From Big Medical Encyclopedia

RYULFA INTENTSIONNAYA SPASM (J. Rulf, is mute. doctor of 20 century; lat. intentio extension, tension; synonym: family cortical spasm, functional family cortical spasm) — the paroxysmal hyperkinesia in the form of tonic, is more rare clonic, myotonia of the extremity arising at the sharp movement. The spasm consistently extends to muscles of the same name, sometimes and the opposite side of a body, resembling an attack of jacksonian superficially epilepsies (see).

Tremnerom is for the first time described in 1906 (E. O. of L. Terner). In 1913 and 1922 Ryulf published the detailed analysis of several cases of this hyperkinesia. In domestic literature a number of such patients described E. F. Kulkov, I. D. Sapir et al., S. N. Davidenkov, S. N. Dotsenko, V. N. Klyuchikov et al., V. S. Lobzin, O. D. Bykov, the Aetiology is not found out. Ryulf observed two families, in to-rykh one of parents and several children were sick. He considered an origin of this hyperkinesia descended (on dominant type) a hyperexcitability of a motive zone of a cerebral cortex. Tremner carried this state to neurosises and by analogy with speech stutter called it «stutter of gait». Nek-ry researchers pointed to R.'s communication and. page with neuroinfections.

The analysis allows to assume a wedge, pictures that R. and. the page results from periodic short-term disturbance of dynamic interaction between cortical and subcrustal motive systems.

The disease arises at children's or youthful age and the wedge, is characterized by manifestations stereotypic. At sharp autokinesia, e.g., at a bystry rising or walking, there is a painless spasm (more often tonic) muscles of foot which is usually extending to a shin, a hip, a trunk, a hand, a neck. sometimes person and language. The spasm proceeds several seconds, sometimes comes over also to the opposite side. Consciousness of the patient at the same time is not broken. The spasm can begin also with a hand (e.g., at playing a grand piano) and to repeat from 6 to 30 times a day, and readiness for a repeated spasm comes in 10 — 30 min. Can prevent generalization of a spasm of the patient special receptions — the slow and careful beginning of autokinesia, suspension of any movement, drawing pain stimulations on any point of a body (pinches, blows, pressing) or active muscular tension (a stiskivaniye of teeth, squeezing by a hand of any subject, etc.). On the other hand, nek-ry patients, making the sharp and strong movements by an extremity, can cause an attack.

Out of attacks organic nevrol. symptoms do not come to light. Are in some cases noted moderate decrease muscle tone (see) and rare choreoathetoid twitchings (see. Hyperkinesias ). The course of a disease stationary, sometimes with gradual reduction of frequency and even the termination of attacks. Patients rather easily adapt to the motive defect, out of an attack feel healthy.

Differential diagnosis carry out with jacksonian epilepsy (see), being a symptom of focal damage of a brain, hysterical spasms (see. Hysteria ).

Treatment symptomatic. Appoint the vitamins of group B, pharmaceuticals improving a hmetabolizm in a brain (amine of bosoms, nootropil), microcompasses-tsiyu (trental, Stugeronum. Cavintonum, komplamin), influencing functions of extrapyramidal system (Norakinum, tspklodol, Seduxenum).

Forecast for life favorable. Patients need rational employment with reduction of emotional and motive loading (work on the conveyor is contraindicated).

See also Spasms .

Bibliography: O. D. O bulls of a role of an infection in an etiology of an intentsionny spasm Ryulfa, Voyen. - medical zhurn., No. 2, page 79, 1971; D about c e N to about S. N. About an intentsionny spasm Ryulfa, Zhurn. neuropath. and psikhiat., t. 56, No. 10, page 818, 1956; V. N. and Astafyev G. V Keys. To the clinical characteristic of an intentsionny spasm of Kulkova — Ryulfa, in the same place, t. 57, No. 5, page 624, 1957; Kulkova E. F. Intentsionnaya Ryulf's spasm, Saturday. nauch. works, posvyashch. to the prof. S. N. Davidenkov, page 50, JI., 1941; Lobzin V. S. To the characteristic of an intentsionny spasm of Ryulf, in book: Vopr. psikhiat. and nevropatol., under the editorship of A. G. Panov, etc., page 489, L., 1962; With and p and r I. D., And r x about R. K. and L and in sh and c And. X. Pristupoobrazny choreoathetosis, Zhurn. neuropath, and psikhiat., t. 19, No. 4, page 43, 1950; And about r d and-novb.iyankov I. Also zabolyavaniye on a nervnat system, page 131, Sofia, are rare 1979 a syndrome; R U 1 f J. Der familiare Rindenkrampf, Arch. Psychiat. Nervenkr., Bd 52, S. 748, 1913; it, Funktio-nelle und familiare Rindenkrampf, ibid., Bd 66, S. 687, 1922.

D. K. Bogorodinsky, A. A. Skoromets.