A Clinical Guide to Epileptic Syndromes and their Treatment, Revised Second Edition

A Clinical Guide to Epileptic Syndromes and their Treatment, Second Edition......Page 1
Title Page......Page 2
Copyright Page......Page 3
Dedication Pages......Page 4
acknowledgements......Page 5
Table of Contents......Page 6
preface to the second edition......Page 14
preface to the revised second edition......Page 15
abbreviations......Page 16
1 General aspects of epilepsies......Page 18
Other formal definitions of epilepsy......Page 19
Medical history......Page 20
Laboratory diagnostic procedures......Page 21
First step: Are the paroxysmal events epileptic seizures?......Page 22
Second step: What type of epileptic seizures?......Page 24
Third step: What is their cause and what is the epileptic syndrome or disease?......Page 25
Epilepsy or epilepsies......Page 27
The ILAE classification of epileptic seizures and epileptic syndromes......Page 28
Gardeners and botanists......Page 30
Author’s clarifications on the ILAE terminology and classifications......Page 32
Incidence and prevalence of epilepsies......Page 33
Mortality and epilepsies......Page 34
References......Page 35
The definition of epileptic seizures......Page 37
Epileptic seizures in accordance with the 1981 ILEA classification......Page 40
Generalised seizures......Page 43
Seizure classification in the new ILAE Task Force reports......Page 44
Debate on the distinction between generalised and focal seizures......Page 47
Generalised tonic–clonic seizures......Page 48
Tonic phase of GTCSs......Page 49
Recovery phase of GTCSs......Page 51
Autonomic changes of GTCS......Page 52
Variants of GTCS......Page 54
Ictal EEG of GTCSs......Page 55
Aetiology of GTCS......Page 56
Pathophysiology......Page 57
Diagnostic tests......Page 59
Aetiology......Page 60
Diagnostic tests......Page 61
Epileptic myoclonus......Page 62
Epileptic negative myoclonus......Page 64
ILAE classification of epileptic myoclonus......Page 65
Atonic seizures......Page 66
Typical absence seizures......Page 67
Clinical manifestations......Page 69
Diagnosing absences and differential diagnosis......Page 70
Atypical absence seizures......Page 71
ILAE terminology and classification of focal seizures......Page 73
Aetiology......Page 75
Management......Page 76
References......Page 77
Definition and classification......Page 80
Generalised tonic–clonic status epilepticus......Page 82
Compensatory phase......Page 83
Pharmacoresistance in GTC-SE......Page 84
Aetiology......Page 85
Generalised myoclonic status epilepticus......Page 86
Absence status epilepticus......Page 87
Impairment of consciousness, memory and higher cognitive functions......Page 88
Precipitating factors......Page 90
Obtundation SE......Page 91
Supplementary motor area SE......Page 92
Clarifications on nomenclature......Page 93
Hippocampal SE......Page 94
Clinical manifestations......Page 95
Definition......Page 96
Out of hospital management......Page 97
Hospital emergency management of GTC-SE......Page 98
Pharmacological treatment of GTC-SE......Page 99
Focal simple and complex SE......Page 101
Benzodiazepines......Page 102
Fosphenytoin......Page 104
Paraldehyde......Page 105
Valproate, levetiracetam and lacosamide......Page 106
References......Page 107
4 Imitators of epileptic seizures......Page 111
Generalised tonic–clonic seizures......Page 112
Prodromes (non-epileptic) preceding the onset of a GTCS......Page 113
Absence seizures......Page 114
Focal epileptic seizures......Page 115
Neurally mediated syncope......Page 116
Reflex anoxic seizures......Page 117
Carotid sinus syncope......Page 118
The long QT syndrome......Page 119
Psychogenic NEPEs imitating epileptic seizures......Page 120
Convulsive psychogenic status epilepticus......Page 121
Hyperventilation syndrome......Page 122
Non-epileptic paroxysmal movement disorders imitating epileptic seizures......Page 123
Jitteriness......Page 124
Gastro-oesophageal re.ux in infants......Page 125
