epileptic encephalopathies
TRANSCRIPT
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Dr. Rahi kiran.BSenior residentDept. of NeurologyGMC Kota
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A group of heterogeneous brain disorders occurring at a critical period of brain development, where frequent abnormal ictal and/or interictal EEG epileptiformactivity is mainly responsible for behavioral, cognitive and motor regression.
(1) electrographic EEG paroxysmal activity that is often aggressive, (2) seizures that are usually multi-form and intractable, (3) cognitive, behavioral, and neurological deficits that may be relentless, (4) sometimes early death.
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Not fully understood, not same for all
Aggressive ictal(seizure) and electrical(EEG) epileptogenic activity in developing brain leading to excessive excitability
which varies acc to age and stage of brain maturity
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In neonates – burst-suppression
Infancy – hypsarrythmia
Child – GSWD
As age advances, these also change
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Epileptic Encephalopathy Syndromes in neonates
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Onset < age 3 m
Male = female
Etiology - Inborn errors of metabolism - mc-nonketotic hyperglycinemia, methylmalonic acidemia.
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Triad of intractable seizures – Erratic myoclonus f/b simple focal seizures f/b Tonic seizures
Erratic myoclonus - shift typically from one part of the body to another in a random, asynchronous fashion.
simple focal seizures –
Tonic seizures - a diffuse tonic contraction, usually extending to the extremities.
Psychomotor devt – abnormal from onset
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Interictal EEG - suppression–burst activity, evolves into atypical hypsarrhythmia after 3 to 5 months of life,
accentuated during sleep
Myoclonus – no EEG expression, follows burst
CT and MR - related to etiology,grossly normal or have asymmetrical enlargement of one hemisphere,
dilatation of the corresponding lateral ventricle, or cortical atrophy
the serum levels of amino acids should be determined, especially glycine and glycerol
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The inter-ictal EEG of early myoclonic encephalopathy is a repetitive suppression–burst pattern without physiological rhythms
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The prognosis is poor - >50% die within weeks or months.
no effective therapy – pyridoxine trial can be tried
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ILAE Definition Very early onset, within the first few months of life, frequent tonic spasms and a
suppression–burst EEG pattern in both the waking and sleeping states.
Onset - 10 days to 3 months of age (occ. Intrauterine).
Etiology – mc – malformations, no familial cases
(hemimegaloencephaly, porencephaly, Aicardi syn, focal cortical dysplasia)
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Tonic spasms - forward tonic flexion lasting 1–10 s that is singular or in long clusters 10–300 times every 24 h.
Alternating hemiconvulsions or GTCS are exceptional.
Myoclonic seizures are rare.
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Burst suppression with pseudorhythmic periodicity,
disappears in 6 months - Most survivors evolve to West syndrome with hypsarrhythmia, or later to LGS.
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prognosis is poor with severe psychomotor retardation
Psychomotor development may be slightly better if the infants do not develop West or Lennox-Gastaut syndrome.
Half of the children are likely to die in infancy or childhood
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No effective treatment
vigabatrin and zonisamide – some benefit
ACTH – not useful
invasive surgery, such as a partial resection or complete hemispherectomy.
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Epileptic Encephalopathy Syndromes in Infancy
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frequent partial seizures of multifocal onset, with autonomic or motor involvement
The interictal EEG reveals multifocal epileptiform activity and slowing, diffuse slowing of the background activity. asymmetry between different recordings
The ictal EEG confirms multifocal onsets,
No burst-suppression
difficult to control with standard AEDs.
Bromides, stiripentol, and clonazepam may be helpful
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Triad of infantile spasms, arrest of psychomotor development, and hypsarrhythmia.
Onset - age of 3 and 12 months.
Male(60%) > female
Etiology – symptomatic(80%), idiopathic cerebral malformations, infection, hemorrhage, hypoxicischemic injury, metabolic
disorders, and genetic conditions,such as Down syndrome, TS
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Epileptic spasms - Clusters of sudden, brief (0.2–2 s), bilateral tonic contractions of the axial and limb muscles involving extension and/or flexion
Occurs in clusters – 1-30 / day, each with 20 – 150 attacks
Precipitating factors- twilight state,sudden loud noises or tactile stimulation,feeding.
