Clinical Guide to Epileptic Syndromes and their Treatment (eBook)

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2010 | 2nd ed. 2010
XIV, 578 Seiten
Springer London (Verlag)
978-1-84628-644-5 (ISBN)

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Clinical Guide to Epileptic Syndromes and their Treatment -  C. P. Panayiotopoulos
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Affecting 4 percent of children and 1-2 percent of the general population, epilepsy is one of the most common neurological disorders. The 1st edition of this guide proved to be the only one of its kind, covering many important aspects of diagnosis and treatment. Due to the continued advances being made in the subject, and building on the sell-out success of the 1st edition this thorough revision reflects the latest report of the ILAE classification core group and the significant progress made in the diagnosis, classification and treatment of the epilepsies.



Chrysostomos Panayiotopoulos is an internationally renowned leader and expert in the field of neurology. He has worked for over 15 years as a Consultant in Clinical Neurophysiology and Epilepsies at St. Thomas' Hospital in London, UK. In addition to this, he holds several honorary titles including Honorary Senior Lecturer at Guys, Kings and St. Thomas' Medical school, Honorary Consultant in Paediatric Epileptology at Guy's Department of Paediatric Neurology, London. He has also been an invited professor to Harvard University and the University of Western Australia, where he was appointed Honorary Director of the Department of Neuropathology.

Dr. Panayiotopoulos has researched prolifically, investigating the clinical and neurophysiological investigations of the muscles, peripheral and central nervous system, with a particular focus on epilepsy. Moreover he has successfully secured more than a quarter of a million pounds of research funding.

Dr Panayiotopoulos has been an active contributor to a number of highly established peer-reviewed journals. He has written over 136 articles in English, in journals such as the Lancet, Brain, Neurology, Annals of Neurology and Archives of Neurology to name but a few. These papers have been cited more than 1000 times in leading journals such as the Lancet, Nature, Neurology and Muscle and Nerve. He has also published over 50 articles in Greek. He has been the invited author of over 44 chapters in neurology books. He has also sat on the editorial board of 'Annals of Saudi Medicine' and acted as a reviewer for: 'Brain', 'Journal of Neurology, Neurosurgery and Psychiatry', 'Muscle and Nerve', 'Epilepsia', 'Lupus', 'Journal of Electroencephalography and Clinical Neurophysiology', 'Epilepsy Research', and 'Seizure'.

Moreover his work on establishing the syndromic diagnosis of epilepsies and the appropriate video-EEG methodology for their diagnosis, has been regarded by some as ground breaking and he is recognised as one the leading authorities on epilepsy. He was appointed as one of the 12 members of the Commission on Classification and Terminology of the International League Against Epilepsy (ILAE) and subsequently as a key advisory member.

Dr. Panayiotopoulos is now Consultant emeritus in the Department of Clinical Neurophysiology and Epilepsies at St. Thomas' hospital, London and also has a consultancy with USB pharma, Brussels, Belgium. He is married with two children.

Author of:

Benign childhood partial seizures and related epileptic syndromes London: John Libbey and Co. (1999))

Early-onset benign childhood occipital seizure susceptibility syndrome: a syndrome to recognise. Epilepsia 1999: 40:621-630


A disorder which affects 4 percent of children and 1-2 percent of the general population, epilepsy is one of the most common neurological disorders. The original edition of C.P. Panayiotopoulos' guide proved to be the only one of its kind, covering many important aspects of diagnosis and treatment. Due to the continued advances being made in the subject, a second edition is now due. The text includes detailed diagnostics, concise descriptions of syndromes, a wealth of practical tips, and is supported by useful tables to enable clear diagnoses.Building on the sell-out success of the first edition this thorough revision reflects the latest report of the ILAE classification core group and the significant progress made in the diagnosis, classification and treatment of the epilepsies.This new edition is indispensable reading for all those who care for patients with epileptic seizures.

Chrysostomos Panayiotopoulos is an internationally renowned leader and expert in the field of neurology. He has worked for over 15 years as a Consultant in Clinical Neurophysiology and Epilepsies at St. Thomas’ Hospital in London, UK. In addition to this, he holds several honorary titles including Honorary Senior Lecturer at Guys, Kings and St. Thomas’ Medical school, Honorary Consultant in Paediatric Epileptology at Guy’s Department of Paediatric Neurology, London. He has also been an invited professor to Harvard University and the University of Western Australia, where he was appointed Honorary Director of the Department of Neuropathology. Dr. Panayiotopoulos has researched prolifically, investigating the clinical and neurophysiological investigations of the muscles, peripheral and central nervous system, with a particular focus on epilepsy. Moreover he has successfully secured more than a quarter of a million pounds of research funding. Dr Panayiotopoulos has been an active contributor to a number of highly established peer-reviewed journals. He has written over 136 articles in English, in journals such as the Lancet, Brain, Neurology, Annals of Neurology and Archives of Neurology to name but a few. These papers have been cited more than 1000 times in leading journals such as the Lancet, Nature, Neurology and Muscle and Nerve. He has also published over 50 articles in Greek. He has been the invited author of over 44 chapters in neurology books. He has also sat on the editorial board of "Annals of Saudi Medicine" and acted as a reviewer for: "Brain", "Journal of Neurology, Neurosurgery and Psychiatry", "Muscle and Nerve", "Epilepsia", "Lupus", "Journal of Electroencephalography and Clinical Neurophysiology", "Epilepsy Research", and "Seizure". Moreover his work on establishing the syndromic diagnosis of epilepsies and the appropriate video-EEG methodology for their diagnosis, has been regarded by some as ground breaking and he is recognised as one the leading authorities on epilepsy. He was appointed as one of the 12 members of the Commission on Classification and Terminology of the International League Against Epilepsy (ILAE) and subsequently as a key advisory member. Dr. Panayiotopoulos is now Consultant emeritus in the Department of Clinical Neurophysiology and Epilepsies at St. Thomas’ hospital, London and also has a consultancy with USB pharma, Brussels, Belgium. He is married with two children. Author of: Benign childhood partial seizures and related epileptic syndromes London: John Libbey and Co. (1999)) Early-onset benign childhood occipital seizure susceptibility syndrome: a syndrome to recognise. Epilepsia 1999: 40:621-630

