Japanese Society of Sleep Research The 43rd Annual Meeting

International Forum

Abnormal Behaviors during Sleep
日本睡眠学会第 43 回定期学術集会

国際フォーラム「睡眠中の異常行動」（日本睡眠学会 認定更新単位取得プログラム 2単位）

Date&Time: July-14(Sat.), 2018 13:30-17:05
Venue: Small Hall, Sapporo Convention Center
日時：2018 年 7 月 14 日（土）13:30 -17:05
会場：札幌コンベンションセンター 小ホール
Organizer:
Shigeru Chiba

Admission free / 参加無料

PROGRAM
Opening Remarks: 13:30-13:35
Makoto Uchiyama, MD, PhD.

Professor, Department of Psychiatry,
Nihon University School of Medicine, Japan

Introduction: 13:35-13:45"How should we examine a patient withabnormal behaviors during sleep?"
Shigeru Chiba, MD, PhD.

Assistant to the President, Professor and Chairperson,
Department of Psychiatry and Neurology,
Asahikawa Medical University, Japan

Special Lectures 1: 13:45-14:30"Sleep-Related Movement Disordersand Epilepsy: Diagnosis andManagement in the U.S."
Clete A. Kushida, MD, PhD, FAASM.

Professor; Division Chief and Medical Director, Psychiatry /Sleep Medicine, Stanford University, USA
Chair: Makoto Uchiyama, MD, PhD.
Professor, Department of Psychiatry,
Nihon University School of Medicine, Japan

Break: 15:15-15:30(Tea, Coffee, Cookies, etc.)

Special Lectures 3: 15:30-16:15"Sleep-related hypermotor
epilepsy: diagnostic criteria, clinicalmanifestations, and differential
diagnostic considerations"
Birgit Frauscher, MD, PhD.

Associate Professor, Montreal Neurological Institute andHospital McGill University, Canada
Chair: Satsuki Watanabe, MD, PhD.
Assistant Professor, Department of Psychiatry,
Saitama Medical University Hospital, Japan

Special Lectures 4: 16:15-17:00"REM Sleep Related Parasomnias Witha Focus on REM Sleep Behavior
Disorder (RBD) and Parasomnia
Overlap Disorder"
Carlos H. Schenck, MD, PhD.

Professor, Psychiatry,
University of Minnesota Medical School,Minnesota Regional Sleep Disorders Center, USA

Special Lectures 2: 14:30-15:15

Chair: Hiroshi Itoh, MD, PhD.
Senior Advisor, The Jikei University School of Medicine,Japan

Federica Provini, MD, PhD.

Closing Remarks: 17:00-17:05
Hiroshi Itoh, MD, PhD.

"Usual and unusual behaviors duringsleep"
Assistant Professor of Neurology, Department of Biomedicaland Neuromotor Sciences University of Bologna, Italy
Chair: Yoshiyuki Tamura, MD, PhD.
Associate Professor, Department of Psychiatry andNeurology, Asahikawa Medical University, Japan

Senior Advisor, The Jikei University School of Medicine,JapanAbnormal Behaviors during Sleep

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Sleep-Related Movement Disorders
and Epilepsy: Diagnosis andManagement in the U.S.
Clete A. Kushida, MD, PhD, FAASM.
Professor; Division Chief and Medical Director,
Psychiatry / Sleep Medicine, Stanford University, USA

Chair: Makoto Uchiyama, MD, PhD.
Professor, Department of Psychiatry,
Nihon University School of Medicine, Japan

Sleep-related movement disorders will be discussed, with an emphasis on sleep andepilepsy throughout the world. Specifically, Dr. Kushida will discuss nocturnal epilepsy,
periodic limb movements in sleep (PLMS), bruxism, REM sleep behavior disorder (RBD),and rhythmic movement disorder (RMD). For PLMS, the distinguishing characteristics
between this condition and restless legs syndrome (RLS) will be covered. In addition,
the criteria for the diagnosis, scoring, and severity of these disorders will be explored;
the differential diagnosis, associated conditions, complications, and pharmacologic
treatments will be described. The sleep study scoring criteria and examples of bruxism will be demonstrated. For nocturnal epilepsy, RBD, and RMD, the sleep study features, diagnostic criteria, and differential diagnosis will be described.
The learning objectives for this lecture include:• To discuss abnormal behavior during sleep from the perspective of sleep and epilepsy centers in the world• To describe the differentiating characteristics of common abnormal behaviors
during sleep• To recognize the sleep study features of these disorders• To learn the key diagnostic criteria of these disorders

