SHYLDERA DISEASE (
P. F. Schilder, amer. neuropathologist and psychiatrist, 1886 — 1940; synonym: a periak-sialny diffusion leukoencephalitis, an intracerebral tsentrolobar-ny symmetric sclerosis, an axial ekstrakortikalny diffusion aplasia, the progressing leucopathy of a brain, the children's perivascular necrosis and a sclerosis of a brain sclerosing the periaksialny diffusion progressing encephalitis, the sclerosing progressing entsefaloleykopatiya) — the inflammatory disease of the central nervous system relating to demyelinating diseases, to group progressing leu - to encephalitis.
It is for the first time described and allocated as an independent disease of a nervous system of the inflammatory nature by Shilder in 1912, to-ry observed during 19 weeks the girl of 14 years with the progressing dementia and symptoms of increase of intracranial pressure. At a pathoanatomical research in this case the extensive centers of demyelination in white matter of both cerebral hemispheres, the massive perivascular infiltrates consisting of lymphocytes and plasmocytes, a large number of the englobing cells of a neuroglia (see), proliferation and a hypertrophy of glio-tsit were found. At the same time axial (axial) cylinders of nerve fibrils were concerning a sokhranna in this connection Shilder called a disease of pe-riaksialny diffusion encephalitis. In the next years this gistokhy. and biochemical researches allowed to carry a number of cases of a similar disease to various types of a hereditary leukodystrophy (see). Profound studying patomorfol. pictures of a disease gave the grounds a nek-eye to researchers to consider Sh. as atypical option of multiple sclerosis (see).
The etiology and pathogeny are not established. It is supposed that Sh., as well as other leukoencephalites (see), is a disease of the infectious and allergic nature. The role of viruses as starting factors of giperergichesky autoimmune process is discussed.
Pathological anatomy. At a macroscopic research the arachnoid membrane of a brain is usually edematous, is sometimes thickened, vessels of a surface of a brain are full-blooded, separate crinkles or shares of a brain are flattened, ventricles are expanded. On cuts of cerebral hemispheres in white matter already at a macroscopic research the multiple centers (in rare instances — single) rounded or irregular shape which are accurately delimited, in places merging among themselves, having pinkish-gray color soft to the touch, and also the centers of yellowish-gray color, plotnovaty, porous (sometimes with cysts) diameter (on the plane of a cut of a brain) from several millimeters to 1 cm and more are visible. These centers of demyelination in different stages of process of the organization are localized in white matter of shares of a brain, is frequent in symmetric sites of both hemispheres, sometimes near a cerebral cortex; at the same time the arc-shaped fibers of a brain remain safe. There are descriptions of cases of localization of the centers also in basal kernels, visual decussation (hiazm), a cerebellum, a trunk of a brain and in a spinal cord.
At gistol. a research of the centers of demyelination find the changes characteristic of different stages of disintegration of a myelin and process of the organization of these centers. In the field of the fresh centers (it is macroscopic they pinkish-gray, soft) granular spheres, and also astrocytes (see the Neuroglia) with hypertrophied cytoplasm (mast cells of Nissl), huge («monstrozny») gliotsita (see the Ganglion) with one or two large hyperchromic kernels of irregular shape are visible; also huge gliotsita meet several faintly painted, puzyrkovidny kernels located excentricly. The axial cylinders of nerve fibrils in the centers of demyelination concerning a sokhranna which many of them bulked up of an izvita, are thickened and hyperchromic, fragmented by places. In process of the organization of the center of demyelination quantity of granular spheres and huge gliotsit umenynayet-sya, fiber-forming ayetrotsita, quantity to-rykh gradually appear increases. The microgliocytes which are also intensively impregnated at special colourings with knotty shoots and large kernels — «cells root crops» come to light. Gradually on site the center of demyelination the glial hem forms, it is frequent with cavities (macroscopically it yellowish-gray color, porous, plotnovaty). Neurons, axons to-rykh appeared within the centers of demyelination, are exposed to retrograde degeneration. In an arachnoid membrane and around vessels of a brain the infiltrates consisting of lymphocytes, not numerous plasmocytes, granular spheres are visible, also proliferation of cells of walls of small vessels, gialiioz and sclerous changes of walls of arteries and veins is observed. In all departments of a brain and especially near the centers of demyelination often find «drainage» (edematous) oligodendroglyocites.
Clinical picture. The disease can develop at any age, but to a thicket children and persons of young age get sick. Frequency of a disease at men and women is identical. Sh.'s beginning. gradual, is more rare acute and even insultopodobny. The current which is steadily progressing sometimes rather stationary with remissions and aggravations.
A wedge, manifestations in an onset of the illness are various. At adults the first sikhmptoma usually are changes of the personality, a behavior disorder, decrease in working capacity, disorders of the highest cortical functions leading to social disadaptation of patients. On this background there are attacks of excitement with a hallucinatory syndrome and psychotic states, to-rye quite often give the grounds to think of the acute beginning of schizophrenia (see). A frequent early symptom are visual disturbances in the form of a gomo-nimny or quadrant hemianopsia (see), and also the central decrease in hearing. In process of a course of a disease the intelligence decreases, apathy increases, edges can be replaced by attacks of excitement. In the subsequent the amaurosis (see the Blindness) owing to retrobulbar neuritis or diffusion demyelination of occipital shares of a brain develops. Polymorphic spasms are observed, is more often than jacksonian type with a loss of consciousness (see. Jacksonian epilepsy). At a research cherepnomozgovy (cranial, T.) nerves the pseudobulbar syndrome comes to light (see. Pseudobulbar paralysis), the central paralysis of face muscles (see. Facial nerve, Trifacial). Belly reflexes, as a rule, disappear. The motive disturbances which are usually beginning with legs are shown by increase in a tone of muscles of legs on pyramidal type in this connection gait becomes spastiko-atakti-cheskoy, and then extend to hands. The central (pyramidal) tetraparesis and tetraplegias (see Paralyses, paresis) with a tendency to increase of a muscle tone are characteristic. Along with heavy motive disturbances the tremor of hands, a choreoathetosis (see Hyperkinesias), hyperkinesias in muscles of language, an asynergia is noted (see the Cerebellum).
