KREYTTSFELDTA-YAKOBA DISEASE (H. G. Creutzfeldt, is mute. neurologist and psychiatrist, 1885 — 1964; A. Jakob, is mute. neurologist, 1884 — 1931; synonym: the pseudosclerosis is spastic, a syndrome of a kortiko-striospinalny degeneration) — the progressing dystrophic disease of bark of a great brain, basal gangliyev and a spinal cord. It is for the first time described by Kreyttsfeldt in 1920 Jacob in 1921 noted a combination at this disease of mental disturbances to symptoms of defeat of front horns of a spinal cord, extrapyramidal and pyramidal system and defined a disease as a spastic pseudosclerosis or encephalopathy with the disseminated centers of a degeneration. W. Spielmeyer suggested to call a disease by name the authors who for the first time described it.
In the world literature more than 250 cases are described To. — I., and 200 of them it is confirmed morphologically.
Etiology and pathogeny are studied insufficiently. One authors consider that the leading role is played by genetically determined predisposition to metabolic disturbances, others assign a big part hron, intoxications. The increasing recognition is gained by the virus theory of an origin of a disease. Gibbs (Page J. Gibbs, 1969) et al. for the first time experimentally reproduced the disease similar with To. — I., by introduction to blood of a chimpanzee of substance of the brain received from patients at a biopsy.
The pathological anatomy
makes the Basis of a pathoanatomical picture dystrophy of cortical and subcrustal gray matter, a cortical and spinal way, cells of front horns of a spinal cord. Death of a large number of neurons of bark and a subcortex is noted. Neurons are subject to a chromatolysis, vacuolation of protoplasm, pycnosis of kernels. In a cerebral cortex III and V layers of nervous cells in a motor zone generally are surprised. In subcrustal nodes the striatal system suffers more than pallidarny. Important morfol, a sign is the glial proliferation which is most expressed in sites of the maximum neyronalny dystrophy.
Gistokhim, a research of neurons reveals reduction of the RNA level and lipids and increase in DNA, reduction of activity of oxidizing and lizosomalny enzymes, acetylcholinesterase and monoamine oxidase. Electronic microscopic examination of a brain established change of an astroglia and presynaptic membranes of axons of nervous cells. Most of authors considers the most essential to intravital diagnosis of a disease dystrophic changes and reduction of number of nervous cells, proliferation of a microglia.
The clinical picture
the Disease arises in 30 — 60 years. Men and women are ill equally often. The wedge, symptoms are extremely variable and depend on a stage of a disease. Conditionally distinguish three stages of development of a disease. In the I (prodromal) stage into the forefront changes of mentality act. Patients become lacking initiative, inert; the mood is changeable — from depressive to excited. There is a constant alarm, negativism, nonsense (see), acoustical and visual hallucinations (see), konfabulez (see), memory sharply decreases. Changes in the motive sphere are insignificant and are characterized by difficulty and weakness of movements in extremities, an ataxy.
In the II stage (the developed wedge, a picture) motive disturbances and mental disorders accrue. Deep dementia is combined with generalized paresis of extremities, extrapyramidal disturbances in the form of choreoathetoid hyperkinesia (see), bulbar and pseudobulbar disturbances (see. Bulbar paralysis , Pseudobulbar paralysis ), segmented motive frustration in the form of muscular atrophies, fascicular twitchings.
In the III (terminal) stage there comes deep weak-mindedness (see). Patients fall into prostration, do not react to surrounding, are disoriented in space and time, lose skills of self-service. Diffusion muscular atrophies, a muscular hypertension, hyperkinesias are expressed. Death comes at the phenomena of deep dementia, a cerebrate rigidity, at spasms, and coma.
On EEG already at early stages of a disease are registered periodic two - and three-phase acute waves. Reduction of amplitude of oscillations, single fastsikulyation is defined on EMG.
Disease is progreduated, duration from 2 to 12 years.
Diagnosis the wedge, manifestations and data gistol, researches of a brain is established on the basis.
Differential diagnosis it is necessary to carry out with a side amyotrophic sclerosis (see. Amyotrophic side sclerosis ), a chorea of Gentington (see. Gentington chorea ), Alzheimer's diseases (see. Alzheimer disease ), Peak (see. Peak disease ).
Unlike a side amyotrophic sclerosis, at To. — I. disturbances of mentality and Extrapyramidal motive disturbances prevail. At a chorea of Gentington usually there are no segmented motive frustration, muscular atrophies, fibrillar and fascicular twitchings. From Alzheimer's diseases and Peak To. — Ya.B. distinguishes a combination of the progressing dementia to symptoms of defeat of pyramidal, extrapyramidal system and the segmented motive device.
Bibliography: Bunina T. L., Borodulina V. A. and Chaykovskaya R. P. About a Creutzfeldt-Jakob disease — Jacoba, Zhurn, a neuropath, and psikhiat., t. 72, No. 7, page 984, 1972, bibliogr.; Creutzfeld H. tiber eine eigenartige herdformige Erkrankung des Zentralnervensystems, Z. ges. Neurol. Psychiat., Bd 57, S. 1, 1920; Davison G. R a b i n e r A. M. Spastic pseudosclerosis (disseminated encephalomye-lopathy, corticopallidospinal degeneration), Arch. Neurol. Psychiat. (Chic.), v. 44, p. 578, 1940; Jacob H., Ru of r-k o s with h W. u. S t r u b e H. Die erbliche Form der Creutzfeldt — Jacobschen Krank-heit, Arch. Psychiat. Nervenkr., Bd 184, S. 653, 1950; Jakob A. tiber eigenartige Erkrankungen der Zentralnervensystems mit bemerkenswerten anatomischen Befunde, Z. ges. Neurol. Psychiat., Bd 64, S. 147, 1921.
L. O. Badalyan.