Juvenile asymmetric segmental spinal muscular atrophy (Hirayama's disease) Three cases without evidence of “flexion myelopathy”.

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Bibliographic Details
Title: Juvenile asymmetric segmental spinal muscular atrophy (Hirayama's disease) Three cases without evidence of “flexion myelopathy”.
Authors: Willeit, J., Kiechl, S., Kiechl-Kohlendorfer, U., Golaszewski, S., Peer, S., Poewe, W.
Source: Acta Neurologica Scandinavica. Nov2001, Vol. 104 Issue 5, p320-322. 3p. 4 Black and White Photographs.
Subjects: Spinal muscular atrophy, Cervical vertebrae diseases
Abstract: Compression of the cervical spinal cord during neck movements (“flexion myelopathy”) was proposed to be the main pathomechanism of juvenile asymmetric spinal muscular atrophy (JASSMA). We present 3 patients with the clinical appearance of JASSMA and typical high-intensity signals in the anterior horn cell region of the lower cervical spinal cord (T2-weighted magnetic resonance images) but without evidence of dynamic spinal cord compression. In all these patients pathomechanism distinct from mechanical damage must be assumed. [ABSTRACT FROM AUTHOR]
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Database: Psychology and Behavioral Sciences Collection
Description
Abstract:Compression of the cervical spinal cord during neck movements (“flexion myelopathy”) was proposed to be the main pathomechanism of juvenile asymmetric spinal muscular atrophy (JASSMA). We present 3 patients with the clinical appearance of JASSMA and typical high-intensity signals in the anterior horn cell region of the lower cervical spinal cord (T2-weighted magnetic resonance images) but without evidence of dynamic spinal cord compression. In all these patients pathomechanism distinct from mechanical damage must be assumed. [ABSTRACT FROM AUTHOR]
ISSN:00016314
DOI:10.1034/j.1600-0404.2001.00074.x