SPECT findings in the Kleine-Levin syndrome.
OBJECTIVE
The Kleine-Levin Syndrome, is a rare disorder with onset during teenage years, but little is known on etiopathogenesis. Seven subjects with Kleine-Levin Syndrome accumulated over time had systematic SPECT studies during (n=5) and out (n=7) of the symptomatic period.


METHODS
Seven boys with symptom onset between 11 and 17 years of age and at least 2 episodes per year were followed for a mean of 6 years.


METHODS
Electroencephalogram awake-asleep, computed tomography scan, and magnetic resonance imaging studies were performed before Tc-99m ECD single photon emission tomography (SPECT) obtained during day 4 or 5 (n=5) and at least 1 month away from the symptomatic period (n=7).


RESULTS
All imaging tests except SPECT were normal. Hypoperfusion of both thalami were seen during the symptomatic period that completely disappeared during the asymptomatic period. Hypoperfusion in other regions were also noted in some, but not all subjects. They persisted during the asymptomatic period in 2 cases over the temporal lobe (2/7 cases), frontal lobe (1/7 cases), and basal ganglia (1/7 cases). The largest amount of persistent hypoperfusion was seen in the subject with longest clinical evolution.


CONCLUSIONS
Hypoperfusion of the thalamus is a consistent finding during the symptomatic period, but perfusion abnormalities may persist even during the asymptomatic period. The longer the duration of the syndrome, the more extended the hypoperfusion regions during the asymptomatic period.
Huang
Yu-Shu
YS
Department of Psychiatry, Chang Gung Memorial University Hospital, Taipei, Taiwan.
Guilleminault
Christian
C
Kao
Pan-Fu
PF
Liu
Feng-Yuan
FY
D000293:Adolescent; D001921:Brain / Q000098:blood supply* / Q000000981:diagnostic imaging / Q000473:pathology; D002560:Cerebrovascular Circulation / Q000502:physiology; D002648:Child; D006970:Disorders of Excessive Somnolence / Q000175:diagnosis / Q000453:epidemiology; D004569:Electroencephalography; D006801:Humans; D017593:Kleine-Levin Syndrome / Q000175:diagnosis* / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D010507:Periodicity; D013702:Temporal Lobe / Q000098:blood supply / Q000000981:diagnostic imaging / Q000473:pathology; D013788:Thalamus / Q000098:blood supply / Q000000981:diagnostic imaging / Q000473:pathology; D015899:Tomography, Emission-Computed, Single-Photon; D014057:Tomography, X-Ray Computed; D014851:Wakefulness / Q000502:physiology