NARCOLEPSY (grech, narke catalepsy, numbness + lepsis attack; synonym: Zhelino's disease, essential narcolepsy) — the disease from group of hypersomnias which is shown day attacks of invincible drowsiness, attacks of full or partial loss of a muscle tone (cataplexy), disturbances of a night dream, changes of the personality inherent to an endocrine psychosyndrome.
The N arises preferential at young age, after 40 years — in isolated cases. Women get sick to more often, and men after 20 years.
A clinical picture
Attacks of invincible drowsiness arise in the afternoon, often suddenly. In some cases they are preceded by weakness, weakness, a headache, can be observed prodromes, reminding aura (see) — paresthesias, feeling of heat, dizziness, pain in epigastric area, bright visual hallucinations. Attacks of drowsiness arise in the conditions of rest, heat, monotonous activity, sometimes at certain one o'clock in the afternoon more often; especially often patients fall asleep during food. Nek-ry patients at the beginning of a disease try to fight against drowsiness, but, feeling that weakness and weakness from it only amplify, soon cease to do it. Attacks are possible in the most various situations, including and life-threatening. Drowsiness is, as a rule, combined with safety of motor acts therefore fallen asleep standing does not fall, going continues to go, the holding subject does not drop it. When approach of drowsiness not so suddenly, patients manage to sit down or lay down. A condition of patients in the period of an attack same, as during a usual dream: muscles relax, sometimes they tell something, make the movements by the head, hands; there can be dreams. The attack usually lasts from one to several minutes, more rare longer, is sometimes estimated several seconds. In day can be from 1 to 100 and more attacks, but 3 — 5 is more often. The attack comes to an end spontaneously, it is easy to wake such patients. With disappearance of drowsiness they test cheerfulness, can continue the interrupted work. Sometimes, being woken, patients for a short time badly comprehend surrounding, are in angry and even in spiteful mood.
The dream at N. superficial, with frequent awakenings, is followed multiple bright, in some cases unpleasant contents and just dreadful, frightening dreams. Patients with clear sleep disorders quite often have gipnagogichesky, visual hallucinations are more often (see). Cataplexies usually arises after attacks of drowsiness appear.
The changes of the personality at N. described by Benedetti (G. Benedetti, 1953), are shown by decrease in mental activity and level of motives, restriction of a focus of interest, fatigue, irritability and sluggishness. Frustration of mood in the form of sub depressions are frequent (see. Depressive syndromes), combined with tearfulness or discontent.
N are inherent various vegetative disturbances with dominance of vagotonic symptoms. Disorders of exchange are shown in the form of obesity. Changes of activity of hemadens meet: most often a thyroid gland, gonads, adrenal glands, a hypophysis (their hypofunction is usually noted). At P. such symptoms as thirst, bulimia, an amenorrhea are possible. A. M. Vane considers characteristic of N. existence though which is unsharply expressed the symptom complex including oculomotor, parkinsonopo-dobny and pyramidal frustration.
In the conditions of elektroeptsefalogra-fichesky inspection in the camera sick N. usually quickly fill up, at the same time the changes corresponding to initial stages of a natural dream, a drowsy state are found, but it is long dragging on, without transition to deeper stages of a dream. Awakening is followed by the same changes on EEG, as well as at healthy people.
Along with N. as separate nozol, a form (essential N.) allocate a narkolegggichesky syndrome (symptomatic N.), to-ry can arise at tumors of a brain, craniocereberal injuries, epidemic encephalitis, endointoxications, diseases of hemadens. Changes of the personality reflect at the same time a basic disease, symptomatic N. is shown generally by attacks of drowsiness.
The etiology and a pathogeny
the Question of N.'s etiology remains unresolved. Most often the cause of illness does not manage to be established. Specify on etiol, value of various factors: inf. diseases (typhus, malaria, measles, etc.), endocrine frustration, genetic predisposition. The somatic and mental disturbances which are found at N. demonstrate defeat of mesodiencephalic educations.
Patofiziol, N.'s basis is, according to I. Pavlov's doctrine, the raised exhaustion of a cerebral cortex with development in it of the braking which is followed by a partial dream. At attacks of drowsiness the braking extending on a cerebral cortex does not occupy cortical department of a motor analyzer; on the contrary, at a cataplexy only this department of a cerebral cortex is surprised.
At symptomatic N. as a result of various patol, processes the same area — the gray matter surrounding caudal departments of the III ventricle and water supply system of a brain is surprised.
Diagnosis establish on the basis a wedge, symptoms and characteristic data of EEG.
Attacks of drowsiness in some cases should be differentiated with small attacks of epilepsy (see), with a pyknolepsy (see), with frustration of a dream of a hysterical origin (see Hysteria).
Treatment has preferential symptomatic character and is directed first of all to elimination of attacks of day drowsiness. Ephedrine and drugs of so-called exciting amines — Phenaminum, Centedrinum are for this purpose used. Tricyclic antidepressants (see), first of all Melipraminum have positive effect. Tricyclic antidepressants exert also positive impact on states, frequent at N., of the lowered mood. For the purpose of its normalization use of amitriptyline is shown (for the night, in connection with clear sedation). Dosages of pharmaceuticals are selected individually, since the minimum doses, especially if treatment is carried out in out-patient conditions. Improvement of a night dream is reached by means of phenobarbital, small doses (3 — 6 — 12 mg) of Tisercinum. Positive takes are yielded by N.'s treatment by the extended dream.
Periodic carrying out courses of fortifying treatment is shown.
The forecast and prevention
the Disease flows usually chronically, for 20 — 30 years and more. In rare instances there can be spontaneous remissions. Frequency of attacks and the arising changes of the personality limit the prof. of a possibility of sick N. and reduce their working capacity in this connection for sick N. there are prof. of restriction. At choice of profession by the patient it is necessary to avoid the uniform, continuous, connected with sitting work. Activity of the patient shall combine a possibility of the movement, switching of attention and short-term breaks for rest.
Forecast symptomatic N. depends on the basic patol, process. At craniocereberal injuries the regreduated current of attacks of drowsiness meets.
See also Cataplexy
Bibliography: Vane A. M. Gipersomniche-sky syndrome (narcolepsy and other forms of pathological drowsiness), M., 1966, bibliogr.; Davidenkov S. N. Clinical lectures on nervous diseases, century 1, L., 1952; Latash L. P. Hypothalamus, adaptive activity and electroencephalogram, M., 1968; The Multivolume guide to neurology, under the editorship of S. N. Davidenkov, t. 6, page 516, L., 1960; Tkachyov R. A. Hereditary form of a narcolepsy, Owls. neuropath., psikhiat, and psychogigabyte., t. 2, century 2, page 60, 1933; it, Features of a current of a narcolepsy, in book: Probl, nevro-patol., under the editorship of S. N. Davidenkov, etc., page 137, M., 1963; Benedetti G. Zur Psychopathologie der Narkolepsie, Mschr. Psychiat. Neurol., Bd 126, S. 135, 1953, Bibliogr.; P or o t A. Manuel alphabeti-que de psychiatrie clinique et therapeutique, p. 398, P., 1969; W i 1 der J. Narkolepsie, Handb, d. Neurol., hrsg. v. O. Bumke u. O. Foerster, Bd 17, S. 87, B., 1935, Bibliogr.
H. G. Shumsky.