LEUKOENCEPHALITIS (leukoencephalitis; Greek leukos white + enkephalos a brain + - itis) — inflammatory and dystrophic defeat of white matter of a brain. L. belong to to demyelinating diseases (see).
For the first time a disease from group L. J. Danson in 1933 described iod the name «subacutely» a form of lethargic encephalitis». In the 4939th Mr. Petya and Dering (H. Pette, G. Doring) reported about encephalitis with hron, the progressing current a little different on a wedge, and patomorfol, to manifestations, having called it a nodular panencephalitis. In 1945 the same disease is described by L. Van Bogaert as «a subacute sclerosing leukoencephalitis». Further Van-Bogart carefully studied clinic and morphology of this hron, the progressing form of encephalitis. With group L. Shilderom described in 1912 (R. of F. Schilder) diffusion periaksialny encephalitis and hemorrhagic L are also similar., about Krom E. W. Hurst in 1941
the Aetiology reported and the pathogeny
Is supposed that L. are diseases of the infectious and allergic nature. The role of myxoviruses, viruses of measles, rage and Herpes zoster as starting factors of giperergichesky autoimmune process is discussed.
Allocate the following kliniko-morfol, forms L.: subacute sclerosing leukoencephalitis of Van-Bogart, periaksialny leukoencephalitis of Shilder, acute hemorrhagic L. At L. demyelinating process is usually combined with defeat of neurons in a varying degree therefore for some of its forms also the term «panencephalitis» is used.
Macroscopic research of a brain at L. reveals expansion of furrows and an atrophy of crinkles. On a cut of hemispheres sites of destruction and demyelination in all departments of a brain are defined various size, it is preferential in white matter, but fascinating various sites of gray matter of bark (fig. 1). In the most struck departments the brain has a spongy consistence, cerebral cavities are moderately expanded.
Gistol, a picture is characterized by diffusion subacute inflammatory reaction with perivascular infiltration by lymphocytes and plasmocytes and focal demyelination (fig. 2). Inflammatory changes are preferential localized in white matter of a brain, sometimes in bark, subcrustal gangliya, a meninx. Normally created myelin (miyelinoklastichesky type of defeat) collapses. Degree of demyelination and destruction of nervous tissue varies in various centers. The separate small centers can merge. At edges of the center of demyelination oligodendrocytes are increased, contain amfofilny inclusions, in more affected areas they completely disappear. Besides, a lot of big meets bizzare a shape of astrocytes hyperchromatic multilobular or several kernels. Axons remain rather safe at early stages of process, later in them there can be dystrophic changes. Neurons of bark of hemicerebrums may contain inclusions of two types: spherical particles to dia. 30 — 40 microns and oblong, or tubular, structures of a little smaller diameter. Inclusions meet with the small duration of a disease more often. Gistokhim, researches find a large amount of protein in inclusions. In most cases find proliferative reaction of a glia. The gliosis can be melkouzelkovy or in the form of the large centers (pseudoneoplasm). Diffusion growth of a fibrous glia leads sometimes to consolidation of marrow so the brain on a section has a cartilaginoid consistence. Walls of arteries and veins are thickened, it is a lot of reticular fibers in an adventitia.
A clinical picture
Psychological disturbances are the earliest display of a disease. In the beginning complaints to increased fatigue, slackness, irritability, instability of mood are noted. Gradually the circle of psychological frustration extends. There is a malignancy, efficiency, greed, selfishness, cruelty, an indiscipline, inertness of thinking. Patients often make unmotivated acts, lose skills of neatness.
Against the background of mental disturbances gradually within several weeks or months the symptomatology progresses focal nevrol: the apraktichesky frustration leading to loss of skills of self-service (see. Apraxia ); Gnostic disturbances (see. Agnosia ); there are disorders of reading, the letter, the account. At some patients visual, auditory hallucinations are observed. Motive disturbances are presented by preferential extrapyramidal frustration in the beginning: rigidity, a phenomenon of «cogwheel» comes to light (see. Trembling paralysis ). Polymorphic hyperkinesias of face muscles, extremities, trunks — a tremor are observed (see. Trembling ), the torsion spasm (see. the Torsion dystonia ), a hemiballism (see. Hyperkinesias ), myoclonias (see). Pyramidal disturbances in typical cases develop at later stages in the form of mono - gemi-or tetraparesis and paralyzes (see. Paralyses, paresis ). Treat often found symptoms of focal defeat static and locomotory ataxy (see) cerebellar or frontal type. Bilateral defeat of cortical and nuclear ways leads to disturbances of phonation, swallowing. Bulbar paralysis develops quite seldom.