Benign non-epileptic myoclonus of early infancy (Fejerman syndrome)......Page 126
Hyperekplexia......Page 127
Non-epileptic paroxysmal kinesigenic choreoathetosis......Page 128
Non-epileptic severe amnesic and confusional attacks imitating epileptic seizures......Page 129
Prolonged confusional states......Page 130
NEPEs occurring during sleep and sleep disorders......Page 131
Hypnagogic myoclonic jerks (sleep starts)......Page 132
Restless legs syndrome and periodic limb movement disorder......Page 133
Sleep drunkenness......Page 134
Narcolepsy......Page 135
Diagnostic procedures......Page 136
Visual, auditory, somatosensory, olfactory, gustatory and autonomic paroxysmal symptoms imitating simple focal seizures......Page 137
Cyclic vomiting syndrome......Page 138
Migraine, migralepsy, basilar migraine with EEG occipital paroxysms and diagnostic errors......Page 139
Basilar migraine......Page 140
Migralepsy......Page 141
Differentiating visual seizures from migraine......Page 143
References......Page 144
ILAE definitions of epilepsies and epileptic syndromes and clarifications of terminology......Page 149
Idiopathic epilepsy syndrome......Page 150
Cryptogenic or probably symptomatic epilepsy syndrome......Page 151
Benign epilepsy syndrome......Page 152
The ILAE 1989 classification of epileptic syndromes......Page 153
Localisation-related (focal) epilepsies and syndromes......Page 154
Epilepsies and syndromes undetermined as to whether they are focal or generalised......Page 155
Syndromic classification of the new diagnostic ILAE scheme......Page 157
References......Page 160
Reasons why the EEG should not be undervalued......Page 161
Sources of error in EEGs......Page 164
Drowsiness, sleep and awakening......Page 166
Other forms of appropriate activation of reflex seizures......Page 168
Value of EEG in stopping AED treatment......Page 169
Improving the EEG contribution to the diagnosis of epilepsies......Page 170
Abnormal EEGs......Page 171
Magnetic resonance imaging......Page 173
X-ray computed tomography......Page 175
Functional neuroimaging......Page 176
Magnetic resonance spectroscopy......Page 178
Co-registration of SPECT/PET with MRI......Page 180
Magnetoencephalography......Page 182
References......Page 184
AED prophylactic treatment......Page 186
Clarifications on terminology of AED treatment......Page 189
Spectrum of efficacy......Page 190
Clinical pharmacokinetics......Page 191
Hepatic enzyme induction and inhibition......Page 192
Mechanisms of action......Page 194
Need for less laboratory testing and other monitoring......Page 195
Frequency of administration and ease of use......Page 196
Newest generation AEDs licensed in the treatment of focal epilepsies: lacosamide and eslicarbazepine......Page 197
Starting AED treatment in newly diagnosed epilepsy......Page 198
Monotherapy......Page 199
Rational polytherapy......Page 200
Total AED withdrawal......Page 201
Generic versus brand or generic to generic AED prescribing......Page 202
Evidence-based recommendations......Page 203
Poverty of reliable RCTs in epilepsies......Page 205
Therapeutic drug monitoring......Page 206
Plasma and saliva TDM......Page 208
Time of sampling......Page 209
Life-threatening ADRs......Page 210
Common CNS-related ADRs......Page 211
Behavioural and psychiatric ADRs to AED......Page 212
Adverse cardiac effects of AEDs......Page 213
Effect of AEDs on oral hormonal contraception......Page 217
Teratogenicity......Page 218
Commonly used older generation AEDs and major congenital malformations......Page 219
Commonly used new generation of broad spectrum AEDs and major congenital malformations......Page 220
Foetal anticonvulsant syndrome and minor anomalies......Page 222
Change in seizure frequency and status epilepticus during pregnancy......Page 223
Seizure deterioration in pregnancy due to changes in plasma AED concentrations......Page 224
Obstetric and other pregnancy-related complications......Page 226
Breastfeeding and AEDs......Page 227
