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LGS and Sleep


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Sagarika Nallu, MD, speaks about LGS and sleep

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LGS and Sleep

  1. 1. Lennox-Gastaut syndrome (LGS) and Sleep Sagarika Nallu, M.D. Assistant professor Pediatric Neurology, Epilepsy & Sleep Medicine Chief of Pediatric Neurology Director of Pediatric Sleep Medicine University of South Florida Morsani College of Medicine
  2. 2. Lennox-Gastaut syndrome (LGS)  Severe form of childhood-onset epilepsy associated with high morbidity and mortality  Peak period between ages 3 and 5 years  More common in males  Annual prevalence of 0.26 per 1000 at age 10 years  Comprises ~4% of childhood epilepsy cases  High mortality rates-Sudden unexpected death in epilepsy (SUDEP) is the most frequent cause of death among patients with Lennox-Gastaut syndrome  Status epilepticus
  3. 3. Classic Lennox-Gastaut syndrome triad Multiple seizure types Cognitive impairment/ regression EEG Pattern-Slow spike-and-wave pattern
  4. 4. Hierarchy of Symptoms Resnick T. Journal of Child Neurology, 2017.
  5. 5. LGS awake EEG: Slow Spike and Wave 11/19/2017
  6. 6. EEG: Paroxysmal Fast Activity 11/19/2017
  7. 7. EEG in LGS Paroxysmal Fast 10Hz Activity -This is associated with tonic seizures- mainly in sleep.  or can occur with minimal clinical manifestations, such as apnea or perhaps truncal rigidity. Can be seen mainly during non-REM sleep. 11/19/2017
  8. 8. EEG in LGS  Since a diagnosis relies so heavily on finding that classic EEG feature and since tonic seizures tend to occur mostly during sleep, a sleep EEG is essential.  These fast rhythms that are essential for diagnosis and are essential in differentiating Lennox-Gastaut Syndrome from other epilepsy syndromes in which slow spike in wave discharges can be seen.  Misdiagnosis or over diagnosis of LGS 11/19/2017
  9. 9. Features of Several Epileptic Disorders Resnick T. Journal of Child Neurology, 2017.
  10. 10. Seizure Triggers 11/19/2017 Missed dose of medication Sleep deprivation Illness Stress, anxiety, overstimulation Hormonal changes Alcohol and drugs of abuse Hyperventilation Flashing lights Temperature extremes Dehydration
  11. 11. Common Sleep Comorbidities  Sleep provides an opportunity to examine and potentially improve epilepsy  Insomnia  Excessive daytime somnolence  Sleep apnea - both Central and Obstructive events  Epilepsy and obstructive sleep apnea (OSA) frequently coexist, and treating OSA in epilepsy patients may reduce seizure frequency and improve daytime sleepiness 11/19/2017
  12. 12. Pharmacologic Treatment  Antiepileptic Drugs  valproic acid  Lamotrigine  Topiramate  Rufinimide  Clobazam  Benzodiazepines  Diazepam  Clonazepam  Clobazam
  13. 13. Medications and sleep  Benzodiazepines are generally safe in low doses but in higher doses and in select patients they may be associated with respiratory depression, hypoventilation, hypoxemia, and obstructive sleep apnea (OSA).  Narcotics can be associated with apneas and hypoventilation with increased risk with higher doses and use of other central nervous system depressants.  Antidepressants may partially improve OSA by suppressing stage rapid eye movement and increasing upper airway tone but do not completely treat OSA. 11/19/2017 Medication Effects on Sleep and Breathing clinics in Chest Medicine 2014 GilbertSedaMD, PhDaSheilaTsaiMDbTeofiloLee-ChiongMDc
  14. 14. Common Sleep Comorbidities  Nocturnal seizures lead to disturbed quality of sleep at night and leads to excessive daytime somnolence  Medications  Polysomnogram or sleep study to rule out sleep apnea.  Obstructive sleep apnea treatment- CPAP/BiPAP, ENT surgery.  Evaluating for Central sleep apnea 11/19/2017
  15. 15. LGS and Sleep Thorough Sleep history Evaluating for sleep disorders Treatment strategies- Eliminating/ minimizing the contributing factors 11/19/2017
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  17. 17. References  Resnick T, Sheth RT. Early Diagnosis and Treatment of Lennox-Gastaut Syndrome. Journal of Child Neurology 2017;32(11):947-955.  Sforza E, Mahdi R, Roche F, et al. Nocturnal interictal epileptic discharges in adult Lennox-Gastaut syndrome: the effect of sleep stage and time of night. Epileptic Disord 2016;18(1):44-50.  Eisensehr I, Parrino L, Noachtar S, et al. Sleep in Lennox– Gastaut syndrome: the role of the cyclic alternating pattern (CAP) in the gate control of clinical seizures and generalized polyspikes. Epilepsy Research 2001;46:241–250.
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  21. 21.  Associated EEG patterns – generalized 1-2hz slow spike and wave (see figure), generalized slowing, paroxysmal activity (recruiting rhythm) during sleep (see figure) 11/19/2017
  22. 22. Psychiatric and Behavioral Characteristics  Attention-deficit hyperactivity disorder (ADHD)  Depression  Anxiety  Aggression  Psychosis
  23. 23. Non-Pharmacologic Therapy  Ketogenic Diet  Surgery  Focal, lobar, or multilobal resection vs hemispherectomy  Vagus nerve stimulation  Corpus callosotomy