Diagnosis of Neurologic Spells: Seizure, Pseudoseizure, Demonic Possession or Witchcraft Jeffrey J. Tramonte, M.D. Baylor Scott & White Healthcare Round Rock Director of Neurology Director of Emory Bellard ALS Clinic July 15, 2014 Goals • Differentiate between seizures and pseudoseizures • Cast out the demons • Understand the limitations of EEG Audience Response System A) Seizure B) Pseudoseizure Case – Recurrent “seizures” • 30 y/o woman referred for seizures – 2 month h/o new onset, frequent generalized convulsions – PHM, ROS, Exam all normal/negative – Work-up is normal • brain MRI, 2 interictal EEGs – Treatment with 3 combined AEDs (CBZ, PHT, VPA) ineffective Case – Recurrent “seizures” • ER presentation with frequent seizures – 1) Treated with Valium and Ativan – 2) Paralyzed, intubated, & sedated due to “agitation” – 3) Central line placed for better venous access • Developed pneumothorax • Chest tube • ICU stay – 4) Repeat workup normal (LP, EEG, MRI) Audience Response System A) Seizure B) Pseudoseizure Case – Recurrent “seizures” • Misdiagnosis and mistreatment of pseudoseizures is dangerous Howell S.J. QJM 1989 Demonic Possession & Witchcraft • Biblical References – Epilepsy viewed as possession by evil spirits • Mark 9:18 Whenever it seizes him, it throws him to the ground. He foams at the mouth, gnashes his teeth and becomes rigid. I asked your disciples to drive out the spirit, but they could not. Movie Clip – The Exorcist Audience Response System A) Seizure B) Pseudoseizure Pseudoseizures vs Seizures • Ictal stuttering is a sign of pseudoseizures – Sensitivity: 8.5% – Specificity: 100% – Pos Pred Value: 100% Vossler D.G. Neurology 2004 Demonic Possession & Witchcraft • Literary References – Othello by Shakespeare (1604) • Othello has a seizure preceded by extreme emotional agitation • Othello acted with “savage madness” • Othello must have been seizing when he killed his wife because he was in a rage – Silas Marner by George Eliot (1861) • Marner’s seizures are viewed as “visits from the Devil” causing him to be accused, found guilty, and cast out by church members Audience Response System A) Seizure B) Pseudoseizure Demonic Possession & Witchcraft • Salem Witch Trials 1691 – 20 Salem villagers were executed for bewitching and collusion with the devil – Signs of bewitching: • • • • Fainting and disordered speech Visual delusions and hallucinations Odd contortions Sensations of prickling, pinching, or skin burning Woolf A. Clin Tox 2000 Demonic Possession & Witchcraft • Salem Witch Trials 1691 – Ergot poisoning theory • 1691-1692 weather conditions favored ergot-producing fungal growth on rye • Crop failure forced villagers to eat freshly harvested, infested rye • The afflicted were nonrandom and lived along rye supply routes • Animals were affected and died (ergotism occurs in grazing animals) • Symptoms consistent with ergotism: seizures, hallucinations, delerium, muscle spasms, and parethesias Woolf A. Clin Tox 2000 Movie Clip – The Exorcist Audience Response System A) Seizure B) Pseudoseizure Pseudoseizures vs Seizures Reuber M. Anaesthesia 2000 Audience Response System A) Seizure B) Pseudoseizure Pseudoseizures vs Seizures • Asynchronous limb jerking is common to pseudoseizures Reuber M. Anaesthesia 2000 Audience Response System A) Seizure B) Cough syncope (convulsive syncope) Movie Clip – The Exorcist Audience Response System A) Seizure B) Pseudoseizure Pseudoseizures vs Seizures • Ictal eye closure is a reliable sign of pseudoseizures – Eyes Closed: (pseudo) • Sensitivity: 96.2% • Specificity: 98.1% • Pos Pred Value: 94.3% – Eyes Open: (real sz’s) • Sensitivity: 98.1% • Specificity: 96.2% • Pos Pred Value: 98.7% Chung S.S. Neurology 2006 Audience Response System A) Seizure B) Pseudoseizure Audience Response System A) Seizure B) Pseudoseizure Pseudoseizures vs Seizures Krumholz A. Neurology 1999 Movie Clip – The Exorcist Audience Response System A) Seizure B) Pseudoseizure Audience Response System A) Seizure B) Pseudoseizure Audience Response System A) Seizure B) Pseudoseizure American Idol 2008 How are seizures diagnosed? • Clinical seizure type (direct observation) • EEG seizure type • EEG interictal expression Commission Report. Epilepsia 1981 Audience Response System 18 year old unaware of daydreaming spells A) Seizure B) Pseudoseizure Childhood Absence Epilepsy The Routine EEG is normal until . . . Childhood Absence Epilepsy Hyperventilation triggers absence seizure How are seizures diagnosed? • Sensitivity of awake EEG – – – – 1st 30 min with photic & HV = 50% 2nd “ “ = 62% 3rd “ “ = 74% 4th “ “ = 85% • Sensitivity of awake & asleep EEG – Repeated 4 times = 92% • Ambulatory EEG monitoring • Video EEG monitoring Binnie C.D. Clin Neurophys 1999 Seizures & Epilepsy: The Bottom Line • Partial seizures (localization-related epilepsies) – Brain MRI for structural etiologies (neoplasm, sclerosis, stroke, vascular malformation, hereditary malformation) • Refer to neurology – Decision making on AEDs, side effects, driving privileges, epilepsy monitoring unit Top Five Take-Home Points 1. Pseudoseizures are commonly misdiagnosed as seizures 2. Ictal eye closure predicts pseudoseizures 3. Ictal stuttering predicts pseudoseizures 4. A single interictal EEG is only 50% sensitive 5. Don’t just sit there bust a move (if you see a patient having a spell, test them) The End
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