DEZHERINA-SOTTA HYPERTROPHIC NEURITIS ( J. J. Dejerine , fr. neuropathologist and anatomist, 1849 — 1917; J. Sottas, fr. neuropathologist, sort. in 1866; neuritis hypertrophicans; grech, hyper-+ trophe food, food; neuritis; synonym: family hypertrophic neuropathy, the progressing hypertrophic intersticial polyneuritis) — the hereditary disease of a nervous system which is shown atrophies of muscles and paresis of distal departments of extremities. Also Sotta is described in 1893 by Zh. Dezherin; it is inherited on autosomal recessively type (see. Inheritance ). S. N. Davidenkov considered D. — This year N as option neural amyotrophy (see).
The pathological anatomy
Patol, process fascinates both peripheral nerves, and substance of a spinal cord. At gistol, a research the hypertrophy of intra neural connecting fabric and growth of cells of an endonevriya with a concentric thickening around the regenerating nerve fibrils is found. Growths an endonevriya prevail over connective tissue changes in a nerve. In muscle fibers puchkovy character of atrophies comes to light. Generalized defeat of myelin fibers, reduction of touch corpuscles (Meyssner's little bodies) in skin is noted. In a spinal cord fibers of back columns regenerate and cells of front horns atrophy.
The clinical picture
the Disease usually begins at early children's and youthful age, however cases of developing of a disease and at adults are described; it is characterized by the slow, progressing current with short-term remissions. It is clinically shown by atrophies of muscles of distal departments of extremities, existence of reinforced nervous trunks, is more often than peripheral nerves. On the course of these trunks painful small knots which appear and increase in volume in process of development of a disease are observed. Along with atrophies paresis of preferential distal departments of extremities, polyneuritic type of disorder of sensitivity come to light (as «gloves», «socks»), spontaneous pains on the course of nerves, are sometimes broken functions of third cranial nerves, in rare instances there are a nystagmus, an ataxia, pseudo-tabic pains, an ataxy. Deep reflexes on extremities at the beginning of a disease can be raised, further disappear. In cerebrospinal liquid insignificant increase in protein is found.
Moderate reduction in the rate of carrying out an impulse on peripheral nerves is characteristic of D. — This year N: it fluctuates from 40 to 45 m/s (norm for efferent fibers of 49 — 62 m/s, for afferent fibers of 55 — 65 m/s). At the issl a giving by means of cutaneous electrodes (see. Electromyography ) the transitional I—II EMG or IIA type (low-amplitude, low-frequency character of curve EMG) comes to light.
Some authors consider that the special form of the progressing hypertrophic intersticial polyneuritis is represented by the neuritis described by G. I. Rossolimo (1899). The disease begins at children's age, is shown by a thickening and morbidity of nerves, an atrophy of muscles with fibrillar twitchings and disorders of sensitivity. In some cases Argayll Robertson's syndrome is found (see. Argaylla-Robertson syndrome ), non-constant nystagmus and diplopia. Other kind of the progressing hypertrophic intersticial polyneuritis — Mari's disease — Boveri, at a cut along with motive and sensitive disorders of peripheral character can be found out intentsionny trembling, a scanning speech what reminds a picture multiple sclerosis (see).
D.'s Diagnosis — is made This year to N on the basis a wedge., genealogical and elektrofiziol, data. The differential diagnosis is carried out with neural amyotrophy of Sharko — Mari, at a cut at some patients nerves can be thickened. Neural amyotrophy is distinguished by a characteristic combination of symptoms and an autosomal and dominant mode of inheritance. — differentiate This year N also with polyneurites of various etiology (they develop more quickly), Fridreykh's disease (see. Ataxy ), amyotrophic side sclerosis (see), myelosyringosis (see), for a cut segmented disorders of superficial sensitivity are typical. The thickening of nerves can be observed also at to a leprosy (see), syphilis (see), neurofibromatosis (see). In difficult cases the diagnosis is established by means of a biopsy of a nerve.
Fortifying, stimulating (ATP, Nerobolum, retabolil; vitamin therapy of B 1 , In 12 , In 6 , E, Aevitum); antikholinesterazny drugs (prozerin, Galantaminum), amino acids are appointed (glutaminic to - that, methionine). Drug treatment is combined with LFK and massage.
Forecast concerning recovery adverse.
Bibliography: Davidenkov S. N. Hereditary diseases of a nervous system, M., 1932; about N e, Clinic and therapy of progressive muscular atrophies, L., 1954; Yusevich Yu. S. An electromyography in clinic of nervous diseases, M., 1958; D yo-j eri pe J. et S about 11 a s, Sur la nevrite interstitielle hypertrophique et progressive de l’enfance, Sem. med. (Paris), p. 129, 1893.
L. O. Badalyan.