Spinal amyotrophy

Spinal amyotrophy is a group of hereditary chronic diseases characterized by progressive atrophic paresis caused by damage to the anterior horns of the spinal cord.

Children's spinal amyotrophy of Verdnig-Hoffmann is manifested in the first year of life. The muscles of the trunk and legs are affected first, later all muscle groups are involved in the process, including the muscles innervated by the cranial nerves. Tendon reflexes disappear. Fascicular twitching is typical. Sensitivity and pelvic functions are not violated. In the late stages of the disease contractures and deformations of the skeleton develop. There is no effective treatment; The disease after a few months or years leads to death.

Benign spinal amyotrophy of Kugelberg - Welander. Atrophic paresis and fascination most often appear in childhood and adolescence. Primary proximal parts of extremities are affected. Gradually the process is generalized, but patients for many years can retain the ability to move independently. Paraclinic findings reveal changes that are characteristic of both neurogenic amyotrophy and myodystrophy. Treatment is symptomatic. The forecast is relatively favorable.