Marie - Boveri disease

Marie-Boveri disease (P. Marie, P. Boveri; synonym Marie-Boveri neuritis) - type family hypertrophic neuritis. Opsona Marie as "a special form of interstitial hypertrophic progressive neuritis of childhood". In the etiology of primary importance is heredity, not in all cases, the disease is shown in full, and may be expressed and abortifacient. At postmortem examination (Boveri) detected changes in the spinal cord (the rear pillars, the pyramid beams beams Flexia) and typical changes in the peripheral nerves. Last thickened; characteristic motoblaze tumors composed of cells and fibers, probably, a product of proliferation of Schwann shells and their nuclei. Belkowski (M Bielschowsky) believes these tumors "peritubular form of neurinomas".
The disease is chronic, slowly progressive, often with a long remissions. The most characteristic symptom is a thickening of the peripheral nerves, well visible and with the feeling. Also note the absence of the tendon reflexes, and sensory impairment, muscular atrophy on the lower extremities with a strong reduction of elektrovozoremontnij, bone changes (kyphoscoliosis, pes varo-equinus excavatus)intentsionnogo jitter, staccato speech. The last two symptom give the clinical picture of the disease Marie-Boveri known similarity with the clinical picture of multiple sclerosis and distinguish this form from hypertrophic neuritis of Dejerine-Cotta, which, in addition, there are changes on the part of the pupils, as well as a shooting pain.
The Outlook for the cure hopeless. Radical treatment does not exist, is only symptomatic therapy. Cm. also Dejerine - Cotta disease.