[A case of granulomatous angiitis of the central nervous system presented with subacute mental deterioration resembling diffuse white matter disease].
We reported a 60-year-old man with granulomatous angiitis of the central nervous system (GACNS) manifesting as subacute mental deterioration. His first symptoms were nausea and vomiting which brought him to a hospital, where no abnormality was found except for gastritis. One month later, he began to feel dizziness and brain tumor was suspected by a neurosurgeon with the MRI findings such as abnormal T2 signal and swelling in his brainstem. While he was followed up, he gradually presented mental change, disorientation and dysmnesia with the abnormal T2 signal spreading over the cerebral white matter bilaterally. Corticosteroid therapy was started based on the suspicion of a lymphoproliferative disease, and his symptoms and the abnormal MRI findings improved. Then he was referred to our department for further evaluation. Because we could not find any evidence of systemic diseases and he had been almost fully recovered, we discontinued the therapy. Soon after that, his mental deterioration as well as the abnormal T2 signal lesions on MRI relapsed. By open brain biopsy, the diagnosis of GACNS was established, and steroid pulse therapy was started. His symptoms and the abnormal T2 signal lesions improved gradually and the steroid was tapered to the maintenance dose without remission. Since the laboratory and imaging findings are not specific for the diagnosis of the angiitis confined to the central nervous system, brain biopsy is recommended for these disorders.
Tashiro
J
J
Department of Neurology, Hakodate Municipal Hospital.
Yokoyama
N
N
Maruo
Y
Y
Kubota
T
T
Niwa
J
J
Shimoyama
N
N
D000208:Acute Disease; D001921:Brain / Q000473:pathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D001523:Mental Disorders / Q000188:drug therapy / Q000209:etiology*; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D020551:Pulse Therapy, Drug; D016896:Treatment Outcome; D020293:Vasculitis, Central Nervous System / Q000150:complications / Q000175:diagnosis* / Q000473:pathology*
D008775:Methylprednisolone