[Giant cell angiitis of the brain]. 1984

R Warzok, and A Oppermann, and G Coulon, and J C Bourrin

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.

UI MeSH Term Description Entries
D008297 Male Males
D001921 Brain The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. Encephalon
D002561 Cerebrovascular Disorders A spectrum of pathological conditions of impaired blood flow in the brain. They can involve vessels (ARTERIES or VEINS) in the CEREBRUM, the CEREBELLUM, and the BRAIN STEM. Major categories include INTRACRANIAL ARTERIOVENOUS MALFORMATIONS; BRAIN ISCHEMIA; CEREBRAL HEMORRHAGE; and others. Brain Vascular Disorders,Intracranial Vascular Disorders,Vascular Diseases, Intracranial,Cerebrovascular Diseases,Cerebrovascular Insufficiency,Cerebrovascular Occlusion,Brain Vascular Disorder,Cerebrovascular Disease,Cerebrovascular Disorder,Cerebrovascular Insufficiencies,Cerebrovascular Occlusions,Disease, Cerebrovascular,Diseases, Cerebrovascular,Insufficiencies, Cerebrovascular,Insufficiency, Cerebrovascular,Intracranial Vascular Disease,Intracranial Vascular Diseases,Intracranial Vascular Disorder,Occlusion, Cerebrovascular,Occlusions, Cerebrovascular,Vascular Disease, Intracranial,Vascular Disorder, Brain,Vascular Disorder, Intracranial,Vascular Disorders, Brain,Vascular Disorders, Intracranial
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013700 Giant Cell Arteritis A systemic autoimmune disorder that typically affects medium and large ARTERIES, usually leading to occlusive granulomatous vasculitis with transmural infiltrate containing multinucleated GIANT CELLS. The TEMPORAL ARTERY is commonly involved. This disorder appears primarily in people over the age of 50. Symptoms include FEVER; FATIGUE; HEADACHE; visual impairment; pain in the jaw and tongue; and aggravation of pain by cold temperatures. (From Adams et al., Principles of Neurology, 6th ed) Aortic Arteritis, Giant Cell,Aortitis, Giant Cell,Arteritis, Temporal,Cranial Arteritis,Horton Disease,Horton Giant Cell Arteritis,Juvenile Temporal Arteritis,Temporal Arteritis,Arteritis, Giant Cell, Horton,Arteritis, Giant Cell, Horton's,Giant Cell Aortic Arteritis,Giant Cell Aortitis,Giant Cell Aortitis, Horton's,Giant Cell Arteritis, Horton,Horton's Disease,Horton's Giant Cell Arteritis,Aortitides, Giant Cell,Arteritides, Cranial,Arteritides, Giant Cell,Arteritides, Juvenile Temporal,Arteritides, Temporal,Arteritis, Cranial,Arteritis, Giant Cell,Arteritis, Juvenile Temporal,Cranial Arteritides,Giant Cell Aortitides,Giant Cell Arteritides,Hortons Disease,Juvenile Temporal Arteritides,Temporal Arteritides,Temporal Arteritides, Juvenile,Temporal Arteritis, Juvenile
D014657 Vasculitis Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body. Angiitis,Angiitides,Vasculitides

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