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Introduction: Kleine-Levin syndrome (KLS) is a rare disorder characterized by recurrent periodic hypersomnia, cognitive disturbances, hyperphagia, and hypersexuality. Although many factors have been associated with its occurrence, little is known about treatment. Herein, we present a case of symptoms similar to KLS thought to be related to vascular occlusion disease. Case description: An 81-year-old woman was admitted to the hospital due to recurring episodes of disturbance of consciousness, cognitive disorder, and hyperphagia for 18 years. She was diagnosed with KLS and basilar artery occlusion. Endovascular and antithrombotic therapy was initiated, and her symptoms fully resolved within 2 weeks of treatment initiation. Conclusion: KLS has diverse clinical presentations and demonstrates variable therapeutic responses. Vascular disease or blood flow disorder may be one possible factor for this disease. This case underscores the need for further research into the etiology and pathogenesis of KLS to promote evidence-based approaches for its diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by recurrent episodes of severe hypersomnolence in association with various degrees of cognitive impairment, perceptive abnormalities, apathy, behavioral disturbances. Some of these symptoms, hypersomnolence, compulsive eating and increased sexual drive may be replaced by their opposites or alternate with them. Remarkably enough, these « atypical symptoms » have never been enlighted nor compared in frequency with corresponding typical symptoms. Besides, KLS is more frequent in males than in females but no review has ever compared the frequency of precipitating factors and symptoms in males and females. To uncover these as yet uninvestigated aspects of KLS, a predesigned template was used to extract precipitating factors and symptoms, in 475 case reports of KLS, comprising 364 males and 111 females. Precipitating factors were more frequently recorded in males (67.31 %) than in females (49.55 %). Recurrent episodes of hypersomnolencee were present in 94.32 % of cases, recurrent insomnia in 1.05 % and alternation of hypersomnolence and insomnia in 4.63 %. Cognitive impairment was present in 67.37 % of cases and absent in 6.95 %. Derealization/altered perception was present in 38.32 % of cases and absent in 1.68 %. Severe apathy was present in 44.63 % of cases. Compulsive eating was present in 59.58 % of cases, absent in 13.26 %, replaced by anorexia in 9.05 %, alternation of compulsive eating and anorexia in 5.68 % and alternation of compulsive eating and no compulsive eating in 8.42 %. Increased sexual drive was present in 33.68 % of cases, absent in 22.74 %, replaced by decreased sexual drive in 1.47 %, alternation of increased sexual drive and no increased sexual drive in 2.95 %. Odd behaviors were present in 45.05 % of cases. Psychiatric features were present in 71.58 % of cases, absent in 2.95 %. Finally, the percentages of precipitating factors and of sleep disorder, apathy, sexual disorder, irritability/agressivity, were higher in males than in females. The frequency of the opposites of hypersomnolence, compulsive eating and increased sexual drive appears to be quite significant. In addition, a systematic comparison of precipitating factors and symptoms in males and females has shown limited differences between sexes. • Naming of Kleine-Levin symptoms must be standardized. • A review of KLS symptoms must include presence, absence and absence of data. • Hypersomnolence, hyperphagia, hypersexuality may be replaced by their opposite or alternate with it. • Comparison of precipitating factors and symptoms between sexes has shown minor differences. • Diagnostic criteria of Kleine-Levin syndrome should be updated. [ABSTRACT FROM AUTHOR]

Sleep is a complex phenomenon regulated by circadian rhythms, homeostatic drive, and various other processes, largely mediated by genetic regulation. The study of sleep has enabled in recent years important discoveries in the prevention and management of cardiovascular, metabolic, and neurodegenerative diseases. The characterization of pleiotropic genetic effects that influence sleep and various neurological disorders could improve understanding of the disease mechanisms. Knowledge of the genetics and epigenetics of hypersomnia might direct new studies and trials towards targeted therapies in the field of precision medicine. Evidence of dysregulated miRNAs acting on target genes involved in various forms of hypersomnia and of their modulatory role in some circadian genes indicates their possible utility in the treatment of diseases such as narcolepsy type 1 and idiopathic hypersomnia. Herein we focus on connections between genetics and some central disorders of hypersomnolence – narcolepsy types 1 and 2 (NT1, NT2), idiopathic hypersomnia (IH), and Kleine–Levin syndrome (KLS) – for a better understanding of their etiopathogenetic mechanisms and a better diagnostic and therapeutic definition. Gene pleiotropism influences neurological and sleep disorders such as hypersomnia; therefore, genetics allows us to uncover common pathways to different pathologies, with potential new therapeutic perspectives. An important body of evidence has accumulated on NT1 and IH, allowing a better understanding of etiopathogenesis, disease biomarkers, and possible new therapeutic approaches. Further studies are needed in the field of epigenetics, which has a potential role in the modulation of biological specific hypersomnia pathways. [ABSTRACT FROM AUTHOR]