Dedication Pages 4
acknowledgements 5
Table of Contents 6
preface to the second edition 14
preface to the revised second edition 15
abbreviations 16
1 General aspects of epilepsies 18
What is epilepsy? Definitions 19
Comment on the new ILAE de.nition 19
Other formal definitions of epilepsy 19
Making the correct diagnosis in epilepsies 20
Medical history 20
‘That’s it!’ phenomenon6 21
Home-made video recordings 21
Laboratory diagnostic procedures 21
Differential diagnosis 22
First step: Are the paroxysmal events epileptic seizures? 22
Second step: What type of epileptic seizures? 24
Third step: What is their cause and what is the epileptic syndrome or disease? 25
Epilepsy or epilepsies 27
The ILAE classification of epileptic seizures and epileptic syndromes 28
On classifications: concepts,clarifications and difficulties in reaching a consensus in the classi.cation of epileptic seizures and syndromes 30
Gardeners and botanists 30
Author’s clarifications on the ILAE terminology and classifications 32
Splitters and lumpers 33
Epidemiology of epilepsies 33
Incidence and prevalence of epilepsies 33
Mortality and epilepsies 34
References 35
2 Epileptic seizures and their classification 37
The definition of epileptic seizures 37
Other useful or ILAE seizure-related terminology 40
Classification of epileptic seizures 40
Epileptic seizures in accordance with the 1981 ILEA classification 40
Partial (or focal) seizures 43
Generalised seizures 43
Seizure classification in the new ILAE Task Force reports 44
Debate on the distinction between generalised and focal seizures 47
Generalised epileptic seizures 48
Generalised tonic–clonic seizures 48
Epidemiology of GTCS 49
Clinical manifestations 49
Ictal events preceding the onset of a GTCS 49
Onset of GTCS proper 49
Tonic phase of GTCSs 49
Intermediate transitory (vibratory) phase of GTCSs 51
Clonic phase of GTCSs 51
Recovery phase of GTCSs 51
Autonomic changes of GTCS 52
Variants of GTCS 54
Complications of GTCSs 55
Ictal EEG of GTCSs 55
Aetiology of GTCS 56
Pathophysiology 57
Generalised tonic seizures 59
Clinical manifestations 59
Aetiology 59
Diagnostic tests 59
Differential diagnosis 60
Management 60
Generalised clonic seizures 60
Clinical manifestations 60
Aetiology 60
Diagnostic tests 61
Differential diagnosis 61
Management 61
Epileptic spasms 61
Clinical manifestations 61
Aetiology 61
Diagnostic tests 61
Differential diagnosis 62
Management 62
Myoclonus 62
Description of myoclonus 62
Classification of myoclonus 62
Epileptic myoclonus 62
Epileptic negative myoclonus 64
ILAE classification of epileptic myoclonus 65
Myoclonus and epileptic syndromes 66
Atonic seizures 66
Clinical manifestations 67
Aetiology 67
Diagnostic tests 67
Differential diagnosis 67
Management 67
Typical absence seizures 67
Clinical manifestations 69
Electroencephalography 70
Pathophysiology of absence seizures 70
Diagnosing absences and differential diagnosis 70
Atypical absence seizures 71
Focal epileptic seizures 73
ILAE terminology and classification of focal seizures 73
Epidemiology 75
Clinical manifestations 75
Aetiology 75
Pathophysiology 76
Diagnostic procedures 76
Prognosis 76
Management 76
Reflex epileptic seizures 77
References 77
3 Status epilepticus 80
Definition and classification 80
Generalised tonic–clonic status epilepticus 82
Physiological stages of GTC-SE 83
Compensatory phase 83
Refractory stage of GTC-SE (established GTC-SE) 84
Pharmacoresistance in GTC-SE 84
Cerebral damage and epileptogenesis 85
Epidemiology of GTC-SE 85
Aetiology 85
Differential diagnosis 86
Prognosis 86
Generalised tonic status epilepticus 86
Generalised myoclonic status epilepticus 86
Idiopathic myoclonic SE 87
Symptomatic/cryptogenic myoclonic SE 87
Absence status epilepticus 87
Typical absence (idiopathic absence) SE 88
Impairment of consciousness, memory and higher cognitive functions 88
Behavioural abnormalities and experiential phenomena 90
Myoclonic jerks in idiopathic absence SE 90
GTCSs associated with idiopathic absence SE 90
Duration and frequency of idiopathic absence SE 90
Post-ictal state 90
Age at onset and sex 90
Precipitating factors 90
Differential diagnosis 91
Atypical absence SE 91
Obtundation SE 91
Situation-related and de novo absence SE 92
Focal status epilepticus 92
Epilepsia partialis continua 92
Supplementary motor area SE 92
Aura continua 93
Dyscognitive focal (psychomotor, complex partial) SE 93
Clarifications on nomenclature 93
Clinical manifestations 94
Prevalence 94
Aetiology and prognosis 94
Differential diagnosis 94
Hippocampal SE 94
Clinical manifestations 95
Electroencephalography 96
Focal SE of frontal lobe origin 96
Focal SE of occipital lobe origin 96
Focal SE of parietal lobe origin 96
Autonomic SE 96
Definition 96
Clinical manifestations 97
Opercular SE 97
Management of status epilepticus 97
Management of GTC-SE and other convulsive SE 97
Out of hospital management 97
Hospital emergency management of GTC-SE 98
General emergency management 99
Pharmacological treatment of GTC-SE 99
Absence SE 101
Self-administration of medication 101
Hospital management 101
Focal simple and complex SE 101
Antiepileptic drugs in the treatment of status epilepticus 102
Benzodiazepines 102
Phenytoin sodium 104
Fosphenytoin 104
Phenobarbital sodium 105
Paraldehyde 105
Valproate, levetiracetam and lacosamide 106
References 107
4 Imitators of epileptic seizures 111
Main types of epileptic seizures and their imitators 112
Generalised tonic–clonic seizures 112
Prodromes (non-epileptic) preceding the onset of a GTCS 113
Ictal events preceding the onset of a GTCS 114
Epileptic myoclonic jerks 114
Absence seizures 114
Tonic seizures 115
Epileptic spasms 115
Epileptic drop attacks 115
Focal epileptic seizures 115
Syncopal attacks imitating epileptic seizures 116
Neurally mediated syncope 116
Vasovagal syncope 117
Reflex anoxic seizures 117
Orthostatic syncope 118
Syncopes induced by Valsalva manoeuvre 118
Micturition syncope 118
Carotid sinus syncope 118
Cardiogenic syncopes 119
The long QT syndrome 119
Syncopes induced by drugs and electrolyte abnormalities 120
Syncopal attacks provoking epileptic seizures: Anoxic epileptic seizures 120
Epileptic seizures imitating syncope 120
Psychogenic NEPEs imitating epileptic seizures 120
Convulsive psychogenic status epilepticus 121
Suffocation in Munchausen syndrome by proxy 122
Panic attacks 122
Hyperventilation syndrome 122
Management of PNEPEs 123
Non-epileptic paroxysmal movement disorders imitating epileptic seizures 123
Tics, compulsions, stereotypies and mannerisms 124
Tremor 124
Non-epileptic movement disorders in neonates and infants imitating seizures 124
Jitteriness 124
Tonic reflex seizures of early infancy 125
Alternating hemiplegia 125
Benign paroxysmal torticollis 125
Rhythmic behavioural movements 125
Self-gratification disorder 125
Gastro-oesophageal re.ux in infants 125
Benign neonatal sleep myoclonus 126
Benign non-epileptic myoclonus of early infancy (Fejerman syndrome) 126
Hyperekplexia 127
Other non-epileptic paroxysmal movement disorders 128
Familial paroxysmal dystonic choreoathetosis 128
Non-epileptic paroxysmal kinesigenic choreoathetosis 128
Episodic ataxia type 1 129
Non-epileptic severe amnesic and confusional attacks imitating epileptic seizures 129
Transient global amnesia 130
Psychogenic (dissociative) fugues 130
Prolonged confusional states 130
Non-epileptic staring spells, childhood preoccupation and daydreaming 131
NEPEs occurring during sleep and sleep disorders 131
Classification of sleep disorders 132
Sleep-related non-epileptic movement disorders 132
Hypnagogic myoclonic jerks (sleep starts) 132
Nocturnal myoclonus 133
Fragmentary hypnic myoclonus 133
Propriospinal myoclonus 133
Benign neonatal sleep myoclonus 133
Facio-mandibular myoclonus 133
Restless legs syndrome and periodic limb movement disorder 133
Rhythmic movements in sleep 134
Sleepwalking and night terrors 134
Paroxysmal nocturnal dystonia (hypnogenic paroxysmal dystonia) 134
Sleep-related non-epileptic behavioural and other disorders 134
Sleep drunkenness 134
Bruxism 135
Catathrenia (nocturnal expiratory groaning) 135
Nocturnal enuresis 135
REM sleep behaviour disorder 135
Sleep-related eating disorder 135
Sudden infant death syndrome (Cot death) 135
Narcolepsy 135
Demographic data 136
Clinical manifestations 136
Aetiology 136
Diagnostic procedures 136
Differential diagnosis 137
Prognosis 137
Management 137
Sleep apnoea 137
Sleep disorders in the elderly 137
Subjective non-epileptic paroxysmal symptoms imitating simple focal seizures 137