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Usual and unusual behaviors
during sleep
Federica Provini, MD, PhD.
Assistant Professor of Neurology, Department of
Biomedical and Neuromotor Sciences University ofBologna, Italy

Chair: Yoshiyuki Tamura, MD, PhD.
Associate Professor, Department of Psychiatry
and Neurology, Asahikawa Medical University,
Japan

Abnormal behaviors during sleep encompass a broad range of manifestations, including Parasomnias and Nocturnal Epileptic Seizures. Parasomnias are a heterogeneous group of manifestations that accompany sleep. They consist of abnormal behaviors during sleep due to an inappropriate activation of cognitive processes orphysiological systems such as the motor and/or autonomic nervous system. According
to the International Classification of Sleep Disorders, Parasomnias are distinguished on
the basis of the stage of sleep in which they appear: (1) Parasomnias arising from NonRapid Eye Movement (NREM) sleep, which comprise Arousal Disorders and Sleeprelated Eating Disorders; (2) Parasomnias associated with Rapid Eye Movement (REM)
sleep; and (3) “Other Parasomnias” occurring in any sleep stage. Some Parasomnias
constitute unusual but physiological manifestations; others may manifest themselvesin a violent way with tragic consequences. This is especially true for REM Behavior
Disorder (RBD) which is characterized by loss of REM sleep atonia resulting in dreamenacting behavior.
Sleep-related hypermotor epilepsy (SHE) is a rare form of focal epilepsy characterized by stereotyped vigorous hyperkinetic features (complex body movements withkicking or cycling of limbs and rocking body movements), usually with vegetative
signs, vocalization, and emotional facial expression. Asymmetric tonic/dystonic seizures with or without head/eye deviation are also observed. Seizures are abrupt in
onset and offset and typically brief (2 minutes). Seizure frequency may be very high,with occurrence either every night or almost every night, usually many times per night.

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Sleep-related hypermotor epilepsy:
diagnostic criteria, clinicalmanifestations, and differential
diagnostic considerations
Birgit Frauscher, MD, PhD.
Associate Professor, Montreal Neurological
Institute and Hospital McGill University, Canada

Chair: Satsuki Watanabe, MD, PhD.
Assistant Professor, Department of Psychiatry,
Saitama Medical University Hospital, Japan

Sleep-related hypermotor epilepsy (SHE, former: nocturnal frontal lobe epilepsy) isa type of epilepsy which is associated with predominantly sleep-related seizures. Itaffects approximately 10 % of epilepsy patient case series. Approximately 70 % ofcases have a frontal lobe origin, whereas in 30 % of cases the origin is extratemporal
ranging from the insula, the temporal lobe, as well as the parietal lobe. Manifestations
are comprised of hypermotor seizures, asymetric tonic seizures, as well as asymetricdystonic seizures. The diagnosis of SHE is often challenging, as in up to 50 % of cases noclear interictal or ictal EEG changes are recorded, and brain imaging is inconclusive.
One of its most important differential diagnosis are the various forms of NREM andREM parasomnias. In many cases the final diagnosis is purely based on history andictal semiology. Stereotypic events, presence of tonic/dystonic posturing as well as asudden onset and offset of clinical symptoms is suggestive of epileptic as opposed to
non-epileptic sleep-related events. This talk will review the in 2016 revised diagnostic
criteria of SHE (Tinuper P et al., 2016), it will discuss the typical seizure semiology of SHE,
highlight the most important differential diagnosis, and it will finally illustrate its characteristic semiological features using case vignettes and video examples. After this talk,participants will be able to identify the most common semiological characteristics ofSHE and be able to differentiate SHE from parasomnias as major differential diagnosis.

