EEG in the Evaluation of Epilepsy. Douglas R. Nordli, Jr., MD
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1 EEG in the Evaluation of Epilepsy Douglas R. Nordli, Jr., MD
2 Contents Epidemiology First seizure Positive predictive value Risk of recurrence Identifying epilepsy Type of epilepsy (background and IEDs) Determining etiology Metabolic disorders Focal structural lesions Assessing treatment Completeness of control Determining when to stop
3 Sensitivity of EEG in Patients with Epilepsy Three large adult studies [(Goodin (1990), Marsan (1970), Salinsky (1987)] Initial EEGs: 29-55% Repeated EEGs: 80-90% Factors Age Epilepsy syndrome Sleep Sleep deprivation Proximity to seizure
4 Patients with First Unprovoked Seizure Study N Age % IED % non-ied (abnormal) % Normal Hopkins 408 Adult 27% 27% 46% FIRST 397 Mixed 50% 6% 44% Van Donselaar 157 Adult 12% 45% 43% King 300 Mixed 43% 25% 32% Shinnar 283 Pediatric 28% 10% 63% Pedley, Mendiratta, Walczak. Current Practice of Clinical Electroencephalography, Lippincott Williams and Wilkins, 2003.
5 Prevalence of IEDs in Normal Subjects Study N Age, yrs General Condition % with IEDs % Developing Seizures Eeg-Olofson Highly screened 1.9% Cavazzuti 3, Screened 3.5% 5.3% Gregory 13, Flight crew 0.5% 2.6% Zivin 6, Inpatient 2.2% 14.1% Bridgers 3, Psychiatry 2.6% Pedley, Mendiratta, Walczak. Current Practice of Clinical Electroencephalography, Lippincott Williams and Wilkins, 2003.
6 Positive Predictive Value (PPV) Ratio Calculation Epilepsy pts with IEDs : Total population with IEDs E x IEDe where (E x IEDe) + [(I-E) x IEDne] E = epilepsy prevalence IEDe = IED prevalence in epilepsy pts IEDne = IED prevalence in non-epilepsy population
7 What is PPV in Population of 1000? E 0.5% IEDe 55% IEDne 4% Calculation.005 x.55 (.005 x.55) + [(1-.005) x.04]
8 What is PPV in Population of 1000? E IEDe IEDne Ratio PPV 0.5% 55% 4% n /43 7% Calculation.005 x.55 (.005 x.55) + [(1-.005) x.04]
9 What is PPV in Population of 1000? E IEDe IEDne Ratio PPV 0.5% 55% 4% n /43 7% 50% 55% 4% n /295 93% Calculation.005 x.55 (.005 x.55) + [(1-.005) x.04]
10 Borusiak et al, Epilepsia 2010 Children with Head Trauma
11 Millichap et al. J Child Neurol 2010 Children with ADD
12 Shinnar et al Risk of Recurrence
13 Shinnar et al Risk of Recurrence
14 Shinnar et al Risk of Recurrence
15 Utility of EEG in Epilepsy
16 Utility of EEG: Case 1 10 year-old boy Single nocturnal convulsion Normal development Normal neurological examination No family history of epilepsy No prior trauma, infection, or febrile seizure
17 Scenario A F7-Ref T3-Ref Scenario B T5-Ref 200 uv 1 sec FP1-Ref F3-Ref C3-Ref P3-Ref O1-Ref
18 Five Epilepsy Patterns NAME EEG BACKGROUND SPIKES GENETICS GENETIC TESTING CLINICALLY USEFUL OUTCOME 1. FAMILIAL EPILEPSIES Normal NONE Autosomal Dominant +/- Mostly Favorable 2. GENETIC GENERALIZED EPILEPSIES NORMAL GENERALIZED AND STEREOTYPED SPIKES ARE STRONGLY GENETIC; EPILEPSY ABOUT 30% NO VERY FAVORABLE, THOUGH SOME REQUIRE LONG RX 3. SELF-LIMITED EPILEPSY WITH FOCAL STEREOTYPED SPIKES NORMAL FOCAL OR MULTIFOACL AND STEROTYPED SPIKES ARE OFTEN AD; EPILEPSY LESS THAN 5% NO VERY FAVORABLE 4a. EPILEPTOGENIC ENCEPHALOPATHIES SLOWED MULTIFOCAL AND PLEOMOPRHIC MIXED YES UNFAVORABLE 4b. SEVERE EPILEPTIC ENCEPHALOPATHIES SLOWED, DISORGANIZED, AND DISCONTINUOUS MULTIFOCAL AND PLEOMOPRHIC USUALLY DE NOVO, RARELY RECESSIVE YES SEVERE 5. FOCAL STRUCTURAL FOCAL SLOWING/ATTENUATIO N FOCAL PLEOMORPHIC NONE NO MIXED
