Leukoencephalitis

Leukoencephalitis is a special form of encephalitis is a viral etiology. For leukoencephalitis typical primary defeat of the white matter of the brain. Leukoencephalitis as subacute sclerosing encephalitis (see) occurs mostly in children aged 4-16 years.
Clinical course. The disease develops gradually. Obstipation cerebral symptoms are rare. Picture of the disease develops gradually from developing dementia (dementia), hyperkinesia (involuntary movements) and autonomic disorders. The earliest symptom is mental illness, which is advancing quickly. Already in the early period there seizures epileptiformnye, but more often they appear on the background deployed disease patterns. In 1,5 -2 months. appear involuntary movements, and the hyperkinesia are varied, often tonic spasms of the muscles of the trunk and extremities, leading to a kind of rotational movements. Often, speech disorders type of sensory or motor aphasia (see). Patients are observed violent laughter and crying. Vegetative disorders manifested by the oiliness of the skin, sweating, salivation, changes in vascular reactions. Gradually develops weight loss, often to the extent of cachexia. The disease is progressing. Death occurs after a few months, and in some cases several years from the onset of the disease.
Treatment. Rational treatment of the disease at the present time does not exist. Some effect can be in the treatment prednisolone (under medical supervision). Shown restorative treatment, vitamin therapy, anti-convulsants (phenobarbital 0.01 g on 1 year of life per day), etc. For the sick must be established good care (they need to be fed, wipe the skin, to follow, in order not to have bedsores).

Leukoencephalitis (from the Greek. leukos - white + encephalitis - inflammatory disease of the brain with the defeat of the white matter. For a long time leukoencephalitis opposed encephalitis with isolated lesions of gray matter (polyencephalic). Recently, the border between the two types of encephalitis gradually erased, and now we can only speak of predominance of the inflammatory process in the white and gray matter of the brain.
To leukoencephalitis initially attributed various lesions of the nervous system as inflammatory and degenerative. As the study of nature encephalitis from this polymorphic groups were selected secondary post-infectious encephalitis, observed mainly in acute childhood infections (measles, rubella, and others), vaccination and allergic encephalitis, and system demyelinating diseases, degenerative, type diffuse sclerosis (a disease Shilder, family advancing leukodystrophy and others). Other primary leukoencephalitis to date difficult to recognize because accurate etiological classification. Diagnostics is possible only on the characteristics of the clinic and pathohistology.
Most closely studied group is the primary L., called subacute sclerosing leukoencephalitis the progressing. As an independent nosological units they were first allocated van Bogart (L. van Bogaert) in 1945, it was Found that the number of encephalitis, described earlier under different names, fully qualify for subacute sclerosing L. They are just variations of this form and differ by the presence or absence of intracellular inclusions, duration and intensity demyelinating process.
Etiology subacute sclerosing leukoencephalitis so far not been established. In favor of the viral nature testify intracellular inclusions, however, the virus is unable to allocate. The disease has seasonal and epidemic foci. There is an increase in cases of subacute course of the disease in many countries. Initially it was thought that the disease occurs only in childhood. Recently it is described in adults, it is less common and occurs less typical.
Pathomorphological subacute sclerosing leukoencephalitis characterized demyelination lesions in the white matter of the brain hemispheres and barrel. The process of demyelination in some cases extends diffuse, but often affects conduction system of the brain in a certain sequence. The first to suffer Associazione system of the big hemispheres, kortiko-trio-poligamia ways and pontocerebellar conductor connection. Pyramid system involved in the process at later stages of the disease. The proliferation of fibrous glia accompanying the demyelination, and inflammatory changes vary depending on the duration of the disease. They expressed stronger with more prolonged course. In cases of rapid currents, along with weak development of demyelination in the foreground there is the proliferation of fibrillar glia, which gave grounds to call encephalitis sclerosing. When a fast-flowing forms often detected and intranuclear eosinophilic inclusion of type A.
The clinical picture is extremely stereotypical. The disease occurs in three main stages. One of the earliest symptoms (first stage) are changes of the higher nervous activity, which appear in children, until quite healthy in somatic and mental respects and is not burdened by pathological heredity. Increases rapidly severe degradation of intelligence with the phenomena agnosia, apraxia and speech disorders. Develop mutism and the complete lack of contact with others.
The second stage is accompanied by a kind hyperkinesia. They may have first character choreiform or myoclonic, but very quickly become the type krupnomasshtabnogo of ballista, very stereotypical kind, with a simultaneous reduction of a number of muscles of the proximal limbs, torso and neck. Their strict rhythm (from 4 to 12 in 1 min). Later joins a kind of hyperkinesis eyeballs: violent lead them to the side and up synchronously with hyperkinesia limbs; at the same time there enforced mouth opening and pushing the language matching the rhythm of the rest of motor discharges. These strict rhythmic and complex hyperkinesia give subacute leukoencephalitis originality, allowing to distinguish it from all other subacute encephalitis. Between attacks motor digits at the beginning is not detected or paralysis of the extremities, no violations on the part of the cranial nerves; there is only muscular hypotonia.
The third stage of the disease is characterized by cessation of hyperkinesia and gradual development decerebration rigidity. Appear vegetative disorders and hyperthermic crisis with increasing temperature up to 40 degrees and above. By this time developed pyramidal symptoms in the form of konusov stop and pathological reflexes. In the cerebrospinal fluid are increasing globulin fraction of protein, while the level of gamma globulin in the blood might not improve. The fundus of the eye in all cases properly. Especially characteristic changes in the electrical activity of the brain with periodic high-voltage discharges observed in all derivations synchronously in both hemispheres. These kind of changes EEG can be different from other forms of encephalitis.
The disease progresses steadily; the process continues from a few months to 2 years, in all cases ending in death. The treatment is unsuccessful. Cm. also Encephalitis.