Werdnig-Hoffmann Progressive Muscular Atrophy

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Werdnig-Hoffmann atrophy is one of the most common inherited forms of progressive muscular dystrophy. This form of the disease was first described in 1874 by the German neurologist Werning and his student Hoffman. This variant of the disease is characterized by damage to central and peripheral motor neurons located in the spinal cord and muscles.

This genetic disorder is associated with a mutation in the PRKCG gene, which encodes the protein phosphorylationase of glycoprotein light chains. When mutations occur, it disrupts the production of proteins in cells, which in turn leads to muscle weakening and atrophy.

One of the first symptoms of Werdnig-Hoffmann muscle wasting disorder is weakness of the arm and leg muscles, which leads to difficulty moving and maintaining balance. On

Werding-Hoffmann progressive muscle atrophy

Werding-Hoffman progressive myotonic peripheral and generalized progressive atrophy. A chronic, slowly progressive disease is a type of degenerative process in the muscles. It can be either congenital or acquired.

The disease affects two groups of paired muscles. Among them are the proximal and distal muscles of the shoulder, forearm, flexors and extensors, and the muscles of the upper limb girdle. The name “proximal” (from the Latin “proximus”) refers to their location closer to the center of the body compared to distant muscles. Those that are located closer to the head or torso are called distal.

In this case, structural changes occur in the muscle fibers, phagocytosis, necrosis, fatty and hydropic degeneration of muscle fibers appear. The processes of transmission of nerve impulses from nerves to muscles and back are disrupted. With a long-term disease, myotonic dystrophy and muscle atrophy develop. Clinical manifestations of progressive Werling-Hoff muscle atrophy Progressive muscle atrophy is accompanied by a decrease in the strength of the affected muscles, their soreness, and deformation along the muscle fiber. In addition, there is a decrease in the elasticity of muscle groups, myopathic