19 Electroclinical Syndromes EEG FEAUTURES NEONATAL INFANCY CHILDHOOD ADOLESCENCE 1. Normal - Benign familial neonatal epilepsy -Benign familial infantile epilepsies -Autosomal dominant nocturnal frontal lobe epilepsy -AD with auditory features -AD familial temporal lobe 2. Generalized stereotyped spikes; normal background - No recognized syndromes -Myoclonic infancy -**Febrile Seizures** - Myoclonic atonic epilepsy -Childhood absence epilepsy -Epilepsy with myoclonic absence -Jeavons - Juvenile absence -Juvenile myoclonic -Epilepsy with GTCs 3. Focal/Multifocal Stereotyped Spikes; Normal Background - No recognized syndromes - **Febrile Seizures** -Panayiotopoulos syndrome -Roladic Epilepsy -Late onset occipital (Gastaut) -With frontal foci 4a. Multifocal Spikes; Background Slowing - No recognized syndromes - Dravet; EFMR - Migrating focal seizures - Non-Progressive Myoclonic Status -Landau Kleffner Syndrome -Continuous Spike Wave Sleep -Progressive Myoclonus Epilepsies 4b. Multifocal spikes, discontinuity, background slowing - EME (Aicardi) - EIEE (Ohtahara) -West syndrome -Late Infantile Epileptic Encephlopathy -Lennox-Gastaut Syndrome Nonsyndromic Epilepsies 5. Focal pleomorphic spikes; focal slowing/attenuation -Epilepsies due to focal structural lesions -Can have homotopic EEG foci
20 Electroclinical Syndromes AGE EEG FEAUTURES NEONATAL INFANCY CHILDHOOD ADOLESCENCE 1. Normal - Benign familial neonatal epilepsy -Benign familial infantile epilepsies -Autosomal dominant nocturnal frontal lobe epilepsy -AD with auditory features -AD familial temporal lobe 2. Generalized stereotyped spikes; normal background 3. Focal/Multifocal Stereotyped Spikes; Normal Background 4a. Multifocal Spikes; Background Slowing - No recognized syndromes - No recognized syndromes - No recognized syndromes -Myoclonic epilepsy infancy -**Febrile Seizures** - **Febrile Seizures** - Dravet; EFMR - Migrating focal seizures - Non-Progressive Myoclonic Status - Myoclonic atonic epilepsy -Childhood absence epilepsy -Epilepsy with myoclonic absence -Jeavons -Panayiotopoulos syndrome -Benign Epilepsy Centro-Temporal Spike -Late onset occipital (Gastaut) -Landau Kleffner Syndrome -Continuous Spike Wave Sleep - Juvenile absence epilepsy -Juvenile myoclonic epilepsy -Epilepsy with GTCs -With frontal foci -Progressive Myoclonus Epilepsies 4b. Multifocal spikes, discontinuity, background slowing - EME (Aicardi) - EIEE (Ohtahara) -West syndrome -Late Infantile Epileptic Encephlopathy -Lennox-Gastaut Syndrome Nonsyndromic Epilepsies 5. Focal pleomorphic spikes; focal slowing/attenuation -Epilepsies due to focal structural lesions -Can have homotopic EEG foci
