Epilepsy Vs Narcolepsy

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Epilepsy Vs Narcolepsy:- case report of a 16 year old girl who keeps losing consciousness with sharp waves on her EEG.

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Epilepsy Vs Narcolepsy

  1. 1. Epilepsy or Narcolepsy? AskTheNeurologist.Com Author Anon
  2. 2. History <ul><li>16 year old woman suffering from sleep deprivation on a holiday </li></ul><ul><li>4 days into trip </li></ul><ul><ul><li>Excessively sleepy in day </li></ul></ul><ul><ul><li>Began “ falling asleep” inappropriately </li></ul></ul><ul><ul><li>On morning of admission, noticed she had bitten tongue to point of bleeding </li></ul></ul><ul><ul><li>During day, frequent sleep-attacks </li></ul></ul>
  3. 3. Sleep attacks <ul><li>Sometimes felt tired beforehand </li></ul><ul><li>Occurred on - talking </li></ul><ul><li>- walking (fell) </li></ul><ul><li>Absence of </li></ul><ul><ul><li>Abnormal movements </li></ul></ul><ul><ul><li>Tongue biting during LOC </li></ul></ul><ul><ul><li>Incontinence </li></ul></ul>
  4. 4. Past History <ul><li>Mild asthma </li></ul><ul><li>Tongue biting since childhood </li></ul><ul><ul><li>Hypnogogic </li></ul></ul><ul><ul><li>? intentional </li></ul></ul><ul><li>Denies </li></ul><ul><ul><li>Seizures </li></ul></ul><ul><ul><li>Cataplexy ( fell, but only after LOC) </li></ul></ul><ul><ul><li>Sleep paralysis </li></ul></ul><ul><ul><li>Hypnogogic hallucinations </li></ul></ul>
  5. 5. Examination <ul><li>Signs of tongue-biting on side of tongue </li></ul><ul><li>Orientated </li></ul><ul><li>Drowsy </li></ul><ul><li>Repeatedly falling asleep </li></ul><ul><li>Neurological examination unremarkable </li></ul>
  6. 6. Investigations <ul><li>Blood : - normal ( CBC, Bioch, ESR) </li></ul><ul><li>Toxic screen negative </li></ul><ul><li>EEG when drowsy / asleep </li></ul><ul><ul><li>Synchronous high amplitude activity </li></ul></ul>
  7. 7. Background 26/8
  8. 8. “ Drowsy” 26/8
  9. 9. Impression following EEG <ul><li>Episodes of loss of consciousness and falling </li></ul><ul><li>Signs of tongue – biting </li></ul><ul><li>Rhythmic high amplitude activity when drowsy </li></ul><ul><li>Hospitalised and anti-epilaptic medication commenced </li></ul>
  10. 10. Course <ul><li>Received diazepam drip and carbamazepine </li></ul><ul><li>Slept well in hospital </li></ul><ul><li>Episodes stopped </li></ul><ul><li>EEG reviewed again </li></ul>
  11. 11. Concept of V-waves <ul><li>Occur at sleep onset from 5 months of age </li></ul><ul><li>Maximal at 4-5 years </li></ul><ul><li>May be present at all ages </li></ul><ul><li>More pronounced during childhood </li></ul><ul><li>Synchronous sharply contoured waves </li></ul><ul><ul><li>Negative phase reversal in midline </li></ul></ul>
  12. 13. EEG tip <ul><li>“ Be suspicious that any “epileptic” activity during sleep, with phase reversal in the midline is normal.” </li></ul><ul><li>“ The coronal montage is especially important during a sleep study, as phase reversal at Cz is sometime the only clue that sharp activity is normal” </li></ul>
  13. 15. “ Drowsy” 26/8
  14. 16. Coronal 26/8 drowsy
  15. 17. Coronal 26/8 low amplitude
  16. 18. Follow-up EEG 28/8
  17. 19. DD <ul><li>Narcolepsy / hypersomnia </li></ul><ul><li>Sleep deprivation </li></ul><ul><li>Epilepsy </li></ul>
  18. 20. Tiredness before LOC Episodes of LOC coincide with v-waves and no other epileptic activity A focus near Cz would be associated with focal seizures of leg ? Tongue biting ? Response to anti-epileptic medications Epilepsy Too severe Recurrence following discharge with adequate sleep History of sleep deprivation Sleep deprivation No other features Age of onset EDS Sleep attacks Narcolepsy Against For
  19. 22. Excessive daytime sleepiness / hypersomnia <ul><li>“ unintentional naps” </li></ul><ul><li>Onset in teens / early twenties </li></ul><ul><li>Sleepiness worst during inactivity </li></ul><ul><li>Improvement following nap </li></ul><ul><li>May complain of </li></ul><ul><ul><li>Inattention /memory disturbance </li></ul></ul><ul><ul><li>Diplopia </li></ul></ul><ul><ul><li>Automatic behaviour </li></ul></ul>
  20. 23. Aspects of REM sleep intrude into wakefulness <ul><li>Cataplexy </li></ul><ul><li>Hypnagogic hallucinations </li></ul><ul><li>Sleep paralysis </li></ul>
  21. 24. Hypnagogic hallucinations <ul><li>Dream-like episodes </li></ul><ul><li>Often frightening </li></ul><ul><li>Occur during drowsiness or onset of sleep </li></ul><ul><li>Usually visual </li></ul><ul><li>May be </li></ul><ul><ul><li>Tactile </li></ul></ul><ul><ul><li>Auditory </li></ul></ul><ul><ul><li>Vestibular (sense of falling) </li></ul></ul>