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REM Sleep Related ParasomniasWith a Focus on REM Sleep Behavior
Disorder (RBD) and Parasomnia
Overlap Disorder
Carlos H. Schenck, MD, PhD.
Professor, Psychiatry, University of Minnesota
Medical School,Minnesota Regional Sleep Disorders Center, USA

Chair: Hiroshi Itoh, MD, PhD.
Senior Advisor, The Jikei University School ofMedicine, Japan

RBD is a parasomnia featuring dream-enacting behaviors emerging with loss of thegeneralized skeletal muscle paralysis of REM sleep, viz. “REM-atonia”. Dream-enactment is often aggressive and violent, commonly resulting in injuries to self and bed
partner. Video-polysomnography (vPSG) is required to establish the diagnosis, withthe objective diagnostic hallmark being the loss of REM-atonia. Pharmacotherapy
with bedtime clonazepam and/or melatonin is highly effective in most reported cases. RBD predominantly affects middle-aged and older men--which most likely reflects
a clinical referral bias on account of more aggressive and injurious RBD behaviors inmen compared to women. This is supported by the first epidemiologic study of vPSGconfirmed RBD in the general population that found an equal gender ratio, alongwith a 1.06% prevalence (Habia-Rubio et al. SLEEP 2017). Longitudinal studies of patients with idiopathic RBD (iRBD) have shown that >80% will develop synucleinopathy
neurodegeneration (Parkinson’s disease [PD]; dementia with Lewy bodies [DLB]; multiple system atrophy [MSA]), with the mean interval from RBD onset to overt neurodegeneration being 12-14 years. Thus, iRBD is now considered to be a prodromal featureand early biomarker of synucleinopathy neurodegeneration, which has accelerated
the search for promising disease-modifying/neuroprotective therapies to be tested8in double-blind trials. RBD is found in approximately 50% of PD patients, in 75% of DLBpatients, and in >90% of MSA patients. Many studies have found that the presenceof RBD in PD is a marker of increased global disease severity of PD (compared to PD
without RBD), involving motor and cognitive dysfunction, visual hallucinations, disease
burden, and diminished quality of life. RBD is present in up to 60% of patients withnarcolepsy type 1. RBD has been found in virtually all categories of neurologic disorders. Experimental animal models of RBD have provided insights into the key brainstem nuclei and pathways subserving REM-atonia that are disrupted by the spectrumof neurological disorders causing RBD. The elegant work of the Luppi group in Lyon,
France (published in BRAIN 2017 and NATURE COMMUNICATIONS 2018) has confirmed
the critical roles played by the sublateral dorsal (subceruleus) nucleus in the pons andthe ventromedial inhibitory nucleus in the medulla. Most antidepressants (esp. SSRIs,venlafaxine, TCAs) can trigger or aggravate RBD. The phenotype of RBD in patients<50 years of age differs from the classic RBD phenotype of middle-aged/older men.
Younger RBD patients have greater gender parity, less severe RBD, greater association
with narcolepsy, and greater association with psychiatric disorders and antidepressant use. RBD in children and adolescents, although rare, is usually associated withnarcolepsy type 1, brainstem tumors, and the Parasomnia Overlap Disorder (POD:RBD + NREM parasomnia [sleepwalking, sleep terrors]), first reported by Schenck et
al. in SLEEP (1997) in a series of 33 patients, with a subsequent growing literature. POD
can be idiopathic or symptomatic, with the known associated conditions with PODincluding i) other parasomnias: sexsomnia; sleep related eating disorder; rhythmic
movement disorder ii) neurologic disorders: PD; narcolepsy; multiple sclerosis; brain tumors; traumatic brain injury; anti-IgLON5 syndrome; Machado-Joseph disease; acuterhomboencephalitis [right pontine tegmentum/medulla lesion]; congenital Mobius
syndrome; Harlequin syndrome; etc.; iii) psychiatric disorders (posttraumatic stress disorder/major depression; chronic alcohol and amphetamine abuse and withdrawal;mixed disorders); and iv) miscellaneous and mixed disorders. Differential diagnosisof RBD includes NREM parasomnias, obstructive sleep apnea (“OSA Pseudo-RBD”),
periodic limb movement disorder (“PLM Pseudo-RBD”), and nocturnal seizures. AnInternational RBD Study Group was founded in 2009 and has held yearly research
symposia and published 9 peer-reviewed journal articles. The first textbook on RBD will
be published in the summer of 2018 (Schenck CH, Högl B, Videnovic A, Eds. Springerverlag).

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