104

Case report

l&r Psychiatry 1996;11:104-105 0 Elsevier, Paris

Kleine-Levin syndrome misdiagnosed as schizophrenia

F Bonnet, F Thibaut *, D Levillain, M Petit

University Department on Schizophrenia Research, Psychiatric Hospital, 4 rue Paul-Eluard, F-76301 Sotteville-Les-Rouen, France

(Received 5 July 1995; accepted 12 July 1995)

Summary - A 27 year-old man initially considered as a schizophrenic patient was later diagnosed as having Kleine-Levin syndrome.

Kleine-Levin syndrome / schizophrenia

INTRODUCTION

Kleine-Levin syndrome is characterized by recur- rent attacks of hypersomnia associated to mega- phagia and abnormal sexual behavior. This disor- der usually occurs in adolescent males. In most cases psychiatric disturbances are present and in some cases they constitute the prominent features which may lead to false diagnosis. Kleine described the first case in 1925. Later, Critchley extensively reviewed the literature and added per- sonal cases in his 1962 monograph which stands as the classic description of this disorder.

We shall report one case of Kleine-Levin syn- drome and show how the conjunction of psychiat- ric symptoms with the characteristic triad led to misdiagnosis which had dramatic consequences as regards therapeutics.

CASE REPORT

h4r P, without any personal psychiatric previous history and whose only previous familial history is an aunt with cyclothymic disorder, presented a first hypersomnia attack when he was 20 years old. It began, suddenly and quickly, after a tonsillectomy; it lasted 8 days and was

not associated with other symptoms, except for exces- sive irritability. Then, the patient went back to a normal scholarship and did not present any specific trouble until the age of 23. This time, he was travelling in a foreign country when, a few hours after having seen a car acci- dent, he presented a hypersomnia attack which sponta- neously ended after 17 days. Hypersomnia was the only known symptom for these two attacks, during which the patient slept an average of 20 hours a day. At age 26, he happened to be at the place where his father drowned 11 years before, he then presented visual hallucinations, a disturbed sexual behavior and mild hypersomnia. Psy- chiatric symptoms (irritability, autistic withdrawal) which were prominent justified the patient’s hospitaliza- tion in a psychiatric ward and turned the diagnosis to schizophrenia.

This patient was given a neuroleptic treatment (halo- peridol, 15 mgld) and had to gradually cease all his pro- fessional occupations during the following months, because of an increasing apragmatism. Considering the observed symptomatology, interpreted as a deficient development of his schizophrenia, the standard neuro- leptic treatment was stopped after several months and replaced by a combination of amisulpride (200 mg/d) and carbamazepine (800 mg/d).

Four months later, in spite of the combination of the

l Correspondence and reprints.

Kleine-Levin syndrome misdiagnosed as schizophrenia 105

neuroleptic treatment with carbamazepine, the patient suffered from a new hypersomnia attack with, as observed in the latter episode, megaphagia and sexual behavior disorders. It spontaneously ended after twenty days. Then the patient, still considered as a schizophrenic patient, moved away from the region when a new attack recurred which led him to hospitalization in our ward.

This typical symptomatology, associating the ele- ments of the characteristic triad that were already observed in the latter attacks, the recurrent evolution of the disease, and the lack of symptoms between the cri- ses, led us to a Kleine-Levin’s syndrome diagnosis. Routine blood tests and magnetic resonance imaging were normal, HLA haplotype dismissed a Gelineau dis- ease. Polysomnographic recording at the beginning of the attack showed a reduction of stages III and IV to 12% (normal range 25%), a decrease in REM sleep latency to 58 minutes (normal range 80 to 120 min), and a REM sleep increased percentage to 26% (normal range 20%). All these data were previously reported in Kleine- Levin syndrome albeit they are not specific (Reynolds et al, 1980). After the introduction of modalinil (a stimu- lant of alpha 1 noradrenergic in central synapse) (200 mgid) at the third day, a clinical efficiency was observed for one day, whereas the polysomnographic recording showed a lack of sleep for 30 hours. Symp- tomatology returned to typical Kleine-Levin’s features for the 15 following days, in spite of continuation of the modafinil treatment at 300 mg per day, whereas poly- somnographic study showed, during the night, a deep slow sleep stage recovery and a better sleep quality.

Globally, our patient presented five attacks between age 20 and age 27. Since Kleine-Levin syndrome was diagnosed, and after the cessation of the neuroleptic treatment, Mr P took up his studies again in order to improve his accountant training.

DISCUSSION

Kleine-Levin syndrome’s typical characteristics are recurrent attacks of hypersomnia, combined with megaphagia and sexual behavior disorders. Megaphagia is of a compulsif type: the patient cannot stop eating, may steal food from other patients or can eat anything he finds (Billard and Cadilhac, 1993). Sexual behavior troubles observed in 27% of the patients are masturbation in public, sexual harassment on unknown people or even rape attempts. However, psychiatric symp- toms can, quite frequently, be associated to this triad (Lemire, 1993). These symptoms can turn the diagnosis to another conclusion, especially if they are of the first rank. Irritability and aggressiveness may be present in about 60% of the cases. Delir- ium (30% of the cases) and hallucinations (of vis-

ual type in general) are also frequently described. Moreover, some symptoms such as autistic with- drawal, disorganized speech, hallucinations (15%), flat or inappropriate affects, stereotyped move- ments and thoughts can be related to schizophre- nia. Diagnosis can be corrected if hypersomnia, which is always present but sometimes as a second rank symptom, and the return to a normal behavior between crises are taken into account. Thus, even in presence of psychiatric symptoms, hypersomnia is a key symptom in the disease and diagnosing a Kleine-Levin syndrome in these atypical cases may prevent the patient from a heavy treatment, which, in our case, had dramatic consequences on his professional occupations.

We tried modafinil at a dose of 200 increased to 300 mg/day. We observed only a relative effi- ciency: the patient’s state improved only for one day (at the third day) and the polysomnographic study showed a lack of sleep for 30 hours. Contin- uation of this treatment for the following days did not lead to further improvement; furthermore, at the end of the fourth day of treatment, the patient went back to hypersomnia which spontaneously ended after 15 days.

As for preventive treatment, we can add our patient’s case to the two found in the literature: a patient had been treated with carbamazepine and did not relapse for 18 months (Savet et al, 1986), and another one received carbarnazepine (600 mg/d) for 18 months with no apparent benefit (Sagar et al, 1990). In our case, the treatment was given for more than seven months for psychiatric reasons, but did not prevent hypemomnia recurrence.

ACKNOWLEDGMENT

The authors thank Prof D Samson-Dollfus (University of Rouen) for the data concerning electroencephalo- graphic investigations.

REFERENCES

Billard M, Cadilhac .I. Les hypersomnies r&xrrentes. Rev Neu- t-01 1988;144:249-58

Critchley M. Periodic hypersomnia and megaphagia in adoles- cent males. Bruin 1962;85:627-56

Lemire I. Revue du syndrome de Kleine-Levin: vers une approche integree. Rev Can Psychiatr 1993;38:277-89

Reynolds CF, Black RS, Coble P, Holzer B, Kupper DJ. Simi- larities in EEG sleep findings for Kleine-Levin syndrome and unipolar depression. Am J Psychiatr 1980;137: 116-8

Sagar RS, Khandelwal SK, Gupta S. Interepisodic morbidity in Kleine-Levin syndrome. Br J Psychiarr 1990;157: 139-l]

Savet A, Robert S, Angeli. Un cas de syndrome de Kleine- Levin stabilise depuis plus d’un an sous carbamaztpine. Presse MM 1986;15:1281