A woman in her 50 s with Kleine–Levin syndrome (KLS) was referred to our sleep clinic for recurrent episodes of sleep hypersomnia lasting for two to 3 days, despite attempts to remain awake. These episodes were unaccompanied by anxiety or depression, increased appetite, increased sex drive, irritability, or hallucinations, and had first appeared during chemotherapy for malignant lymphoma. Video polysomnography revealed mild obstructive sleep apnea syndrome (apnea–hypopnea index 7.9/h), but no other abnormalities. All blood tests and brain imaging investigations, including brain MRI and A 123I-ioflupane SPECT, yielded normal results. Oral L-carnitine was found to be effective for shortening the period of hypersomnolence, reducing the degree of hypersomnolence, and prolonging the inter-episode period. Since it has been reported that alkylating agents may induce carnitine deficiency, the present observations appear to support the involvement of carnitine in the onset of KLS. [ABSTRACT FROM AUTHOR]

A sleep disorder is characterised by alteration in normal pattern of sleep enough to interfere with the normal functioning of an individual. Sleep disorders are broadly classified as primary sleep disorders such as dyssomnias, hypersomnia and parasomnias and secondary due to medical and mental disorders such as anxiety or substance abuse. We report the case of a 12-year-old boy who presented to us with episodic hypersomnolence, hyperphagia and behavioural abnormalities and was initially managed as epilepsy at another hospital. He was subsequently diagnosed as primary sleep disorder, Kleine–Levin syndrome. We also describe the diagnostic approach to such a case and the crucial role of functional brain imaging in it.

The aim of this review is to give updated information to pediatric neurologists on the correct diagnostic approach and treatment of excessive daytime sleepiness (EDS) in children and adolescents. Due to the change in the society habits, EDS is becoming an emerging problem for the health system. At the present there are few articles specifically devoted to the evaluation of EDS. EDS is often reported in several manuscripts as a side effect of other sleep disorders (obstructive sleep apnea, circadian disorders, etc.) or of the use of drugs or of the substance abuse or as a consequence of bad sleep habits and poor sleep hygiene. EDS, especially in children, may manifest with paradoxical symptoms like hyperactivity, inattention, and impulsiveness. However, common sign of EDS in children are the propensity to sleep longer than usual, the difficulty waking up in the morning, and falling asleep frequently during the day in monotonous situation. The diagnosis should include subjective (sleep diaries, questionnaires) and objective (polysomnography, multiple sleep latency test, etc.) instruments to avoid misdiagnosis. Narcolepsy is the most studied central disorder of hypersomnolence, and it is a predominantly pediatric disease with a peak age of onset in prepuberty but the diagnosis is often delayed especially in mild forms. The early and correct treatment of narcolepsy and of other form of EDS is extremely important since late and inappropriate treatments can affect the psychosocial development of the children and adolescents. • The excessive daytime sleepiness (EDS) is underreported by parents and underdiagnosed by physicians. • EDS in children and adolescents may present paradoxically with inattention and hyperactivity. • In 25%–40% of cases EDS is caused by other sleep disorders. • Narcolepsy is a rare disorder with onset of symptoms (EDS and cataplexy) in childhood or adolescence. • Treatment of EDS and cataplexy includes stimulants, sodium oxybate, pitolisant and antidepressants. [ABSTRACT FROM AUTHOR]