Visual, auditory, somatosensory, olfactory, gustatory and autonomic paroxysmal symptoms imitating simple focal seizures 137
Cyclic vomiting, paroxysmal vertigo or motion sickness 138
Cyclic vomiting syndrome 138
Benign positional paroxysmal vertigo 139
Benign paroxysmal vertigo of childhood 139
Motion sickness 139
Migraine, migralepsy, basilar migraine with EEG occipital paroxysms and diagnostic errors 139
Visual aura of migraine 140
Basilar migraine 140
Migralepsy 141
Visual seizures 143
Differentiating visual seizures from migraine 143
Cerebrovascular NEPEs imitating epileptic seizures 144
References 144
5 Epileptic syndromes and their classification 149
ILAE definitions of epilepsies and epileptic syndromes and clarifications of terminology 149
Epileptic syndrome 150
Epilepsy disease 150
Idiopathic epilepsy syndrome 150
Symptomatic epilepsy syndrome 151
Cryptogenic or probably symptomatic epilepsy syndrome 151
Benign epilepsy syndrome 152
Genetic epilepsy 153
The ILAE 1989 classification of epileptic syndromes 153
Localisation-related (focal) epilepsies and syndromes 154
Generalised epilepsies and syndromes 155
Epilepsies and syndromes undetermined as to whether they are focal or generalised 155
Special syndromes 157
Epilepsies with seizures precipitated by speci.c modes of activation 157
Syndromic classification of the new diagnostic ILAE scheme 157
References 160
6 EEG and brain imaging 161
EEG in the diagnosis and management of epilepsies 161
The value of routine inter-ictal or ictal EEGs in epilepsies 161
Reasons why the EEG should not be undervalued 161
Reasons why the EEG should not be overvalued 164
Sources of error in EEGs 164
Physiological activators of EEG abnormalities 166
Drowsiness, sleep and awakening 166
Hyperventilation 168
Intermittent photic stimulation 168
Other forms of appropriate activation of reflex seizures 168
Video-EEG recording should be made routine clinical practice 169
The role of EEG technologists 169
The significance of the EEG after the .rst afebrile seizure 169
Value of EEG in stopping AED treatment 169
Improving the EEG contribution to the diagnosis of epilepsies 170
Illustrative cases of good EEG practice 171
Normal routine EEGs 171
Abnormal EEGs 171
Brain imaging in the diagnosis and management of epilepsies 173
Magnetic resonance imaging 173
X-ray computed tomography 175
Functional neuroimaging 176
Recommendations for neuroimaging of patients with epilepsy 178
Ideal practice 178
Minimum standards 178
Functional neuroimaging in clinical practice 178
Functional MRI 178
Magnetic resonance spectroscopy 178
Single photon emission CT 180
PET with [18F].uorodeoxyglucose and 15O-labelled water 180
PET with specific ligands 180
Co-registration of SPECT/PET with MRI 180
Magnetoencephalography 182
References 184
7 Principles of therapy in the epilepsies 186
AED prophylactic treatment 186
Clarifications on terminology of AED treatment 189
Choosing the best AED option 190
Seizure specificity 190
Strength of efficacy 190
Spectrum of efficacy 190
Safety, tolerability and adverse reactions 191
Clinical pharmacokinetics 191
Hepatic enzyme induction and inhibition 192
Carbonic anhydrase inhibitors 194
Pharmacodynamics 194
Drug–drug interactions 194
Mechanisms of action 194
Need for as little titration as possible 195
Need for less laboratory testing and other monitoring 195
Frequency of administration and ease of use 196
Cost of treatment 197
Newest generation AEDs licensed in the treatment of focal epilepsies: lacosamide and eslicarbazepine 197
Newly diagnosed epilepsy 198
Starting AED treatment in newly diagnosed epilepsy 198
Monotherapy 199
Rational polytherapy 200
Converting from polytherapy to monotherapy 201
Total AED withdrawal 201
Generic versus brand or generic to generic AED prescribing 202
Evidence for AED treatment recommendations in clinical practice 203
Evidence-based recommendations 203
Poverty of reliable RCTs in epilepsies 205
Evidence-based recommendations in this book 206
Therapeutic drug monitoring 206
Plasma and saliva TDM 208
Clinical applications 209
Time of sampling 209
Considerations of adverse antiepileptic drug reactions in the treatment of epilepsies 210
Life-threatening ADRs 210
Common CNS-related ADRs 211
Behavioural and psychiatric ADRs to AED 212
Non-CNS ADRs of AEDs 213
Adverse cardiac effects of AEDs 213
Principles of management in women with epilepsies 217
Oral hormonal contraception and AEDs 217
Effect of AEDs on oral hormonal contraception 217
Effect of oral hormonal contraception on AEDs 218
Pregnancy 218
Teratogenicity 218
Commonly used older generation AEDs and major congenital malformations 219
Commonly used new generation of broad spectrum AEDs and major congenital malformations 220
The AAN/AES assessments of AEDrelated MCMs 222
Foetal anticonvulsant syndrome and minor anomalies 222
Post-natal cognitive effects of foetal exposure to AEDs 223
Change in seizure frequency and status epilepticus during pregnancy 223
Seizure deterioration in pregnancy due to changes in plasma AED concentrations 224
Effect of seizures on mother and the unborn baby 226
Obstetric and other pregnancy-related complications 226
Adverse perinatal outcomes 227
Folic acid supplementation for the prevention of major congenital malformations 227
Breastfeeding and AEDs 227
Principles of AED therapy in women of childbearing age in clinical practice 228
Commencing AED therapy in women of childbearing age with newly diagnosed focal epilepsy 229
Commencing AED therapy in women of childbearing age with newly diagnosed generalised epilepsy 230
Preconception 231
Principles of management in the elderly with epileptic seizures 232
Difficulties in diagnosing epileptic seizures in the elderly 232
Frequency of seizures and their severity in the elderly 233
EEG and other investigative procedures in the elderly 233
Physiological and other changes in the elderly that may affect AEDs 234
Principles of AED treatment in the elderly 234
Surgery for epilepsies 235
Criteria for surgical referral 236
Drug-resistant epilepsy for the purposes of surgical referral 236
The concept of surgically remediable epileptic syndromes 236
Strategy of a surgical work-up 237
Types of surgical procedures 237
Curative (definitive) surgery 237
Focal resective procedure (lesionectomy) 237
Cerebral hemispherectomy 238
Palliative (functional) surgery 238
Corpus callosotomy 238
Multiple subpial transections 238
Vagus nerve stimulation 239
Efficacy 239
Adverse reactions 239
Technical aspects 239
Surgical procedure and cost 239
What is the place of VNS in the treatment of epilepsies? 240
Ketogenic diet 241
Indications and efficacy 241
Rationale and types of ketogenic diet 241
The Atkins diet 241
Adverse effects of the ketogenic diet 241
Corticosteroids in the treatment of childhood epilepsies 242
Preparation, doses and regimens of corticosteroids 242
Adverse effects and monitoring of corticosteroid treatment 242
References 242
8 Neonatal epileptic seizures and neonatal epileptic syndromes 249
Neonatal epileptic seizures 249
Clarifications on classification and terminology 249
Demographic data 249
Clinical manifestations 250
Subtle seizures 250
Motor seizures 250
Autonomic ictal manifestations 252
Duration of neonatal seizures 252
Non-epileptic neonatal seizures 252
Aetiology 252
Pathophysiology 254
Diagnostic procedures 254
Brain imaging 254
Electroencephalography 254
Inter-ictal EEG 254
Ictal EEG 255
Electroclinical dissociation or decoupling response 255
Electrical seizure patterns of usually poor prognosis 257
Post-ictal EEG 257
Differential diagnosis 257
Prognosis 257
Management 258
Do electrographic (electrical) seizures need treatment? 259
Neonatal epileptic syndromes 259
Clarifications on classification 260
Benign familial neonatal seizures 260
Demographic data 260
Clinical manifestations 260
Aetiology 260
Diagnostic procedures 261
Genetic testing 261
Electroencephalography 261
Differential diagnosis 261
Prognosis 261
Management 262
Benign neonatal seizures (non-familial) 262
Clarifications on classification 262
Demographic data 262
Clinical manifestations 262
Aetiology 262
Diagnostic procedures 263
Electroencephalography 263
Differential diagnosis 263
Prognosis 264
Management 264
Early myoclonic encephalopathy 264
Clarifications on classification 264
Demographic data 264
Clinical manifestations 264
Aetiology 265
Pathophysiology 265
Diagnostic procedures 265
Electroencephalography 266
Differential diagnosis 266
Prognosis 266
Management 266
Ohtahara syndrome 266
Clarifications on classification 266
Demographic data 267
Clinical manifestations 267
Aetiology 267
Pathophysiology 267
