Cataplexy

Classification according to ICD-10
G47.4	Narcolepsy and cataplexy
ICD-10 online (WHO version 2019)

Cataplexy ( ancient Greek κατά kata "from ... down", ancient Greek πλῆξις plēxis "strike down , blow"). (not to be confused with catalepsy or catatonia ) is the medical term for the short-term loss of muscle tone without clouding of consciousness caused by emotions (joy, laughter, shame, enthusiasm, anger, excitement or fright) .

After the clinical picture of cataplexy in connection with the neuronally caused disturbance of the sleep-wake regulation ( narcolepsy ) had been described by Gélineau in 1880, initially without a precise distinction between the individual symptoms, in 1902 von Löwenfeld used the term cataplexy for episodes of the affective induced loss of muscle tone established. Cataplexy is one of the four symptoms collectively known as narcoleptic tetrad and, in addition to excessive daytime sleepiness, the leading symptom of narcolepsy. Cataplexy can also occur in the Niemann Pick type C disease ; however, only about 20% of those affected suffer from it.

Course of a cataplexy

Triggers are individually different affective stimuli , with laughter, jokes and subtle comments being among the triggers for the majority of those affected. Anger, shock and the feeling of suddenly being the center of attention are at least some of the occasional triggers for a significant proportion of those affected. A certain relaxation seems to be necessary for cataplexy to occur, which makes targeted observation difficult.

In the course of the cataplectic attack builds up within seconds. The EEG shows that brain activity at the beginning of the cataplectic attack resembles wakefulness, but later resembles REM sleep . In REM sleep, too, the muscle tone is switched off as a natural process so that dream contents are not converted into real muscle movements ( sleep paralysis ). Cataplexy is one of the REM-associated symptoms.

Both the entire skeletal muscles and only individual muscle groups can be affected during the attack. The facial muscles are almost always affected, smooth muscles, respiratory and tongue and throat muscles, however, never, so that the important vital functions are not interrupted. Often only the facial muscles (only clumsy speech is possible), the knees (sagging of the body, in almost 70%), the neck (head swings, in almost 60%), and the hands (dropping objects, in almost 50%) ) affected. In severe cases, the person cannot stand on their feet and falls completely.

The duration of the individual attack is a few seconds to a few minutes, it ends suddenly and completely. The cataplectic attack cannot be interrupted from the outside. Cataplexies are often described as bilateral, but are unilateral in around 20%.

Severe fatigue increases the likelihood of it occurring in 75% of those affected. Around half of those affected feel it when an attack is imminent. Series of cataplexies occur in 20% of those affected. Even if the potential risk of accidents and injuries is high, only just under half of those affected have injured so far, but some of them have suffered multiple injuries and 20% have suffered serious injuries, according to the result of a related investigation. Since those affected can hardly actively intervene in what is happening themselves, the severity of the injuries ultimately depends on the situation.

In contrast to syncope and epileptic seizures , consciousness is always retained during cataplexy. However, other symptoms of narcolepsy ( hypnagogic hallucination , sleep attacks) can follow immediately.

Investigation methods

The presence of cataplexy is determined by taking a targeted medical history and using special questionnaires. If there are uncertainties about the differential diagnosis, the determination of the hypocretin level in the CSF and HLA typing may be necessary in individual cases.

In terms of differential diagnosis, it must be differentiated from seizure disorders and somatoform disorders on a neurotic basis. It needs to orthostatic dysregulation , syncope, myoclonus , dissociative seizures, epilepsy, transient ischemic attacks are thought and more.

Atypical phenomena similar to cataplexy have also been described in healthy individuals. In these people and in the presence of other hypersomnic diseases, however, a feeling of weakness or trembling of the knees is most frequently mentioned, but slackening of the facial features and in the jaw area, in contrast to narcoleptics, rarely.

Cataplexy therapy

In the treatment of narcolepsy with cataplexy , the “behavior-modifying measures”, improvement of coping strategies, sleep hygiene and individually adapted daytime sleep episodes, which are cited as non-drug therapy options in the guideline of the German Society for Neurology (DGN) , are mainly aimed at daytime sleepiness. In relation to cataplexies, a coping strategy , i.e. the establishment of life processes according to the specific symptom manifestations, should be seen in avoiding trigger situations for cataplexies, which in many cases cannot be implemented in view of the triggers.

Cataplexy is treated with drugs such as clomipramine , sodium oxybate (gammahydroxybutyrate; trade names for monopreparations: Somsanit , Xyrem ), selective serotonin reuptake inhibitors (SSRIs) or other tricyclic antidepressants .

Depending on the dosage, cataplexy can be reduced by up to 90%, but return to the previous level after the drug is discontinued. With some drugs habituation effects occur. The sudden discontinuation of certain medications can lead to an increase in the frequency and severity of the cataplexy and to periods of cataplexy, the status cataplectic, that last for hours or days .

literature

• Susanne Schäfer: The "sleeping sickness" narcolepsy. An experience report about laughing, paralysis and sleeping in cardboard boxes . Free Spiritual Life Publishing House, Stuttgart 1998, ISBN 978-3-7725-1744-0 .

Web links

• Cataplexy. In: Orphanet (Rare Disease Database).

Individual evidence

1. ↑ J. Gélineau: De la narcolepsie . In: Gazette des hôpitaux , 53, 1880, pp. 626-628
2. ↑ L. Löwenfeld: About narcolepsy . In: Münch. Med. Wochenschr. , 49, 1902, pp. 1041-1045
3. ^ RE Yoss, DD Daly: Criteria for the diagnosis of the narcoleptic syndrome . In: Proc Staff Meetings Mayo Clin , 32, 1957, p. 320
4. ↑ Orphanet; Niemann-Pick disease type C
5. ↑ ^{a b c} Sebastiaan Overeem, Sofie J. van Nues, Wendy L. van der Zande, Claire E. Donjacour, Petra van Mierlo, Gert Jan Lammers: The clinical features of cataplexy: A questionnaire study in narcolepsy patients with and without hypocretin- 1 deficiency . In: Sleep Medicine . Vol. 12, No. 1 , 2011, p. 12–18 , doi : 10.1016 / j.sleep.2010.05.010 , PMID 21145280 (English).
6. ↑ ^{a b c} Mayer, Geert et al: Narcolepsy: Diagnosis and Therapy . In: Deutsches Ärzteblatt , 2001, 98, pp. A249 – A254
7. ↑ ^{a b c} S1 guideline narcolepsy of the German Society for Neurology (DNG). In: AWMF online (as of 2008)
8. ^ Christian Sturzenegger, Claudio L. Bassetti: The clinical spectrum of narcolepsy with cataplexy: a reappraisal . In: Journal of Sleep Research . Vol. 13, No. 4 , 2004, p. 395-406 , doi : 10.1111 / j.1365-2869.2004.00422.x , PMID 15560774 (English).
9. ↑ ^{a b} S3 guideline for non-restful sleep / sleep disorders of the German Society for Sleep Research and Sleep Medicine (DGSM). In: AWMF online (as of 2009)

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