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  • Title: [Stark-Kaeser type of chronic scapulo-peroneal amyotrophy. Apropos of 10 cases].
    Author: Serratrice G, Gastaut JL, Pellissier JF, Pouget J.
    Journal: Rev Neurol (Paris); 1976 Dec; 132(12):823-32. PubMed ID: 1013569.
    Abstract:
    The authors described 10 cases of scapulo-peroneal amyotrophy of spinal origin of the Stark-Kaeser type. The main characteristics are a frequently dominant autosomal heredity, onset in the muscles of the legs, scapulp-peroneal weakness and amyotrophy with extension sometimes to the bulbar muscles, areflexia without sensory disturbances, an electromyogram of the neurogenic type with a normal conduction rate, neurogenic histological aspects with normal peripheral nerve. It does not usually result in severe disability. This syndrome is related to the scapulo-peroneal or facio-scapulo-peroneal myopathies and the scapulo-peroneal neuropathies. The problem of the boundaries between these disorders and their association does not alter the fact that the concept of spinal amyotrophy of the Stark-Kaeser type should be kept as a reference group.
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