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Kleine-Levin syndrome
Epidemiology:
1) rare
2) onset is typically around adolescence to the late teens
3) 4 times more common in males than in females
Pathology:
1) basal frontal lobe dysfunction
2) temporal lobe epilepsy
3) septal nucleus injury
4) hypothalamus areas regulating sleep & appetite may be involved
Clinical manifestations:
1) recurring periods of excessive drowsiness & sleep (up to 20 hours per day)
2) symptoms may last for days to weeks
3) excessive food intake
4) irritability
5) disorientation
6) lack of energy
7) phonophobia
8) hallucinations (some)
9) hypersexuality
10) affected persons are normal between episodes
11) depression & amnesia may occur after an attack (temporary)
12) may be weeks or more before symptoms reappear
Differential diagnosis:
- cyclic re-occurrence of sleepiness during the premenstrual period in teenaged girls
Management:
1) no definitive treatment
2) stimulants, amphetamines, methylphenidate & modafinil may be useful to treat sleepiness
3) lithium or carbamazepine may be useful for mood disorder
4) responses to treatment have often been limited
5) prognosis
a) appears to be benign
b) does not impact on intellect or physical function
c) symptoms usually improve or disappear with age
General
syndrome
References
- NINDS Kleine-Levin Syndrome Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Kleine-Levin-Syndrome-Information-Page