[MIM 148 840]

Rare: 1 - 2/1.106. Neurological disease that results in episodes of sudden and recurrent hypersomnia associated with

cognitive and behavioral disorders.

More specifically:

-        episodes of hypersomnia: sleep lasting 15-21 hours per day

-        behavioral disorders: binge eating (66%), hyperorality, sexual  (53%) or social disinhibition

-        cognitive problems: dreamlike state, altered perceptions, apathy, confusion;  occasional hallucinations or depressive disorders.

These events are sometimes accompanied by signs of autonomic dysfunction: hypo - or hypertension, bradycardia or tachycardia, irregular breathing.

The patient returns to a normal state between episodes but often has a high BMI.

Different subtypes:

-        primary form (90%): unknown origin; more frequent in males, onset in adolescence (except a reported case at the age of 4 years), often in the course of a banal infection; the episodes last for an average of 10 to 13 days and repeat every 3-4 months. Episodes tend to lengthen with age unless the condition appears before 12 or after 20 years. Sometimes associated with von Willebrand or Klinefelter syndrome, polycystic kidney disease, mental retardation

-        secondary forms (10%): following a genetic disease (Prader-Willi, Asperger), a cerebral vascular disorder, an infection, an autoimmune phenomenon (multiple sclerosis) or a paraneoplastic syndrome; the age of onset is later, episodes of hypersomnia are longer and more frequent.

During an episode:

-        EEG: often slowed down (70%), no epileptic activity.

-        polysomnography: hypersomnia or hypo-alertness

-        CTscan:  thalamo-hypothalamic, frontal and cingulate hypoperfusion,

Treatment: none is effective; lithium can be useful.

Anesthetic implications:

obesity; no cases reported in the literature: increased risk of an attack of hypersomnia after general anesthesia ?

References : 

• Arnulf I, Rico TJ, Mignot E. 
  Diagnosis, disease course, and management of patients with Kleine-Levin syndrome. 
  Lancet-Neurol 2012; 11: 918-28.
• Das S, Gupta R, Dhyani M, Raghuvanshi S. 
  Kleine-Levin syndrome: a case report and review of literature. 
  Pediatr Neurol 2014; 50: 411-6. 
• Ben-Menachem E, Winder MJ. 
  Kleine-Levin syndrome and general anesthesia : a case report.
  J Clin Anesth 2021 ; 75 :110486
  
  

Updated: October 2021