Abstract
We report an autopsy case of amyotrophic lateral sclerosis (ALS) clinically diagnosed
as spinal progressive muscular atrophy (SPMA). The patient was a Japanese woman without
hereditary burden. She developed muscle weakness of the distal part of the left lower
extremity at age 42, followed by muscle weakness and atrophy of the right lower extremity
and upper extremities. At age 57, she needed transient ventilatory support. Slight
weakness in the facial muscles and fasciculation of the tongue appeared at age 60.
At age 61, she died of sudden respiratory arrest. During the clinical course, neurological
examination revealed neither Babinski signs nor hyperreflexia. The neuropathological
examination revealed not only neuronal loss with gliosis in the facial nucleus, hypoglossal
nucleus, and anterior horns of the spinal cord, but also loss of Betz cells and degeneration
of the pyramidal tracts. Based on these clinicopathological findings and review of
literature, we conclude that sporadic ALS mimicking SPMA is present.
Keywords
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Article info
Publication history
Accepted:
November 30,
1998
Received in revised form:
October 20,
1998
Received:
May 14,
1998
Identification
Copyright
© 1999 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