Kleine–Levin syndrome (KLS) is characterized by episodes of hypersomnia. Additionally, these patients can present with hyperphagia, hypersexuality, abnormal behavior, and cognitive dysfunction. Functional neuroimaging studies such as fMRI-BOLD, Positron Emission Tomography (PET) or SPECT help us understand the neuropathological bases of different disorders. We conducted a systematic review to investigate the neuroimaging features of KLS patients and their clinical correlations. This systematic review was conducted by following the Meta-Analysis of Observational Studies in Epidemiology (MOOSE) and PRISMA protocol reporting guidelines. We aim to investigate the clinical correlation with neuroimaging among patients with KLS. We included only studies written in the English language in the last 20 years, conducted on humans; 10 studies were included. We excluded systematic reviews, metanalysis, and case reports. We found that there are changes in functional imaging studies during the symptomatic and asymptomatic periods as well as in between episodes in patients with K.L.S. The areas most reported as affected were the hypothalamic and thalamic regions, which showed hypoperfusion and, in a few cases, hyperperfusion; areas such as the frontal, parietal, occipital and the prefrontal cortex all showed alterations in cerebral perfusion. These changes in cerebral blood flow and regions vary according to the imaging (SPECT, PET SCAN, or fMRI) and the task performed while imaging was performed. We encountered conflicting data between studies. Hyper insomnia, the main feature of this disease during the symptomatic periods, was associated with decreased thalamic activity. Other features of K.L.S., such as apathy, hypersexuality, and depersonalization, were also correlated with functional imaging changes. There were also findings that correlated with working memory deficits seen in this stage during the asymptomatic periods. Hyperactivity of the thalamus and hypothalamus were the main features shown during the asymptomatic period. Additionally, functional imaging tends to improve with a longer course of the disease, which suggests that K.L.S. patients outgrow the disease. These findings should caution physicians when analyzing and correlating neuroimaging findings with the disease.

Kleine-Levin syndrome (KLS) is a rare illness of recurrent episodes of hypersomnia associated with hyperphagia, cognitive dysfunctions and behavioural abnormalities. KLS, a disorder often reported in young males has been described in fewer females. It is rare in patients with age beyond second decade. We report a case of a female in early thirties who presented with recurrent episodes of excessive sleepiness and hyperphagia with cognitive dysfunctions. Her eating and subsequent weight gain was so prominent that she was referred to addiction treatment centre. She was managed with Lithium and Modafinil.

Kleine-Levin syndrome is a rare disease characterized by periodic hypersomnia, cognitive impairment, and behavioral disorders, such as hyperphagia and hypersexuality. It is common among adolescents and males. It has an unknown etiology. Two patients were discussed with the backings of literature in order to increase the awareness of clinicians, considering that there is a limited information regarding the syndrome.

Kleine-Levin syndrome (KLS) is a rare, recurring sleep disorder that easily ignored. Episodic upward-gaze palsy is an uncommon manifestation observed in patients of KLS, which further complicates this disorder. Although peripheral microbial infection have been recognized as most common triggers for KLS, the underlying pathophysiology of this disorder remains unclear. We reported a unique case of KLS elicited by acute encephalitis, which was confirmed by pleocytosis of cerebrospinal fluid at the early stage. The cerebrospinal fluid returned to normal over time while the attacks continued to recur frequently. Episodic upward-gaze palsy was observed during attacks and clinical symptoms were exacerbated following a subsequent COVID-19 infection. This report presents a classic KLS case with distinctive characteristics, which should facilitate more accurate and earlier diagnosis for clinicians. Furthermore, it provides a new perspective for understanding the pathogenesis of this rare disease., Citation: Lv H, Long X, Lv Y, Zhou J. Acute encephalitis induced Kleine-Levin syndrome with episodic vertical gaze dysfunction during hypersomnia episodes. J Clin Sleep Med . 2024;20(9):1555-1556., (© 2024 American Academy of Sleep Medicine.)

