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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and neurophysiological features of 18 cases of chronic asymmetrical spinal muscular atrophy are described. These were patients presenting with asymmetrical neurogenic atrophy involving one or more limbs who had no evidence of pyramidal tract dysfunction after 3 or more years of symptoms. There were twice as many males as females and the mean age of onset of the disorder was about 32 years. None of the patients had bulbar involvement. The tendon reflexes tended to be depressed. The distribution of muscle weakness in the limbs was very variable, and only slowly progressive. In 5 cases symptoms and signs were confined to the hands and forearms. Motor nerve conduction velocities to wasted muscles were slightly reduced but there was no evidence of generalised neuropathy. A diagnosis of chronic asymmetrical spinal muscular atrophy, as opposed to that of classical motor neurone disease, is favoured by an age of onset under 40 years, an absence of pyramidal signs or bulbar involvement after 3 years or more of symptoms, and depressed or absent tendon reflexes. The 2 conditions appear to be clinically distinct and prognosis is considerably better in chronic asymmetrical spinal muscular atrophy. The aetiology of this condition in unknown; it may be of relevance that 2 patients in this series had close relatives with Werdnig-Hoffmann disease.
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PMID:Chronic asymmetrical spinal muscular atrophy. 685 45

The number of motor cells was significantly reduced in the C8 segment of the cervical spinal cord in all 12 cases of amyotrophic lateral sclerosis (ALS), in the C6 and/or C8 segments in 1 case of adult onset spinal muscular atrophy, 2 cases of Werdnig-Hoffmann (W-H) disease, 3 of 4 cases of chronic polyneuropathy and in 1 case of poliomyelitis and 1 of ossification of the posterior longitudinal ligament in the cervical spine (OPLL). The numbers of motor cells were normal in the C6 or C8 segment in 6 cases of muscular dystrophy, except in one case of congenital muscular dystrophy, who showed reduced numbers of the motor cells. Examination of the distribution of motor cells per 500 micrometers thickness in serial sections revealed that reduction in numbers of the motor cells was diffuse and symmetrical in half the cases of ALS and W-H disease and in the cases of chronic polyneuropathy and congenital dystrophy; diffuse but asymmetrical in the other ALS and W-H disease cases and in a case of adult spinal muscular atrophy, and localized and asymmetrical in the cases of poliomyelitis and OPLL. In muscular dystrophy the distribution of motor cells showed segmental variations similar to controls.
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PMID:Morphometric quantification of the cervical limb motor cells in various neuromuscular diseases. 742 Jan 21