differentiating nocturnal epilepsy from...
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Differentiating Nocturnal Epilepsy
From Parasomnias December 5, 2011
Jennifer L. DeWolfe, DO Assistant Professor of Neurology
Director, UAB Neurology Sleep Services
Director, BVAMC Sleep Center
University of Alabama at Birmingham
American Epilepsy Society | Annual Meeting
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Disclosure
• Within the past 12 months
UCB, Inc.
GlaxoSmithKline
• Current Support
Schwarz Pharma
Valeant
(not currently)
Speakers Bureau
Speakers Bureau
Clinical Research
Clinical Research
American Epilepsy Society | Annual Meeting
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Learning Objectives
• Parasomnias should be considered in the differential diagnosis of nocturnal behaviors
• Parasomnias occur frequently in people with neurological diseases, including epilepsy
• Differentiate nocturnal seizures from parasomnias and discuss when to refer for evaluation
American Epilepsy Society | Annual Meeting
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Nocturnal Behaviors • Generalized or partial epileptic seizures
• Parasomnias
• Normal sleep variants (hypnic jerks)
• Sleep-related movement disorders
– Rhythmic movement disorder, Periodic limb
movements in sleep, bruxism
• Somniloquy (NREM, REM)
• Sleep enuresis (NREM, REM)
• Post-arousal behaviors (OSA, other primary
sleep d/o)
• Psychogenic events (appears asleep)
Bazil, Carl. Seminars in Neurology, Volume 24, Number 3, 2004.
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• Undesirable behavioral, autonomic, and
experiential phenomena (emotions,
perceptions, dreaming) occur during
–Entry into sleep
–Any sleep stage (NREM and REM)
–During partial or full arousals from any sleep stage
International Classification of Sleep Disorders, 2nd Ed. Am Academy Sleep Med. 2005:298.
Parasomnias
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NREM Parasomnias:
Disorders of Arousal
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30y/o RHM recurrent nocturnal behaviors since
childhood
• Hx sleepwalking, sleep talking
• Events may vary; however some event types are recurrent
• Duration: few minutes
• Minimal to Complex behaviors
– From sleep, sometimes gets up out of bed and walks
– Mimics eating, may have conversations, hallucinations
– Usually appears frightened, sweaty, has grabbed wife’s arm when she tried to gently shake him awake
• No dream recall if woken
• Amnestic of events
Case 1
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CASE 1 VIDEO
Patient consent obtained to use video for teaching purposes
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Case 1 • Interevent EEG: normal
• Event: disoriented speech, fear, tachycardia from
N3 Sleep
– theta > delta slowing
– with continued interaction, disorientation resolves,
appears to return to clinical baseline as EEG
demonstrates return to normal waking background
• Amnesia of event
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Case 1
• Diagnosis: NonREM Arousal Parasomnia – Features of Confusional Arousal
• Disorientation, confusion, altered speech
– Features of Sleep Terrors • Fear response, tachycardia
– History nocturnal behaviors, sleepwalking
• Likely has multiple Arousal Parasomnias
• Events have been controlled by avoiding sleep deprivation and clonazepam 1mg qhs
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1. The AASM Manual for the Scoring of Sleep and Associated Events: Rules, Terminology and Technical Specifications.
2007. 2. Picture from slide by Susan Harding, MD.
NREM Disorders of Arousal
• Slow wave sleep
– Can be light NREM sleep if forced awake
• N3 Sleep: 20% or more delta activity per 30 second epoch
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1. Aldrich M, editor. Parasomnias. Oxford: Oxford University Press; 1999. 2. Schenck, Carlos. Sleep Runners. 2007. 3.
Derry CP, Duncan JS, Berkovic SF. Epilepsia. 2006 Nov;47(11):1775-91.
NREM Disorders of Arousal
• First half of the night
• Once a night
• Common childhood, infrequently persists into
adulthood
• Strong family history
• Amnesia of event
– may recall dream fragments in am
• Can be induced in healthy people
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1. Aldrich M, editor. Parasomnias. Oxford: Oxford University Press; 1999. 2. Schenck, Carlos. Sleep Runners. 2007. 3.
