臨床神経学

<シンポジウム(1)―5―2>鉄と神経疾患

脳表ヘモジデリン沈着症の臨床

山脇 健盛

広島大学大学院医歯薬保健学研究院応用生命科学部門脳神経内科学〔〒734―8551 広島市南区霞1―2―3〕

Superficial siderosis (SS) of the central nervous system is a rare condition in which hemosiderin is deposited in the subpial layer of the brain and/or spinal cord. It is supposed that hemosiderin deposition is a result of recurrent or persistent hemorrhage in the subarachnoid space. The causes of hemorrhage are tumor, vascular abnormality, injury, dural defect, and others. The source of hemorrhage is not apparent despite of extensive examinations in about a half of the patients with SS. Patients with SS usually reveal slowly progressive and irreversible cerebellar ataxia and/or sensorineural hearing loss. MRI of T2WI or T2*WI demonstrates characteristic linear low intensity along surface of the brain and the spinal cord. There are two types of SS. One is a classical type, in which low intensity of MRI is diffuse and symmetrical. The other is a localized type. We attempted to make a clinical criteria of SS according to the world literature. Then, the criteria was applied to cases (53 cases of classical type and 7 cases of localized type) which are collected from Japanese nationwide questionnaires. The causes and symptoms of Japanese SS are similar with those of Western countries.
Full Text of this Article in Japanese PDF (415K)

(臨床神経, 52:947−950, 2012)
key words:脳表ヘモジデリン沈着症,MRI,T2*強調画像,感音性難聴,小脳失調

(受付日:2012年5月23日)