CITATION: Lachman, A. 2014. Kleine-Levin syndrome as a neuropsychiatric presentation: A case report and review. South African Journal of Psychiatry, 20(2):60-62, doi:10.7196/SAJP.494.The original publication is available at http://www.sajp.org.zaKleine-Levin syndrome (KLS) is a rare disorder characterised by hypersomnia, hyperphagia and behavioural disturbances. It typically
occurs in adolescent men and may mimic a variety of neuropsychiatric conditions. Here we report what appears to be the first published
case of KLS in South Africa and trace the clinical course, investigations and diagnostic difficulties encountered in the management of this
condition. An overview of the literature guiding the diagnosis, differentials and treatment is discussed.http://www.sajp.org.za/index.php/sajp/article/view/494Publisher's versio

Lachman, Anusha

South African Journal of Psychiatry

Anusha Lachman

Crossref

60    SAJP  -  July 2014  Vol. 20  No. 2 
CASE REPORT
Kleine-Levin syndrome (KLS) is considered an excep-
tionally rare,[1] recurrent condition characterised by 
hyper somnia, hyperphagia and behavioural disturbances 
including hypersexuality, depression, irritability and 
derealisation.[1,2] It occurs typically in adolescent men, 
who are often misdiagnosed with depression, bipolar disorder, psy-
chosis or seizure disorders. Patients are asymptomatic between 
attacks and return to baseline, with normal or nonspecific results 
of investigations. The disorder is at the interface of neurology 
and psychiatry, and no objective biochemical, neuropathological 
or structural imaging alterations have been identified. This paper 
describes a case of typical KLS, the first to be documented in South 
Africa (SA), and illustrates diagnostic difficulties in its presentation. 
Case report
Over the course of 2 years, patient A presented to a tertiary 
hospital with six episodes of excessive sleepiness (hypersomnia) 
accompanied by blurred vision and headaches. These episodes were 
followed by changes in appetite, irritability of mood and periods of 
hyper sexuality. His first presentation to hospital was in 2011, but on 
further enquiry the family reported similar episodes beginning in 
2008 (at the age of 10 years), where he would complain of headaches 
and blurred vision, followed by deep sleep, sometimes in excess of 
15 hours, before returning to his normal level of activity.
In 2011, at the age of 14 years, patient A was admitted to a psy-
chiatric ward following an episode of aggression, hypersexuality 
and running away from home. A week before his admission he 
reported not sleeping well and being stressed while preparing for 
examinations. He was observed to be disorientated and fluctuating 
in his mental state, with periods of poor arousability. Investigations 
for delirium included two normal electroencephelograms (EEGs), a 
normal computed tomography (CT) scan of the brain, and a negative 
toxicology screen for illicit substances. He was empirically treated 
with sodium valproate for a seizure disorder and his aggression and 
hypersexuality improved with an atypical antipsychotic (risperidone).
After discharge, he continued to function well at school but was 
re admitted with a second episode of aggression, hypersomnia, 
headaches and confusion in January 2012. This time he was treated 
for a postictal psychosis, which settled on an increased dose of 
risperidone. All investigations at this stage were normal. A week 
following discharge, he was readmitted with a relapse. This episode 
settled quickly with no adjustments in medication.
In July 2012, he presented again to a psychiatric ward following 
headaches, irritable mood, paranoia and blurred vision during 
his mid-year examinations at school. During this admission, 
haematological investigations and cerebrospinal fluid (CSF) 
examinations were all normal. An ophthalmological investigation was 
also normal. While in the ward, he developed swelling of the joints 
with restricted range of movement in both knee joints. After a review 
by a rheumatologist, a diagnosis of juvenile oligoarthritis was made, 
and he started receiving treatment with salazopyrine. 
His combined diagnoses on discharge included a psychotic disorder 
due to a general medical condition, complex partial seizures and 
oligoarthritis. Outpatient appointments were regularly attended and 
the complaints varied from ongoing blurred vision to intermittent 
joint pain. His social and academic functioning remained intact. 
