Abstract
Five patients with monomelic spinal muscular atrophy are described. Clinical features included insidious onset of wasting and weakness of one limb, lack of involvement of the cranial nerves, brain stem, pyramidal tracts and sensory system, and a stable condition over a period of 4-20 years. Clinical findings, electromyography and/or muscle biopsy were consistent with anterior horn cell lesion. Central cavities were excluded by magnetic resonance imaging studies of the spinal cord. Computed tomography of skeletal musculature and electromyography indicated more diffuse lower motor neuron involvement by revealing abnormalities in clinically unaffected muscles in 4 of the 5 patients. Myokymic discharges were found in the affected limb of 1 patient.
Citations
Oct 1, 1994·Muscle & Nerve·P D Donofrio
Jan 1, 1992·Pediatric Radiology·G C LiuC W Yang
Nov 1, 1994·Journal of the Neurological Sciences·A Di MuzioA Uncini
Sep 1, 1994·Neuromuscular Disorders : NMD·R KayM S Schwartz
Jun 4, 2005·Journal of the Neurological Sciences·Michael D Weiss
Dec 1, 2005·Journal of Clinical Neuromuscular Disease·Jenny P LiaoBarend P Lotz
Jan 1, 1989·Acta Neuropathologica·J F CummingsP F Bookbinder
May 14, 2004·Journal of the Neurological Sciences·A NaliniE Ratnavalli
May 24, 2014·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Atchayaram NaliniAravinda Hanumanthapura Ramalingaiah
Jun 28, 2017·World Neurosurgery·Daniel LewisJohn Leach
Oct 1, 1993·Journal of Child Neurology·S AysunO E Ozcan
May 5, 2009·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Bo ZhouDong Zhou
Mar 28, 2008·Neuropathology and Applied Neurobiology·L StevensY Mounier
May 1, 1990·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·J OryemaS Spiegel
Mar 5, 2003·Acta Neurologica Scandinavica·M Gourie-Devi, A Nalini
Oct 6, 2000·Arquivos de neuro-psiquiatria·M R De Freitas, O J Nascimento
Mar 3, 2021·Brain and Behavior·Lulu WangDaojun Hong