Download A Clinical Gde. to Epileptic Syndromes, Their Trtmt. 2nd ed. - C. Panayiotopoulos (Springer, 2010) WW PDF

TitleA Clinical Gde. to Epileptic Syndromes, Their Trtmt. 2nd ed. - C. Panayiotopoulos (Springer, 2010) WW
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LanguageEnglish
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Total Pages627
Table of Contents
                            A Clinical Guide to Epileptic Syndromes and their Treatment, Second Edition
Front-matter
	Title Page
	Copyright Page
	Dedication Pages
	acknowledgements
	Table of Contents
	preface to the second edition
	preface to the revised second edition
	abbreviations
1 General aspects of epilepsies
	What is epilepsy? Definitions
		Comment on the new ILAE definition
		Other formal definitions of epilepsy
	Making the correct diagnosis in epilepsies
		Medical history
			‘That’s it!’ phenomenon6
			Home-made video recordings
		Laboratory diagnostic procedures
		Differential diagnosis
			First step: Are the paroxysmal events epileptic seizures?
			Second step: What type of epileptic seizures?
			Third step: What is their cause and what is the epileptic syndrome or disease?
		Epilepsy or epilepsies
	The ILAE classification of epileptic seizures and epileptic syndromes
		On classifications: concepts,clarifications and difficulties in reaching a consensus in the classi.cation of epileptic seizures and syndromes
			Gardeners and botanists
		Author’s clarifications on the ILAE terminology and classifications
			Splitters and lumpers
	Epidemiology of epilepsies
		Incidence and prevalence of epilepsies
		Mortality and epilepsies
	References
2 Epileptic seizures and their classification
	The definition of epileptic seizures
	Other useful or ILAE seizure-related terminology
	Classification of epileptic seizures
		Epileptic seizures in accordance with the 1981 ILEA classification
			Partial (or focal) seizures
			Generalised seizures
		Seizure classification in the new ILAE Task Force reports
		Debate on the distinction between generalised and focal seizures
	Generalised epileptic seizures
		Generalised tonic–clonic seizures
			Epidemiology of GTCS
			Clinical manifestations
				Ictal events preceding the onset of a GTCS
				Onset of GTCS proper
				Tonic phase of GTCSs
				Intermediate transitory (vibratory) phase of GTCSs
				Clonic phase of GTCSs
				Recovery phase of GTCSs
				Autonomic changes of GTCS
				Variants of GTCS
				Complications of GTCSs
				Ictal EEG of GTCSs
				Aetiology of GTCS
				Pathophysiology
		Generalised tonic seizures
			Clinical manifestations
			Aetiology
			Diagnostic tests
			Differential diagnosis
			Management
		Generalised clonic seizures
			Clinical manifestations
			Aetiology
			Diagnostic tests
			Differential diagnosis
			Management
		Epileptic spasms
			Clinical manifestations
			Aetiology
			Diagnostic tests
			Differential diagnosis
			Management
		Myoclonus
			Description of myoclonus
			Classification of myoclonus
			Epileptic myoclonus
			Epileptic negative myoclonus
			ILAE classification of epileptic myoclonus
			Myoclonus and epileptic syndromes
		Atonic seizures
			Clinical manifestations
			Aetiology
			Diagnostic tests
			Differential diagnosis
			Management
		Typical absence seizures
			Clinical manifestations
			Electroencephalography
			Pathophysiology of absence seizures
			Diagnosing absences and differential diagnosis
		Atypical absence seizures
	Focal epileptic seizures
		ILAE terminology and classification of focal seizures
			Epidemiology
			Clinical manifestations
			Aetiology
			Pathophysiology
			Diagnostic procedures
			Prognosis
			Management
	Reflex epileptic seizures
	References
3 Status epilepticus
	Definition and classification
	Generalised tonic–clonic status epilepticus
		Physiological stages of GTC-SE
			Compensatory phase
			Refractory stage of GTC-SE (established GTC-SE)
				Pharmacoresistance in GTC-SE
				Cerebral damage and epileptogenesis
		Epidemiology of GTC-SE
		Aetiology
		Differential diagnosis
		Prognosis
	Generalised tonic status epilepticus
	Generalised myoclonic status epilepticus
		Idiopathic myoclonic SE
		Symptomatic/cryptogenic myoclonic SE
	Absence status epilepticus
		Typical absence (idiopathic absence) SE
			Impairment of consciousness, memory and higher cognitive functions
			Behavioural abnormalities and experiential phenomena
			Myoclonic jerks in idiopathic absence SE
			GTCSs associated with idiopathic absence SE
			Duration and frequency of idiopathic absence SE
			Post-ictal state
			Age at onset and sex
			Precipitating factors
			Differential diagnosis
		Atypical absence SE
		Obtundation SE
		Situation-related and de novo absence SE
	Focal status epilepticus
		Epilepsia partialis continua
		Supplementary motor area SE
		Aura continua
		Dyscognitive focal (psychomotor, complex partial) SE
			Clarifications on nomenclature
			Clinical manifestations
			Prevalence
			Aetiology and prognosis
			Differential diagnosis
		Hippocampal SE
			Clinical manifestations
			Electroencephalography
		Focal SE of frontal lobe origin
		Focal SE of occipital lobe origin
		Focal SE of parietal lobe origin
		Autonomic SE
			Definition
			Clinical manifestations
		Opercular SE
	Management of status epilepticus
		Management of GTC-SE and other convulsive SE
			Out of hospital management
			Hospital emergency management of GTC-SE
				General emergency management
				Pharmacological treatment of GTC-SE
		Absence SE
			Self-administration of medication
			Hospital management
		Focal simple and complex SE
		Antiepileptic drugs in the treatment of status epilepticus
			Benzodiazepines
			Phenytoin sodium
			Fosphenytoin
			Phenobarbital sodium
			Paraldehyde
			Valproate, levetiracetam and lacosamide
	References
4 Imitators of epileptic seizures
	Main types of epileptic seizures and their imitators
