2/14/2019 Disclosures Epilepsy Vikram Rao, MD, PhD NeuroPace, Inc.: Consultant Diagnosis & Management Upsher Smith Laboratories: Served on Scientific Advisory Board Switch Bio, Inc.: Served on Scientific Advisory Board 2019 Neurona, Inc.: Consultant Susannah Cornes, MD Susannah B. Cornes, MD None Associate Professor of Clinical Neurology Vikram R. Rao, MD, PhD Assistant Professor of Clinical Neurology Epilepsy Diagnosis and • Seizures: paroxysmal, stereotyped spells of Management altered movement, sensation, experience, Patient Preferences and/or consciousness resulting from excessive brain electrical activity • Epilepsy: recurrent, unprovoked seizures 4 th most common neurological disorder (migraine, stroke, AD) ∼ 1% of the population (3.4 million in U.S.) Multisource Data 1 in 26 people will develop epilepsy in their lifetime through clinical vignettes • Etiology: stroke, tumor, trauma, infection, genetic, metabolic, developmental, neurodegenerative, cryptogenic, … Institute of Medicine Report (2012); www.cureepilepsy.org; www.cdc.gov; Clinical Diagnosis Zack and Kobau, MMWR Morb Mortal Wkly Rep ( 2017) 66:31 1
2/14/2019 CASE 1: Audience Response System 33 RHM h/o HTN, HL p/w 6-months of stereotyped spells Internal head sensa�on → R face numb and weak, dysarthria → R hand/arm numb and weak, tremoring At this point, the MOST likely diagnosis is: movements → R leg weak; no loss of awareness 67% Duration: 4–8 min, Frequency: 2-3x/month A. Jacksonian epilepsy Workup B. Cardioembolic TIA Head CT, brain MRI, TTE, Holter, carotid U/S, routine EEG all normal C. Complex migraine Lupus anticoagulant positive; started on lovenox → warfarin 18% D. Call-Fleming syndrome 7% Spells persisted despite therapeutic INR 4% 4% E. Other vascular phenomenon Focal seizures suspected, Keppra started Trileptal added with minimal improvement Jacksonian epilepsy Cardioembolic TIA Complex migraine Call-Fleming syndrome Other vascular phenom... Referred to UCSF for refractory epilepsy Confidential – 9/21/15 6 UCSF Evaluation UCSF Evaluation MRA CTA Red flags: - spells involve mostly negative symptoms (numbness, weakness) - right arm shaking described as oscillatory (not clonic) - relatively long duration (>4 min) - too stereotyped for cardioembolic process - no response to two antiepileptic drugs CTP Additional history: - >90% of events occurred while in a hot shower or upon standing Further workup: Brain MRI/A: High-grade left M1 stenosis, no infarcts - Brain MRI/A ordered CTA: confirmed 70% left M1 stenosis CTP: left MCA territory ischemia Tapered off Keppra, Trileptal; started on aspirin, statin, permissive HTN; referred to UCSF Neurovascular 2
2/14/2019 CASE 2: UCSF Treatment 54 RHF with 28 years of spells of R hemibody paresthesias Indescribable aura, electrical head sensation that shoots MRA down right hemibody, occ. falls, no loss of awareness Duration: 10 sec, Frequency: catamenial, clusters up to 100/day; Triggers: stress, alcohol, sleep deprivation Workup Head CT, brain MRI, sleep-deprived EEG, carotid U/S, TCD all normal Improved spell frequency on Dilantin, Keppra added without benefit Diagnosed with “psychosomatic” hemisensory syndrome Advised to reduce stress, improve diet/exercise, ensure good sleep 33/34 patients with extensive Wingspan stent to left M1 workup negative, 1 patient with Aspirin + Plavix corona radiata stroke Spells resolved completely She presented to UCSF for a second opinion UCSF Evaluation Audience Response System Red flags: At this point, the MOST likely diagnosis is: - consistent aura before symptoms - falls with some episodes 54% - no psychiatric risk factors for non-epileptic spells A. Multiple sclerosis - spells improved on Dilantin B. Psychogenic non-epileptic - reported triggers (stress, alcohol, sleep deprivation) are common 29% for seizures spells C. Focal sensory seizures Additional history: 10% 7% - prior EEGs had not been done during symptomatic periods 0% D. TIA TIA Multiple sclerosis Focal sensory seizures Catamenial migraine Psychogenic non-epilept.. Further workup: E. Catamenial migraine - 48-hour ambulatory EEG ordered, timing to be based on catamenial variation in spells Confidential – 9/21/15 13 3
