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はじめに
中枢神経系原発悪性リンパ腫は,中枢神経内に原発したと診断できる節外性リンパ腫の一型である1)。わが国の全国集計では全原発性脳腫瘍のうち3.1%を占め,近年増加傾向である2)。中枢神経系原発悪性リンパ腫は,ほとんどがB細胞系非ホジキンリンパ腫で,T細胞系やその他のリンパ腫は稀である。初発症状は多彩で,巣症状,頭蓋内圧亢進症状,知能・精神症状が多いが3),視力障害で発症する症例も稀に報告されており4),頻度は4%程度との報告もある5)。また,中枢神経系原発悪性リンパ腫は,多彩な症状と非特異的画像所見から,早期診断が難しく,診断までに長期間を要することが多い5)。近年,髄液中IL-10測定が,中枢神経系原発悪性リンパ腫と他の脳腫瘍との鑑別において有用であると報告されている6)。
今回筆者らは,髄液中IL-10が高値であることから中枢神経系原発悪性リンパ腫を疑い,脳生検にてびまん性大細胞型B細胞リンパ腫と診断した症例を経験したので報告する。
Abstract
We report a case of primary central nervous system lymphoma (PCNSL) that presented with visual disturbance. A 76-year-old man developed decreased bilateral visual acuity. He was diagnosed with bilateral retrobulbar neuritis by an ophthalmologist. Treatment with high-dose corticosteroids was initiated and resulted in mild improvement of visual acuity. However, the patient gradually became apathetic and bradykinetic, experiencing difficulty performing the activities of daily living; he was admitted to our hospital because of this progressive illness. Neurological examination revealed bradyphrenia and bradykinesia with frontal lobe release signs, disorientation, and ideomotor apraxia. Magnetic resonance imaging showed abnormal signals in the bilateral basal ganglia and thalamus. Cerebrospinal fluid (CSF) examination revealed no pleocytosis and slightly elevated protein levels: β2-microglobulin level was mildly increased, and IL-10 level in the CSF was markedly elevated. These findings suggested a diagnosis of PCNSL, and a brain biopsy specimen was obtained from the left caudate head. Pathological findings indicated diffuse large B-cell lymphoma. Nonspecific neurological manifestations and radiological findings can make the diagnosis of PCNSL difficult and result in delayed diagnosis. Visual impairment has been suggested as a feature of PCNSL, and an elevated IL-10 level in the CSF may be a useful marker for diagnosing PCNSL.
(Received: June 27, 2011, Accepted: May 23, 2012)
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