Diagnostic procedures 267
Electroencephalography 267
Differential diagnosis 268
Prognosis 268
Management 268
References 269
9 Idiopathic epileptic seizures and syndromes in infancy 271
Febrile seizures 271
Clarifications on classification 271
Demographic data 272
Clinical manifestations 272
Seizures 272
Risk factors of a first febrile seizure 273
Risk factors for recurrence 273
Aetiology 273
Pathophysiology 274
Diagnostic procedures 274
Electroencephalography 274
Differential diagnosis 274
Prognosis 275
Intellectual and behavioural outcome 276
Management 276
Acute management of a child with a febrile seizure 276
Prophylactic management 276
Supportive family management 277
Epilepsy with febrile seizures plus 277
Clarifications on classification 277
Demographic data 277
Clinical manifestations 277
Aetiology 278
Diagnostic procedures 278
Differential diagnosis 278
Prognosis 279
Management 279
Benign infantile seizures 279
Considerations on classification 279
Demographic data 279
Clinical manifestations 279
Aetiology 279
Diagnostic procedures 280
Electroencephalography 280
Differential diagnosis 281
Prognosis 281
Management 281
Myoclonic epilepsy in infancy 281
Clarifications on classification 281
Demographic data 281
Clinical manifestations 281
Precipitating factors 283
Aetiology 283
Diagnostic procedures 283
Differential diagnosis 283
Prognosis 283
Management 284
References 284
10 Epileptic encephalopathies in infancy and early childhood 287
Clarifications on classification 288
West syndrome 288
Clarifications on classification 288
Demographic data 288
Clinical manifestations 288
Aetiology 290
Diagnostic procedures 291
Electroencephalography 292
Ictal EEG 292
Differential diagnosis 294
Prognosis 294
Management 295
Dravet syndrome 295
Clarifications on classification 295
Demographic data 295
Clinical manifestations 296
Seizure-precipitating factors 297
Aetiology 297
Diagnostic procedures 297
Electroencephalography 298
Differential diagnosis 298
Prognosis 298
Management 299
Lennox–Gastaut syndrome 299
Clarifications on classification 299
Demographic data 300
Clinical manifestations 300
Aetiology 305
Pathophysiology 305
Diagnostic procedures 305
Electroencephalography 306
Differential diagnosis 306
Prognosis 308
Management 310
AED treatment 310
Older AEDs 311
Newer AEDs 311
Hormonal and other non-AED treatment 313
Non-pharmacological treatments 313
Treatment of status epilepticus in Lennox-Gastaut syndrome 314
Treatment-induced aggravation of seizures, cognition and behaviour 314
Attention to seizure precipitants 314
Educational management 315
Landau–Kleffner syndrome 315
Considerations on classification 315
Demographic data 316
Clinical manifestations 316
Linguistic abnormalities 316
Cognitive and behavioural abnormalities 316
Seizures 316
Aetiology 317
Pathophysiology 317
Diagnostic procedures 318
Electroencephalography 318
Differential diagnosis 318
Prognosis 318
Management 320
Epileptic encephalopathy with continuous spike-and-wave during sleep 321
Clarifications on classification 321
Demographic data 321
Clinical manifestations 321
Aetiology 322
Pathophysiology 323
Diagnostic procedures 323
Electroencephalography 323
Differential diagnosis 324
Prognosis 325
Management 325
Myoclonic encephalopathy in non-progressive disorders 325
Clarifications on classification 326
Demographic data 326
Clinical manifestations 326
Aetiology 326
Pathophysiology 326
Diagnostic procedures 326
Electroencephalography 326
Differential diagnosis 327
Prognosis 327
Management 327
Atypical benign partial epilepsy of childhood 327
Clarifications on nomenclature 327
Demographic data 327
Clinical manifestations 327
Diagnostic procedures 328
Differential diagnosis 328
Prognosis 328
Management 328
Hypothalamic epilepsy 329
Clarifications on classification 329
Demographic data 329
Clinical manifestations 329
Aetiology 330
Pathophysiology 330
Diagnostic procedures 330
Electroencephalography 331
Differential diagnosis 331
Prognosis 332
Management 332
References 333
11 Severe neocortical epileptic syndromes in infancy and childhood 339
Kozhevnikov–Rasmussen syndrome 339
Clarifications on nomenclature 339
The formal ILAE nomenclature 339
Historical facts 340
Misconceptions 340
Demographic data 340
Clinical manifestations 340
Aetiology 341
Diagnostic procedures 342
Electroencephalography 342
Differential diagnosis 342
Prognosis 344
Management 344
Migrating focal seizures of infancy 344
Considerations on classification 344
Demographic data 344
Clinical manifestations 344
Aetiology 345
Diagnostic procedures 345
Electroencephalography 345
Prognosis 345
Management 345
Hemiconvulsion–hemiplegia epilepsy 345
Considerations on classification 346
Demographic data 346
Clinical manifestations 346
Aetiology 346
Diagnostic procedures 346
Electroencephalography 346
Prognosis 346
Management 347
References 347
12 Benign childhood focal seizures and related epileptic syndromes 350
Considerations on classification 350
Benign childhood epilepsy with centrotemporal spikes 351
Considerations on nomenclature 351
Demographic data 351
Clinical manifestations 352
Status epilepticus 352
Aetiology 353
Diagnostic procedures 353
Electroencephalography 353
Evolution and prognosis 357
Management 357
Panayiotopoulos syndrome 358
Considerations on classification 358
Demographic data 358
Clinical manifestations 358
Conventional seizure symptoms 360
Aetiology 361
Pathophysiology 361
Diagnostic procedures 362
Electroencephalography 362
Magnetoencephalography 366
Differential diagnosis 366
Prognosis 367
Management 367
Idiopathic childhood occipital epilepsy of Gastaut 368
Considerations on classification 368
Demographic data 368
Clinical manifestations 368
Aetiology 369
Pathophysiology 369
Diagnostic procedures 370
Electroencephalography 370
Differential diagnosis 373
Differentiating ICOE-G from PS 373
Prognosis 374
Management 374
Other phenotypes of BCSSS 374
Benign childhood seizures with affective symptoms 374
Demographic data 374
Clinical manifestations 374
Aetiology 375
Diagnostic procedures 375
Electroencephalography 375
Prognosis 375
Management 375
Benign childhood epilepsy with parietal spikes and frequent ESESs 375
Benign childhood focal seizures associated with frontal or midline spikes 375
Benign infantile focal epilepsy with midline spikes and waves during sleep 375
Benign childhood seizure susceptibility syndrome: A unified concept 376
Febrile seizures and BCSSS 377
Benign (isolated) focal seizures of adolescence 378
Considerations on classification 378
Demographic data 379
Clinical manifestations 379
Diagnostic procedures 379
Differential diagnosis 379
Prognosis and management 379
Management of benign childhood focal seizures 379
Acute management of a child with prolonged seizures 380
Prophylactic AED treatment of benign childhood focal seizures 380
Stopping medication 381
Parental attitude and education 381
By Thalia Valeta 381
References 382
13 Idiopathic generalised epilepsies 387
Considerations on classification 387
Syndromes of IGE recognised by the ILAE 388
Epilepsy with myoclonic–astatic seizures 388
Considerations on classification 389
Demographic data 389
Clinical manifestations 390
Aetiology 392
Diagnostic procedures 392
Electroencephalography 392
Differential diagnosis 392
Prognosis 393
Management 393
Childhood absence epilepsy 393
Considerations on classification 393
Demographic data 396
Clinical manifestations 396
Aetiology 396
Diagnostic procedures 396
Electroencephalography 396
Differential diagnosis 397
Prognosis 398
Management 398
Epilepsy with myoclonic absences 398
Considerations on classification 398
Demographic data 399
Clinical manifestations 399
Aetiology 399
Diagnostic procedures 399
Electroencephalography 399
Differential diagnosis 399
Prognosis 399
Management 399
Juvenile absence epilepsy 401
Considerations on classification 401
Demographic data 402
Clinical manifestations 402
Seizure-precipitating factors 402
Aetiology 402
Diagnostic procedures 402
Electroencephalography 402
Differential diagnosis 403
Prognosis 403
Management 404
Juvenile myoclonic epilepsy 404
Demographic data 404
Clinical manifestations 405
Seizure-precipitating factors 406
Aetiology 408
Diagnostic procedures 409
Electroencephalography 409
Differential diagnosis 409
Prognosis 410
Management 410
Pharmacological treatment 410
Prevention of GTCSs and termination of myoclonic and absence status epilepticus 411
Duration of AED treatment and withdrawal of medication 411
Epilepsy with GTCSs only 411
Considerations on classification 411
Demographic data 412
Clinical manifestations 412