21 Electroclinical Syndromes EEG FEAUTURES NEONATAL INFANCY CHILDHOOD ADOLESCENCE 1. Normal - Benign familial neonatal epilepsy -Benign familial infantile epilepsies -Autosomal dominant nocturnal frontal lobe epilepsy -AD with auditory features -AD temporal lobe 2. Generalized stereotyped spikes; normal background - No recognized syndromes -Myoclonic epi infancy -**Febrile Seizures** - Myoclonic atonic epilepsy -Childhood absence epilepsy -Epilepsy with myoclonic absence -Jeavons - Juvenile absence -Juvenile myoclonic -With GTCs 3. Focal/Multifocal Stereotyped Spikes; Normal Background - No recognized syndromes - **Febrile Seizures** -Panayiotopoulos syndrome -Rolandic -Late onset occipital (Gastaut) -With frontal foci 4a. Multifocal Spikes; Background Slowing - No recognized syndromes - Dravet; EFMR - Migrating focal seizures - Non-Progressive Myoclonic Status -Landau Kleffner Syndrome -Continuous Spike Wave Sleep -Progressive Myoclonus Epilepsies 4b. Multifocal spikes, discontinuity, background slowing 5. Focal pleomorphic spikes; focal slowing/attenuation - EME (Aicardi) - EIEE (Ohtahara) -West syndrome -Late Infantile Epileptic Encephlopathy -Epilepsies due to focal structural lesions -Can have homotopic EEG foci Nonsyndromic Epilepsies -Lennox-Gastaut Syndrome F A M I L I A L
22 Five Epilepsy Patterns NAME EEG BACKGROUND SPIKES GENETICS GENETIC TESTING CLINICALLY USEFUL OUTCOME 1. FAMILIAL EPILEPSIES Normal NONE Autosomal Dominant +/- Mostly Favorable 2. GENETIC GENERALIZED EPILEPSIES NORMAL GENERALIZED AND STEREOTYPED SPIKES ARE STRONGLY GENETIC; EPILEPSY ABOUT 30% NO VERY FAVORABLE, THOUGH SOME REQUIRE LONG RX 3. SELF-LIMITED EPILEPSY WITH FOCAL STEREOTYPED SPIKES NORMAL FOCAL OR MULTIFOACL AND STEROTYPED SPIKES ARE OFTEN AD; EPILEPSY LESS THAN 5% NO VERY FAVORABLE 4a. EPILEPTOGENIC ENCEPHALOPATHIES SLOWED MULTIFOCAL AND PLEOMOPRHIC MIXED YES UNFAVORABLE 4b. SEVERE EPILEPTIC ENCEPHALOPATHIES SLOWED, DISORGANIZED, AND DISCONTINUOUS MULTIFOCAL AND PLEOMOPRHIC USUALLY DE NOVO, RARELY RECESSIVE YES SEVERE 5. FOCAL STRUCTURAL FOCAL SLOWING/ATTENUATIO N FOCAL PLEOMORPHIC NONE NO MIXED
23 Scenario A F7-Ref T3-Ref Scenario B T5-Ref 200 uv 1 sec FP1-Ref F3-Ref C3-Ref P3-Ref O1-Ref
24 Utility of EEG: Case 2 6 month-old boy Single paroxysmal event: paused, eyes horizontally deviate Normal gestation, delivery and peri-natal course Visual contact, reaches for objects, social smile
25 Scenario A Scenario B Fp1-F3 F3-C3 C3-P3 P3-O1 Fp2-F4 F4-C4 C4-P4 P4-O2 Fp1-F7 F7-T3 T3-T5 T5-O1 Fp2-F8 F8-T4 T4-T6 T6-O2 200 uv 1 sec Fz-Cz Cz-Pz
26 Five Epilepsy Patterns NAME EEG BACKGROUND SPIKES GENETICS GENETIC TESTING CLINICALLY USEFUL OUTCOME 1. FAMILIAL EPILEPSIES Normal NONE Autosomal Dominant +/- Mostly Favorable 2. GENETIC GENERALIZED EPILEPSIES NORMAL GENERALIZED AND STEREOTYPED SPIKES ARE STRONGLY GENETIC; EPILEPSY ABOUT 30% NO VERY FAVORABLE, THOUGH SOME REQUIRE LONG RX 3. SELF-LIMITED EPILEPSY WITH FOCAL STEREOTYPED SPIKES NORMAL FOCAL OR MULTIFOACL AND STEROTYPED SPIKES ARE OFTEN AD; EPILEPSY LESS THAN 5% NO VERY FAVORABLE 4a. EPILEPTOGENIC ENCEPHALOPATHIES SLOWED MULTIFOCAL AND PLEOMOPRHIC MIXED YES UNFAVORABLE 4b. SEVERE EPILEPTIC ENCEPHALOPATHIES SLOWED, DISORGANIZED, AND DISCONTINUOUS MULTIFOCAL AND PLEOMOPRHIC USUALLY DE NOVO, RARELY RECESSIVE YES SEVERE 5. FOCAL STRUCTURAL FOCAL SLOWING/ATTENUATIO N FOCAL PLEOMORPHIC NONE NO MIXED
