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Is juvenile spinal muscular atrophy of the distal upper limbs due to cervical flexion myelopathy? A case report
Author(s) -
Siebner H. R.,
Keinath S.,
Bischoff C.,
Conrad B.
Publication year - 1997
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.1997.tb00350.x
Subject(s) - medicine , myelopathy , magnetic resonance imaging , spinal cord , myelography , atrophy , anatomy , spinal cord compression , cervical vertebrae , progressive muscular atrophy , cord , radiology , surgery , pathology , disease , amyotrophic lateral sclerosis , psychiatry
A 22‐year‐old woman developed a slowly progressive symmetric weakness and muscular atrophy of distal upper limbs at the age of 17. Radiography during anteflexion and retroflexion showed a hypermobile cervical spine with a maximum at the C5/6 disc level. Cervical myelography and postmyelographic computed tomography (CT) of the lower cervical spine demonstrated a remarkable anterior shift of the dural sac during anteflexion resulting in anteroposterior compression of the lower spinal cord. Postmyelographic CT and magnetic resonance imaging (MRI) revealed atrophy of the lower spinal cord with bilateral cystic lesions. We suppose that repetitive straining and compression of the lower cervical cord during neck flexion of the hypermobile cervical spine caused selective necrosis of anterior horn cells with secondary cystic transformation. Mechanically induced flexion myelopathy should be considered in all young patients presenting with muscular atrophy of the distal upper limb. Functional CT myelography or dynamic MRI of the cervical spine are appropriate to demonstrate lower spinal cord compression during flexion.