Kozhevnikov syndrome (epilesia partialis continua) CK

The disease was first described by A.Ya. Kozhevnikov in 1895. In the original description, it is noted that epilesia partialis continua is characterized by a combination of frequent, almost constant motor Jackson paroxysms with local myoclonic jerks.

The frequency of occurrence is unknown.
Etiological factors are diverse: the most common cause of SC is tick-borne encephalitis, which is also noted Kozhevnikov; In addition, injuries, tumors, cerebrovascular accidents can also cause SC.

The age of the manifestation – from child to old.
The leading clinical syndrome is one-sided partial motor paroxysms, occurring with intact consciousness and manifested by clonic twitches of the face, one limb or half of the body. Often the attacks are accompanied by a “Jacksonian march.” The duration of paroxysm is 1-2 minutes, the frequency is 1-2 times / day.
Myoclonias are stereotypical, permanent, occur more often in the muscles of the face and in those parts of the limbs where the Jackson march begins.
In the neurological status – more often hemiparesis, due to the underlying disease, motor disorders do not progress with the course of the disease.

There are no mental changes.
The criteria for the diagnosis of Kozhevnikov syndrome are: 1) simple partial motor seizures;
2) focal myoclonias;
3) focal neurological disorders (hemiparesis);
4) non-progressive course.
Treatment. The drugs of choice are konvulex and other valproates and carbamazepines in therapeutic doses. Often there is resistance to anticonvulsants. According to Rasmussen (1991) and Bancaud (1992), surgical correction allows to completely stop epileptic paroxysms.
The forecast is relatively favorable, taking into account the non-progressive course and the absence of changes in mental functions.

local_offerevent_note April 10, 2019

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