		Generalised tonic–clonic seizures
			Prodromes (non-epileptic) preceding the onset of a GTCS
			Ictal events preceding the onset of a GTCS
		Epileptic myoclonic jerks
		Absence seizures
		Tonic seizures
		Epileptic spasms
		Epileptic drop attacks
		Focal epileptic seizures
	Syncopal attacks imitating epileptic seizures
		Neurally mediated syncope
			Vasovagal syncope
			Reflex anoxic seizures
			Orthostatic syncope
			Syncopes induced by Valsalva manoeuvre
			Micturition syncope
			Carotid sinus syncope
		Cardiogenic syncopes
			The long QT syndrome
			Syncopes induced by drugs and electrolyte abnormalities
		Syncopal attacks provoking epileptic seizures: Anoxic epileptic seizures
		Epileptic seizures imitating syncope
	Psychogenic NEPEs imitating epileptic seizures
		Convulsive psychogenic status epilepticus
		Suffocation in Munchausen syndrome by proxy
		Panic attacks
		Hyperventilation syndrome
		Management of PNEPEs
	Non-epileptic paroxysmal movement disorders imitating epileptic seizures
		Tics, compulsions, stereotypies and mannerisms
		Tremor
		Non-epileptic movement disorders in neonates and infants imitating seizures
			Jitteriness
			Tonic reflex seizures of early infancy
			Alternating hemiplegia
			Benign paroxysmal torticollis
			Rhythmic behavioural movements
			Self-gratification disorder
			Gastro-oesophageal re.ux in infants
			Benign neonatal sleep myoclonus
			Benign non-epileptic myoclonus of early infancy (Fejerman syndrome)
			Hyperekplexia
		Other non-epileptic paroxysmal movement disorders
			Familial paroxysmal dystonic choreoathetosis
			Non-epileptic paroxysmal kinesigenic choreoathetosis
			Episodic ataxia type 1
	Non-epileptic severe amnesic and confusional attacks imitating epileptic seizures
		Transient global amnesia
		Psychogenic (dissociative) fugues
		Prolonged confusional states
		Non-epileptic staring spells, childhood preoccupation and daydreaming
	NEPEs occurring during sleep and sleep disorders
		Classification of sleep disorders
		Sleep-related non-epileptic movement disorders
			Hypnagogic myoclonic jerks (sleep starts)
			Nocturnal myoclonus
			Fragmentary hypnic myoclonus
			Propriospinal myoclonus
			Benign neonatal sleep myoclonus
			Facio-mandibular myoclonus
			Restless legs syndrome and periodic limb movement disorder
			Rhythmic movements in sleep
			Sleepwalking and night terrors
			Paroxysmal nocturnal dystonia (hypnogenic paroxysmal dystonia)
		Sleep-related non-epileptic behavioural and other disorders
			Sleep drunkenness
			Bruxism
			Catathrenia (nocturnal expiratory groaning)
			Nocturnal enuresis
			REM sleep behaviour disorder
			Sleep-related eating disorder
			Sudden infant death syndrome (Cot death)
		Narcolepsy
			Demographic data
			Clinical manifestations
			Aetiology
			Diagnostic procedures
			Differential diagnosis
			Prognosis
			Management
		Sleep apnoea
		Sleep disorders in the elderly
	Subjective non-epileptic paroxysmal symptoms imitating simple focal seizures
		Visual, auditory, somatosensory, olfactory, gustatory and autonomic paroxysmal symptoms imitating simple focal seizures
		Cyclic vomiting, paroxysmal vertigo or motion sickness
			Cyclic vomiting syndrome
			Benign positional paroxysmal vertigo
			Benign paroxysmal vertigo of childhood
			Motion sickness
	Migraine, migralepsy, basilar migraine with EEG occipital paroxysms and diagnostic errors
		Visual aura of migraine
			Basilar migraine
			Migralepsy
		Visual seizures
			Differentiating visual seizures from migraine
	Cerebrovascular NEPEs imitating epileptic seizures
	References
5 Epileptic syndromes and their classification
	ILAE definitions of epilepsies and epileptic syndromes and clarifications of terminology
		Epileptic syndrome
		Epilepsy disease
		Idiopathic epilepsy syndrome
		Symptomatic epilepsy syndrome
		Cryptogenic or probably symptomatic epilepsy syndrome
		Benign epilepsy syndrome
		Genetic epilepsy
	The ILAE 1989 classification of epileptic syndromes
		Localisation-related (focal) epilepsies and syndromes
		Generalised epilepsies and syndromes
		Epilepsies and syndromes undetermined as to whether they are focal or generalised
		Special syndromes
			Epilepsies with seizures precipitated by speci.c modes of activation
	Syndromic classification of the new diagnostic ILAE scheme
	References
6 EEG and brain imaging
	EEG in the diagnosis and management of epilepsies
		The value of routine inter-ictal or ictal EEGs in epilepsies
			Reasons why the EEG should not be undervalued
			Reasons why the EEG should not be overvalued
		Sources of error in EEGs
		Physiological activators of EEG abnormalities
			Drowsiness, sleep and awakening
			Hyperventilation
			Intermittent photic stimulation
			Other forms of appropriate activation of reflex seizures
		Video-EEG recording should be made routine clinical practice
		The role of EEG technologists
		The significance of the EEG after the .rst afebrile seizure
		Value of EEG in stopping AED treatment
		Improving the EEG contribution to the diagnosis of epilepsies
		Illustrative cases of good EEG practice
			Normal routine EEGs
			Abnormal EEGs
	Brain imaging in the diagnosis and management of epilepsies
		Magnetic resonance imaging
		X-ray computed tomography
		Functional neuroimaging
		Recommendations for neuroimaging of patients with epilepsy
			Ideal practice
			Minimum standards
		Functional neuroimaging in clinical practice
			Functional MRI
			Magnetic resonance spectroscopy
			Single photon emission CT
			PET with [18F].uorodeoxyglucose and 15O-labelled water
			PET with specific ligands
			Co-registration of SPECT/PET with MRI
		Magnetoencephalography
	References
7 Principles of therapy in the epilepsies
	AED prophylactic treatment
		Clarifications on terminology of AED treatment
		Choosing the best AED option
