Microscopic polyangitis presenting with temporal arteritis and multiple cranial neuropathies.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis affects vessels of various diameters in various tissues or organs, sometimes associated with neurological complications. A 77-year-old man developed dysphagia, hoarseness, dysgeusia, gait unsteadiness, and right temporalgia; neurological examination revealed multiple cranial neuropathies. Laboratory studies demonstrated severe inflammatory responses, elevation of perinuclear ANCA, and mild proteinuria. Magnetic resonance imaging of the brain showed dural enhancement in the cerebellar tentorium. Biopsy revealed necrotizing glomerulonephritis in the kidney, and temporal arteritis without giant cells in the temporal artery. The patient was diagnosed with microscopic polyangitis presenting with temporal arteritis and multiple cranial nerve involvement, and was treated with predonisolone, after which the symptoms and laboratory data showed improvement. This is the first case of ANCA-associated vasculitis with pathologically verified lesions in the temporal artery as well as in the kidney. Thus, ANCA-associated vasculitis may simultaneously affect large vessels such as temporal artery, as well as microvessels in the kidney, nerves and other organs.
Morinaga
Akiyoshi
A
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Takaramachi 13-1, Kanazawa, Japan. amorinag@med.kanazawa-u.ac.jp
Ono
Kenjiro
K
Komai
Kiyonobu
K
Yamada
Masahito
M
D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D003389:Cranial Nerve Diseases / Q000276:immunology* / Q000473:pathology*; D013700:Giant Cell Arteritis / Q000276:immunology* / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male
D019268:Antibodies, Antineutrophil Cytoplasmic