Seizure-precipitating factors 412
Aetiology 412
Diagnostic procedures 412
Electroencephalography 412
Differential diagnosis 413
Prognosis 413
Management 413
Other probable syndromes of IGE to consider 414
IGE with absences of early childhood 414
Perioral myoclonia with absences 415
Considerations on classification 415
Demographic data 415
Clinical manifestations 415
Aetiology 415
Diagnostic procedures 415
Electroencephalography 415
Differential diagnosis 417
Prognosis 417
Management 417
IGE with phantom absences 417
Considerations on classification 417
Demographic data 418
Clinical manifestations 418
Aetiology 419
Diagnostic procedures 419
Electroencephalography 419
Differential diagnosis 419
Prognosis 419
Management 419
Autosomal dominant cortical tremor, myoclonus and epilepsy 420
Epidemiology 420
Aetiology 420
Clinical manifestations 420
Diagnostic procedures 420
Prognosis 420
Differential diagnosis 420
Management 421
AED treatment of IGEs 421
Treatment of newly diagnosed IGEs 423
IGEs: RCTs and evidence- based recommendations 423
Older AEDs in IGEs 424
Newer AEDs useful in IGEs 425
Management of patients with difficult to treat IGEs 425
Drug withdrawal 426
Management of status epilepticus in IGEs 426
References 426
14 Familial (autosomal dominant) focal epilepsies 432
Autosomal dominant nocturnal frontal lobe epilepsy 435
Demographic data 435
Clinical manifestations 435
Circadian distribution 436
Precipitating factors 436
Aetiology 436
Diagnostic procedures 436
Prognosis 436
Differential diagnosis 436
Management 436
Familial (autosomal dominant) temporal lobe epilepsy 437
Familial mesial temporal lobe epilepsy 437
Demographic data 437
Clinical manifestations 437
Aetiology 438
Diagnostic procedures 438
Electroencephalography 438
Differential diagnosis 438
Prognosis 438
Management 439
Familial lateral temporal lobe epilepsy 439
Demographic data 439
Clinical manifestations 439
Aetiology 439
Diagnostic procedures 439
Prognosis 439
Differential diagnosis 439
Management 440
Familial focal epilepsy with variable foci 440
Demographic data 440
Clinical manifestations 440
Aetiology 440
Diagnostic procedures 440
Electroencephalography 440
Prognosis 440
Management 440
Other possible familial (autosomal dominant) focal epilepsies not yet recognised 441
Autosomal dominant rolandic epilepsy and speech dyspraxia 441
Focal epilepsy with pericentral spikes 441
References 442
15 Symptomatic and cryptogenic (probably symptomatic) focal epilepsies 444
Symptomatology and classification 444
Temporal lobe epilepsies 445
Semiology of TLE 445
Subjective ictal clinical manifestations 445
Ascending epigastric aura 446
Fear 446
Complex internal sensations (experiential, mental, intellectual or psychic symptoms, dreamy states) 447
Déjà vu 448
Jamais vu, jamais entendu and jamais veçu 449
Auditory hallucinations and illusions 449
Olfactory and gustatory hallucinations 449
Visual hallucinations and illusions 450
Other subjective ictal manifestations 450
Objective ictal symptoms 450
Automatisms 450
Definitions of automatisms 451
Autonomic disturbances and ictus emeticus 452
Language and speech ictal disturbances 452
Motor arrest, staring and temporal lobe absence 452
Motor manifestations 453
Gelastic seizures of TLE 453
Amnesic seizures 453
Catamenial temporal lobe seizures 453
Post-ictal symptoms 454
Differentiating temporal lobe seizures from other extratemporal seizures on the basis of post-ictal symptoms 454
Mesial TLE with hippocampal sclerosis 454
Clarifications on classification 454
Demographic data 456
Clinical manifestations 456
Simple focal seizures 457
Complex focal seizures 457
Lateralising signs of ictal and post-ictal symptoms 457
GTCSs 458
Post-ictal symptoms 458
Complex focal status epilepticus of temporal lobe origin 458
Neurological, mental state and behaviour 458
Febrile convulsions and other initial precipitating events 458
Aetiology 458
Hippocampal sclerosis 459
Hippocampal sclerosis and temporal lobe epilepsy: cause or consequence? 459
Pathophysiology 459
Diagnostic procedures 459
Inter-ictal EEG 460
Ictal EEG 460
Differential diagnosis 460
Prognosis 461
Neurosurgical series 461
Community studies 461
Management 462
AED treatment 462
Neurosurgical treatment 462
MTLE defined by specific aetiologies other than hippocampal sclerosis 463
Lateral temporal lobe epilepsy 463
Clinical manifestations 463
Aetiology 464
Diagnostic procedures 464
Differential diagnosis 464
Management 464
Frontal lobe epilepsies 465
Considerations on classification 465
Demographic data 466
Clinical manifestations 467
Seizures from the motor cortex 467
Seizures from the supplementary sensorimotor area 468
Hypermotor seizures155 of bizarre bilateral, asymmetrical tonic posturing and movements 468
Somatosensory or other ill-de.ned auras (not epigastric), vocalisations and speech arrest that are common ictal manifestations 468
Vocalisations 469
Other frontal lobe seizures of particular clinical interest 469
Focal status epilepticus of frontal lobe origin 469
Aetiology 469
Diagnostic procedures 469
Differential diagnosis 470
Prognosis 470
Management 471
Epilepsia partialis continua of Kozhevnikov 471
Clarifications on classification 471
Demographic data 472
Clinical manifestations 472
Other types of seizures 473
Neurological signs and symptoms 473
Aetiology 473
Pathophysiology 473
Diagnostic procedures 473
Differential diagnosis 474
Prognosis 477
Management 477
Parietal lobe epilepsies 477
Clarifications on classification 477
Demographic data 477
Clinical manifestations 477
Somatosensory seizures 477
Disturbances of body image and somatic illusions 478
Other ictal subjective symptoms 479
Seizure spreading to extraparietal regions 479
Duration of seizures 479
Post-ictal manifestations 479
Epileptogenic localisation 479
Precipitating factors 479
Aetiology 479
Diagnostic procedures 480
Electroencephalography 480
Differential diagnosis 481
Management 481
Occipital lobe epilepsies 482
Clarifications on classification 482
Terminology 482
Demographic data 482
Clinical manifestations 482
Elementary visual hallucinations 483
Complex visual hallucinations, visual illusions and other symptoms from more anterior ictal spreading 485
Palinopsia 485
Sensory hallucinations of ocular movements 485
Ictal blindness 485
Tonic deviation of the eyes, oculoclonic seizures and epileptic nystagmus 486
Repetitive eyelid closures, eyelid .uttering and eyelid blinking 486
Consciousness 486
Ictal or post-ictal headache 486
Seizure spreading 487
Aetiology 487
Coeliac disease and occipital epilepsy 487
Eclampsia and occipital seizures 488
Lafora disease and occipital seizures 488
Pathophysiology 488
Diagnostic procedures 488
Electroencephalography 488
Inter-ictal EEG 489
Ictal EEG 489
Differential diagnosis 489
Differentiating ictal deviation of eyes of occipital versus extra-occipital origin 490
Differentiating idiopathic from symptomatic occipital epilepsy 491
Prognosis 491
Management 491
AED therapy of focal epilepsies 491
RCTs and evidence- based recommendations in focal epilepsies 492
Older AEDs 493
Newer AEDs 493
Monotherapy of focal epilepsies 493
The best choice among AED licensed for monotherapy in focal epilepsies 494
Rational polytherapy in focal epilepsies 494
Conclusions in the use of newer AEDs in focal epilepsies 496
References 498
16 Reflex seizures and related epileptic syndromes 506
Clarifications on classification 506
Definitions 506
The precipitating stimulus 507
The response to the stimulus 508
Clinical types of reflex seizure 508
Reflex-electroclinical events and the role of the EEG 508
Visually induced seizures and epilepsies 509
Photosensitivity, epileptic seizures and epileptic syndromes 509
Clarifications on classification 509
Demographic data 511
Clinical manifestations 511
Generalised seizures 511
Focal seizures 512
Precipitating factors 512
Self-induced photic reflex seizures 512
Aetiology 512
Diagnostic procedures 513
Prognosis 514
Management 514
Avoidance or prevention of the provocative stimulus may be the only treatment 514
AED treatment 516
Idiopathic photosensitive occipital lobe epilepsy 518
Clarifications on classification 518
Demographic data 519
Clinical manifestations 519
Precipitating factors 519
Diagnostic procedures 519
Electroencephalography 519
Aetiology 521
Differential diagnosis 521
Prognosis 521
Management 521
Jeavons syndrome 522
Considerations on classification 522
Demographic data 523
Clinical manifestations 523
Precipitating factors 523
Self-induction in Jeavons syndrome 523
Aetiology 525
Pathophysiology 525
Diagnostic procedures 525
Electroencephalography 525
