted the intake of prednisolon and provoked exacerbation of the disease with motor and sensor disturbances. 21/2 years after the beginning of the symptomatology he suddenly developed cerebral coma and died. The autopsy revealed ischemic necrosis of both the left and the right thalamus, hemorrhage with destruction of the left-sided thalamus, nucleus caudatus, internal capsula and rupture into the lateral ventricle. Histologically, preparations from all parts of the brain revealed granulomatous angiitis of arteries, capillaries and veins with fibrinoid necrosis, infiltration of lymphocytes, histiocytes, epitheloid and plasma cells as well as numerous giant cells. Microaneurysms were found frequently. The inflammatory process showed various stages of the development indicating that more and more vessels had been involved at different times. Contrasting to giant cell arteritis of A. temporalis, in giant cell angiitis of the brain the blood sedimentation rate is frequently normal. In all reported cases, vessels of the leptomeninges were involved. Therefore, leptomeningeal biopsy is regarded as diagnostic procedure. The own observation as well as data of the literature demonstrate that giant cell angiitis of the brain is distinct from temporal arteritis (Horton's disease), not only because of differences in location, but also because of differences in age incidence and prognosis. It is supposed that giant cell angiitis is not a nosological entity, but the expression of different etiologic and pathogenetic mechanisms.