Syringomyelia(redirected from Morvan disease)
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in humans, a chronic, progressive disease of the nervous system characterized by the formation of cavities in the spinal cord, by the proliferation of neuroglia, and by sensitivity, motor, and trophic disorders. The disease was first described by the French physician Olivier d’Angers in 1824.
The occurrence of syringomyelia is related to the improper embryonic development of the central canal and the posterior median cervical septum of the spinal cord, which is possibly influenced by heredity. Environmental factors, including trauma, infections, and intoxications, may also cause the disease. Usually striking at a young age, syringomyelia is initially characterized by intense pain in the cervicobrachial area and the arm, by numbness in various parts of the body, and by decreased sensitivity to pain and temperature (possibly resulting in burns). Gradually the muscles begin to atrophy, with the hands affected first, becoming clawhands. The skin becomes dry and cyanotic, and the bones become brittle. Other elements of the disease are curvature of the spine, pathological fractures, and trophic diseases of the joints. The spread of the disease to the brain stem results in syringobulbia, which is characterized by paresis of the soft palate, speech and swallowing disturbances, and atrophy of the tongue muscles.
The diagnosis of syringomyelia is facilitated by anomalies in an individual’s physical development, including asymmetry of the face and lid splits and spina bifida occulta. The disease is treated with repeated roentgenotherapy, doses of Neostigmine (or galanthamine), and ultrasound, which provides temporary relief in the early stages of the disease. In some cases, syringomyelia is treated surgically.
REFERENCEShamburov, D. A. Siringomieliia. Moscow, 1961.
V. B. GEL’FAND