Derry CP, Duncan JS, Berkovic SF. Epilepsia. 2006 Nov;47(11):1775-91.
NREM Disorders of Arousal
• EEG during event
– Slow alpha and theta (incomplete arousal)
• Commonly associated with comorbid arousal
parasomnia or primary sleep d/o
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Pathophysiology
• Physiological dysfunction in neuronal regulation
of generalized cortical activation
• Increase in sleep instability and arousal
oscillation
• Increased slow wave sleep fragmentation
– Especially in first NREM-REM cycle
• Worsened by sleep deprivation
– Due to increased N3 sleep
1. Mahowald MW, Schenck CH, et al. Arch Neurol. 1992 Jun;49(6):604-7. 2. Mahowald MW, Schenck CH, et al. J Forensic
Sci. 2003;48(5):1158-62.
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Predisposing, Precipitating Factors
• Genetic factors - predominant role
• Rotating shifts/Night shift
• Recovery from sleep deprivation (increased N3)
• Forced awakenings
• Stress
• Other sleep disorders
• Psychiatric disorders – Anxiety, Depression, Bipolar disorder
• Alcohol use/abuse, Drug abuse, Medications
1. Derry CP, Duncan JS, Berkovic SF. Epilepsia. 2006 Nov;47(11):1775-91. 2. Schenck, Carlos. Sleep Runners. 2007. 3.
Yogarajah M, Powell HW, et al. J Neurol Neurosurg Psychiatry. 2009 Mar;80(3):305-10.
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• Autonomic, behavioral
manifestations
• Unresponsive, piercing scream,
incoherent vocalization
• If awakened may be confused,
disoriented, +/- violent
• Adults may have dream
imagery, dream enactment, and
subsequent recall for episodes
• Amnesia
Sleep Terrors
1. Derry CP, Duncan JS, Berkovic SF. Epilepsia. 2006 Nov;47(11):1775-91. 2. Schenck, Carlos. Sleep Runners. 2007. 3.
Yogarajah M, Powell HW, et al. J Neurol Neurosurg Psychiatry. 2009 Mar;80(3):305-10.
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Confusional Arousals
• Disoriented to surroundings, slow speech,
decreased responsiveness, confused behavior
– Simple and non-goal directed
– Complex and prolonged
– May be inappropriate, vigorous, resistive, sexual,
violent or murderous (especially if forced awakenings)
• Minutes to hours
1. Derry CP, Duncan JS, Berkovic SF. Epilepsia. 2006 Nov;47(11):1775-91. 2. Schenck, Carlos. Sleep Runners. 2007. 3.
Yogarajah M, Powell HW, et al. J Neurol Neurosurg Psychiatry. 2009 Mar;80(3):305-10. 4. Mahowald MW, Schenck CH, et
al. Arch Neurol. 1992 Jun;49(6):604-7. 2.
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Somnambulism • Sleepwalking
• Sudden arousal slow wave sleep
• Altered consciousness, wide-eyed
stare
• May leave room
• Limited patchy memory, if any
• Complex behaviors
– cook, eat, dress, agitation,
violence, urinate
– Sleep Driving
• Induced by alcohol, medication
(lithium, zolpidem, anticholinergics)
1. Derry CP, Duncan JS, Berkovic SF. Epilepsia. 2006 Nov;47(11):1775-91. 2. Schenck, Carlos. Sleep Runners. 2007. 3.
Yogarajah M, Powell HW, et. al. J Neurol Neurosurg Psychiatry. 2009 Mar;80(3):305-10.
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REM Parasomnias
• Nightmares
• REM Sinus Arrest
• Sleep Paralysis
• Nocturnal Groaning (Catathrenia)
• REM Behavior Disorder
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REM Behavior Disorder • Disruption of normal atonia (paralysis) and
impaired suppression of movement generators
during REM Sleep
• Acts out dreams
• Can be violent, self-injurious
• Clinical diagnosis confirmed with PSG
– REM without atonia, +/- simple or complex behaviors
• Can be induced or worsened by alcohol or
medications (SSRIs)
1. The AASM Manual for the Scoring of Sleep and Associated Events: Rules, Terminology and Technical Specifications.
2007. 2. Trotti, LM. Drugs Aging, 2010. 27(6): p. 457-70.