Principles of AED therapy in women of childbearing age in clinical practice......Page 228
Commencing AED therapy in women of childbearing age with newly diagnosed focal epilepsy......Page 229
Commencing AED therapy in women of childbearing age with newly diagnosed generalised epilepsy......Page 230
Preconception......Page 231
Difficulties in diagnosing epileptic seizures in the elderly......Page 232
EEG and other investigative procedures in the elderly......Page 233
Principles of AED treatment in the elderly......Page 234
Surgery for epilepsies......Page 235
The concept of surgically remediable epileptic syndromes......Page 236
Focal resective procedure (lesionectomy)......Page 237
Multiple subpial transections......Page 238
Surgical procedure and cost......Page 239
What is the place of VNS in the treatment of epilepsies?......Page 240
Adverse effects of the ketogenic diet......Page 241
References......Page 242
Demographic data......Page 249
Motor seizures......Page 250
Aetiology......Page 252
Inter-ictal EEG......Page 254
Electroclinical dissociation or decoupling response......Page 255
Prognosis......Page 257
Management......Page 258
Neonatal epileptic syndromes......Page 259
Aetiology......Page 260
Prognosis......Page 261
Aetiology......Page 262
Differential diagnosis......Page 263
Clinical manifestations......Page 264
Diagnostic procedures......Page 265
Clarifications on classification......Page 266
Electroencephalography......Page 267
Management......Page 268
References......Page 269
Clarifications on classification......Page 271
Seizures......Page 272
Aetiology......Page 273
Differential diagnosis......Page 274
Prognosis......Page 275
Prophylactic management......Page 276
Clinical manifestations......Page 277
Differential diagnosis......Page 278
Aetiology......Page 279
Electroencephalography......Page 280
Clinical manifestations......Page 281
Prognosis......Page 283
References......Page 284
10 Epileptic encephalopathies in infancy and early childhood......Page 287
Clinical manifestations......Page 288
Aetiology......Page 290
Diagnostic procedures......Page 291
Ictal EEG......Page 292
Prognosis......Page 294
Demographic data......Page 295
Clinical manifestations......Page 296
Diagnostic procedures......Page 297
Prognosis......Page 298
Clarifications on classification......Page 299
Clinical manifestations......Page 300
Diagnostic procedures......Page 305
Differential diagnosis......Page 306
Prognosis......Page 308
AED treatment......Page 310
Newer AEDs......Page 311
Non-pharmacological treatments......Page 313
Attention to seizure precipitants......Page 314
Considerations on classification......Page 315
Seizures......Page 316
Pathophysiology......Page 317
Prognosis......Page 318
Management......Page 320
Clinical manifestations......Page 321
Aetiology......Page 322
Electroencephalography......Page 323
Differential diagnosis......Page 324
Myoclonic encephalopathy in non-progressive disorders......Page 325
Electroencephalography......Page 326
Clinical manifestations......Page 327
Management......Page 328
Clinical manifestations......Page 329
Diagnostic procedures......Page 330
Differential diagnosis......Page 331
Management......Page 332
References......Page 333
The formal ILAE nomenclature......Page 339
Clinical manifestations......Page 340
Aetiology......Page 341
Differential diagnosis......Page 342
Clinical manifestations......Page 344
Hemiconvulsion–hemiplegia epilepsy......Page 345
Prognosis......Page 346
References......Page 347
Considerations on classification......Page 350
Demographic data......Page 351
Status epilepticus......Page 352
Electroencephalography......Page 353
Management......Page 357
Clinical manifestations......Page 358
Conventional seizure symptoms......Page 360
Pathophysiology......Page 361
Electroencephalography......Page 362
Differential diagnosis......Page 366
Management......Page 367
Clinical manifestations......Page 368
Pathophysiology......Page 369
Electroencephalography......Page 370
