Neurological causes of excessive daytime sleepiness. Professor Adam Zeman Royal Devon and Exeter Hospital University of Exeter Medical School

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1 Neurological causes of excessive daytime sleepiness Professor Adam Zeman Royal Devon and Exeter Hospital University of Exeter Medical School

2 Excessive daytime sleepiness Physiological sleep deprivation, poor sleep hygiene Pathological Sleep apnoea Narcolepsy and idiopathic hypersomnolence Recurrent hypersomnias Circadian rhythm sleep disorders Sleep related movement disorders Medication/drug-related hypersomnia General medical disorders Psychiatric disorders Neurological disorders

3 Sleep deprivation Impaired performance and productivity Impaired attention, executive functioning, learning and memory Impaired mood and relationships Accidents eg 30% fatal crashes in NYC Metabolic syndrome immunity mortality

4 Neurological EDS Narcolepsy Idiopathic hypersomnia Kleine-Levin syndrome Circadian rhythm disorders

5 Neurological EDS Narcolepsy Idiopathic hypersomnia Kleine-Levin syndrome Circadian rhythm disorders

6 Daytime sleepiness: Epworth Sleepiness Scale Sitting and reading Watching TV Sitting inactive in a public place eg theatre, meeting Passenger in a car for an hour Lying down to rest in the afternoon Sitting and talking to someone Sitting quietly after lunch In a car while stopped in traffic 0 = would never dose 1 = slight chance of dosing 2 = moderate chance 3 = high chance

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8 Narcolepsy Excessive daytime sleepiness Cataplexy sleep paralysis hypnagogic hallucinations Poor nocturnal sleep Automatic behaviour Circa 4/10,000

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10 Narcolepsy: investigations Polysomnography/MSLT Mean sleep latency <8 minutes 2 sleep-onset REM periods

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15 Narolepsy: investigations Polysomnography/MSLT Mean sleep latency <8 minutes 2 sleep-onset REM periods CSF hypocretin <100 pg/ml diagnostic of narcolepsy

16 Narcolepsy sleepiness Modafinil Dexamfetamine cataplexy Antidepressants Both Xyrem

17 Narcolepsy audit Joanne Veale n = female 17 teen onset 9 20s 3 30s 1 50s 6 always sleepy 1 family history of narcolepsy

18 Excessive sleepiness All sleepy Mean ESS19/24 34/36 nap 31/34 find naps refreshing

19 Cataplexy 30/36 cataplexy 24/30 laughter most common trigger Also anger, anxiety, stress 26/36 hypnagogic hallucinations 23/36 sleep paralysis 28/36 restless sleep 10/36 automatic behaviour

20 Cataplexy 30/36 cataplexy 24/30 laughter most common trigger Also anger, anxiety, stress 26/36 hypnagogic hallucinations 23/36 sleep paralysis 28/36 restless sleep 10/36 automatic behaviour

21 Occupational Impact 14/26 left work 8/18 stopped driving 4/18 short distances only

22 CSF Hypocretin Level + HLA typing 12 CSF Hypocretin 11 < 110pg/ml 7 < 50pg/ml 1 300pg/ml 10/10 HLA type DQB1-0602

23 Xyrem 11/36 Xyrem 9/11 extremely effective mean ESS decrease 10.5 points

24 Xyrem It s so nice to have my life back at last and for that I'd like to thank you I feel like I have a new head.. I feel so awake and alert Fantastic, brilliant

25 Xyrem 2/11 discontinued the medication 2 nauseated with dose increases Falls in 2 patients

26 Neurological EDS Narcolepsy Idiopathic hypersomnia Klein Levin syndrome Circadian rhythm disorders

27 Idiopathic hypersomnia Typical features: Long unrefreshing sleep Difficult to wake in the morning High ESS Long, unrefreshing naps Decreased sleep latency on MSLT, no REM onset sleep periods May benefit from Modafinil and Dexamphetamine

28 Neurological EDS Narcolepsy Idiopathic hypersomnia Kleine-Levin syndrome Circadian rhythm disorders

29 Kleine-Levin Syndrome Teenage onset Sleep hours/day + Eat voraciously on awakening Cognitive impairment during attack, hypersexuality Cyclical occurrence 3-4 times/year Episodes last days to weeks

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31 Neurological EDS Narcolepsy Idiopathic hypersomnia Kleine-Levin syndrome Circadian rhythm disorders

32 Circadian rhythm disorders Delayed sleep phase Advanced sleep phase Irregular sleep-wake Free-running (non-entrained, non-24 hour) Jet-lag Shift-work

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34 Circadian rhythm disorders Treatment Phototherapy Pharmacotherapy Chronotherapy

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36 Circadian rhythm disorders Treatment Phototherapy Pharmacotherapy Chronotherapy

37 Excessive daytime sleepiness Physiological sleep deprivation, poor sleep hygiene Pathological Sleep apnoea Narcolepsy and idiopathic hypersomnolence Recurrent hypersomnias Circadian rhythm sleep disorders Sleep related movement disorders Medication/drug-related hypersomnia General medical disorders Psychiatric disorders Neurological disorders

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