2/14/2019 CASE 3: UCSF Evaluation 52-year old man h/o OSA, two cousins with epilepsy Onset of spells at age 16 Ambulatory EEG No clear ictal correlate Hypermotor nocturnal events involving brief (<15 sec) Frequent left parietal spikes to patient’s symptoms tonic extensor posturing and flailing limb movements; Duration: <15 sec; Frequency: multiple times every night Workup Brain MRI reportedly normal Routine EEG: negative Sleep study: ?REM behavior disorder, no seizure activity Epilepsy considered possible, treated with 3 antiepileptic drugs Auras can be EEG-negative Symptoms presumed epileptic Referred to UCSF for pre-surgical evaluation Seizure-free on oxcarbazepine Localizing seizures in the brain UCSF Evaluation Video-EEG: no interictal spikes, 3 seizures without EEG correlate PET-CT: negative Neuropsychology: non-lateralizing patterns of deficits 3T Brain MRI: Scalp EEG 3T MRI PET MEG SPECT Wada Stimulation Stereo-EEG mapping Grid electrodes Transmantle focal cortical Highly epileptogenic lesion dysplasia (likely FCD IIb) High likelihood of overlap with eloquent cortex 4
2/14/2019 UCSF Evaluation Audience Response System Intracranial EEG: 122 subdural electrodes (grid/strips/depths) At this point, the MOST likely appropriate next step ?: 51% A. Lesionectomy 39% B. Intracranial EEG C. Implant responsive - Seizure onset zone localized to the dysplasia and adjacent neurostimulator 7% 3% interhemispheric cortex 0% - Electrical stimulation mapping revealed overlap with D. Implant vagus nerve stimulator Lesionectomy Intracranial EEG eloquent (leg) motor area Implant responsive neur... Implant vagus nerve stim... Cognitive behavioral the... E. Cognitive behavioral therapy - Patient unwilling to accept treatment-related neurological deficits Confidential – 9/21/15 20 Electrodes Advanced treatments for seizures • Record from seizure focus/foci • Stimulate to reduce seizures Resection Laser Devices Leads • Strips or depths Responsive Neurostimulator • Cranially implanted • Electronics and battery 5
2/14/2019 Chronic electrocorticography Responsive neurostimulation RNS leads Electrocorticogram of seizure https://... One year after responsive stimulation was enabled, patient is completely seizure-free CASE 4: Intracranial Monitoring 28 RHF with onset of epilepsy at age 12 Right leg tingling and ascending numbness, right leg stiffens and jerks, falls, no loss of awareness Duration: 60 sec; Frequency: 3x/week, secondarily generalizes to convulsion 1x/month EEG Source Localization Workup 3T Brain MRI: normal Video-EEG: dorsal view - Interictal: left centro-parietal spikes - Ictal: left centro-parietal seizure onset - Seizure onset from centroparietal region PET-CT: dorsal-medial parietal hypometabolism PET - Stimulation mapping revealed overlap with eloquent (leg) inferior sensorimotor cortex Treated with 3 antiepileptic drugs view - Patient willing to risk neurological defict for an intervention with highest chance of seizure freedom Intracranial EEG for precise seizure localization 6
2/14/2019 Resective surgery Audience Response System Tailored neocortical resection Which one of the following is generally associated with the HIGHEST rate of seizure freedom?: 84% A. Thalamic deep brain stimulation B. Addition of a fourth antiepileptic drug C. Responsive neurostimulation D. Resective surgery 5% 5% 5% 0% E. Multiple subpial transections Surgical pathology: Focal cortical dysplasia IIb Resective surgery Thalamic deep brain stim... Responsive neurostimula... Addition of a fourth anti... Multiple subpial transect... Clinical outcome: Seizure-free x 9 months, minimal deficits Confidential – 9/21/15 29 CASE 5: Audience Response System 21yo W p/w new onset shaking spells 2w s/p cesarian in the setting of severe pre-eclampsia Post-partum depression, anxiety, behavioral changes At this point, the MOST likely diagnosis is: Placed on risperidone for post-partum psychosis 37% Interactive between frequent episodes of shaking A. Eclampsia B. Psychogenic nonepileptic Work-Up: 24% 22% CT, MRI normal; CTX, Vanc, Flagyl, Acyclovir, Dilantin; LP normal spells 10% C. Seizure provoked by 8% Additional history: Keppra added; noted to be increasingly sedated so Dilantin weaned. antipsychotics Risperidone stopped due to abnormal buccal movements. D. Paraneoplastic encephalitis Eclampsia Paraneoplastic encephalitis New onset epilepsy Psychogenic nonepileptic... Seizure provoked by anti... Transferred to UCSF for Video-monitoring to diagnose spells. E. New onset epilepsy Confidential – 9/21/15 33 7
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