			Seizure specificity
			Strength of efficacy
			Spectrum of efficacy
			Safety, tolerability and adverse reactions
			Clinical pharmacokinetics
				Hepatic enzyme induction and inhibition
				Carbonic anhydrase inhibitors
			Pharmacodynamics
			Drug–drug interactions
			Mechanisms of action
			Need for as little titration as possible
			Need for less laboratory testing and other monitoring
			Frequency of administration and ease of use
			Cost of treatment
		Newest generation AEDs licensed in the treatment of focal epilepsies: lacosamide and eslicarbazepine
		Newly diagnosed epilepsy
			Starting AED treatment in newly diagnosed epilepsy
		Monotherapy
		Rational polytherapy
			Converting from polytherapy to monotherapy
		Total AED withdrawal
		Generic versus brand or generic to generic AED prescribing
		Evidence for AED treatment recommendations in clinical practice
			Evidence-based recommendations
				Poverty of reliable RCTs in epilepsies
			Evidence-based recommendations in this book
		Therapeutic drug monitoring
			Plasma and saliva TDM
			Clinical applications
			Time of sampling
		Considerations of adverse antiepileptic drug reactions in the treatment of epilepsies
			Life-threatening ADRs
			Common CNS-related ADRs
			Behavioural and psychiatric ADRs to AED
			Non-CNS ADRs of AEDs
			Adverse cardiac effects of AEDs
	Principles of management in women with epilepsies
		Oral hormonal contraception and AEDs
			Effect of AEDs on oral hormonal contraception
			Effect of oral hormonal contraception on AEDs
		Pregnancy
		Teratogenicity
			Commonly used older generation AEDs and major congenital malformations
			Commonly used new generation of broad spectrum AEDs and major congenital malformations
			The AAN/AES assessments of AEDrelated MCMs
			Foetal anticonvulsant syndrome and minor anomalies
			Post-natal cognitive effects of foetal exposure to AEDs
			Change in seizure frequency and status epilepticus during pregnancy
			Seizure deterioration in pregnancy due to changes in plasma AED concentrations
			Effect of seizures on mother and the unborn baby
			Obstetric and other pregnancy-related complications
			Adverse perinatal outcomes
			Folic acid supplementation for the prevention of major congenital malformations
		Breastfeeding and AEDs
		Principles of AED therapy in women of childbearing age in clinical practice
			Commencing AED therapy in women of childbearing age with newly diagnosed focal epilepsy
			Commencing AED therapy in women of childbearing age with newly diagnosed generalised epilepsy
			Preconception
	Principles of management in the elderly with epileptic seizures
		Difficulties in diagnosing epileptic seizures in the elderly
		Frequency of seizures and their severity in the elderly
		EEG and other investigative procedures in the elderly
		Physiological and other changes in the elderly that may affect AEDs
		Principles of AED treatment in the elderly
	Surgery for epilepsies
		Criteria for surgical referral
			Drug-resistant epilepsy for the purposes of surgical referral
			The concept of surgically remediable epileptic syndromes
		Strategy of a surgical work-up
		Types of surgical procedures
			Curative (definitive) surgery
				Focal resective procedure (lesionectomy)
				Cerebral hemispherectomy
			Palliative (functional) surgery
				Corpus callosotomy
				Multiple subpial transections
	Vagus nerve stimulation
		Efficacy
		Adverse reactions
		Technical aspects
		Surgical procedure and cost
		What is the place of VNS in the treatment of epilepsies?
	Ketogenic diet
		Indications and efficacy
		Rationale and types of ketogenic diet
			The Atkins diet
		Adverse effects of the ketogenic diet
	Corticosteroids in the treatment of childhood epilepsies
		Preparation, doses and regimens of corticosteroids
		Adverse effects and monitoring of corticosteroid treatment
	References
8 Neonatal epileptic seizures and neonatal epileptic syndromes
	Neonatal epileptic seizures
		Clarifications on classification and terminology
		Demographic data
		Clinical manifestations
			Subtle seizures
			Motor seizures
			Autonomic ictal manifestations
			Duration of neonatal seizures
			Non-epileptic neonatal seizures
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Brain imaging
			Electroencephalography
				Inter-ictal EEG
				Ictal EEG
				Electroclinical dissociation or decoupling response
				Electrical seizure patterns of usually poor prognosis
				Post-ictal EEG
		Differential diagnosis
		Prognosis
		Management
			Do electrographic (electrical) seizures need treatment?
	Neonatal epileptic syndromes
		Clarifications on classification
	Benign familial neonatal seizures
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Genetic testing
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Benign neonatal seizures (non-familial)
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Early myoclonic encephalopathy
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Ohtahara syndrome
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	References
9 Idiopathic epileptic seizures and syndromes in infancy
	Febrile seizures
		Clarifications on classification
		Demographic data
		Clinical manifestations
			Seizures
			Risk factors of a first febrile seizure
			Risk factors for recurrence
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
			Intellectual and behavioural outcome
		Management
			Acute management of a child with a febrile seizure
			Prophylactic management
			Supportive family management
	Epilepsy with febrile seizures plus
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Benign infantile seizures
		Considerations on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Myoclonic epilepsy in infancy
		Clarifications on classification
		Demographic data
		Clinical manifestations
			Precipitating factors