Kleine–Levin syndrome (KLS) is a rare central disorder of daytime hypersomnolence and is often characterized by a relapsing and remitting course, recurrent episodes of excessive sleep lasting from 12 to 20 hours a day, and symptoms including hyperphagia, hallucinations, derealization, disorientation, and hypersexuality. There are numerous perioperative considerations in dealing with KLS that include challenges during induction of anesthesia, delayed emergence, postoperative sleep disorders, and delirium. However, due to its rare occurrence, the anesthetic considerations of KLS remain poorly described. This case report outlines the anesthetic considerations and management of a young female patient with KLS who underwent transnasal excision of a trigeminal schwannoma under general anesthesia. [ABSTRACT FROM AUTHOR]

Kleine–Levin syndrome (KLS) is characterized by episodes of hypersomnia. Additionally, these patients can present with hyperphagia, hypersexuality, abnormal behavior, and cognitive dysfunction. Functional neuroimaging studies such as fMRI-BOLD, Positron Emission Tomography (PET) or SPECT help us understand the neuropathological bases of different disorders. We conducted a systematic review to investigate the neuroimaging features of KLS patients and their clinical correlations. This systematic review was conducted by following the Meta-Analysis of Observational Studies in Epidemiology (MOOSE) and PRISMA protocol reporting guidelines. We aim to investigate the clinical correlation with neuroimaging among patients with KLS. We included only studies written in the English language in the last 20 years, conducted on humans; 10 studies were included. We excluded systematic reviews, metanalysis, and case reports. We found that there are changes in functional imaging studies during the symptomatic and asymptomatic periods as well as in between episodes in patients with K.L.S. The areas most reported as affected were the hypothalamic and thalamic regions, which showed hypoperfusion and, in a few cases, hyperperfusion; areas such as the frontal, parietal, occipital and the prefrontal cortex all showed alterations in cerebral perfusion. These changes in cerebral blood flow and regions vary according to the imaging (SPECT, PET SCAN, or fMRI) and the task performed while imaging was performed. We encountered conflicting data between studies. Hyper insomnia, the main feature of this disease during the symptomatic periods, was associated with decreased thalamic activity. Other features of K.L.S., such as apathy, hypersexuality, and depersonalization, were also correlated with functional imaging changes. There were also findings that correlated with working memory deficits seen in this stage during the asymptomatic periods. Hyperactivity of the thalamus and hypothalamus were the main features shown during the asymptomatic period. Additionally, functional imaging tends to improve with a longer course of the disease, which suggests that K.L.S. patients outgrow the disease. These findings should caution physicians when analyzing and correlating neuroimaging findings with the disease. [ABSTRACT FROM AUTHOR]

Introduction: Kleine-Levin syndrome (KLS) is a rare and debilitating disorder presenting with periodic hypersomnolence, cognitive, psychiatric and behavioral disturbances. In the absence of biomarkers it can be difficult to diagnose. Rare LMOD3 variants in a family and in seven sporadic cases with KLS have been described. Here we report a patient and her family with an unclassified, familial, periodic central disorder of hypersomnolence (CDH) in whom the presence of a LMOD3 gene variant was assessed.Case Description: The female patient presented since early adulthood with recurrent episodes of hypersomnolence. Over more than 20 years of follow-up the diagnoses of idiopathic hypersomnia, KLS and hypersomnia associated with a psychiatric condition were made. The family history is positive for periodic hypersomnolence and psychiatric conditions. The patient, her symptomatic mother and her asymptomatic sister carried a Proline for Histidine substitution at codon 552 of the LMOD3-gene. This variant was previously reported in two sporadic KLS patients and its frequency in the general population is below 0.02%.Discussion: We report the association of periodic hypersomnia with a polymorphism of the LMOD3-gene in a patient with atypical KLS and a positive family history. Further research is needed to assess the pathological and predictive value of LMOD3 variants in KLS. [ABSTRACT FROM AUTHOR]

Background: Kleine-Levin syndrome (KLS) is a rare sleep disorder with at least two episodes of hypersomnia coincidence with at least one cognitive, eating, perceptive and disinhibited symptoms and normal inter-episodes. These symptoms are not explained by another sleep, medical, neurological, psychiatric disorders and substance or drug use. Case Presentation: Here we report a young female with personality disorder and obsessive)compulsive disorder who had KLS. Her symptoms appeared in the past 1.5 years ago, while she had an episode of hypersomnia lasting for 5 days. She had 4 attacks; each one lasted up to 2-7 days. We found that overriding KLS symptoms on underlying main psychiatric or personality disorders complicates diagnosis. All neurological examinations during episode and further investigation were in normal range. Conclusion: We suggest that taking a complete history and mental state examination in the episode and inter episode phase helps to diagnosis both KLS and comorbid psychiatric disorders. [ABSTRACT FROM AUTHOR]

