Nagayama M, Shinohara Y, Takeoka T, Haida M, Ikeda A, Yamamoto I, Sato O
Department of Neurology, Tokai University, School of Medicine, Kanagawa, Japan.
Eur Neurol. 1992;32(5):260-3. doi: 10.1159/000116837.
This is the first report to describe the coexistence of two rare diseases, intracranial paramedian hourglass-shaped dermoid and steatocystoma multiplex. A 46-year-old female with a history of steatocystoma multiplex, bradydactylies and kyphosis showed oscillopsia, ataxia and hemifacial spasm. MRI findings suggested a giant dermoid cyst extending from the left middle temporal fossa to the cerebellopontine angle, and this was confirmed surgically. We propose the involvement of some genetic factor or pathological process common to both disorders, steatocystoma multiplex and dermoid. From the clinical point of view, patients with cystic skin lesion should also be checked for intracranial lesion.
这是首篇描述两种罕见疾病——颅内旁正中沙漏形皮样囊肿和多发性皮脂囊肿共存的报告。一名患有多发性皮脂囊肿、短指畸形和脊柱后凸病史的46岁女性出现了视振荡、共济失调和半面痉挛。磁共振成像（MRI）结果提示一个巨大的皮样囊肿从左中颞窝延伸至桥小脑角，手术证实了这一情况。我们推测这两种疾病——多发性皮脂囊肿和皮样囊肿，存在一些共同的遗传因素或病理过程。从临床角度来看，有囊性皮肤病变的患者也应检查是否存在颅内病变。