		Aetiology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	References
10 Epileptic encephalopathies in infancy and early childhood
	Clarifications on classification
	West syndrome
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
			Ictal EEG
		Differential diagnosis
		Prognosis
		Management
	Dravet syndrome
		Clarifications on classification
		Demographic data
		Clinical manifestations
			Seizure-precipitating factors
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Lennox–Gastaut syndrome
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
			AED treatment
			Older AEDs
			Newer AEDs
			Hormonal and other non-AED treatment
			Non-pharmacological treatments
			Treatment of status epilepticus in Lennox-Gastaut syndrome
			Treatment-induced aggravation of seizures, cognition and behaviour
			Attention to seizure precipitants
			Educational management
	Landau–Kleffner syndrome
		Considerations on classification
		Demographic data
		Clinical manifestations
			Linguistic abnormalities
			Cognitive and behavioural abnormalities
			Seizures
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Epileptic encephalopathy with continuous spike-and-wave during sleep
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Myoclonic encephalopathy in non-progressive disorders
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Atypical benign partial epilepsy of childhood
		Clarifications on nomenclature
		Demographic data
		Clinical manifestations
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Hypothalamic epilepsy
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	References
11 Severe neocortical epileptic syndromes in infancy and childhood
	Kozhevnikov–Rasmussen syndrome
		Clarifications on nomenclature
			The formal ILAE nomenclature
			Historical facts
			Misconceptions
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Migrating focal seizures of infancy
		Considerations on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Prognosis
		Management
	Hemiconvulsion–hemiplegia epilepsy
		Considerations on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Prognosis
		Management
	References
12 Benign childhood focal seizures and related epileptic syndromes
	Considerations on classification
	Benign childhood epilepsy with centrotemporal spikes
		Considerations on nomenclature
		Demographic data
		Clinical manifestations
			Status epilepticus
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Evolution and prognosis
		Management
	Panayiotopoulos syndrome
		Considerations on classification
		Demographic data
		Clinical manifestations
			Conventional seizure symptoms
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
			Magnetoencephalography
		Differential diagnosis
		Prognosis
		Management
	Idiopathic childhood occipital epilepsy of Gastaut
		Considerations on classification
		Demographic data
		Clinical manifestations
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
			Differentiating ICOE-G from PS
		Prognosis
		Management
	Other phenotypes of BCSSS
		Benign childhood seizures with affective symptoms
			Demographic data
			Clinical manifestations
			Aetiology
			Diagnostic procedures
				Electroencephalography
			Prognosis
			Management
		Benign childhood epilepsy with parietal spikes and frequent ESESs
		Benign childhood focal seizures associated with frontal or midline spikes
		Benign infantile focal epilepsy with midline spikes and waves during sleep
	Benign childhood seizure susceptibility syndrome: A unified concept
		Febrile seizures and BCSSS
	Benign (isolated) focal seizures of adolescence
		Considerations on classification
		Demographic data
		Clinical manifestations
		Diagnostic procedures
		Differential diagnosis
		Prognosis and management
	Management of benign childhood focal seizures
		Acute management of a child with prolonged seizures
		Prophylactic AED treatment of benign childhood focal seizures
		Stopping medication
		Parental attitude and education
			By Thalia Valeta
	References
13 Idiopathic generalised epilepsies
	Considerations on classification
	Syndromes of IGE recognised by the ILAE
	Epilepsy with myoclonic–astatic seizures
		Considerations on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Childhood absence epilepsy
		Considerations on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Epilepsy with myoclonic absences
		Considerations on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Juvenile absence epilepsy
		Considerations on classification
		Demographic data
		Clinical manifestations
			Seizure-precipitating factors
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Juvenile myoclonic epilepsy
		Demographic data
		Clinical manifestations
			Seizure-precipitating factors
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
			Pharmacological treatment
			Prevention of GTCSs and termination of myoclonic and absence status epilepticus
			Duration of AED treatment and withdrawal of medication
	Epilepsy with GTCSs only
		Considerations on classification
		Demographic data
		Clinical manifestations
			Seizure-precipitating factors
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Other probable syndromes of IGE to consider
	IGE with absences of early childhood
	Perioral myoclonia with absences
		Considerations on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	IGE with phantom absences
		Considerations on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Autosomal dominant cortical tremor, myoclonus and epilepsy
		Epidemiology
		Aetiology
		Clinical manifestations
		Diagnostic procedures
		Prognosis
		Differential diagnosis
		Management
	AED treatment of IGEs
		Treatment of newly diagnosed IGEs
			IGEs: RCTs and evidence- based recommendations
			Older AEDs in IGEs
			Newer AEDs useful in IGEs
		Management of patients with difficult to treat IGEs
		Drug withdrawal
