Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait.

  title={Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait.},
  author={William R. Kennedy and Milton Alter and Joo Ho Sung},
  volume={18 7},
Neurology 1968;18:671-680 Progressive spinal muscular atrophy, juvenile proximal spinal muscular atrophy (Kugelberg-Welander), and infantile muscular atrophy (Werdnig-Hoffman) comprise a group of diseases by virtue of their pathological similarity. Chronic degeneration of the lower motor neurons and neurogenic atrophy of the skeletal muscle are common to all. The diseases differ in mode of inheritance, age of onset, distribution of muscular atrophy, and prognosis. Some investigators have… 

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[Two brothers with very late onset of muscle weakness in X-linked recessive spinal and bulbar muscular atrophy].
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Bulbar Spinal Muscular Atrophy
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Hereditary proximal spinal and bulbar motor neuron disease of late onset. A report of six cases.
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Chronic proximal spinal muscular atrophy.


Five patients with muscular atrophy resembling this syndrome are seen, except for the following two points: onset of the disorder in adults; and presence of the syndrome of bulbar palsy in three of the five cases.
Proximal spinal muscular atrophy.
  • M. Gross
  • Medicine, Psychology
    Journal of neurology, neurosurgery, and psychiatry
  • 1966
Three cases of muscular atrophy can occur in young people which clinically resemble limb girdle muscular dystrophy but which electromyographically and histologically can be shown to be due to damage to the spinal motor neurone.
Infantile muscular atrophy.
The purpose of the present article is to call attention to the wide spectrum of its onset and severity and to correlate these features with the pathological changes.
Kugelberg-welander syndrome with dominant inheritance.
A family with dominant inheritance is reported in this paper, and eight members of the family were examined; information about the others was provided by surviving individuals.