Intracranial paramedian hourglass-shaped dermoid associated with hereditary steatocystoma multiplex.

https://arctichealth.org/en/permalink/ahliterature4128
Source
Eur Neurol. 1992;32(5):260-3
Publication Type
Article
Date
1992
Author
M. Nagayama
Y. Shinohara
T. Takeoka
M. Haida
A. Ikeda
I. Yamamoto
O. Sato
Author Affiliation
Department of Neurology, Tokai University, School of Medicine, Kanagawa, Japan.
Source
Eur Neurol. 1992;32(5):260-3
Date
1992
Language
English
Publication Type
Article
Keywords
Brain Neoplasms - complications - radiography
Dermoid Cyst - complications - radiography
Epidermal Cyst - complications - genetics
Female
Humans
Magnetic Resonance Imaging
Middle Aged
Skin Diseases - complications - genetics
Tomography, X-Ray Computed
Abstract
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.
PubMed ID
1521546 View in PubMed
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