Differential diagnosis 525
Prognosis 526
Management 526
Pattern-sensitive epilepsy 526
Demographic data 527
Clinical manifestations 527
Environmental stimuli 527
Aetiology 528
Pathophysiology 528
Diagnostic procedures 528
Prognosis 529
Management 529
Fixation-off sensitivity 529
Clinical and EEG correlations in patients with FOS 529
Pathophysiology 530
Scotosensitive epilepsy 530
Techniques for documenting FOS 530
Complex reflex epilepsies 531
Seizures induced by thinking and praxis 531
Primary (idiopathic) reading epilepsy 531
Clarifications on classification 531
Demographic data 531
Clinical manifestations 531
Precipitating factors 532
Aetiology 533
Pathophysiology 533
Diagnostic procedures 533
Electroencephalography 533
Prognosis 533
Management 533
Startle seizures 533
Clarifications on classification 534
Demographic data 534
Clinical manifestations 534
Aetiology 534
Diagnostic procedures 534
Electroencephalography 534
Differential diagnosis 534
Prognosis 535
Management 535
Hot water epilepsy 535
Clarifications on classification 535
Demographic data 535
Clinical manifestations 535
Aetiology 536
Pathophysiology 536
Diagnostic procedures 536
Electroencephalography 536
Differential diagnosis 536
Prognosis 536
Management 537
References 537
17 Diseases frequently associated with epileptic seizures 541
Clarifications on classification 542
Progressive myoclonic epilepsies 543
Clarifications on nomenclature 543
Unverricht disease 544
Demographic data 544
Clinical manifestations 545
Aetiology 546
Diagnostic procedures 546
Differential diagnosis 546
Prognosis 546
Management 546
Lafora disease 548
Demographic data 548
Clinical manifestations 548
Aetiology 549
Pathology 549
Diagnostic procedures 549
Differential diagnosis 550
Prognosis 550
Management 550
Neuronal ceroid lipofuscinoses 550
Demographic data 550
Clinical manifestations 550
Aetiology 551
Pathology 552
Diagnostic procedures 553
Differential diagnosis 553
Prognosis 553
Management 554
Sialidoses (type I and II) 555
Demographic data 555
Clinical manifestations 555
Aetiology 555
Diagnostic procedures 556
Differential diagnosis 556
Prognosis 556
Management 556
Myoclonic epilepsy associated with ragged-red fibres 557
Demographic data 557
Clinical manifestations 557
Aetiology 557
Diagnostic procedures 558
Differential diagnosis 558
Prognosis 558
Management 558
Dentatorubral-pallidoluysian atrophy 559
Demographic data 559
Clinical manifestations 559
Aetiology 559
Pathology 560
Diagnostic procedures 560
Differential diagnosis 560
Prognosis 560
Management 560
Angelman syndrome 560
Demographic data 561
Clinical manifestations 561
Aetiology 561
Diagnostic procedures 562
Differential diagnosis 562
Prognosis 563
Management 563
Management of myoclonus 563
Epileptic myoclonus 563
Progressive myoclonic epilepsies 564
Antimyoclonic pharmacological agents 565
Older antimyoclonic AEDs 565
Newer antimyoclonic AEDs 566
Antimyoclonic AEDs in development 566
Other pharmacological agents 566
References 567
18 Pharmacopoeia 572
Acetazolamide 573
Authorised indications 573
Clinical applications 573
Dosage and titration 573
Main ADRs 573
Mechanism of action 573
Pharmacokinetics 573
Drug interactions 574
Main disadvantages 574
Benzodiazepines 574
Main ADRs 574
Tolerance, dependence and withdrawal syndrome 574
Main mechanism of action 575
Carbamazepine 575
Authorised indications 575
Clinical applications 576
Dosage and titration 576
Main ADRs 576
Considerations in women 577
Main mechanisms of action 577
Pharmacokinetics 577
Drug interactions 578
With other AEDs 578
With non-AEDs 578
Main disadvantages 578
Clobazam 578
Authorised indications 578
Clinical applications 578
Dosage and titration 579
Main ADRs 579
Main mechanism of action 579
Pharmacokinetics 579
Drug interactions 579
Main disadvantages 579
Clonazepam 580
Authorised indications 580
Clinical applications 580
Dosage and titration 580
Main ADRs 580
Main mechanism of action 580
Pharmacokinetics 580
Drug interactions 580
Main disadvantages 580
Eslicarbazepine acetate 581
Authorized indications 581
Clinical applications 581
Dosage and titration. 581
Main ADRs 581
Considerations in women 581
Main mechanisms of action 582
Pharmacokinetics 582
Drug interactions 582
Main disadvantages 582
Ethosuximide 583
Authorised indications 583
Clinical applications 583
Dosage and titration 583
Main ADRs 583
Considerations in women 583
Main mechanisms of action 583
Pharmacokinetics 583
Drug interactions 583
Main disadvantages 583
Other available succinimides 583
Felbamate 584
Authorised indications 584
Clinical applications 584
Dosage and titration 584
Main ADRs 584
Considerations in women 585
Main mechanisms of action 586
Pharmacokinetics 586
Drug interactions 586
Main disadvantages 586
Gabapentin 586
Authorised indications 586
Clinical applications 586
Dosage and titration 587
Main ADRs 587
Considerations in women 587
Main mechanisms of action 587
Pharmacokinetics 587
Drug interactions 587
Main disadvantages 587
Lacosamide 588
Authorised indications 588
Clinical applications 588
Dosage and titration 588
Main ADRs 588
Considerations in women 588
Main mechanisms of action 589
Pharmacokinetics 589
Drug interactions 589
Main disadvantages 589
Lamotrigine 589
Authorised indications 589
Clinical applications 590
Dosage and titration 590
Main ADRs 591
Considerations in women 592
Main mechanisms of action 592
Pharmacokinetics 593
Drug interactions 593
Main disadvantages 593
Levetiracetam 594
Authorised indications 594
Clinical applications 594
Dosage and titration 595
Main ADRs 595
Considerations in women 596
Main mechanisms of action 596
Pharmacokinetics 596
Drug interactions 597
Main disadvantages 597
Oxycarbazepine 597
Authorised indications 597
Clinical applications 598
Dosage and titration 598
Main ADRs 598
Considerations in women 599
Main mechanisms of action 599
Pharmacokinetics 599
Drug interactions 599
Main disadvantages 599
Phenobarbital 600
Authorised indications 600
Current main applications 600
Dosage and titration 600
Main ADRs 600
Considerations in women 600
Main mechanisms of action 600
Pharmacokinetics 600
Drug interactions 600
Main disadvantages 601
Other available main barbiturate agents 601
Phenytoin 601
Authorised indications 601
Clinical applications 601
Dosage and titration 601
Main ADRs 601
Considerations in women 602
Main mechanisms of action 602
Pharmacokinetics 602
Drug interactions 602
Disadvantages 602
Other available phenytoin-related agents 602
Pregabalin 603
Authorised indications 603
Clinical applications 603
Dosage and titration 603
Main ADRs 603
Considerations in women 603
Main mechanisms of action 603
Pharmacokinetics 604
Drug interactions 604
Main disadvantages 604
Rufinamide 604
Authorised indications 604
Clinical applications 604
Dosage and titration 604
Main ADRs 604
Considerations in women 605
Mechanism of action 605
Pharmacokinetics 605
Drug interactions 605
Main disadvantages 605
Stiripentol 606
Authorised indications 606
Clinical applications 606
Dosage and titration 606
Main ADR 606
Considerations in women 606
Main mechanisms of action 606
Pharmacokinetics 606
Drug interactions 606
Main disadvantage 606
Sulthiame 607
Authorised indications 607
Clinical applications 607
Dosage and titration 607
Main ADRs 607
Considerations in women 607
Mechanism of action 607
Pharmacokinetics 607
Drug interactions 608
Main disadvantages 608
Tiagabine 608
Authorised indications 608
Clinical applications 608
Dosage and titration 608
Main ADRs 608
Considerations in women 608
Main mechanisms of action 608
Pharmacokinetics 609
Drug interactions GTCSs in patients 10 of age effectiveness
Main disadvantages 609
Topiramate 609
Authorised indications 609
Clinical applications 609
Dosage and titration 610
Main ADRs 610
Considerations in women 611
Main mechanisms of action 612
Pharmacokinetics 612
Drug interactions 612
Main disadvantages 612
Valproate 612
Authorised indications 612
Clinical applications 613
Dosage and titration 613
Main ADRs 613
Considerations in women 614
Main mechanisms of action 614
Pharmacokinetics 615
Drug interactions 615
Main disadvantages 616
Vigabatrin 616
Authorised indications 616
Clinical applications 616
Dosage and titration 616
Main ADRs 617
Main mechanisms of action 617
Pharmacokinetics 617
Drug interactions 617
Considerations in women 617
Main disadvantages 617
Zonisamide 618
Authorised indications 618
Clinical applications 618
Dosage and titration 618
Main ADRs 619
Considerations in women 619
Main mechanisms of action 619
Pharmacokinetics 619
Interaction with other drugs 620
Main disadvantages 620
References 620

