The Psychiatric Symptomatology

in Kleine-Levin Syndrome
Nahit Motavalli Mukaddes, MD
Behiye Alyanak, MD
Meltem Erocal Kora, MD
Ozgur Polvan, MD
Istanbul University

ABSTRACT: The Kleine-Levin syndrome is a rare disorder with its main symptoms
being periodic hypersomnolence and excessive eating accompanied by behavioral
changes. The dominance of the behavioral and psychological symptoms may obscure
the diagnosis. In this article the diagnostic process and the psychiatric symptomatology
of two adolescent male patients with Kleine-Levin syndrome is discussed.
KEY WORDS: Kleine-Levin; Psychiatric Symptomatology; Hypersomnolence; Eating
Disorders; Hyperphagia.

The Kleine-Levin syndrome is a neuropsychiatric disorder with un-

known etiology. In this quite rare disorder the major symptoms in-
clude periodic hypersomnia and excessive eating.1 The diagnosis is
delayed or spontaneous remission occurs before a diagnosis is made.
Historically, Kleine first described periodical somnolence in a group
of patients in 1925 and then Levin defined episodic hypersomnia and
accompanying intense hunger which lasted for a couple of days to
weeks in 1936.
Although the syndrome was described 70 years ago, due to the low
prevalence rate of the disorder, studies could not go beyond being case
presentations. In these cases the etiologies most commonly implicated
were diencephalic dysfunction, mild depigmentation in locus ceruleus
and substantia nigra, elevated levels of 5HIAA and 5HT in CSF, en-
largement of the third ventricle, and mild diffuse abnormality in EEG
(increased delta wave activity).2-5 Two cases in which the onset of

Received November 4, 1997; For Revision January 15, 1998; Accepted July 9, 1998.
Address correspondence to Dr. Nahit Motavalli Mukaddes, PK 151 Tesvikiye, Istan-
bul, 80212, Turkey.
Child Psychiatry and Human Development, Vol. 29(3), Spring 1999
© 1999 Human Sciences Press, Inc. 253
254 Child Psychiatry and Human Development

symptoms were found to be related to head injury have been reported

as well.3
This disorder is usually seen in males in the second decade of their
lives. Episodic hypersomnia is accompanied by intense hunger and
various psychological symptoms. The syndrome can present with 1 to
12 episodes annually.6 The first episode is usually triggered with an
influenza like disease or a physical stressor. The episodes themselves
and the rich and diversified psychiatric symptomatology make the di-
agnosis difficult. The most frequent psychological symptoms in this
syndrome are irritability, excitation, motor uneasiness, and social,
and sexual disinhibition.6,7 Many authors point to the benignity of the
course of the disorder which means that the episodes' duration and
severity decrease with age and that interepisodic functioning remains
normal.2-9
Two cases which were difficult to diagnose initially due to the domi-
nance of the psychiatric symptomatology will be described.

Case 1
G.A. is a 15-year-old boy attending the eighth grade. He is the second child
of a family with four children. His father is 44 years old, has completed pri-
mary school and is working as a driver. His mother is a 36-year-old housewife;
she is a graduate of primary school. There is nothing significant in the boy's
birth and postnatal history. His psychomotor development was normal. There
are no previous reports of any medical problems.
The patient was brought to the clinic with complaints of school refusal,
refusing to go out of the house, refusing to meet with people, and preferring to
stay isolated. It was learned that he spent his time at home in anxiety, lying
down on his back, and putting his feet up on the wall while lowering his head
down from the bed. In the first psychiatric interview he hardly cooperated; his
affect was flattened and he gave short answers to the questions like "yes" and
"no." Blocks were apparent in his thought content and speech. Two months
prior to the psychiatric referral he was seen by a physician because of high
fever with apathy and sleepiness. He made fun of the physician and spat in
his face. The physician prescribed antiflu medications, and recovered in one
week. 20 days later the patient became sleepy and refused to go to school. He
started painting over the photographs of famous statesmen in his school
books, writing "Jerk" under them. He also refused to see his friends, ate un-
usual foods very rapidly. He ate sour salted apples, cucumbers, and water-
melon rinds.
The symptoms in this case made organic etiology and psychosis two possi-
bilities. Blood measurements, an EEG, and psychometric evaluations were
performed. No pathology was observed in the blood tests. The possible physi-
Nahit Motavalli Mukaddes et al. 255

ological posterior slow wave pattern in the EEG was considered to be in the
normal range. The Rorschach test revealed a schizoid type of a reaction.
While he was being evaluated the symptoms disappeared. In a three month
period he had four episodes of hypersomnolance, abnormal eating attitudes,
social isolation, increase in sexual interests and masturbation, and absurd
behavioral signs which lasted for approximately one week every 20-25 days.
His average daily time for sleep in the episodes were 17 hours. Between the
episodes the patient was exactly like his premorbid condition; there was noth-
ing significant in his psychiatric status, and it was notable to observe that he
could not remember some of his experiences during the episodes. He stated
that he was almost in a dream at the beginning of the episodes. These percep-
tions were interpreted as derealization. The patient was given the diagnosis
of the Kleine-Levin syndrome, and lithium 900 mg/day was started (blood
level: 0.6 mEq/L). In the following eight months of his treatment he and his
family reported he was free of his symptoms. In the symptom free period, his
total WISC-R score was 87. However, he still had two to three day "min-
iepisodes" every month in which he reported mild apathy and a desire for
salted cucumbers and sour apples. For the last four months of his treatment,
these miniepisodes have disappeared and he has returned to his premorbid
condition.

