Paralysis Progressive Juvenile

Progressive juvenile paralysis

Progressive juvenile paralysis (PPJ) is a form of muscular dystrophy characterized by progressive damage to the muscles of the limbs and trunk.

The disease was first described in 1890 by the German neurologist I. Goffman. It occurs predominantly in boys aged 5-15 years.

The reasons for PYU are not completely clear. The hereditary nature of the disease is assumed to be associated with mutations in genes encoding proteins involved in the regulation of muscle tone.

Main symptoms of PPY:

1. Gradual weakness and atrophy of the leg muscles, further spreading to the muscles of the arms, torso and neck.

2. Gait disturbances, frequent falls.

3. Joint contractures.

4. Spinal deformities.

5. Dysfunction of the pelvic organs.

6. Cognitive impairment (about a third of patients).

Diagnosis is based on the clinical picture, EMG data, MRI and DNA analysis. Treatment is mainly symptomatic and aimed at slowing progression - exercise therapy, orthopedic devices, drugs that improve neuromuscular conduction. The prognosis is unfavorable; most patients lose the ability to move independently and care for themselves by the age of 20.