Muscle atrophy of Haran - DuChene

Spinal progressive amyotrophy adults was first highlighted by Dyushanom (G. C. A. Duchenne) and Aran (F. A. Aran) as independent nosological units. However, later it turned out that this form is a national group, which includes diseases of different etiology. Gradually began to allocate different amyotrophic syndromes, the etiology of which can be attributed to the already known diseases. Part diseases attributed to the group amyotrophic syphilis, another part to those of syringomyelia, part - to amyotrophic lateral sclerosis. As the study of neuroinfections revealed the possibility of a similar syndromes with chronic progressive course caused by the virus of tick-borne encephalitis. Became known late amyotrophic form of epidemic encephalitis. Clinically similar syndromes occur in diseases of the spine (spondylitis, hypertrophic pachymeningitis, dicity), neuritis cervical-brachial plexus, with slow current intramedullary tumors of the spinal cord.
However, after you have selected all of these related forms remained a group of diseases that represents the primary spinal muscular atrophy adults. Although this form is not yet finally determined, however, you can assume that it exists as a separate disease. Muscle atrophy of Haran - DuChene is rare, similar to amyotrophy werdnig-Hoffmann, but develops in the later periods of life; more often in sporadic cases, although there is a family predisposition. The etiology is unclear. The assumption of viral nature has no convincing evidence. As with all atrophy, of great importance is the harmfulness of the external environment infection, trauma, cooling and other
Pathological studies reveal chronic-degenerative process in the cells of the front horns, especially intensively expressed in the cervical part of spinal cord regeneration fiber front roots and peripheral nerves of beam muscle atrophy. The main symptom of the disease is slowly progressive muscle atrophy that starts in the distal upper limb. Atrophy small hand muscles (the muscles of the exaltation of the thumb and little finger and interosseous) and develops deformation in the form of "claws", or "the hand of Aran - DuChene" (Fig. 5). Atrophy develops more often in the right hand, in the future become symmetric and spread to the muscles of the forearm, shoulder, neck and torso, accompanied by qualitative changes of elektrovozoremontnij and data biopsy confirmed their neurogenic origin. Tendon reflexes disappear with the rise atrophy, in parallel with a decrease in muscle strength. Sensitivity is not suffering. The very slow development of the disease (sometimes for 20 years), the absence of a pyramid bulbar symptoms and disorders distinguish it from amyotrophic lateral sclerosis, with which it has much in common. The forecast is unfavorable. No effective treatment.

Fig. 5. Atrophy of the muscles of the hand