"General aspects of epilepsies (p. 1-2)

Epileptic seizures and epileptic syndromes have high prevalence and incidence rates affecting all ages and all races of both sexes. They constitute an important part of the everyday clinical practice of general and specialist health care professionals.

Patients with epileptic seizures and their families are entitled to diagnosis, prognosis and management that are specific and precise. Medical diagnosis is the identi?cation of a disease by investigation of its symptoms and history, which provides a solid basis for the treatment and prognosis of the individual patient. Accurate diagnosis is the golden rule in medicine and epilepsies should not be an exception to this.

Current practice that limits the diagnosis to ‘epilepsy’ or ‘seizures’ is unsatisfactory to the patient and physician alike, and may result in avoidable morbidity and mortality. Such a non-speci?c diagnostic label fails to provide guidance on important items such as severity of disease, prognosis, short- and long-term therapeutic decisions, and genetics (research and counselling), which are all factors that crucially affect personal, family and social life, education and career choices of patients. ‘Epilepsy’ is not a single disease entity. Epilepsies are many syndromes and diseases that have a multitude of different manifestations and causes. Epileptic syndromes and diseases are now largely well de?ned and easy to diagnose.

De?ning the type of epilepsy should be considered mandatory because it offers the best guide to both management and prognosis. The short- and long-term management of epilepsies is syndrome related and differs markedly between the various syndromes, thereby emphasising the need for accurate diagnosis. The bene?ts of syndromic diagnosis over seizure/symptom diagnosis, or an inclusive diagnosis such as ‘epilepsy’, far outweigh any morbidity from miscategorisation that may arise in dif?cult cases. Unspecified diagnosis in epilepsies commonly results in avoidable morbidity and sometimes mortality.