		Management of status epilepticus in IGEs
	References
14 Familial (autosomal dominant) focal epilepsies
	Autosomal dominant nocturnal frontal lobe epilepsy
		Demographic data
		Clinical manifestations
			Circadian distribution
			Precipitating factors
		Aetiology
		Diagnostic procedures
		Prognosis
		Differential diagnosis
		Management
	Familial (autosomal dominant) temporal lobe epilepsy
	Familial mesial temporal lobe epilepsy
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Familial lateral temporal lobe epilepsy
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
		Prognosis
		Differential diagnosis
		Management
	Familial focal epilepsy with variable foci
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Prognosis
		Management
	Other possible familial (autosomal dominant) focal epilepsies not yet recognised
		Autosomal dominant rolandic epilepsy and speech dyspraxia
		Focal epilepsy with pericentral spikes
	References
15 Symptomatic and cryptogenic (probably symptomatic) focal epilepsies
	Symptomatology and classification
	Temporal lobe epilepsies
		Semiology of TLE
			Subjective ictal clinical manifestations
				Ascending epigastric aura
				Fear
				Complex internal sensations (experiential, mental, intellectual or psychic symptoms, dreamy states)
				Déjà vu
				Jamais vu, jamais entendu and jamais veçu
				Auditory hallucinations and illusions
				Olfactory and gustatory hallucinations
				Visual hallucinations and illusions
				Other subjective ictal manifestations
			Objective ictal symptoms
				Automatisms
				Definitions of automatisms
				Autonomic disturbances and ictus emeticus
				Language and speech ictal disturbances
				Motor arrest, staring and temporal lobe absence
				Motor manifestations
				Gelastic seizures of TLE
			Amnesic seizures
			Catamenial temporal lobe seizures
		Post-ictal symptoms
			Differentiating temporal lobe seizures from other extratemporal seizures on the basis of post-ictal symptoms
	Mesial TLE with hippocampal sclerosis
		Clarifications on classification
		Demographic data
		Clinical manifestations
			Simple focal seizures
			Complex focal seizures
			Lateralising signs of ictal and post-ictal symptoms
			GTCSs
			Post-ictal symptoms
			Complex focal status epilepticus of temporal lobe origin
			Neurological, mental state and behaviour
			Febrile convulsions and other initial precipitating events
		Aetiology
			Hippocampal sclerosis
				Hippocampal sclerosis and temporal lobe epilepsy: cause or consequence?
			Pathophysiology
		Diagnostic procedures
			Inter-ictal EEG
			Ictal EEG
		Differential diagnosis
		Prognosis
			Neurosurgical series
			Community studies
		Management
			AED treatment
			Neurosurgical treatment
	MTLE defined by specific aetiologies other than hippocampal sclerosis
	Lateral temporal lobe epilepsy
		Clinical manifestations
		Aetiology
		Diagnostic procedures
		Differential diagnosis
		Management
	Frontal lobe epilepsies
		Considerations on classification
		Demographic data
		Clinical manifestations
			Seizures from the motor cortex
			Seizures from the supplementary sensorimotor area
				Hypermotor seizures155 of bizarre bilateral, asymmetrical tonic posturing and movements
				Somatosensory or other ill-de.ned auras (not epigastric), vocalisations and speech arrest that are common ictal manifestations
				Vocalisations
			Other frontal lobe seizures of particular clinical interest
			Focal status epilepticus of frontal lobe origin
		Aetiology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Epilepsia partialis continua of Kozhevnikov
		Clarifications on classification
		Demographic data
		Clinical manifestations
			Other types of seizures
			Neurological signs and symptoms
		Aetiology
		Pathophysiology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Parietal lobe epilepsies
		Clarifications on classification
		Demographic data
		Clinical manifestations
			Somatosensory seizures
			Disturbances of body image and somatic illusions
			Other ictal subjective symptoms
			Seizure spreading to extraparietal regions
			Duration of seizures
			Post-ictal manifestations
			Epileptogenic localisation
			Precipitating factors
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Management
	Occipital lobe epilepsies
		Clarifications on classification
			Terminology
		Demographic data
		Clinical manifestations
			Elementary visual hallucinations
			Complex visual hallucinations, visual illusions and other symptoms from more anterior ictal spreading
			Palinopsia
			Sensory hallucinations of ocular movements
			Ictal blindness
			Tonic deviation of the eyes, oculoclonic seizures and epileptic nystagmus
			Repetitive eyelid closures, eyelid .uttering and eyelid blinking
			Consciousness
			Ictal or post-ictal headache
			Seizure spreading
		Aetiology
			Coeliac disease and occipital epilepsy
			Eclampsia and occipital seizures
			Lafora disease and occipital seizures
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
				Inter-ictal EEG
				Ictal EEG
		Differential diagnosis
			Differentiating ictal deviation of eyes of occipital versus extra-occipital origin
			Differentiating idiopathic from symptomatic occipital epilepsy
		Prognosis
		Management
	AED therapy of focal epilepsies
		RCTs and evidence- based recommendations in focal epilepsies
		Older AEDs
		Newer AEDs
		Monotherapy of focal epilepsies
			The best choice among AED licensed for monotherapy in focal epilepsies
			Rational polytherapy in focal epilepsies
			Conclusions in the use of newer AEDs in focal epilepsies
	References
16 Reflex seizures and related epileptic syndromes
	Clarifications on classification
		Definitions
		The precipitating stimulus
		The response to the stimulus
			Clinical types of reflex seizure
			Reflex-electroclinical events and the role of the EEG
	Visually induced seizures and epilepsies
