CHRONIC SPINAL MUSCULAR ATROPHY IN ADULTS .2. OTHER FORMS

Nineteen patients with long-standing muscular wasting are described in whom the clinical and investigative data suggest a chronic disorder of anterior horn cells. They have been presented in a number of broad groups based upon the distribution of weakness and it is suggested that this grouping may bear some relationship to an aetiological classification, although exceptions probably occur. Patients showing a symmetrical distribution of weakness most commonly have a family history of the disease, and a genetically-determined metabolic defect is probably responsible in most of these cases. Several metabolic defects may occur, and some possible factors influencing the resulting distribution of weakness have been discussed. Some of the remaining patients are probably suffering from conventional" motor neurone disease of unusually slow progression. The results of investigation are discussed and further examples are described showing in some cases EMG features suggestive of a myopathic disorder. © 1969."