He had one episode of hypersomnia over a weekend, followed by a 
voracious appetite, but this resolved and he returned to school with 
no apparent cognitive fallouts. 
On 1 January 2013, he began complaining of anxiety, with blurred 
vision, and became very distressed and clingy with his mother. An 
episode of sleepiness followed, which lasted 20 hours, together with 
a change in his appetite, after which he was brought to hospital. He 
reported feeling confused and sleepy, and presented as perplexed 
with an irritable mood during the interview. He was compliant 
with his medication regimen, which included sodium valproate, 
risperidone and salazopyrine. He settled quickly in the ward. All 
investigations, including a urine toxicology, were unremarkable. 
He was reviewed by the rheumatologist, an ophthalmologist and a 
neurologist and discharged with an appointment for a sleep EEG 
Kleine-Levin syndrome as a neuropsychiatric presentation: 
A case report and review
A Lachman, MB ChB, FCPsych (SA), MMED (Psych), MPhil (Child Psych), Cert Child Psych (SA)
Tygerberg Hospital, Cape Town; Department of Psychiatry, Stellenbosch University, Tygerberg, Cape Town, South Africa
Corresponding author: A Lachman (anusha@sun.ac.za)
Kleine-Levin syndrome (KLS) is a rare disorder characterised by hypersomnia, hyperphagia and behavioural disturbances. It typically 
occurs in adolescent men and may mimic a variety of neuropsychiatric conditions. Here we report what appears to be the first published 
case of KLS in South Africa and trace the clinical course, investigations and diagnostic difficulties encountered in the management of this 
condition. An overview of the literature guiding the diagnosis, differentials and treatment is discussed.
S Afr J Psych 2014;20(2):60-62. DOI:10.7196/SAJP.494
 July 2014  Vol. 20  No. 2     -  SAJP    61
CASE REPORT
as an outpatient. An additional diagnosis 
of bipolar disorder not-otherwise-specified 
was entertained despite the lack of clear 
mood symptoms and no family history of 
mood or psychiatric disorders.
A month post discharge, he presented once 
again with an acute onset of hypersomnia 
(lasting 2 days) followed by emotional 
outbursts, including irritability, clingy beha-
viour, aggression and sexual disinhibition. He 
was fully compliant on medication, denied 
any substance abuse, and all investigations 
(including inflammatory markers) were nor-
mal. Subsequent investigations included 
a single photon emission CT (SPECT) of the 
brain (Fig. 1), which showed reduced perfusion 
to the right fron tal, paracingulate posterior 
parietal and right parieto-occipital cortices. 
Perfusion was also reduced bilaterally to the 
temporal poles. A neurology consultation was 
requested and, following expert opinion, KLS 
was diagnosed. 
He was discharged on only salazopyrine 
and risperidone at a low dose. However, his 
return to school was plagued by episodes 
of anxiety, poor concentration and memory 
impairment. A month later he was readmitted 
with hypersomnia, poor arousability and 
confusion. All investigations, including mag -
netic resonance imaging (MRI) of the brain, 
were normal. He was reviewed by rheuma-
tology and medical genetics departments, 
who found examination unremarkable. 
Methylphenidate was initiated as a short 
course to help with the sleepiness. Despite 
resolution of the sleepiness, he remained 
irritable, intrusive, anxious and agg ressive. 
There were no perceptual disturbances, but 
clear impairments in memory and attention. 
Guided by anecdotal reports and cases 
documented in the literature,[2,3] he was given 
a trial of lithium carbonate after which his 
mood symptoms began to settle. A repeat 
SPECT of the brain showed no change to 
the areas of hypoperfusion. He is currently 
receiving individual educational facilitation 
to bridge him into mainstream schooling 
after a year of interrupted educational input.
Discussion
KLS diagnosis is difficult because the condition 
is seen infrequently and is often complicated 
by the presence of neurological and psychiatric 
symptoms. [4] The International Classification 
of Sleep Disorders (1990)[5] modified the 
diagnostic criteria for KLS and defined it as 
a syndrome composed of recurring episodes 
of undue sleepiness lasting for some days, 
which may or may not be associated with 
hyperphagia and abnormal behaviour. 