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REM Behavior Disorder Associated Neurological Disorders • -synucleinopathies
– Dementia with Lewy Bodies (92%)
– Multiple System Atrophy (69-90%)
– Parkinson’s disease (15-65%)
• Tauopathies – Corticobasal degeneration – sporadic case studies
– Alzheimer’s disease
• Epilepsy (>60 y/o) (12%) – Male, sleep-related seizures, cryptogenic seizures
• Psychiatric disorders – depression, antidepressant induced RBD
• Narcolepsy
1. Berry R, et al. Clinical Sleep Disorders. 2005:196-197. 2. Ochoa JG, et al. Clinical Sleep Disorders. 2005:230-232. 3. Gagnon,
JF, et al. Sleep. 2006;29(10)1321-1325. 4. Gagnon JF, et al. Neurology. 2002;59:148-52. 5. Iranzo A, et al. Neurology
2005;65:247-52. 6. Plazzi G, et al. Neurology. 1997;48:1094-7. 7. Comella CI, et al. Neurology. 1998;51:528. 8. Manni R, et al.
Epilepsy Res. 2007. 9. Ebrahim IO, et al. J Clin Sleep Med. 2005;1(1):43-7.. 10. Zoccolella, S., et al. Sleep Med Rev.
2011;15(1):41-50.
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Case 2 49 y/o RHM with partial seizures controlled on
CBZ, OSA controlled with CPAP, c/o two years of new nocturnal behaviors
• Habitual Seizure Semiology: – “Bad smell” then stares unresponsive x 30 seconds, then back to
baseline. Never generalized.
– Always during the day, seizure free x 5 years
• Current nocturnal events: – Variable thrashing during sleep, usually wee hours
– If awoken, recalls recurrent dream of being attacked by his mother
– Has fallen out of bed, fractured hand on night stand, broken lamp
– 2 episodes sleep walking – woke in kitchen, dining room
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Case 2 Polysomnogram
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Case 2 Video
Patient consent obtained to use video for teaching purposes Patient consent obtained to use video for teaching purposes
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Case 2
Diagnosis: REM Behavior Disorder
• Discussed safe sleeping environment
• Failed trial alternate antidepressant
– Good control of depression, persistent behaviors
– Continued psychiatry and counseling follow up
• Added clonazepam 0.5mg at night, titrated up to 1.5 mg at
night – controlled behaviors
• Remains seizure free on unchanged AEDs
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• Minimize precipitating factors
– Alcohol, medications
• Safety of the sleep environment
– Lock guns separate from ammunition
– Remove sharp objects from bedroom
– Mattress and boxsprings on floor
– Bedpartner in other room until behaviors controlled
– Door or floormat alarms
– Bed away from window
Parasomnia Treatment
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• Do not forcefully awaken patient
• Gently guide them back to bed
• Sleep hygiene, maintain regular sleep-wake schedule, sufficient total sleep time
• Stress reduction, counseling if indicated
• Relaxation techniques
• Psychotherapy
• Self-hypnosis
1. Schenck, Carlos. Sleep Runners. 2007. 2. Mahowald MW, Schenck CH, et al. 1992 Jun;49(6):604-7.
Parasomnia Treatment
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• Medications
– Potentially injurious behaviors
– Frequent or disruptive events
• NREM Parasomnias
– Benzodiazepines
• May worsen obstructive sleep apnea
– Imipramine in children
• REM Behavior D/O
– Benzodiazepines
– Melatonin
Parasomnia Treatment
1. Schenck, Carlos. Sleep Runners. 2007. 2. Mahowald MW, Schenck CH, et al. Arch Neurol. 1992 Jun;49(6):604-7. 3. Trotti,
LM. Drugs Aging. 2010;27(6):457-70.
