Gowers-Welander Form Myopathies

Govers-Welander form of myopathy is a rare hereditary muscular dystrophy characterized by predominant damage to the muscles of the shoulder and pelvic girdle.

The disease was first described by the English neurologist William Govers in 1884. Later, in 1951, American neuropathologist Lars Welander studied this form of myopathy in detail and gave it a name.

Main clinical manifestations:

1. Weakness and atrophy of the muscles of the shoulder and pelvic girdle, and, to a lesser extent, the muscles of the proximal limbs.

2. Onset of the disease in adolescence or young adulthood.

3. Slowly progressive course.

4. The ability for self-care is preserved.

5. There are no dysfunctions of the respiratory and cardiovascular systems.

Diagnosis is based on the clinical picture, EMG data and molecular genetic studies. Treatment is symptomatic. The prognosis is relatively favorable.

Gower-Wellender myopathy is a rare genetically determined form of muscular dystrophy that was first described in 1952. It belongs to a group of inherited diseases called mitochondrial diseases.

This type of myopathy is considered the most severe of all forms of muscular dystrophy. It is characterized by nonspecific syndromes of innate metabolism, which manifests itself in Devic Steno syndrome, urinary elimination and malabsorption, and thyroiditis. Patients also often have mental disorders that begin in childhood. Diseases of the joints, heart, gastrointestinal tract, as well as disorders of bioenergy metabolism often occur in carriers of the myotonic dystrophy gene. However, the pathogenetic mechanisms underlying the disease in the Govers-Welander form remain poorly understood.

The first described form of the disease was identified in the American neurologist William Alvin Gover and the Swedish neurologist Ludwig Welander, together with colleagues they worked to identify the causes of mortality among young athletes after competitions. This type of muscular dystrophic heredity was first described in 1783 by the Swedish doctor Nils Fred Hilmars and the American doctor John Helper.