Differentiating ICOE-G from PS......Page 373
Clinical manifestations......Page 374
Benign infantile focal epilepsy with midline spikes and waves during sleep......Page 375
Benign childhood seizure susceptibility syndrome: A unified concept......Page 376
Febrile seizures and BCSSS......Page 377
Considerations on classification......Page 378
Management of benign childhood focal seizures......Page 379
Prophylactic AED treatment of benign childhood focal seizures......Page 380
By Thalia Valeta......Page 381
References......Page 382
Considerations on classification......Page 387
Epilepsy with myoclonic–astatic seizures......Page 388
Demographic data......Page 389
Clinical manifestations......Page 390
Differential diagnosis......Page 392
Considerations on classification......Page 393
Electroencephalography......Page 396
Differential diagnosis......Page 397
Considerations on classification......Page 398
Management......Page 399
Considerations on classification......Page 401
Electroencephalography......Page 402
Prognosis......Page 403
Demographic data......Page 404
Clinical manifestations......Page 405
Seizure-precipitating factors......Page 406
Aetiology......Page 408
Differential diagnosis......Page 409
Pharmacological treatment......Page 410
Considerations on classification......Page 411
Electroencephalography......Page 412
Management......Page 413
IGE with absences of early childhood......Page 414
Electroencephalography......Page 415
Considerations on classification......Page 417
Clinical manifestations......Page 418
Management......Page 419
Differential diagnosis......Page 420
AED treatment of IGEs......Page 421
IGEs: RCTs and evidence- based recommendations......Page 423
Older AEDs in IGEs......Page 424
Management of patients with difficult to treat IGEs......Page 425
References......Page 426
14 Familial (autosomal dominant) focal epilepsies......Page 432
Clinical manifestations......Page 435
Management......Page 436
Clinical manifestations......Page 437
Prognosis......Page 438
Differential diagnosis......Page 439
Management......Page 440
Focal epilepsy with pericentral spikes......Page 441
References......Page 442
Symptomatology and classification......Page 444
Subjective ictal clinical manifestations......Page 445
Fear......Page 446
Complex internal sensations (experiential, mental, intellectual or psychic symptoms, dreamy states)......Page 447
Déjà vu......Page 448
Olfactory and gustatory hallucinations......Page 449
Automatisms......Page 450
Definitions of automatisms......Page 451
Motor arrest, staring and temporal lobe absence......Page 452
Catamenial temporal lobe seizures......Page 453
Clarifications on classification......Page 454
Clinical manifestations......Page 456
Lateralising signs of ictal and post-ictal symptoms......Page 457
Aetiology......Page 458
Diagnostic procedures......Page 459
Differential diagnosis......Page 460
Community studies......Page 461
Neurosurgical treatment......Page 462
Clinical manifestations......Page 463
Management......Page 464
Considerations on classification......Page 465
Demographic data......Page 466
Seizures from the motor cortex......Page 467
Somatosensory or other ill-de.ned auras (not epigastric), vocalisations and speech arrest that are common ictal manifestations......Page 468
Diagnostic procedures......Page 469
Prognosis......Page 470
Clarifications on classification......Page 471
Clinical manifestations......Page 472
Diagnostic procedures......Page 473
Differential diagnosis......Page 474
Somatosensory seizures......Page 477
Disturbances of body image and somatic illusions......Page 478
Aetiology......Page 479
Electroencephalography......Page 480
Management......Page 481
Clinical manifestations......Page 482
Elementary visual hallucinations......Page 483
Ictal blindness......Page 485
Ictal or post-ictal headache......Page 486
Coeliac disease and occipital epilepsy......Page 487
Electroencephalography......Page 488
Differential diagnosis......Page 489
