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Chronic asymmetrical spinal muscular atrophy.

A E Harding, P G Bradbury, N M Murray

    Journal of the Neurological Sciences
    |April 1, 1983
    PubMed
    Summary

    Chronic asymmetrical spinal muscular atrophy presents unique features distinct from motor neurone disease. This condition, affecting limbs asymmetrically, shows a better prognosis and specific diagnostic criteria.

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    Area of Science:

    • Neurology
    • Clinical Neuroscience
    • Genetics

    Background:

    • Spinal muscular atrophy (SMA) typically presents with symmetrical muscle weakness.
    • Chronic asymmetrical spinal muscular atrophy (CASMA) is a rare variant with distinct clinical and neurophysiological characteristics.
    • Differentiating CASMA from classical motor neurone disease (MND) is crucial for prognosis and management.

    Purpose of the Study:

    • To describe the clinical and neurophysiological features of 18 patients with CASMA.
    • To establish diagnostic criteria that distinguish CASMA from MND.
    • To compare the prognosis of CASMA with that of classical MND.

    Main Methods:

    • Retrospective analysis of 18 patients diagnosed with CASMA.
    • Clinical examination focusing on limb involvement, pyramidal signs, and bulbar function.
    • Neurophysiological studies including motor nerve conduction velocities.
    • Comparison of clinical features with classical MND cases.

    Main Results:

    • Patients presented with asymmetrical neurogenic atrophy in one or more limbs, with no pyramidal tract dysfunction after 3+ years.
    • Mean age of onset was 32 years, with twice as many males affected.
    • None had bulbar involvement; tendon reflexes were depressed. Muscle weakness was slowly progressive and variable.
    • Motor nerve conduction velocities were slightly reduced in affected muscles, without generalized neuropathy.
    • Key diagnostic indicators for CASMA include onset under 40, absence of pyramidal/bulbar signs after 3+ years, and depressed reflexes.

    Conclusions:

    • CASMA is clinically distinct from classical MND, characterized by asymmetrical limb involvement and a better prognosis.
    • Specific features like age of onset, lack of bulbar/pyramidal signs, and reflex changes aid in differentiating CASMA from MND.
    • The etiology of CASMA remains unknown, though a potential genetic link is suggested by family history in some cases.

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