Onset of the condition occurs typically in 
adolescence (81%) although younger onset 
of the first episode has been reported. [2] 
Aet iology and pathogenesis of KLS is un -
known, but intermittent dysfunction in the 
hypothalamus is likely.[6] Inflammatory pro-
cesses,[6] neurotransmitter (serotonin and dopa-
mine) metabolism abnormalities[7] and 
viral infections[2] have all been suggested 
as possible contributors to KLS. Frequently, 
neurological conditions such as encephalitis, 
narcolepsy and circadian rhythm disorders 
may be considered in cases of KLS.[6,7]
Recorded precipitating factors for episodes 
include: upper respiratory tract infections, 
streptococcal infections, gastroenteritis, viral 
infections (chicken pox, Epstein Barr virus), 
substance use (cannabis and alcohol), head 
trauma and sleep deprivation or stress.[8] Our 
patient reported no stressors other than exam 
pressure and had a single episode of sleep 
deprivation preceding an attack. In addition, 
his only possible identifiable precipitant was 
considered to be the juvenile oligoarthritis. 
Clinically, as demonstrated by this case, KLS 
may often be misdiagnosed and treated as 
bipolar disorder or a seizure disorder, despite 
there being an absence of evidence to support 
either diagnosis. Because of the cyclical 
nature of the presentation and disturbances 
in sleep and inhibitions, several authors have 
attempted to find a relationship between 
KLS and bipolar disorder.[6,8] The range 
of disturbed behaviours reported is wide, 
encompassing features suggestive of mild 
clouding of consciousness (disorientation), 
mood symptoms (irritability, dysphoria, im -
pulsiveness, depression), overarousal/rest-
lessness, disinhibition or psychotic features. [1,9] 
The striking presentation with behavioural 
disturbances warranting repeated invest-
igations perhaps overshadowed the diagnosis 
in our patient, and the clue which lay in the 
symptom of repeated hypersomnia. 
KLS is a diagnosis of exclusion, with symp-
toms that may mimic many more commonly 
occurring conditions that need to be explored 
first. In a systematic review of 186 case studies 
of KLS, Arnulf et al.[8] reported that most 
medical tests in KLS patients were aimed 
at eliminating epilepsy (EEG), focal brain 
lesions (brain imaging) and encephalitis (CSF 
analysis) as potential causes, and even when 
done during the acute episodes, results were 
unremarkable. Radiological investigations 
are usually normal, with routine MRI and 
EEG also being non-specific.[1] The most 
frequent reporting of abnormalities appear 
to be recorded on SPECT imaging[2]  with 
reported perfusion deficits in the right or 
left thalamus, hypothalamus, basal ganglia, 
mesial temporal lobes and frontal lobe. [2,10] In 
our case, a SPECT done on patient A during 
an episode showed reduced perfusion to the 
right frontal, paracingular, posterior parietal, 
right parieto-occipital cortices and bilateral 
temporal poles. The interepisodic SPECT, 
however, showed no significant change to the 
pattern of perfusion deficits. 
Evidence suggests that the course of 
KLS is often benign, with spontaneous 
improvement over several years although 
interepisodic morbidity especially resulting 
from mood disorders, and mild but long-
lasting memory disturbances have been 
documented.[1,9]  Episodes are less frequent 
towards the end of the disease course, and 
patients are considered cured only if they do 
not experience an episode for ≥6 years.[1,2] A 
high frequency of episodes in the beginning 
Fig. 1. SPECT brain Tc99m (March 2013): A = reduced right frontal and paracingular cortex; B = reduced 
posterior parietal and right parieto-occipital cortex; C = reduced perfusion bilateral temporal poles.
