[Clinical electrodiagnostic findings in peroneal muscular atrophy (author's transl)]. 1976

B Leven

43 patients and 27 relatives suffering from a hypertrophic type of peroneal muscular atrophy have been examined. Following the classification of peroneal muscular atrophy by Dyck and Lambert early age of onset and considerable reduction of nerve conduction velocity served as criteria for allotting them to the hypertrophic type. In all patients a typical deformity of the foot developed before the age of 10. Among the relatives there were some without this deformity and with only slight clinical signs. Assessing consistency and thickness of peripheral nerves by palpation proved difficult and unreliable. These findings also varied considerably between members of one family. The motor nerve conduction velocity was less than one half of the normal on average. Even those patients with only minor clinical signs had a nerve conduction velocity reduced by one fifth. In about 60% of the patients there was dominant inheritance, in 3 it was recessive and in about one third nothing was known. 10 of the patients without dominant inheritance had rather severe neurologic disturbance and a kyphoscoliosis. Whether this is hypertrophic neuritis of Dejerine und Sottas is discussed. Data form the literature differ. Another common type of peroneal muscular atrophy is the neuronal type. In contrast to the hypertrophic type it generally begins in adults and nerve conduction velocity is hardly affected.

UI MeSH Term Description Entries
D007738 Kyphosis Deformities of the SPINE characterized by an exaggerated convexity of the vertebral column. The forward bending of the thoracic region usually is more than 40 degrees. This deformity sometimes is called round back or hunchback. Hyperkyphosis,Hyperkyphoses,Kyphoses
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009133 Muscular Atrophy Derangement in size and number of muscle fibers occurring with aging, reduction in blood supply, or following immobilization, prolonged weightlessness, malnutrition, and particularly in denervation. Atrophy, Muscle,Neurogenic Muscular Atrophy,Neurotrophic Muscular Atrophy,Atrophies, Muscle,Atrophies, Muscular,Atrophies, Neurogenic Muscular,Atrophies, Neurotrophic Muscular,Atrophy, Muscular,Atrophy, Neurogenic Muscular,Atrophy, Neurotrophic Muscular,Muscle Atrophies,Muscle Atrophy,Muscular Atrophies,Muscular Atrophies, Neurogenic,Muscular Atrophies, Neurotrophic,Muscular Atrophy, Neurogenic,Muscular Atrophy, Neurotrophic,Neurogenic Muscular Atrophies,Neurotrophic Muscular Atrophies
D009431 Neural Conduction The propagation of the NERVE IMPULSE along the nerve away from the site of an excitation stimulus. Nerve Conduction,Conduction, Nerve,Conduction, Neural,Conductions, Nerve,Conductions, Neural,Nerve Conductions,Neural Conductions
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004568 Electrodiagnosis Diagnosis of disease states by recording the spontaneous electrical activity of tissues or organs or by the response to stimulation of electrically excitable tissue. Electrodiagnoses
D005260 Female Females
D005531 Foot Deformities, Acquired Distortion or disfigurement of the foot, or a part of the foot, acquired through disease or injury after birth. Acquired Foot Deformities,Acquired Foot Deformity,Deformities, Acquired Foot,Deformity, Acquired Foot,Foot Deformity, Acquired

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