Amyotrophic Familial Spastic Paraplegia

Publisher Website
Google Scholar
Abstract
A report is given of an unusual hereditary disorder of the nervous system occurring in 3 siblings. Their disease began when they were between the ages of 3 and 5 and progressed slowly with the following features: spastic paresis appearing initially in the legs, gradually extending to the upper extremities, and eventually involving lower cranial nerves; the development of progressive, generalized muscular wasting, probably of neurogenic type, during adolescence; marked skeletal deformities; no impairment of sensation or coordination; normal intelligence. Special studies, including eeg., emg., and muscle biopsy were performed. It is concluded that these cases represent transitional forms of the more commonly known types of hereditary degenerative diseases of the nervous system, and it is suggested that classification of these particular disorders be made according to the predominant neurologic abnormalities present in the particular case under consideration, such abnormalities usually being those 1st to appear clinically at onset of the disease.
Keywords