62    SAJP  -   July 2014  Vol. 20  No. 2 
(as in patient A) is thought to be associated with a shorter duration 
of disease.[2]
Patients wish to alleviate the symptoms because of the crippling 
nature of the acute phase and the fact that episodes can stretch for 
weeks or months at a time, impairing their quality of life. Owing to 
the rarity of the disease, consistent reports or guidelines on treatment 
are sparse. There are no randomised, placebo-controlled trials of 
pharmacological treatment for KLS.[3] Drug trials of stimulants such 
as methylphenidate and amphetamine[2,8]  have been suggested for 
symptomatic treatment during episodes of hypersomnia. Where 
psychotic symptoms are present, risperidone has been reported as 
most beneficial.[2,3] Preventative strategies have been suggested for 
use in individuals who experience ≥4 episodes a year.[2,3] Lithium has 
been the most frequently used treatment and reported as significantly 
beneficial in improving abnormal behaviour and reducing the 
duration of episodes while decreasing relapses.[3,11] 
Patient A is currently largely asymptomatic on a therapeutic dose of 
lithium and risperidone, and only has complaints of minor memory 
difficulties at school. His last episode was 3 months prior to the 
initiation of lithium.
 
Conclusion
This report appears to be the first published case in SA of an 
adolescent with KLS presenting with neuropsychiatric symptoms 
to an acute psychiatric service. KLS is difficult to diagnose at 
first presentation because more serious neurological disorders need 
to be excluded. However, in adolescent males who present with 
periodic hypersomnia and psychiatric or behavioural disturbances 
with recurrent presentations yielding normal investigations, a 
diagnosis of KLS should be considered. The clinical manifestations 
as highlighted in this case emphasise the need for neurologists 
and psychiatrists to collaborate, especially in the management of 
diagnostically difficult adolescents. 
Consent. Written consent was obtained from the adolescent and his 
parents for publication, and approved by the Human Research and 
Ethics Committee of Stellenbosch University, ethics reference number 
C13/07/002.
Acknowledgements. The author wishes to thank Profs Jonathan 
Carr of the Department of Neurology and James Warwick of the 
Department of Nuclear Medicine, Tygerberg Hospital, Stellenbosch 
University and Dr Eddy Lee-Pan of the Department of Neurology, 
University of Cape Town, for their assistance in managing the case 
and drafting this manuscript.
References
1. Ramdurg S. Kleine-Levin syndrome: Etiology, diagnosis and treatment. Ann Indian Acad 
Neurol 2010;13(4):241-246. [http://dx.doi.org/10.4103/0972-2327.74185]
2. Arnulf I, Floo TJ, Mignot E. Diagnosis, disease course and management of patients with 
Kleine-Levin Syndrome. Lancet Neurol 2012:11(10):918-928. [http://dx.doi.org/10.1016/
S1474-4422(12)70187-4]
3. Oliveira MM, Conti C, Saconato H, Prado GF. Pharmacological treatment for Kleine-
Levin syndrome. Cochrane Database Sys Rev 2013;8. [http://dx.doi.org/10.1002/14651858.
CD006685.pub3]
4. Bonnet F, Thibaut F, Levillain D, Petit M. Kleine-Levin syndrome misdiagnosed as 
schizophrenia. Eur Psychiatry 1996;11(2):104-105. [http://dx.doi.org/10.1016/0924-
9338(96)84788-2]
5. Thorpy MJ, Chairman, Diagnostic Classification Steering Committee. International 
Classification of Sleep Disorders: Diagnostic and Coding Manual. American Sleep Disorders 
Association, 1990.
6. Papacostas SS, Hadjivasilis V. Kleine-Levin syndrome: Report of a case and review 
of the literature. Eur Psychiatry 2000:15(4):231-235. [http://dx.doi.org/10.1016/S0924-
9338(00)00239-X]
7. Cawthorn P. A disorder unique to adolescence? The Kleine Levin syndrome. J Adolesc 
1990;13(4):401-406. [http://dx.doi.org/10.1016/0140-1971(90)90032-3]
8. Arnulf I, Zeitzer JM, File J, et al. Kleine-Levin syndrome: A systematic review of 186 cases in the 
literature. Brain 2005;128(12):2763-2776. [http://dx.doi.org/10.1093/brain/awh620]
9. Pike M, Stores G. Kleine-Levin syndrome: A cause of diagnostic confusion. Arch Dis Child 
1994;71(4):355-357. [http://dx.doi.org/10.1136/adc.71.4.355]
10. Hong SB, Joo EY, Tae WS. Episodic diencephalic hypoperfusion in Kleine-Levin syndrome. 
Sleep 2006;29(8):1091-1093.
