F i g . 1 Ax i a l T1 we i g h t e d b r a i n MR I i ma g e t a k e n b e f o r e t r e a t me n t s h o ws i s o -i n t e n s e l e s i o n i n t h e l e f t o r b i t a l a p e x a n d s p h e n o i d i t i s ( A) , b u t o n T2 we i g h t e d i ma g e t h e l e f t o r b i t a l a p e x l e s i o n i s i n a pp a r e n t ( B ) . C o r o n a l T1 we i g h t e d i ma g e wi t h G d -DTPA e n h a n c e me n t s h o ws t h i c k e n e d d u r a ma t e r o f t h e c e r e b r u m a n d c e r e b r a l f a l x ( C , D) . ( TOS HI B A VI S ART 1 . 5 T, A, C , D: TR＝ 5 2 0 , TE＝ 1 5 , B : TR＝ 4 , 6 0 0 , TE＝ 1 2 0 ) F i g . 2 Ax i a l T1 we i g h t e d i ma g e t a k e n 4 2 d a y s a f t e r t r e a t me n t ( A) , a n d T2 we i g h t e d i ma g e ( B ) s h o w d e c r e a s e d l e s i o n i n t h e l e f t o r b i t a l a p e x a n d i

A 42-year-old woman with bronchial asthma was admitted to our hospital due to sensory dominant mononeuritis multiplex lasting for more than 6 months. At that time, her eosinophil count was 761/μl and her sural nerve biopsy showed no findings suggestive of vasculitis. Four months later, she experienced sudden convulsions and right hemiparesis due to left lobular parietal subcortical hemorrhage, when her eosinophil count was elevated to 3,257/μl. Numerous microbleeds and small infarctions were also detected in the intracerebral areas of different regions with MRI. Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis of the small vessels, commonly affecting the peripheral nerves. Subarachnoid hemorrhage in patients with EGPA is extremely rare. Steep elevation of the eosinophil count may release certain cytokines, causing cerebral hemorrhage.

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小笠原真志 2) 西野 一三 2) 藤田 信也 1) * 要旨：症例は 71 歳女性．II 型呼吸不全の原因精査のため当科に入院した．60 歳代後半から体幹の筋力低下に 気づいていたが，日常生活は自立していた．両親がいとこ婚で姉に類症があり，傍脊柱筋，大腿屈筋と縫工筋に 選択的な筋萎縮を認めた．筋生検では筋障害は軽度であったが，遺伝子解析で SELENON(SEPN1)遺伝子に未報 告のホモ接合型バリアント(c.227T>C p.Phe76Ser)を認めた．Selenoprotein 関連ミオパチーは，幼少期まで に発症することがほとんどで，本症例は呼吸不全で顕在化した高齢発症の初めての報告である．

al. Brachyury, a crucial regulator of notochordal development is a novel biomarker for chordomas. J Pathol 2006;209:157-165. 14) Sangoi AR, Dulai MS, Beck AH, et al. Distinguishing chordoid meningiomas from their histologic mimics: an immuno-histochemical evaluation. Am J Surg Pathol 2009;33:669-681. 15) Alshammari J, Monnier P, Daniel RT, et al. Clival chordoma with an atypical presentation: a case report. J Med Case Rep 2012; 6:410. 16) Feng K, Qiuhang Z, Qiuyi Q. Transclival cerebrospinal fluid rhinorrhea as the initial presenting symptom of a tiny intradural chordoma. J Clin Neurosci 2010;17:1083-1085. Abstract A case of chordoma presenting as recurrent bacterial meningitis with cerebrospinal fluid leakage