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Seizures vs. Parasomnias
• Seizures – Sleep and Wake
– May cluster in sleep
– Adult & childhood onset
– Stereotyped
– Usually brief
– Amnesia (complex
partial seizure or
secondarily generalized)
• Parasomnias – Only sleep related
– Usually 1 event/night
– Childhood onset (NREM)
vs. Adult onset (REM
Behavior D/O)
– Usually not stereotyped
– May be prolonged
– Typically have amnesia
of events (although RBD
may recall associated dream if
awoken during behaviors)
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Sleep Complaints in PWE • Poor sleep hygiene can disrupt sleep, contribute to
insomnia and sleep deprivation1,3
• Fatigue 63%3
• Parasomnias 60%3
– most sleep-wake transition (NREM and REM less common)
• Sleep Maintenance Insomnia 52%3
• Disrupted sleep 38%1,2,4,5
• Excessive Sleepiness 37-39%3,5
• Sleep Onset Insomnia 34%3
• Obstructive sleep apnea symptoms 20-33%3,6,7
– Higher in PWE > 50 y/o7
• Restless legs symptoms 18%3
1. Vaughn, BV, D’Cruz OF. Semin Neurol. 2004 Sep;24(3):301-13. 2. Bazil, CW. Epilepsy Behav. 2003;4 Suppl 2:S39-45. 3. Khatami, R, et
al. Seizure. 2006;15(5):299-306. 4. deWeerd A, et al. Epilepsia. 2004;45:1397–404. 5. Xu, X, et al. Epilepsia. 2006;47(7):1176-83. 6.
Malow BA, et al. Neurology. 2000;55(7):1002-1007. 7. Chihorek, AM, et al. Neurology. 2007;69:1823-1827.
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When to Refer for Sleep Evaluation? • Sleep complaints
– Excessive sleepiness
• Review medications, AEDs
• Screen for sleep disorders
– Insomnia
• Restless legs symptoms, limb movements during sleep,
AEDs, underlying mood d/o, OSA symptoms
• Sleep hygiene
– Snoring, witnessed apneic events
– Poor sleep quality
• Nocturnal epileptiform discharges/seizures, nocturnal
awakenings, primary sleep d/o
• Sleep hygiene
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When to Refer for Sleep Evaluation?
• Breakthrough seizures – Previously well controlled
– New events
• Nocturnal Events – Self injurious behaviors
– Sleep-wake transition behaviors
– Dream enactment
– Sleep walking
– Confusional arousals
• Parasomnias can be difficult to distinguish from
epileptic seizures
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Polysomnogram
• Polysomnogram with extended EEG monitoring
–10-20 Electrode placement (less temporal
coverage)
–Very frequent nocturnal events
–Non-stereotyped events
–Strong suspicion for comorbid sleep disorders
–Less expensive than vEEG
DeWolfe, J and Malow, B. Approach to sleep-related seizure identification and management in ed. Matheson,J. Sleep Disorders
Treatment. 2010.
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Continuous Video EEG Monitoring
• Continuous Video EEG Monitoring
– Stereotyped events, seizure risk factors, history central
nervous system disorders, history of epilepsy
– Nocturnal events occur less frequently (weekly, monthly)
– Polysomnogram captured stereotyped events
w/without epileptiform transients
– Adult onset
– Additional electrode coverage, glued, activation
procedures
– Consider electro-oculography and chin EMG
DeWolfe, J and Malow, B. Approach to sleep-related seizure identification and management in ed. Matheson,J. Sleep Disorders
Treatment. 2010.
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• Long differential diagnosis of nocturnal behaviors
• History may narrow the differential
• Stereotyped events (especially automatisms), clustered
events, adult onset: consider epilepsy
• Parasomnias are common and frequently occur with other
sleep disorders
• Comorbid sleep disorders and seizures are not uncommon
• Polysomnography with extended EEG monitoring can
diagnose and distinguish between epileptic seizures and
sleep disorders
• Video EEG monitoring for less frequent events, longer
duration of monitoring, extended temporal lobe coverage
Summary