	Photosensitivity, epileptic seizures and epileptic syndromes
		Clarifications on classification
		Demographic data
		Clinical manifestations
			Generalised seizures
			Focal seizures
			Precipitating factors
			Self-induced photic reflex seizures
		Aetiology
		Diagnostic procedures
		Prognosis
		Management
			Avoidance or prevention of the provocative stimulus may be the only treatment
			AED treatment
	Idiopathic photosensitive occipital lobe epilepsy
		Clarifications on classification
		Demographic data
		Clinical manifestations
			Precipitating factors
		Diagnostic procedures
			Electroencephalography
		Aetiology
		Differential diagnosis
		Prognosis
		Management
	Jeavons syndrome
		Considerations on classification
		Demographic data
		Clinical manifestations
			Precipitating factors
			Self-induction in Jeavons syndrome
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Pattern-sensitive epilepsy
		Demographic data
		Clinical manifestations
			Environmental stimuli
		Aetiology
			Pathophysiology
		Diagnostic procedures
		Prognosis
		Management
	Fixation-off sensitivity
		Clinical and EEG correlations in patients with FOS
			Pathophysiology
		Scotosensitive epilepsy
		Techniques for documenting FOS
	Complex reflex epilepsies
	Seizures induced by thinking and praxis
	Primary (idiopathic) reading epilepsy
		Clarifications on classification
		Demographic data
		Clinical manifestations
			Precipitating factors
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Prognosis
		Management
	Startle seizures
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	Hot water epilepsy
		Clarifications on classification
		Demographic data
		Clinical manifestations
		Aetiology
			Pathophysiology
		Diagnostic procedures
			Electroencephalography
		Differential diagnosis
		Prognosis
		Management
	References
17 Diseases frequently associated with epileptic seizures
	Clarifications on classification
	Progressive myoclonic epilepsies
		Clarifications on nomenclature
	Unverricht disease
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Lafora disease
		Demographic data
		Clinical manifestations
		Aetiology
			Pathology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Neuronal ceroid lipofuscinoses
		Demographic data
		Clinical manifestations
		Aetiology
			Pathology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Sialidoses (type I and II)
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Myoclonic epilepsy associated with ragged-red fibres
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Dentatorubral-pallidoluysian atrophy
		Demographic data
		Clinical manifestations
		Aetiology
			Pathology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Angelman syndrome
		Demographic data
		Clinical manifestations
		Aetiology
		Diagnostic procedures
		Differential diagnosis
		Prognosis
		Management
	Management of myoclonus
		Epileptic myoclonus
		Progressive myoclonic epilepsies
		Antimyoclonic pharmacological agents
			Older antimyoclonic AEDs
			Newer antimyoclonic AEDs
			Antimyoclonic AEDs in development
			Other pharmacological agents
	References
18 Pharmacopoeia
	Acetazolamide
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Mechanism of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Benzodiazepines
		Main ADRs
		Tolerance, dependence and withdrawal syndrome
		Main mechanism of action
	Carbamazepine
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
			With other AEDs
			With non-AEDs
		Main disadvantages
	Clobazam
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Main mechanism of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Clonazepam
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Main mechanism of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Eslicarbazepine acetate
		Authorized indications
		Clinical applications
		Dosage and titration.
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Ethosuximide
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
		Other available succinimides
	Felbamate
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Gabapentin
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Lacosamide
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Lamotrigine
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Levetiracetam
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Oxycarbazepine
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Phenobarbital
		Authorised indications
		Current main applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
		Other available main barbiturate agents
	Phenytoin
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Disadvantages
		Other available phenytoin-related agents
	Pregabalin
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Rufinamide
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Mechanism of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Stiripentol
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADR
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantage
	Sulthiame
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Mechanism of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Tiagabine
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions GTCSs in patients  10 of age; effectiveness
		Main disadvantages
	Topiramate
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Valproate
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Main disadvantages
	Vigabatrin
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Main mechanisms of action
		Pharmacokinetics
		Drug interactions
		Considerations in women
		Main disadvantages
	Zonisamide
		Authorised indications
		Clinical applications
		Dosage and titration
		Main ADRs
		Considerations in women
		Main mechanisms of action
		Pharmacokinetics
		Interaction with other drugs
		Main disadvantages
	References
                        