27 Electroclinical Syndromes EEG FEAUTURES NEONATAL INFANCY CHILDHOOD ADOLESCENCE 1. Normal - Benign familial neonatal epilepsy -Benign familial infantile epilepsies -Autosomal dominant nocturnal frontal lobe epilepsy -AD with auditory features -AD familial temporal lobe 2. Generalized stereotyped spikes; normal background - No recognized syndromes -Myoclonic infancy -**Febrile Seizures** - Myoclonic atonic epilepsy -Childhood absence epilepsy -Epilepsy with myoclonic absence -Jeavons - Juvenile absence -Juvenile myoclonic -Epilepsy with GTCs 3. Focal/Multifocal Stereotyped Spikes; Normal Background - No recognized syndromes - **Febrile Seizures** -Panayiotopoulos syndrome -Roladic Epilepsy -Late onset occipital (Gastaut) -With frontal foci 4a. Multifocal Spikes; Background Slowing - No recognized syndromes - Dravet; EFMR - Migrating focal seizures - Non-Progressive Myoclonic Status -Landau Kleffner Syndrome -Continuous Spike Wave Sleep -Progressive Myoclonus Epilepsies 4b. Multifocal spikes, discontinuity, background slowing - EME (Aicardi) - EIEE (Ohtahara) -West syndrome -Late Infantile Epileptic Encephlopathy -Lennox-Gastaut Syndrome Nonsyndromic Epilepsies 5. Focal pleomorphic spikes; focal slowing/attenuation -Epilepsies due to focal structural lesions -Can have homotopic EEG foci
28 Determining Etiology Focal slowing, attenuation, or both Cortical malformations Congenital vascular lesions Tumors Peculiar fast rhythms Lissencephaly Hemimegancephaly Genetic etiologies, for example: Rett Angelman Fragile X Metabolic Disorders, for example: Maple syrup urine disease Neuronal ceroid lipofuscinosis Alper s
29 Etiology: Case 3 5 Year-Old Girl
30 Etiology: Case 4
31 Etiology: Case 5 2 year-old girl with recurrent episodes of staring, pause in behavior Clusters of epileptic spasms
32 Interictal EEG Fp1-F7 F7-T3 T3-T5 T5-O1 Fp2-F8 F8-T4 T4-T6 T6-O2 200 uv 1 sec
33
34 Assessing Treatment
35 Assessing Treatment
36 Relapse After AED Withdrawal Study Population Abnormality Risk of Relapse? Percent with Abnormality suffering relapse Medical Research Council Mixed Features analyzed individually + NR Shinnar Children Features analyzed individually + 35% Emerson Children IEDs, focal slowing + 57% Pedley, Mendiratta, Walczak. Current Practice of Clinical Electroencephalography, Lippincott Williams and Wilkins, 2003.
37 EEG in Pediatric Epilepsy Pros Relatively inexpensive Safe Equipment widely available Results have been well-studied Can be repeated Useful in a wide variety of clinical situations Cornerstone of epilepsy syndrome Cons Technically demanding Requires expertise to interpret correctly Results often are etiologically non-specific
38 Role of EEG in Epilepsy Risk recurrence Excluding non-epileptic causes Establish epilepsy syndrome Etiology Assessing treatment Contributes to decision to stop treatment Single most useful test for epilepsy
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