Kleine-Levin syndrome is a rare disease characterized by periodic hypersomnia, cognitive impairment, and behavioral disorders, such as hyperphagia and hypersexuality. It is common among adolescents and males. It has an unknown etiology. Two patients were discussed with the backings of literature in order to increase the awareness of clinicians, considering that there is a limited information regarding the syndrome. [ABSTRACT FROM AUTHOR]

Introduction: Kleine-Levin syndrome (KLS) is a rare disorder characterized by recurrent periodic hypersomnia, cognitive disturbances, hyperphagia, and hypersexuality. Although many factors have been associated with its occurrence, little is known about treatment. Herein, we present a case of symptoms similar to KLS thought to be related to vascular occlusion disease., Case Description: An 81-year-old woman was admitted to the hospital due to recurring episodes of disturbance of consciousness, cognitive disorder, and hyperphagia for 18 years. She was diagnosed with KLS and basilar artery occlusion. Endovascular and antithrombotic therapy was initiated, and her symptoms fully resolved within 2 weeks of treatment initiation., Conclusion: KLS has diverse clinical presentations and demonstrates variable therapeutic responses. Vascular disease or blood flow disorder may be one possible factor for this disease. This case underscores the need for further research into the etiology and pathogenesis of KLS to promote evidence-based approaches for its diagnosis and treatment., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Kong and Li.)

Kleine-Levin syndrome (KLS) is a rare disorder characterized by severe episodic hypersomnia, with cognitive impairment accompanied by apathy or disinhibition. Pathophysiology is unknown, although imaging studies indicate decreased activity in hypothalamic/thalamic areas during episodes. Familial occurrence is increased, and risk is associated with reports of a difficult birth. We conducted a worldwide caseâ?'control genome-wide association study in 673 KLS cases collected over 14 y, and ethnically matched 15,341 control individuals. We found a strong genome-wide significant association (rs71947865, Odds Ratio [OR] = 1.48, P = 8.6 Ã-- 10-9) within the 3'region of TRANK1 gene locus, previously associated with bipolar disorder and schizophrenia. Strikingly, KLS cases with rs71947865 variant had significantly increased reports of a difficult birth. As perinatal outcomes have dramatically improved over the last 40 y, we further stratified our sample by birth years and found that recent cases had a significantly reduced rs71947865 association. While the rs71947865 association did not replicate in the entire follow-up sample of 171 KLS cases, rs71947865 was significantly associated with KLS in the subset follow-up sample of 59 KLS cases who reported birth difficulties (OR = 1.54, P = 0.01). Genetic liability of KLS as explained by polygenic risk scores was increased (pseudo R² = 0.15; P < 2.0 Ã-- 10-22 at P = 0.5 threshold) in the follow-up sample. Pathway analysis of genetic associations identified enrichment of circadian regulation pathway genes in KLS cases. Our results suggest links between KLS, circadian regulation, and bipolar disorder, and indicate that the TRANK1 polymorphisms in conjunction with reported birth difficulties may predispose to KLS. [ABSTRACT FROM AUTHOR]

Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by periodic hypersomnia and behavioral or cognitive disturbances. Although prolonged emergence from general anesthesia and postoperative hypersomnia may occur in a patient with KLS, there is little information about the safe anesthetic management of these patients. We describe the case of a 22-year-old female previously diagnosed with KLS who was scheduled to have her third molars extracted under general anesthesia. Because the patient had symptoms of periodic hypersomnia and hyperphagia, the surgery was scheduled during a KLS crisis interval. General anesthesia was induced with propofol, remifentanil, and rocuronium, and maintained with desflurane and remifentanil. To prevent overuse of anesthetic agents, an electroencephalogram (EEG)-based depth of anesthesia monitor (SedLine; Masimo Corporation) was used intraoperatively. A neuromuscular monitor was also used to carefully titrate use of a neuromuscular blocking agent. After surgery, sugammadex was administered, and the patient quickly emerged within 10 minutes, as also confirmed by the EEG monitor. She had no KLS recurrence postoperatively. When anesthetizing patients with KLS, an EEG-based depth of anesthesia monitor and neuromuscular monitor may be warranted to ensure complete emergence from general anesthesia. In addition, elective surgery should be planned during crises intervals. [ABSTRACT FROM AUTHOR]