Spasms – flexor – extensor – mc Flexor – salaam spasmsExtensor – like moro reflex
Psychomotor delay – from the onset
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interictal -Gibbs and Gibbs –hypsarrhythmic EEG.(2/3 rds) Disorganized pattern with asynchronous, very high amplitude slowing and frequent
multifocal spike and sharp wave discharges
Sleep – REM – normal NREM – more synchronous
By 2-4yrs - LGS
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Ictal EEG – mc – high voltage, gen. slow wave
episodic,low amplitude slow wave
diffuse attenuation(electrodecremental ictal EEG pattern).
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Brief flexor spasm associated with high voltage slow wave discharge
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Good if treated early
Mortality - 5%.
60% - develop other seizure types – LGS
50%-permanent motor disabilities
2/3rd -severe cognitive and psychological impairment.
5–12% - normal mental and motor development.
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First line ACTH and vigabatrin (TS)
No role of pyridoxine
valproate, levetiracetam, topiramate, zonisamide, lamotrigine, and benzodiazepines
ketogenic diet is helpful in most cases
Hemispherectomy - medically intractable
No specific AED has been shown to affect long-term developmental outcome
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Onset – day 1 to 5yrs
Female : male – 2: 1
Etiology – 50 % - a/w angelman syn, 20% - birth asphyxia
c/b – fixed, non-progressive encephalopathylong-lasting recurrent episodic erratic myoclonic status epilepticus with atypical absence seizures.
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interictal EEG-multifocal epileptiform discharges and background slowing
Ictal EEG - continuous generalized slow spike and wave, or an absence pattern
Myoclonic status – improves but with residual disability
Treatment myoclonic status – BZDs Others - valproate with ethosuximide or clobazam
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Onset - always <1yr of life, with a peak age of 5 m.
Male : female – 2:1
Etiology – 50% genetic, SCN1A mutation – mostly sporadic
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Tetrad of seizuresEarly onset infantile febrile clonic convulsionsMyoclonic jerksAtypical absencesComplex focal seizures.
In second year - more frequent and persistent, partial & myoclonic jerks start, not a/w fever, ppted by – hot and light, gradual regression of milestones
Precipitating Factors - Febrile illnesses , warm environment (hot baths), Photic and pattern stimulation.
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Inter-Ictal EEG
Normal till 18mnths age
Initial -20% show normal BG with photoparoxysmal discharges of spikes/ polyspikes-slow waves.
Within 1 year, EEG - abnormal slow background with frequent asymmetricalparoxysms of polyspikes (GPSWD)
Ictal EEG
Varies according to seizuretype.
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Brain scans are usually normal. SCN1A” mutation - 70 per cent of children
Treatment very resistant – initial - Phenobarbital, sodium valproate Other - stiripentol, topiramate ,clonazepam ,clobazam, LEV combination of sodium valproate with either topiramate or stiripentol may be the
most helpful. c/I – PHT,CBZ,LMT short course of a steroid (called prednisolone) and the ketogenic diet may also be
helpful.
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Epileptic Encephalopathy Syndromes in Childhood
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1–4% of childhood epilepsies.
Onset - Starts between 1 and 7 yrs with a peak at 3–5 years.
Male > female
Triad-
Polymorphic intractable seizure that are mainly tonic (80%), atonic (50%)and
atypical absence seizures (70%), Myoclonic Jerks(11–28%) – sleep & awake
Cognitive and behavioural abnormalities.
EEG with paroxysms of fast activity and slow (less than 2.5 Hz) generalised spike-
wave discharges (GSWD).
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symptomatic - 70–78% of patients with LGS –
encephalitis and/or meningitis,
tuberous sclerosis,
brain malformations
(e.g., cortical dysplasias),
Idiopathic –
▪ normal psychomotor development occurred prior to the onset of symptoms,
▪ no underlying disorders or definite presumptive causes are present,
▪ no neurologic or neuroradiologic abnormalities are found.
birth injury
hypoxia-ischemia injury,
frontal lobe lesions
trauma.
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ILAE inclusion criteria –
Atleast 2 seizure types – tonic, atonic, atypical absence(mandatory)
EEG – GSWD with episodic fast activity
Impaired intellectual functioning
Not imp. – onset age, imaging, etiology
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Tonic seizures – 80 – 100% mc & most characteristic,
Symmetrical, brief, awake & NREM, not in REM
Atonic – 50%
Atypical absence – 2/3rd
Myoclonic jerks – 11-28%
Drop attacks -
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Inter-ictal EEG – abnormal BG – fragmented alpha, excess slow waves
Ictal EEG –
a) tonic – paroxysmal fast activity
b) Atonic – gen. polyspikes, slow GSWD
c) Atypical absence - <2.5Hz GSWD
d) Myoclonic – gen. polyspikes with/without slow waves
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Tonic seizure started clinically with a scream and episodic nystagmusThe ictal EEG consisted of an high-amplitude generalised sharp and
slow waves at approximately 1 Hz.