Document Text Contents
Page 2

A Clinical Guide to

Epileptic Syndromes

and their Treatment

CP Panayiotopoulos

ABC

Revised Second Edition
Based on the ILAE classifications

and practice parameter guidelines

Page 313

Epileptic Encephalopathies in Infancy and Early Childhood 301

Treatments for Lennox–Gastaut
syndrome

First-line drugs (in order of priority)

sedative effects and is particularly useful as an add-

on to valproate.

sedative than other benzodiazepines.

widely used in clinical practice.

serious adverse reactions.

sometimes fatal, adverse reactions.

Second-line drugs

and probably tonic seizures in combination with

valproate.

periods of status epilepticus.

in patients for whom other treatments are of little

benefit.

Non-pharmacological treatments

but expectations should be kept low.

Drop attacks are more responsive to felbamate,

lamotrigine, rufinamide, and topiramate; vagus nerve

stimulation and corpus callosotomy are surgical options.

Hormonal and
other non-AED treatment
Corticosteroids and ACTH may be helpful
particularly in idiopathic/cryptogenic Lennox–

Gastaut syndrome, particularly at onset (as for West
syndrome)151 and in periods of marked seizure
exaggeration.124,150 Given their long-term adverse
effects, they should not be tried more than once
or twice in the course of the disease and only at
times of desperation.124 They are also used to treat
episodes of nonconvulsive status epilepticus not
responding to conventional AEDs.150,151

Intravenous immunoglobulin was found to be useful
in a few case reports and sometimes improvement
was evident after the first dose.150 It may be tried
at times of seizure exacerbations not responding to
other medications. Intravenous immunoglobulin is
costly and administration is inconvenient but it does
not interfere with other drugs and is usually well
tolerated.
Amantadine, tryptophane, flumazenil, imipramine and
other non-epileptic drugs have had limited success
in some patients and they may even exacerbate
seizures.