There are numerous disorders of known or presumed neurologic origin that result in excessive daytime sleepiness, collectively known as the central disorders of hypersomnolence. These include narcolepsy types 1 and 2, idiopathic hypersomnia, Kleine–Levin syndrome, and hypersomnia due to or associated with medical disease, neurologic disease, psychiatric disease, medications or substances, and insufficient sleep durations. This chapter focuses on the treatment of nonnarcoleptic hypersomnia syndromes, from those that are commonly encountered in neurologic practice, such as hypersomnia due to Parkinson's disease, to those that are exceedingly rare but present with dramatic manifestations, such as Kleine–Levin syndrome. The level of evidence for the treatment of sleepiness in these disorders is generally lower than in the well-characterized syndrome of narcolepsy, but available clinical and randomized, controlled trial data can provide guidance for the management of each of these disorders. Treatments vary by diagnosis but may include modafinil/armodafinil, traditional psychostimulants, solriamfetol, pitolisant, clarithromycin, flumazenil, sodium oxybate, melatonin, methylprednisolone, and lithium. [ABSTRACT FROM AUTHOR]

Non-24-hour sleep-wake rhythm disorder (N24SWD) typically presents in patients with visual impairments that disrupt the ability to entrain to the 24 hour solar cycle. We discuss a 43 year old sighted man who presented with periodic daytime hypersomnia and nighttime insomnia, occasionally leading to <3 hours of sleep per day. Previous polysomnography showed an apnea hypopnea index of 6.2 events per hour. A sleep log of 3 months showed irregular time of sleep onset, and an average of 3 hours of sleep per day. Wrist actigraphy confirmed N24SWD. A trial of tasimelteon 20 mg/day resulting in improved daytime hypersomnia (pre-Epworth Sleepiness Scale (ESS) = 21/24, post-ESS = 5/24; a score of > 10/24 is considered sleepy). Follow-up actigraphy showed marked resolution of phase delay with an average of five hours of sleep. The case demonstrates that tasimelteon is a possible treatment for N24SWD in sighted individuals., Competing Interests: Declaration of competing interest The authors received have no financial support or conflicts of interest to report for the paper., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Le syndrome de Kleine-Levin est un trouble neuropsychiatrique appartenant à la catégorie des hypersomnies d'origine centrale. Sa prévalence est estimée à 1 à 5 cas par million d'habitant. Il débute électivement au moment de la puberté (85 % des cas se déclarent au cours de la deuxième décennie). La clinique se caractérise par la survenue d'épisodes d'hypersomnie récurrents d'une durée de quelques jours à plusieurs semaines, associée à des symptômes neurologiques, psychiatriques ou comportementaux. Ce polymorphisme clinique peut être trompeur en présence de symptômes psychiatriques « bruyants » et retarder le diagnostic et le traitement. La cause du syndrome est encore inconnue : des hypothèses auto-immune, inflammatoire ou génétique ont été avancées. La prise en charge consiste à éviter l'alcool, les infections et le manque de sommeil, à utiliser des corticoïdes en crise et du lithium ou du valproate en prévention. Nous présentons le parcours de soins d'un adolescent de 15 ans, qui illustre l'intrication des symptômes neurologiques et psychiatriques et la nécessité de connaître cette sémiologie aux confins de la neuropsychiatrie qui fera éviter des parcours diagnostiques chaotiques. Kleine-Levin syndrome is a rare (1 to 5 cases per million inhabitants) neuropsychiatric disorder belonging to central hypersomnia. The onset frequently occurs around puberty, including 85 % of cases beginning during the second decade. The disorder is characterized by remittent-recurrent episodes (lasting from a few days to several weeks) of hypersomnia associated with neurological, psychiatric and behavioral symptoms. This clinical polymorphism may mimic psychiatric disorders to the point of delaying the diagnosis and treatment. The cause of the syndrome is still poorly understood in spite of autoimmune, inflammatory or genetic hypotheses. The management includes avoiding alcohol intake, infection and sleep deprivation, as well as IV steroids during episodes, and long-term prevention using valproate and lithium therapy. We report the case of a 15-year-old teenager who illustrates how psychiatric and neurological symptoms can be mixed and delay diagnosis. [ABSTRACT FROM AUTHOR]