11. Muratori F, Bertini N, Masi G. Efficacy of lithium treatment in Kleine-Levin syndrome. Eur 
Psychiatry 2002;17(4):232-233. [http://dx.doi.org/10.1016/S0924-9338(02)00661-2]
CASE REPORT


English

Kleine-Levin syndrome as a neuropsychiatric presentation: A case report and review

Stellenbosch University SUNScholar Repository

60    SAJP  -  July 2014  Vol. 20  No. 2 CASE REPORTKleine-Levin syndrome (KLS) is considered an excep-tionally rare,[1] recurrent condition characterised by hyper somnia, hyperphagia and behavioural disturbances including hypersexuality, depression, irritability and derealisation.[1,2] It occurs typically in adolescent men, who are often misdiagnosed with depression, bipolar disorder, psy-chosis or seizure disorders. Patients are asymptomatic between attacks and return to baseline, with normal or nonspecific results of investigations. The disorder is at the interface of neurology and psychiatry, and no objective biochemical, neuropathological or structural imaging alterations have been identified. This paper describes a case of typical KLS, the first to be documented in South Africa (SA), and illustrates diagnostic difficulties in its presentation. Case reportOver the course of 2 years, patient A presented to a tertiary hospital with six episodes of excessive sleepiness (hypersomnia) accompanied by blurred vision and headaches. These episodes were followed by changes in appetite, irritability of mood and periods of hyper sexuality. His first presentation to hospital was in 2011, but on further enquiry the family reported similar episodes beginning in 2008 (at the age of 10 years), where he would complain of headaches and blurred vision, followed by deep sleep, sometimes in excess of 15 hours, before returning to his normal level of activity.In 2011, at the age of 14 years, patient A was admitted to a psy-chiatric ward following an episode of aggression, hypersexuality and running away from home. A week before his admission he reported not sleeping well and being stressed while preparing for examinations. He was observed to be disorientated and fluctuating in his mental state, with periods of poor arousability. Investigations for delirium included two normal electroencephelograms (EEGs), a normal computed tomography (CT) scan of the brain, and a negative toxicology screen for illicit substances. He was empirically treated with sodium valproate for a seizure disorder and his aggression and hypersexuality improved with an atypical antipsychotic (risperidone).After discharge, he continued to function well at school but was re admitted with a second episode of aggression, hypersomnia, headaches and confusion in January 2012. This time he was treated for a postictal psychosis, which settled on an increased dose of risperidone. All investigations at this stage were normal. A week following discharge, he was readmitted with a relapse. This episode settled quickly with no adjustments in medication.In July 2012, he presented again to a psychiatric ward following headaches, irritable mood, paranoia and blurred vision during his mid-year examinations at school. During this admission, haematological investigations and cerebrospinal fluid (CSF) examinations were all normal. An ophthalmological investigation was also normal. While in the ward, he developed swelling of the joints with restricted range of movement in both knee joints. After a review by a rheumatologist, a diagnosis of juvenile oligoarthritis was made, and he started receiving treatment with salazopyrine. His combined diagnoses on discharge included a psychotic disorder due to a general medical condition, complex partial seizures and oligoarthritis. Outpatient appointments were regularly attended and the complaints varied from ongoing blurred vision to intermittent joint pain. His social and academic functioning remained intact. He had one episode of hypersomnia over a weekend, followed by a voracious appetite, but this resolved and he returned to school with no apparent cognitive fallouts. On 1 January 2013, he began complaining of anxiety, with blurred vision, and became very distressed and clingy with his mother. An episode of sleepiness followed, which lasted 20 hours, together with a change in his