Non-pharmacological treatments
The ketogenic diet is undergoing a mini-renaissance
in epileptic encephalopathies (see page 228)
including the Lennox–Gastaut syndrome.120,167–169
In a recent report,167 the effect of a ketogenic diet
was studied in a blinded randomised cross over
study in 20 children with intractable Lennox–
Gastaut syndrome. The patients fasted for 36
hours and then were randomised to receive the
classic ketogenic diet in conjunction with a
solution containing either 60 g/day of glucose to
negate the ketosis or saccharin. A crossover to the
ketogenic diet with the alternate solution occurred
following the sixth day and a repeat fast. After
administration of the solution, there was moderate
evidence of a reduction in parent-reported
seizures between the glucose and saccharin arms,
with a median difference of 1.5 seizures per day
(p=0.07). There was no reduction in the number
of EEG-identified events, with a median reduction
of 7 events per day (p=0.33). Ketosis was not
completely eliminated in the glucose-added
arm.167 A supplementary study168 attempted to
clarify whether the effectiveness of the ketogenic

Page 314

302 A Clinical Guide to Epileptic Syndromes and their Treatment

diet could be explained by a placebo effect or by
parental expectations and commitment. In this
study, the additional glucose did not significantly
alter the frequency of EEG-assessed events, but
did decrease the frequency of parent-reported
“drop” seizures (P=0.07). Fasting had substantial
effects on both drop attacks and EEG-assessed
events. The diet remained effective in decreasing
seizures at 12 days, 6 months, and 12 months.168
The popular Atkins diet may be a less restrictive
alternative when appropriately modified.170

Vagus nerve stimulation in childhood epileptic
encephalopathies, including Lennox–Gastaut syn-
drome, has been found to be effective, particularly
in tonic and atonic seizures, and to improve
the quality of life of these patients.120,150,171–172
It has been assessed to be as good as corpus
callosotomy.172 However, the results of another
promising study report when follow-up
assessments were made suggests that caution may
be warranted.173

Neurosurgery: Corpus callosotomy is the only
surgical procedure for devastating atonic seizures
with traumatic falls (drop attacks).151,152 Resective
neurosurgery is appropriate in the few cases with
distinctively localised epileptogenic lesions.174

Treatment of status epilepticus in
Lennox-Gastaut syndrome120,150,151

Episodes of non-convulsive status epilepticus are is
common and may last for hours to weeks; attempts
should be made to prevent these as much as possible.
Home-administered benzodiazepines are the first
option in treating impending or established non-
convulsive status epilepticus. Midazolam (buccal
or nasal) and diazepam (rectal) are preferred; some
authorities also recommend oral intake of relatively
high doses of clonazepam, clobazam or nitrazepam,
although these are not of proven efficacy by oral
administration. Hospital management includes
intravenous administration of mainly nitrazepam,
phenytoin, diazepam, clonazepam or midazolam.
Intravenous immunoglobulins or corticosteroids
may also be used when the status epilepticus is
prolonged and resistant to AED. The treatment

of status epilepticus is detailed in chapter 3 (page
82).

Intravenous benzodiazepines may, rarely, induce tonic

status epilepticus.

Treatment-induced aggravation of
seizures, cognition and behaviour
Treatment-induced aggravation of seizures,
cognition and behaviour is a major problem
with Lennox–Gastaut syndrome. It is much more
common than reported and may be a significant
cause of the bad prognosis of Lennox–Gastaut
syndrome. The effect is difficult to detect and
often assumed to be a spontaneous fluctuation or
part of the progress of the disease, and is hard
to attribute to a specific treatment, particularly if
the treatment concerned has improved other types
of seizure. Parents often value highly a treatment
that improves major seizures at the cost of adverse
effects.
Gabapentin is contraindicated because it worsens
seizures. However, any one of the AEDs cited in
the above section has the potential to aggravate
seizures, cognition and behaviour or to have
other serious physical consequences. Seizures
may also be increased by sedative AEDs that affect
alertness.

Finding the right balance of risk versus bene�t of any

treatment and for each individual patient is probably

more demanding in Lennox–Gastaut syndrome than for

any other condition.

Attention to seizure precipitants
Detection and prevention of seizure facilitating factors
are part of the appropriate management of Lennox–
Gastaut syndrome.151 Intercurrent febrile illnesses,
vomiting, changes in treatment regimens, sedation
and psychological stress may facilitate seizures and
status epilepticus. Children with Lennox–Gastaut
syndrome are particularly vulnerable in an unstable
and non-stimulating environment in which they
experience irregular patterns of sleep, diet and
medication.

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