Bedřich Roth was a Czech neurologist who dedicated his professional life to the study of sleep medicine. Shortly after WW2 he initiated a research program with an exclusive focus on narcolepsy and the hypersomnias. At the young age of 38 years, he published his first book. It was in Czech but had extensive English and Russian summaries. In 1967 he spent a study period in Marseille with Professor Henri Gastaut, who was very impressed by this bright and hardworking young neurologist. Dr. Roth, with his colleague Sona Nevšímalová, was the first to publish on the high frequency of depression in patients with narcolepsy and hypersomnia. He was the first person to define Idiopathic Hypersomnia and, along with Allan Rechtschaffen, to document the high frequency of sleep drunkenness in such patients. His many contributions, and his 65th birthday, were celebrated in 1984. Dr. Roth developed Hodgkin's lymphoma and passed away in November 1989. A conference celebrating his life and career took place in Prague in 2009. In 2015 the annual worldwide Idiopathic Hypersomnia Awareness Week organized by Michelle Chadwick celebrated the 35th anniversary of his 1980 book "Narcolepsy and Hypersomnia". Dr. Roth was the first physician to dedicate his entire career to sleep medicine and his publications predated by more than two decades those of all other authors. Bedřich Roth should therefore be considered to be the true father of sleep medicine. He was known widely for his integrity, humility, honesty, and his perseverance to achieve his goals. [ABSTRACT FROM AUTHOR]

Abstract: Frequently, patients with multiple sclerosis (MS) experience comorbid psychiatric symptoms. Mental disorders primarily occur simultaneously with or after an MS diagnosis; however, the probability of them being the initial manifestation of the disease is rare. We describe the case of a 22-year-old man who had previously been hospitalized because a single psychotic episode alongside symptoms of Kleine-Levin syndrome and a diagnosis of "acute and transient psychotic disorders." Two years later, he was diagnosed with MS. A literature review of the possibility of a psychiatric episode overshadowing an MS diagnosis is then presented. Clinicians should always consider the possibility of an underlying organicity in a case of psychiatric presentation with atypical features, with special attention being given during the investigation process. This approach will lead to the early diagnosis of an organic disease, which can be treated accordingly and as early as possible. [ABSTRACT FROM AUTHOR]

"The Daredevils of Sassoun" is an Armenian national epic poem originating from the eighth century CE. In its "David of Sassoun" branch we found an intriguing description of a strange sleep pattern of Msrah Melik - David's enemy. In one of the main episodes he was described as being in deep sleep for three days while David was attacking his men. Melik's guards tell David that he still needs to complete his 7-day-long sleep. The pattern duration, periodic occurrence and some additional signs described in the poem's text clearly resemble Kleine-Levin syndrome. To the best of our knowledge, this could be the first description of Kleine-Levin syndrome. [ABSTRACT FROM AUTHOR]

Advances in neuroimaging open up the possibility for new powerful tools to be developed that potentially can be applied to clinical populations to improve the diagnosis of neurological disorders, including sleep disorders. At present, the diagnosis of narcolepsy and primary hypersomnias is largely limited to subjective assessments and objective measurements of behavior and sleep physiology. In this review, we focus on recent neuroimaging findings that provide insight into the neural basis of narcolepsy and the primary hypersomnias Kleine-Levin syndrome and idiopathic hypersomnia. We describe the role of neuroimaging in confirming previous genetic, neurochemical, and neurophysiological findings and highlight studies that permit a greater understanding of the symptoms of these sleep disorders. We conclude by considering some of the remaining challenges to overcome, the existing knowledge gaps, and the potential role for neuroimaging in understanding the pathogenesis and clinical features of narcolepsy and primary hypersomnias. [ABSTRACT FROM AUTHOR]