In August 2008, Orphanet
published a document compiled by Professors Arnulf, Lecendreux, Franco and Dauvilliers, specialised in neurology and sleep pathologies. This report deals with the characteristics of the Kleine-Levin Syndrome.
It is a very rare neurologic disease (1 or 2 cases out of 1 million) which results mainly in episodes of hypersomnia and loss of the sense of reality. Treatments are quasi-non-existent and not very effective, which makes it difficult to deal with it.
Characteristics of the Kleine-Levin Syndrome
- This document of Orphanet lists the main symptoms of Kleine-Levin Syndrome:
- The patient sleeps between 18 and 24 hours per day,
- Sleep disturbed by intense dreams and hallucinations,
- Cognitive disorders,
- Difficulty of expressing themselves and of concentrating on reading,
- Cannot take definite choice or carry out 2 actions at the same time,
- Temporal and space confusion, partial amnesia,
- Feeling a loss of the sense of reality,
- Feeling of disconnection between the body and the spirit,
- Impression of being "in a dream",
- Behavioral and psychic disorders
- Sexual dishinibition,
- Irritability and apathy,
- Suicidal desire,
- Meningitis symptoms: cephalgia, photophobia, fever, nauseas.
Induced and worsening factors
- Factors of vulnerability:
- Male sex (2/3 of the patients),
- Perinatal problems or of development,
- Patient of Ashkenazi origin.
- Starting factors:
- Season: autumn and winter,
- Influenza and angina,
- Alcohol, unusual stress,
- Infectious agents: Asian flu, chickenpox, streptococcus,
- Cranial trauma,
- Use of marijuana,
- School holidays or respite after a period of examination.
Other forms of the syndrome
- SKL (Kleine-Levin Syndrome) idiopathic, without another symptom before the disease or apart from the crises.
- People suffering from another serious neurological disease.
- Benign forms during which only two to three episodes take place without continuation.
- Serious forms because of their frequency or the duration of the episodes.
Evolution of the disease
- Normal state at the end of the episode.
- The duration of an episode varies from two to 270 days.
- Between the episodes, the periods of respite last an average of 3,5 to 4 months.
- Cures spontaneously in the large majority of the cases.
- Does not concern vital prognosis.
Causes, treatment and advices
Almost unknown factors
- The authors of the document explain that the causes of the syndrome are not studied much:
- No virus was identified,
- Delay in the development of the child,
- Ashkenazi Jewish origin.
Treatment and advices
- The majority of the treatments are disappointing, but some were beneficial for isolated cases:
- Somnolence: amantadine at the beginning of an attack,
- Psychotic symptoms: Risperidone,
- Distress: anxiolytic,
- Treatment with lithium or sodium valproate in case of recurrence.
- Prevent the patient from driving a car,
- If brutal depressive symptoms appear, make sure of the proximity of a family member,
- Avoid drinking alcohol,
- An assessment of sleep and brain scan by a SISCOM process.
The Kleine-Levin syndrome
, Orphanet, August 2008.
Photographic copyright: Elifranssens - Dreamstime.com
Le syndrome de Kleine-Levin
El síndrome de Kleine-Levin (Orphanet)