Differentiating ictal deviation of eyes of occipital versus extra-occipital origin......Page 490
AED therapy of focal epilepsies......Page 491
RCTs and evidence- based recommendations in focal epilepsies......Page 492
Monotherapy of focal epilepsies......Page 493
Rational polytherapy in focal epilepsies......Page 494
Conclusions in the use of newer AEDs in focal epilepsies......Page 496
References......Page 498
Definitions......Page 506
The precipitating stimulus......Page 507
Reflex-electroclinical events and the role of the EEG......Page 508
Clarifications on classification......Page 509
Generalised seizures......Page 511
Aetiology......Page 512
Diagnostic procedures......Page 513
Avoidance or prevention of the provocative stimulus may be the only treatment......Page 514
AED treatment......Page 516
Clarifications on classification......Page 518
Electroencephalography......Page 519
Management......Page 521
Considerations on classification......Page 522
Self-induction in Jeavons syndrome......Page 523
Differential diagnosis......Page 525
Pattern-sensitive epilepsy......Page 526
Environmental stimuli......Page 527
Diagnostic procedures......Page 528
Clinical and EEG correlations in patients with FOS......Page 529
Techniques for documenting FOS......Page 530
Clinical manifestations......Page 531
Precipitating factors......Page 532
Startle seizures......Page 533
Differential diagnosis......Page 534
Clinical manifestations......Page 535
Prognosis......Page 536
References......Page 537
17 Diseases frequently associated with epileptic seizures......Page 541
Clarifications on classification......Page 542
Clarifications on nomenclature......Page 543
Demographic data......Page 544
Clinical manifestations......Page 545
Management......Page 546
Clinical manifestations......Page 548
Diagnostic procedures......Page 549
Clinical manifestations......Page 550
Aetiology......Page 551
Pathology......Page 552
Prognosis......Page 553
Management......Page 554
Aetiology......Page 555
Management......Page 556
Aetiology......Page 557
Management......Page 558
Aetiology......Page 559
Angelman syndrome......Page 560
Aetiology......Page 561
Differential diagnosis......Page 562
Epileptic myoclonus......Page 563
Progressive myoclonic epilepsies......Page 564
Older antimyoclonic AEDs......Page 565
Other pharmacological agents......Page 566
References......Page 567
18 Pharmacopoeia......Page 572
Pharmacokinetics......Page 573
Tolerance, dependence and withdrawal syndrome......Page 574
Authorised indications......Page 575
Main ADRs......Page 576
Pharmacokinetics......Page 577
Clinical applications......Page 578
Main disadvantages......Page 579
Main disadvantages......Page 580
Considerations in women......Page 581
Main disadvantages......Page 582
Other available succinimides......Page 583
Main ADRs......Page 584
Considerations in women......Page 585
Clinical applications......Page 586
Main disadvantages......Page 587
Considerations in women......Page 588
Authorised indications......Page 589
Dosage and titration......Page 590
Main ADRs......Page 591
Main mechanisms of action......Page 592
Main disadvantages......Page 593
Clinical applications......Page 594
Main ADRs......Page 595
Pharmacokinetics......Page 596
Authorised indications......Page 597
Main ADRs......Page 598
Main disadvantages......Page 599
Drug interactions......Page 600
Main ADRs......Page 601
Other available phenytoin-related agents......Page 602
Main mechanisms of action......Page 603
Main ADRs......Page 604
Main disadvantages......Page 605
Main disadvantage......Page 606
Pharmacokinetics......Page 607
Main mechanisms of action......Page 608
Clinical applications......Page 609
Main ADRs......Page 610
Considerations in women......Page 611
Authorised indications......Page 612
Main ADRs......Page 613
Main mechanisms of action......Page 614
Drug interactions......Page 615
Dosage and titration......Page 616
Main disadvantages......Page 617
Dosage and titration......Page 618
Pharmacokinetics......Page 619
References......Page 620