Case 2
MA is a 14-year-old boy, attending the first year of high school. His father
is a 40-year-old teacher. His mother is a 39-year-old housewife. He is their
only child. The patient complained of hypersomnolance, diminished interests
and pleasure, unhappiness, eating too much, and suicidal ideas starting one
year ago. First he had difficulty waking up in the morning and going to
school; he then became sleepy in the school. Whenever he was awakened by
others he had difficulty concentrating; he only wanted to eat with an intense
sense of hunger when he was awake. After a few days he failed to attend
school because of an increasing sleepiness. He said that if he was not awak-
ened he could sleep for 17-18 hours and when awake he felt unhappy. Be-
cause of his diminished sense of well-being and suicidal ideas he has seen a
psychiatrist.
The clinician MA first saw diagnosed depression and prescribed fluvox-
amine. His presenting symptoms subsided in one week only to resume after a
symptom free 20-day period. This second episode lasted for nine days and
waned except for the symptoms of unhappiness and difficulty concentrating.
The one and a half months' trial of fluvoxamine treatment was considered
unsuccessful, and a trial of trazadone for three consecutive months was pre-
scribed with the diagnosis of "atypical depression." However during this pe-
riod the symptoms of hypersomnolance, hyperphagia, megaphagia, and de-
pression persisted. Losing their faith in the treatment methods tried, the
family decided not to give him medications for four or five months, try alter-
native forms of treatment, and withdraw him from school. After this period
they visited our clinic for another trial.
256 Child Psychiatry and Human Development

MA's past history revealed normal psychomotor development, an outgoing,

hard working and a lively personality structure, and nothing significant in his
personal or family relations. His IQ scores were in the normal range. In the
first clinical interview, although the presenting symptoms of depressive mood,
diminished interests and pleasure, hypersomnia, lowered self-esteem and sui-
cidal ideas made depression a possible diagnosis, the episodic nature and the
unresponsiveness to antidepressants brought Kleine-Levin syndrome to mind.
After ruling out any problems with routine blood tests, lithium 900 mg/day
was started (blood level: 0.7 mEq/L). In a 13-month-period with this treat-
ment, the patient has had only four "miniepisodes" which lasted for two days
each. These "miniepisodes" consisted only of mild increase in sleepiness and
diminished interest in activities with no accompanying hyperphagia and sui-
cidal thoughts. It has been 18 months now that he is on the medication and
MA has started going to school again and seems to be totally symptom free.

Discussion

Both of the cases presented are typical examples of the Kleine-

Levin syndrome both in terms of the age for onset and episodic hy-
persomnia and hyperphagia. The sexual disinhibition encountered in
the first patient was absent in the second one. Although Orlosky de-
scribes sexual disinhibition as the second most common symptom in
his series of 33 Kleine-Levin patients, other authors state that some
cases may not exhibit it and that incomplete case presentations are
more frequent than the complete cases.10,11 A review of the available
case presentations in the literature revealed that motor uneasiness,
irritability, bizarre behavioral patterns, and depressive symptoms
can be observed along with social and sexual disinhibition.6 The two
cases we discussed show differences in terms of psychiatric symp-
toms. We have not been able to determine any relation between our
patients' premorbid personality structure and psychiatric morbidity
in the family and the symptoms exhibited during the episodes. Our
treatment strategy was based on previous reports that lithium has
been used with beneficial effects.3,12 Because spontaneous remissions
are reported, consideration of no treatment may be possible in cases
with mild symptoms. However since our patients demonstrated se-
vere impairments in functioning, we could not consider "no treat-
ment." The responses to lithium in both of the cases was attained
with blood levels recommended for major psychiatric affective and
conduct disorders. The residual "miniepisodes" did not cause a deteri-
oration in functioning so the overall response to lithium therapy was
good. The disorder is mainly seen in the adolescent years causing a
Nahit Motavalli Mukaddes et al. 257

period of loss of functioning which is very important in those years.

Even these two reasons verify the need to make extensive research in
understanding the disorder.

Summary

The Kleine-Levin Syndrome with excessive eating and sleepiness as

its major symptoms is a rare disorder of adolescence. Periodicity of
the symptoms, unusual psychiatric features, and the overall rarity of
the syndrome cause problems in both the diagnosis and treatment
processes.
Although the prognosis is fairly good, due to the striking and vary-
ing symptomatology, it is important for the clinicians to be aware of
this distinct entity. Adolescents presenting with hunger and sleep
problems should be carefully evaluated with a detailed history. Mis-
diagnosis and delays in diagnosis can interfere with patient compli-
ance and result in exposure to unnecessary medications. Case results
indicate that lithium is a drug of choice for the treatment of this puz-
zling but rare disorder of adolescence.

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