  22. 25. Sleep paralysis <ul><li>Profound weakness </li></ul><ul><li>May be at onset of sleep or on waking </li></ul><ul><li>May cause fear / feeling of choking </li></ul><ul><li>Is intrusion of ( protective) REM sleep paralysis into wakefulness </li></ul><ul><li>Sleep paralysis and hypnagogic hallucinations may occur following sleep deprivation </li></ul>
  23. 26. Cataplexy <ul><li>Sudden muscle weakness, no LOC </li></ul><ul><li>Triggered by intense emotion </li></ul><ul><ul><li>Laughter </li></ul></ul><ul><ul><li>Anger </li></ul></ul><ul><li>Similar concept to sleep paralysis </li></ul><ul><li>Present in 60% of narcolpetics </li></ul><ul><li>Usually occurs 3-5 years following onset of sleepiness </li></ul>
  24. 27. Lab. Findings <ul><li>Polysomnography </li></ul><ul><li>Multiple sleep latency test ( MSLT) </li></ul>
  25. 28. Polysomnography <ul><ul><li>Evaluates sleep quality </li></ul></ul><ul><ul><li>Excludes other causes of sleepiness: </li></ul></ul><ul><ul><ul><li>Obstructive Sleep apnea </li></ul></ul></ul><ul><ul><ul><li>Periodic leg movements </li></ul></ul></ul><ul><ul><ul><li>REM sleep behaviour disorder </li></ul></ul></ul>
  26. 29. MSLT <ul><li>Performed day after polysomnography </li></ul><ul><li>Given opportunity to nap 2 hours </li></ul><ul><li>Narcoleptics fall asleep within 5 minutes </li></ul><ul><ul><li>Normal = 10-15 minutes </li></ul></ul><ul><li>Naps often contain REMs (SOREMs) </li></ul>
  27. 30. Diagnosing Narcolepsy <ul><li>Chronic sleepiness </li></ul><ul><ul><li>AND </li></ul></ul><ul><ul><li>Either: - Cataplexy </li></ul></ul><ul><ul><li>OR: - > 2 SOREMs in MSLT d </li></ul></ul><ul><li>Idiopathic hypersomnia </li></ul><ul><ul><li>Chronic sleepiness without either of other criteria </li></ul></ul>
  28. 31. Secondary causes of narcolepsy <ul><li>Posterior hypothalamic lesions </li></ul><ul><ul><li>Tumour </li></ul></ul><ul><ul><li>Stroke / AVM </li></ul></ul><ul><ul><li>Sarcoidosis </li></ul></ul><ul><ul><li>Paraneoplastic ( anti-Ma) </li></ul></ul><ul><ul><li>MS </li></ul></ul>
  29. 32. Genetics of narcolepsy <ul><li>1 / 2000 </li></ul><ul><li>Usually sporadic </li></ul><ul><li>HLA DR2, DQ1 ( DQB1 * 0602) </li></ul>
  30. 33. Role of orexin ( hypocretin) <ul><li>Orexin-containing neurons found in posterior and lateral hypothalamus </li></ul><ul><li>Innervate aminergic annd cholinergic neurons that provoke wakefulness </li></ul>
  31. 34. Brown = Tyr OH’lase staining of Locus Coerulius neuron Black = terminals immunoreactive for orexin
  32. 35. Evidence for orexin hypothesis <ul><li>Intraventricular injection of orexin causes wakefulness </li></ul><ul><li>Knockout mice ( orexin or receptor) have disease resembling narcolepsy </li></ul><ul><li>Doberman dogs with orexin receptor mutations have narcolepsy and cataplexy </li></ul><ul><li>Absence of CSF orexin correlates well with cataplexy </li></ul>
  33. 37. Cataplexy and Sleep paralysis:- <ul><li>Intraventricular injection of orexin causes wakefulness </li></ul><ul><li>Knockout mice ( orexin or receptor) have disease resembling narcolepsy </li></ul><ul><li>Doberman dogs with orexin receptor mutations have narcolepsy and cataplexy </li></ul><ul><li>Absence of CSF orexin correlates well with cataplexy </li></ul>
  34. 38. Sleep paralysis = opposite problem i.e. in RBD lose the protective paralysis which is inappropriately active in sleep paralysis and cataplexy.
  35. 41. Features of RBD <ul><li>Violent thrashing or yelling accompanied by nightmares during sleep. </li></ul><ul><li>Behavioral outbursts associated with dream mentation and intermittent loss of the muscle atonia that normally characterizes the REM sleep state. </li></ul>
  36. 42. RBD associations <ul><li>Can be associated with, or even precede, other neurologic disorders, especially parkinsonian states. </li></ul><ul><li>38% of patients with isolated RBD developed a parkinsonian disorder at a mean of 3.7 years after the diagnosis of RBD. </li></ul><ul><li>One-third of patients with (PD) show RBD on polysomnography </li></ul><ul><li>Strong association with all synucleinopathies </li></ul><ul><ul><li>PD, MSA and DLB </li></ul></ul><ul><li>RBD with dementia or PD’ism is highly predictive of synucleinopathy at postmortem. </li></ul><ul><li>Responds to clonazepam, dopaminergic drugs </li></ul>

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