The EEG returned to its pre- ictal state after approximately 1 min
from the onset of theseizure.
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10–30% of cases develop from West syndrome or other epileptic encephalopathies.
Bad prognosis
5% - die
90%- seizures in adult life also
Almost all – cognitive impairment- more in symptomatic cases
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AEDs – First line – all types - VPA, clobazam, zonisamide, rufinamide, topiramate & felbamate (serious adverse effects)
Lamotrigine – all but myoclonic, LEV – all but tonic
PHT – tonic clonazepam – myoclonic
Drop attacks – most difficult to treat- topiramate, felbamate,Lamotrigine, rufinamide
Atonic ,Atypical absence, Myoclonic – better
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ACTH & steroids – in SE
ketogenic diet-refractory to AED
Surgical-
corpus callosotomy – resistant drop attacks
vagus nerve stimulation – tonic & atonic
focal cortical resection
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LGS EM-AS of Doose
type Focal Generalised
Main types Tonic, atonic, atypical absence Myoclonic, atonic
tonic common never
atypical absence common uncommon
Devt. Before onset abnormal normal
West syndrome common uncommon
Background EEG abnormal normal
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“Partly reversible, epileptic encephalopathy of childhood manifesting with acquired verbal auditory agnosia and fluctuating course of the linguistic disturbances that occur together with other cognitive and neuropsychological behaviouralabnormalities.”
Age at onset : 2–8 yrs with a peak at 5–7 yrs.
male-to female ratio, 2 : 1
Etiology - unknown
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Linguistic abnormalities- normal developmental milestones before aphasia symptoms
All pts will have language abnormalities, 3/4th seizures
First symptom – verbal auditory agnosia, later all types of aphasia
Imp. Feature - fluctuating course
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Cognitive & Behavioural problems- 3/4th – ADHD
Seizures – 3/4th , nocturnal,
Types not well defined - (a/e tonic)
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Posterior temporal lobe foci of sharp-slow waves, multifocal and bi-synchronous
CSWS also occur(not needed for diagnosis) – also persist in REM sleep
Length of CSWS has strict correlation with language recovery
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Seizures,EEG abnormalities and language disturbance usually remit by 15yrs of age.
50% of patients relatively normal life with 10–20% achieving complete normalisation.
50% left with permanent sequelae that may be very severe.
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AEDs 1st line – VPA + clobazam
Phenobarbital, carbamazepine, and phenytoin – worsen EEG Discharges
Valproic acid, ethosuximide, and benzodiazepine alone or in combination – effective
clobazam and levetiracetam to be the most efficacious in the treatment of ESES.
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corticosteroids & ACTH – if AED fail
IVIG & ketogenic diet – no proven benefit
surgical intervention with multiple subpial transections (MSTs) – mainly in cases with secondary b/l synchrony
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Continuous spike-andwave during sleep. All discharges vanished after receiving ACTH
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Partly reversible, age-related childhood epileptic encephalopathy c/b “subclinical” spikes and waves occurring almost continuously during slow sleep and appearing every night for a variable length of time in children
triad of: EEG CSWS Seizures(except tonic seizures). Neuropsychological and motor impairment.
Onset of seizures - 2 m to12 yrs, with a peak at 4–5 yrs.
Male : female – 2/3rd
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seizure types- u/l or b/l clonic seizures, GTCS, absence, partial motor seizures.
Tonic seizures, however, never occur.
About 60% of patients also exhibit several types of Seizures
The majority of affected children never return to normal levels, particularly in the verbal area and attention
MRI brain – abnormal in many
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Inter-ictal – fronto or centro-temporal slow spikes
The typical EEG changes appear 1 year to 2 years after the first seizure and are associated with behavioral deterioration.
characteristic feature-continuous B/L spike-wave discharges on the EEG during NREM sleep(cause Unknown)
SWI – spike wave index - > 50 % NREM
> 85 % - poor prognosis
< 85% - better performance
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I stage is before the discovery of CSWS The EEG shows multi-focal spikes and bisynchronous generalised sharp or spike-wavedischarges. First seizure is usually nocturnal
II stage is when CSWS is found Increase in seizures and the appearance or deterioration of neuropsychological symptoms (frontal & temporal)that prompt a sleep EEG. Tonic seizures never occur Continuous bilateral and diffuse slowspikes-waves of 1.5–2 Hz during NREM
sleep is the defining EEG patternof ECSWS.