A constant symptom of a disease are spasms (see). They can appear at different stages of a disease. Small and abortal convulsive attacks are most characteristic, generalized big attacks are more rare. In a late stage of a disease trophic and vegetative frustration develop: cachexia, decubituses, disturbances of thermal control, profuse sweat etc. In an end-stage patients are immobilized, is sometimes observed cerebrate rigidity (see).
Current of L. can be steadily progressing or remittiruyushchy. In the latter case the wedge, a picture can remind multiple sclerosis (see).
At an electroencephalography register periodic paroxysmal activity with an interval 5 — 15 sec. at the same time in the majority of assignments in the form of slow (1 — 2 in 1 sec.) vysokovoltazhny waves.
In blood the leukocytosis, increase in fraction of gamma-globulin is defined, the antiserum capacity to a clumsy virus or to myxoviruses is usually raised (virus jc, sv-40).
In cerebrospinal liquid the cytosis and increase in protein content in most cases is not observed. However at an electrophoretic research of proteins find out that gamma-globulin makes up to 40 and more percent from total quantity of protein, and the fraction of albumine is lowered. Colloid reactions give the maximum flocculation in the first test tubes (paralytic type of reaction of Lange).
Treatment shall be complex. Hormonal and symptomatic therapy is shown. The positive effect is gained at purpose of corticosteroids. Treatment by glucocorticoids (Prednisolonum) should be begun in an early stage patol, process taking into account a rhythm of hormonal activity of adrenal glands. Hormonal therapy is supplemented antiallergic (Dimedrol, Pipolphenum, Suprastinum, Diazolinum) and anticonvulsant drugs. The drugs reducing a muscle tone (Mydocalmum, Ametinum, Midantanum, Cyclodolum, etc.), vitamins of group B and other symptomatic means are shown. Use of active therapy can detain disease and promote remissions for several years.
At steadily progressing current patients perish in 2 — 12 months after emergence of the first symptoms. At a remittiruyushchy current the disease lasts up to 3 years and more, and remissions can continue from several months to several years during which honor symptoms of a disease or completely are absent.
Features of separate forms of a leukoencephalitis
Subacute sclerosing leukoencephalitis of Van-Bogart. At patomorfol, a research of a brain of patients with this form L., as a rule, intracellular inclusions are found. Extent of defeat decreases in the direction from bark to phylogenetic more ancient educations, but more often than at other forms, the trunk and a spinal cord is surprised.
Wedge, feature of this form L. early manifestation and dominance of extrapyramidal disturbances (A hyperkinetic form) is, to the Crimea only at late stages pyramidal symptoms join. Epileptic seizures are not characteristic.
Periaksialny diffusion leukoencephalitis of Shilder. Patomorfol, feature in comparison with other L. and multiple sclerosis is rather early dystrophy of axons. This form differs from previous in dominance of pyramidal symptoms and frequent epileptic seizures. Big attacks are usually observed. Development of retrobulbar neuritis of optic nerves or the central form of the blindness connected with demyelination of occipital shares is characteristic (see. Shildera disease ).
Acute hemorrhagic leukoencephalitis. On a wedge, and patomorfol, to signs this form L. it is similar to viral and postvaccinal encephalitis. At a pathoanatomical research reveal wet brain, on cuts in substance of a brain — the big centers soft pinkish-sulfur or yellowish coloring with multiple dot hemorrhages. Gistol. the picture is characterized by a fibrinous necrosis of walls of small vessels, generally the venules surrounded with exudate of fibrin, inflammatory cells and ring-shaped hemorrhagic zones. In the same perivascular zones — demyelination with the moderated or expressed destruction of axons. At the earliest stages perivascular infiltrates are presented to hl. obr. neutrophils, however find many lymphocytes in older centers and plasmocytes.
Clinic of acute hemorrhagic L. it is characterized by extremely acute beginning, fulminant increase of weight of symptoms of damage of a brain. Persons of both sexes aged from 20 up to 40 years get sick. Duration of a current is from 2 days to 2 weeks. Developed a wedge, the picture is preceded by the catarral phenomena in a pharynx, fever with a leukocytosis in peripheral blood. In 2 — 4 days there is a headache, muscle tension of a neck, consciousness is broken, the coma sometimes develops. Focal or generalized spasms, motive disturbances in a look gemi-or tetraplegias, pseudobulbar paralysis are characteristic. On an eyeground — hypostasis of a disk (nipple) of an optic nerve. Forms are seldom observed subacute and hron. By means of EEG and arteriography focal changes can be found. In cerebrospinal liquid — the expressed pleocytosis at the expense of polymorphonuclear leukocytes, also lymphocytes meet; protein content is increased to 1 g/l and more; often the xanthochromia of cerebrospinal liquid comes to light, it is microscopically possible to find single erythrocytes.
The outcome is usually lethal.
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L. O. Badalyan.