appetite, after which he was brought to hospital. He reported feeling confused and sleepy, and presented as perplexed with an irritable mood during the interview. He was compliant with his medication regimen, which included sodium valproate, risperidone and salazopyrine. He settled quickly in the ward. All investigations, including a urine toxicology, were unremarkable. He was reviewed by the rheumatologist, an ophthalmologist and a neurologist and discharged with an appointment for a sleep EEG Kleine-Levin syndrome as a neuropsychiatric presentation: A case report and reviewA Lachman, MB ChB, FCPsych (SA), MMED (Psych), MPhil (Child Psych), Cert Child Psych (SA)Tygerberg Hospital, Cape Town; Department of Psychiatry, Stellenbosch University, Tygerberg, Cape Town, South AfricaCorresponding author: A Lachman (anusha@sun.ac.za)Kleine-Levin syndrome (KLS) is a rare disorder characterised by hypersomnia, hyperphagia and behavioural disturbances. It typically occurs in adolescent men and may mimic a variety of neuropsychiatric conditions. Here we report what appears to be the first published case of KLS in South Africa and trace the clinical course, investigations and diagnostic difficulties encountered in the management of this condition. An overview of the literature guiding the diagnosis, differentials and treatment is discussed.S Afr J Psych 2014;20(2):60-62. DOI:10.7196/SAJP.494 July 2014  Vol. 20  No. 2     -  SAJP    61CASE REPORTas an outpatient. An additional diagnosis of bipolar disorder not-otherwise-specified was entertained despite the lack of clear mood symptoms and no family history of mood or psychiatric disorders.A month post discharge, he presented once again with an acute onset of hypersomnia (lasting 2 days) followed by emotional outbursts, including irritability, clingy beha-viour, aggression and sexual disinhibition. He was fully compliant on medication, denied any substance abuse, and all investigations (including inflammatory markers) were nor-mal. Subsequent investigations included a single photon emission CT (SPECT) of the brain (Fig. 1), which showed reduced perfusion to the right fron tal, paracingulate posterior parietal and right parieto-occipital cortices. Perfusion was also reduced bilaterally to the temporal poles. A neurology consultation was requested and, following expert opinion, KLS was diagnosed. He was discharged on only salazopyrine and risperidone at a low dose. However, his return to school was plagued by episodes of anxiety, poor concentration and memory impairment. A month later he was readmitted with hypersomnia, poor arousability and confusion. All investigations, including mag -netic resonance imaging (MRI) of the brain, were normal. He was reviewed by rheuma-tology and medical genetics departments, who found examination unremarkable. Methylphenidate was initiated as a short course to help with the sleepiness. Despite resolution of the sleepiness, he remained irritable, intrusive, anxious and agg ressive. There were no perceptual disturbances, but clear impairments in memory and attention. Guided by anecdotal reports and cases documented in the literature,[2,3] he was given a trial of lithium carbonate after which his mood symptoms began to settle. A repeat SPECT of the brain showed no change to the areas of hypoperfusion. He is currently receiving individual educational facilitation to bridge him into mainstream schooling after a year of interrupted educational input.DiscussionKLS diagnosis is difficult because the condition is seen infrequently and is often complicated by the presence of neurological and psychiatric symptoms. [4] The International Classification of Sleep Disorders (1990)[5] modified the diagnostic criteria for KLS and defined it as a syndrome composed of recurring episodes of undue sleepiness lasting for some days, which may or may not be associated with hyperphagia and abnormal behaviour. Onset of the condition occurs typically in adolescence (81%) although younger onset of the first episode has been reported. [2] Aet iology and pathogenesis of KLS is un -known, but intermittent dysfunction in the hypothalamus is likely.