Important reminder

Traditional medical teaching and attitudes to the diagnosis and management of epilepsies often differ from those applied in other medical conditions. This should be corrected. Physicians who rightly seek bedside con?rmation of muscle fatigability in a patient with a clear-cut history of myasthenia gravis, should also request to view the seizures, which if frequent can be easily captured even by mobile phones. Physicians who rightly emphasise the differential diagnosis between spinal muscular atrophies and limb girdle muscular dystrophy should give the same emphasis to the differentiation between absence seizures of idiopathic generalised epilepsies and complex focal seizures. Major paediatric journals that often emphasise a rare disease should at least give the same space to highlighting the fact that childhood autonomic status epilepticus is a common and costly cause of misdiagnosis and mismanagement, adversely affecting thousands of children around the world (see page 81). one"

Erscheint lt. Verlag 10.5.2010
Zusatzinfo XIV, 578 p. 100 illus. in color.
Verlagsort London
Sprache englisch
Themenwelt Medizin / Pharmazie Medizinische Fachgebiete Neurologie
Schlagworte brain • brain imaging • Childhood • Electroencephalography (EEG) • Epilepsie • Epilepsy • Epileptic • Epilepticus • Seizures
ISBN-10 1-84628-644-1 / 1846286441
ISBN-13 978-1-84628-644-5 / 9781846286445
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