Highway. can proceed with dominance of mental disorders, a dysarthtia (see), an ataxy (see), with change of a tone of muscles (see the Tone) on plastic (extrapyramidal) type. In an end-stage sick Sh. are completely immobilized, the cachexia is sharply expressed (see).
At Sh. children. quite often begins sharply, insultopodobno. Rise in temperature, a headache, nausea, sometimes pains in a neck are noted. The child becomes sluggish, the speech poor with elements of a pseudobulbar dysarthtia (see). There are behavior disorders, attacks of inadequate excitement, to-rye are replaced by block. Decrease in hearing and a vision disorder is characteristic. Unlike adults, at children motive disturbances are more often shown by a static and dynamic ataxy (see), and the central paralyzes have character of monoplegias and gemiplegiya more often (see the Hemiplegia). In process of a course of a disease there are partial spasms with a loss of consciousness, dominance of a toniko-clonic component. Progreduated disease leads to mental and heavy motive disturbances, in an end-stage frequent myoclonic spasms in all body develop. In some cases at Sh. children. can proceed with symptoms of increase in intracranial pressure and on a wedge, to remind a picture a tumor of a brain.
At a research of an eyeground not rough symmetric hypostasis of disks of optic nerves comes to light. Characteristic of Sh. the central amaurosis — a blindness is at preservation of reaction of pupils to light. Often the gomonimny or quadrant hemianopsia is defined. On EEG (see Elektroentsefalografiya) diffusion symmetric disorganization of a rhythm of bioelectric activity of a brain is registered. At an ekhoentsefalografiya (see) shift of median structures of a brain is not noted. Pressure of cerebrospinal liquid normal, is observed slight increase of protein content, change of reaction of Lange (see. Cerebrospinal liquid). At a computer tomography (see the Tomography computer) a brain sites of decrease in density in the field of frontal and occipital lobes, the corresponding localizations of the large centers of demyelination sometimes are defined.
The variety a wedge, manifestations is caused by the diffusion nature of demyelinating process in a brain, various sizes of the centers of demyelination and their localization, and also unequal degree of manifestation of the inflammatory phenomena.
It is difficult to establish the diagnosis in connection with variety of klin.proyavleniye of III., exclusive rarity of a disease. Clinical picture Sh. can imitate many other diseases of c. N of page, including a tumor of a brain (see), tubercular meningitis (see), Konovalov's disease — Wilson (see Gepato-tserebraljnaya dystrophy), a leukodystrophy (see), postvaccinal encephalitis (see), syphilis of a nervous system (see Syphilis), etc.
Treatment. There is no specific treatment. In some cases process is stabilized at treatment by steroid hormones in combination with the hyposensibilizing and resorptional therapy.
Forecast adverse. Bibliography: Gusev E. I. Leykoentse-falit Shildera, in book: Diseases of a nervous system, under the editorship of P. V. Melnichuk, t. 1 „page 277, M., 1982; Markov D. A. and Leonovich A. L. Multiple sclerosis, page 91, M., 1978; With and in e N to about S. N. Multiple sclerosis and diffusion periak-sialny encephalitis, Kiev, 1966; F and Tax Code e l I. I. Diffuzny periaksial-ny encephalitis Shildera, Zhurn. neuropath. and psikhiat., t. 60, No. 9, page 1089, 1960; H and l and with about in And. A. Leucoencephalitis. diffusa scleroticans atypica (Schil-der'a type), Owls. psikhonevrol., No. 6, page 33. 1932; Adams R. D. a. K u b i k C. S. Symposium on multiple sclerosis and demye-linating diseases, morbid anatomy of demye-linative diseases, Amer. J. Med., v. 12, p. 510, 1952; Adams R. D. a. Victor M. Principles of neurology, N. Y. a. o. «1977; Brain W. R. Brain’s clinical neurology, p. 465, Oxford a. o., 1978; Ferrer I. o. Schilder's disease, Child’s Brain, v. 8, p. 294, 1981; Gr i-sold W., Jellinger K. u. V about 1 1-m e r R. Morbus Schilder bei 54 jahringer Frau mit klinischer Remission, Nervenarzt * Bd 53, S. 164, 1982; Handbook of clinical neurology, ed. by P. J. Vinken a. Bruyn. v. 9, p. 469, Amsterdam a. o., 1975; Lhermitte F. e. a. Les formes cavi-taires de la sclerose en plaques et de la maladie de Schilder, Rev. neurol., t. 137, p. 589, 1981; Schilder P. Zur Kenn-tnis der sogenannten diffusen Sklerose, Z „ges. Neurol. Psychiat., Bd 10, S. 1, 1912. L. O. Badalyan; V. A. Morgunov (stalemate. An.).