[6] Inflammatory pro-cesses,[6] neurotransmitter (serotonin and dopa-mine) metabolism abnormalities[7] and viral infections[2] have all been suggested as possible contributors to KLS. Frequently, neurological conditions such as encephalitis, narcolepsy and circadian rhythm disorders may be considered in cases of KLS.[6,7]Recorded precipitating factors for episodes include: upper respiratory tract infections, streptococcal infections, gastroenteritis, viral infections (chicken pox, Epstein Barr virus), substance use (cannabis and alcohol), head trauma and sleep deprivation or stress.[8] Our patient reported no stressors other than exam pressure and had a single episode of sleep deprivation preceding an attack. In addition, his only possible identifiable precipitant was considered to be the juvenile oligoarthritis. Clinically, as demonstrated by this case, KLS may often be misdiagnosed and treated as bipolar disorder or a seizure disorder, despite there being an absence of evidence to support either diagnosis. Because of the cyclical nature of the presentation and disturbances in sleep and inhibitions, several authors have attempted to find a relationship between KLS and bipolar disorder.[6,8] The range of disturbed behaviours reported is wide, encompassing features suggestive of mild clouding of consciousness (disorientation), mood symptoms (irritability, dysphoria, im -pulsiveness, depression), overarousal/rest-lessness, disinhibition or psychotic features. [1,9] The striking presentation with behavioural disturbances warranting repeated invest-igations perhaps overshadowed the diagnosis in our patient, and the clue which lay in the symptom of repeated hypersomnia. KLS is a diagnosis of exclusion, with symp-toms that may mimic many more commonly occurring conditions that need to be explored first. In a systematic review of 186 case studies of KLS, Arnulf et al.[8] reported that most medical tests in KLS patients were aimed at eliminating epilepsy (EEG), focal brain lesions (brain imaging) and encephalitis (CSF analysis) as potential causes, and even when done during the acute episodes, results were unremarkable. Radiological investigations are usually normal, with routine MRI and EEG also being non-specific.[1] The most frequent reporting of abnormalities appear to be recorded on SPECT imaging[2]  with reported perfusion deficits in the right or left thalamus, hypothalamus, basal ganglia, mesial temporal lobes and frontal lobe. [2,10] In our case, a SPECT done on patient A during an episode showed reduced perfusion to the right frontal, paracingular, posterior parietal, right parieto-occipital cortices and bilateral temporal poles. The interepisodic SPECT, however, showed no significant change to the pattern of perfusion deficits. Evidence suggests that the course of KLS is often benign, with spontaneous improvement over several years although interepisodic morbidity especially resulting from mood disorders, and mild but long-lasting memory disturbances have been documented.[1,9]  Episodes are less frequent towards the end of the disease course, and patients are considered cured only if they do not experience an episode for ≥6 years.[1,2] A high frequency of episodes in the beginning Fig. 1. SPECT brain Tc99m (March 2013): A = reduced right frontal and paracingular cortex; B = reduced posterior parietal and right parieto-occipital cortex; C = reduced perfusion bilateral temporal poles.62    SAJP  -   July 2014  Vol. 20  No. 2 (as in patient A) is thought to be associated with a shorter duration of disease.[2]Patients wish to alleviate the symptoms because of the crippling nature of the acute phase and the fact that episodes can stretch for weeks or months at a time, impairing their quality of life. Owing to the rarity of the disease, consistent reports or guidelines on treatment are sparse. There are no randomised, placebo-controlled trials of pharmacological treatment for KLS.[3] Drug trials of stimulants such as methylphenidate and amphetamine[2,8]  have been suggested for symptomatic treatment during episodes of hypersomnia. Where psychotic symptoms are present, risperidone has been reported as most beneficial.[2,3] Preventative strategies have been suggested for use in individuals who experience ≥4 episodes a year.