Myoclonus epilepsy

Description:

Myoclonus epilepsy (epilepsy myoclonic) - the hereditary and degenerative disease of a nervous system which is characterized by a combination of myoclonic hyperkinesias to epileptic seizures and the chronic progressing current leading to weak-mindedness and extrapyramidal rigidity.

Symptoms Myoclonus epilepsy:

the abolevaniye meets at girls more often. The first clinical signs usually appear at the advanced school age in the form of generalized convulsive attacks of epileptic character. In several months myoclonic hyperkinesias are found. The return ratio of symptoms is observed much less often. Myoclonias happen asymmetrical, without constancy of localization. At distribution of a myoclonia on muscles - synergists there is a bystry movement of an extremity of various amplitude. Hyperkinesias in any one muscular group, a thicket in proximal department of a hand begin; further they extend to all extremities, muscles of a neck, a trunk, the person. The myoclonia of language conducts to a dysarthtia. The active movements, physical tension, emotions strengthen a myoclonic hyperkinesia. At rest, especially during sleep, it decreases or completely passes. Expressiveness of a myoclonia fluctuates in various days and even within several hours (accrue at bright lighting and in sunny days). At a part of patients the hyperkinesia disturbs food, standing, walking. Sometimes it passes into the myoclonic status.

Epileptic seizures proceed with consciousness disturbance, toniko-clonic spasms and repeat with various frequency. In process of development of a disease, strengthening of a myoclonia epileptic seizures urezhatsya. Gradually symptoms of weak-mindedness accrue, extrapyramidal rigidity against the background of which myoclonias become less expressed develops. The course of a disease long, progressing, sometimes with bystry increase of symptoms (malignant) and with slower and its easy development. The forecast at both types of a current adverse.

Diagnosis of a disease is based on a myoclonia combination to epileptic seizures and detection of myoclonic little bodies (Lafora's little body) in hepatic cells. The diagnosis at detection of low level of a mukopola of saccharites in blood serum is confirmed. The myoclonus epilepsy is differentiated from kozhevnikovsky epilepsy (see a tick-borne Encephalitis) at which the hyperkinesia has constant character, and attacks more often happen focal. Besides, myoclonias at kozhevnikovsky epilepsy are usually observed in paretichny muscles, against the background of their hypotonia, a hypotrophy and an areflexia. At a cerebellar myoclonic dyssynergia of Hunt hyperkinesias and epileptic seizures are combined with an ataxy.

Reasons Myoclonus epilepsy:

It is inherited on autosomal recessively type. It is supposed that primary biochemical defect is connected with disturbance of synthesis of a mukopola of saccharites. Pathoanatomical changes of degenerative character are noted generally in a gear kernel of a cerebellum, cerebellar legs, the lower olives. To a lesser extent the pale sphere, a visual hillock, bark of hemicerebrums are surprised. Changes of a liver are observed.

Treatment Myoclonus epilepsy:

As primary biochemical defect at a myoclonus epilepsy is not established, carry out only symptomatic therapy. Appoint anticonvulsant drugs (phenobarbital, benzonal, clonazepam, sometimes valproic acid drugs - Depakinum, Convulexum). Hyperkinesias decrease a little after reception of Sibazonum (Seduxenum). Also nootropic drugs (Pantogamum, piracetam), glutaminic acid, group B vitamins are shown.