Тhe clinical case of benign monomelic amyotrophy of the lower limb

Abstract

The benign monomelic amyotrophy of the lower limb is a slowly progressive disease that is clinically manifested by muscle atrophy in only one lower limb. This disease is quite rare, while it is most common in Asian countries (about 80 cases have been described). According to the literature a total of 16 cases of benign monomelic amyotrophy of the lower limb were described in Europe by 2000. Etiology and pathogenesis have not been reliably studied now. The article presents a clinical case of the development of this disease in a 42-year-old patient. The patient was admitted with complaints of weakness in the right leg and its decrease in volume. During the period of hospitalization, diff erential diagnosis was carried out with amyotrophic lateral sclerosis, progressive muscle atrophy, Hirayama disease, vascular and paraneoplastic processes. According to the results of a comprehensive laboratory and instrumental examination, the diagnosis was fi rst established: benign monomelic amyotrophy of the lower limb. The father of a patient who had atrophy of the muscles of the left lower limb would also be examined. According to the data of hereditary anamnesis, an assumption was made about the presence of the phenomenon of anticipation in the inheritance of benign monomelic amyotrophy of the lower limb. This article describes for the fi rst time a case of benign monomelic amyotrophy of the lower limb in the domestic literature, as well as a familial case of this disease all over the world.