Goffmann myopathy

Hoffman myopathy Hoffman myopathy is a form of dystrophic myotonia, characterized by severe muscle weakness, predominant localization of the pathological process in the muscles of the proximal legs and changes in gait. However, children and adults with Hoffmann myopathy may have muscle involvement in the trunk, neck, shoulders, and proximal arms. If the distal muscle of the forearm (psilateria) is involved in the pathological process, thin hand syndrome is characteristic.

Symptoms Symptoms characteristic of Hoffman myopathy. In severe cases of pathology, pseudoparalytic syndrome may appear due to atrophy of muscle fibers. The disease can also lead to the development of pathologies such as orthostatic pulmonary edema and Hoffmann-Oberst syndrome, manifested by fibrillation of the ventricles of the heart. Symptoms characteristic of the disease are: * progressive muscle weakness in the lower extremities; * weakness and atrophy of the serratus anterior muscle; * increased tone in the calf muscles; * predominantly asymmetric atrophy of the muscles of the lower extremities.