III stage is after clinical and EEG remission starts –2-7yrs from onset, seizures remit in all pts, EEG normalises, left with residual deficits
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Seizures remit in all by 10 – 15yrs age – irrespective of etiologyGood prognosis
Cognition & behaviour – incomplete recovery
Early treatment - better
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may or may not respond - benzodiazepines, valproate, ethosuximide, carbamazepine, phenytoin.
Only benzodiazepines and adrenocorticotrophic hormone have been reported to suppress the electrical Status
TOC - benzodiazepines + valproate
long-lasting improvement of the language function - multiple subpial transectionsof the region of focal epileptic discharges
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LKS CSWS
Seizures 3/4th All
Language Verbal auditory agnosia Expressive
Bahavioral Common All
CSWS onEEG 80% 100%
Spike localisation Temporal Frontal
Normal life 50% 25%
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Other Severe Epileptic Encephalopathies
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Both sexes are equally affected
Etiology- chronic viral infection, immune response to infection,immune-mediated causes.
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typically shows –
1. Focal motor seizures or epilepsia partialis continua (EPC)
2. Generalized seizures contralateral to the ictal and interictal epileptiform activity
3. Unilateral hemispheric cortical brain atrophy on MRI (abnormal signal T2/FLAIR and atrophy or hyperintense signal of the ipsilateral caudate head)
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MRI - to exclude other causes of focal seizures
EEG -
response to treatment is very poor
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“A previously normal child, aged 2–15 years, within a few days of developing a FOU, develops an acute prolonged perisylvian refractory convulsive status epilepticus, persisting for more than 1 month”
Later develops chronic pharmacoresistant epilepsy and cognitive dysfunction
Cause unknown, may be immune-mediated.
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Interictal EEG - diffuse slowing
Ictal EEG - fast activity intermixed with spikes
MRI brain - normal or may show b/l hippocampal abnormality
seizures are resistant to AEDs
Steroids/ immunotherapy may be tried
prognosis is poor
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Early Myoclonic Encephalopathy- Suspected if –
Early Continuous shifting Segmental and erratic myoclonus f/b focal seizures, rarely tonic seizures
burst short, suppression long on EEG
Ohtahara Syndrome
Early Tonic seizures, focal seizures
Burst suppression EEG with Pseudo-rhythmic appearance
burst long, suppression short on EEG
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Dravet Syndrome
Intractable myoclonic jerks and MR within 1-2 yrs from onset along with
febrile seizures –
prolonged > 15min
unilateral
mainly clonic
frequent
ppted by low fever
early onset (<1yr)
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West syndrome Infantile spasms, clustering on arousal
Lennox–Gastaut Syndrome Multiple seizure types with preexisting or newly developing cognitive and
behavioural problems
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Epileptic Encephalopathies
Neonates(Burst suppression on EEG)
Infants(Hypsarrythmia on EEG) Children
(GSWD on EEG)
EME Ohtahara syn West synDravet syn LGS LKS CSWS
LKS CSWS
Seizures 3/4th All
Language Verbal auditory agnosia
Expressive
Bahavioral Common All
CSWS onEEG
80% 100%
Spike localisation
Temporal Frontal
Normal life 50% 25%
Infantile spasmsHypsarrythmia30 % LGSDOC–ACTH + vigabatrin
Multiple seizures-tonic,
atonic, atypical absence
EEG-GSWD with paroxysmal fast activity
SCN1A mut –70%Atypical febrile seizuresStiripentol-recently approved
MC cause-Inborn errors of metabolismImp – erratic myoclonus, no tonic spasams
MC-structural abnormalitiesImp-tonic seizures, rarely myoclonus
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The spectrum from neonatal to early childhood is c/b variable clinical and EEG features – reflecting developmental age rather than underlying etiology.
Early treatment & prevention of seizure activity gives better cognitive outcomes and quality of life
As these are drug resistant, further studies are needed
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Khan S, Baradie RA. Epileptic Encephalopathies: An Overview Hindawi Publishing Corporation,Epilepsy Research and Treatment. Volume 2012, 8 pages doi:10.1155/2012/403592
Bradley’s textbook of Neurology, 7th edition
Covanis A. Epileptic encephalopathies (including severe epilepsy syndromes).Epilepsia, 53(Suppl. 4):114–126, 2012 doi: 10.1111/j.1528-1167.2012.03621.x
eMedicine@medscape:Epileptic and Epileptiform Encephalopathies