Glial bundles of nerve roots in werdnig-hoffmann disease
β Scribed by Samuel M. Chou
- Publisher
- John Wiley and Sons
- Year
- 1980
- Tongue
- English
- Weight
- 440 KB
- Volume
- 8
- Category
- Article
- ISSN
- 0364-5134
No coin nor oath required. For personal study only.
β¦ Synopsis
Retraction nystagmus indicates the presence of a lesion in the rostra1 periaqueductal-midbrain region [31. It may occur spontaneously or on deviation of gaze from the midposition, most commonly on attempts to look upward. In the patient described here, retraction nystagmus occurred only on bilateral simultaneous cold caloric stimulation.
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## Abstract Autopsy examination of a 31/4βyearβold child with a severe congenital hypomyelination neuropathy showed the anterior spinal nerve roots and motor cranial nerves to be almost devoid of myelin in their subarachnoid course. The posterior spinal nerve roots and peripheral nerves were less s
Spinal muscular atrophy (SMA) or Werdnig-Hoffmann disease is the second most common neuromuscular disease, with 25% of cases presenting in infancy. Deletions in the survival motor neuron gene are believed responsible for autosomal-recessive SMA. SMA affects about 1 in 10,000 births. Symptomatic newb