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

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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.

Keywords: magnetic resonance imaging; monomelic amyotrophy; myelopathy; spinal muscular atrophy

Document Type: Research Article

Affiliations: 1: Neurology, 2: Paediatrics, and 3: Magnetic Resonance and Spectroscopy, University of Innsbruck, Austria

Publication date: November 1, 2001


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