[2,3] Lithium has been the most frequently used treatment and reported as significantly beneficial in improving abnormal behaviour and reducing the duration of episodes while decreasing relapses.[3,11] Patient A is currently largely asymptomatic on a therapeutic dose of lithium and risperidone, and only has complaints of minor memory difficulties at school. His last episode was 3 months prior to the initiation of lithium. ConclusionThis report appears to be the first published case in SA of an adolescent with KLS presenting with neuropsychiatric symptoms to an acute psychiatric service. KLS is difficult to diagnose at first presentation because more serious neurological disorders need to be excluded. However, in adolescent males who present with periodic hypersomnia and psychiatric or behavioural disturbances with recurrent presentations yielding normal investigations, a diagnosis of KLS should be considered. The clinical manifestations as highlighted in this case emphasise the need for neurologists and psychiatrists to collaborate, especially in the management of diagnostically difficult adolescents. Consent. Written consent was obtained from the adolescent and his parents for publication, and approved by the Human Research and Ethics Committee of Stellenbosch University, ethics reference number C13/07/002.Acknowledgements. The author wishes to thank Profs Jonathan Carr of the Department of Neurology and James Warwick of the Department of Nuclear Medicine, Tygerberg Hospital, Stellenbosch University and Dr Eddy Lee-Pan of the Department of Neurology, University of Cape Town, for their assistance in managing the case and drafting this manuscript.References1. Ramdurg S. Kleine-Levin syndrome: Etiology, diagnosis and treatment. Ann Indian Acad Neurol 2010;13(4):241-246. [http://dx.doi.org/10.4103/0972-2327.74185]2. Arnulf I, Floo TJ, Mignot E. Diagnosis, disease course and management of patients with Kleine-Levin Syndrome. Lancet Neurol 2012:11(10):918-928. [http://dx.doi.org/10.1016/S1474-4422(12)70187-4]3. Oliveira MM, Conti C, Saconato H, Prado GF. Pharmacological treatment for Kleine-Levin syndrome. Cochrane Database Sys Rev 2013;8. [http://dx.doi.org/10.1002/14651858.CD006685.pub3]4. Bonnet F, Thibaut F, Levillain D, Petit M. Kleine-Levin syndrome misdiagnosed as schizophrenia. Eur Psychiatry 1996;11(2):104-105. [http://dx.doi.org/10.1016/0924-9338(96)84788-2]5. Thorpy MJ, Chairman, Diagnostic Classification Steering Committee. International Classification of Sleep Disorders: Diagnostic and Coding Manual. American Sleep Disorders Association, 1990.6. Papacostas SS, Hadjivasilis V. Kleine-Levin syndrome: Report of a case and review of the literature. Eur Psychiatry 2000:15(4):231-235. [http://dx.doi.org/10.1016/S0924-9338(00)00239-X]7. Cawthorn P. A disorder unique to adolescence? The Kleine Levin syndrome. J Adolesc 1990;13(4):401-406. [http://dx.doi.org/10.1016/0140-1971(90)90032-3]8. Arnulf I, Zeitzer JM, File J, et al. Kleine-Levin syndrome: A systematic review of 186 cases in the literature. Brain 2005;128(12):2763-2776. [http://dx.doi.org/10.1093/brain/awh620]9. Pike M, Stores G. Kleine-Levin syndrome: A cause of diagnostic confusion. Arch Dis Child 1994;71(4):355-357. [http://dx.doi.org/10.1136/adc.71.4.355]10. Hong SB, Joo EY, Tae WS. Episodic diencephalic hypoperfusion in Kleine-Levin syndrome. Sleep 2006;29(8):1091-1093.11. Muratori F, Bertini N, Masi G. Efficacy of lithium treatment in Kleine-Levin syndrome. Eur Psychiatry 2002;17(4):232-233. [http://dx.doi.org/10.1016/S0924-9338(02)00661-2]CASE REPORT

Kleine-Levin syndrome as a neuropsychiatric presentation : a case report and review

Kleine-Levin syndrome (KLS) is a rare disorder characterised by hypersomnia, hyperphagia and behavioural disturbances. It typically occurs in adolescent men and may mimic a variety of neuropsychiatric conditions. Here we report what appears to be the first published case of KLS in South Africa and trace the clinical course, investigations and diagnostic difficulties encountered in the management of this condition. An overview of the literature guiding the diagnosis, differentials and treatment is discussed

Kleine-Levin syndrome as a neuropsychiatric presentation : a case report and review

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