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Epilepsy and Learning Difficulties
Epilepsy and Learning Difficulties
Often un-noticed Vast differential diagnosis of aetiology Is it only a problem in the symptomatic group? Epileptic encephalopathy Will it get better if the seizures go away? Is it epilepsy??
Epilepsy- epidemiology
Prevalence rates 3.6-6.5/1000 in Europe Increased risk of 11% with either CP or LD
LD, IQ50-70: 15%, IQ<50: 30%
LD and concurrent CP increases risk of epilepsy to 48% Neurocutaneous syndromes associated with very high rates of epilepsy
91% in Tuberous Sclerosis
Cerebral palsy
Risks of epilepsy: 4 limb involvement 50-94% Hemiplegia 22-47% Diplegia 16-27% EEG abnormalities common in children with CP +/- epilepsy
Epileptic encephalopathy
Neonatal syndromes West syndrome
2/3 severe cognitive and psychological impairment 5-12% normal cognitive development
Dravet syndrome Myoclonic astatic syndrome Landau-Kleffner syndrome Lennox Gastaut syndrome.
LGS - prognosis
Poor outcome associated with:
Early age of onset Symptomatic aetiology Frequent tonic seizures Repeated episodes of NCS Consistently slow EEG background
Aetiology
Learning difficulties
Seizures
Aetiology???????????
Dilemma regarding diagnosis.
Aetiology
Genetic Metabolic Structural Neurocutaneous Tumour Infective Immune mediated
Gene mutations
Sodium channel/ SCN1A
Chromosomal
1p36, Wolf-Hirschhorn T21, Angelman syndrome Fragile X Ring chromosome 20
Aetiology
Genetic Metabolic Structural Neurocutaneous Tumour Infective Immune mediated
Mitochondrial Biotinidase deficiency Peroxisomal Lysosomal Creatine disorders Purine disorders Amino/organic acids Vitamin responsive epilepsies .
Aetiology
Genetic Metabolic Structural Neurocutaneous Tumour Infective Immune mediated
Aetiology
Genetic Metabolic Structural Neurocutaneous Tumour Infective Immune mediated
Aetiology
Genetic Metabolic Structural Neurocutaneous Tumour Infective Immune mediated
Aetiology
Genetic Metabolic Structural Neurocutaneous Tumour Infective Immune mediated
Aetiology
Genetic Metabolic Structural Neurocutaneous Tumour Infective Immune mediated NMDAR VGK GAD Hashimoto encephalitis Rassmussen encephalitis
Some syndromes.
NREM
REM
ESES
Age dependant
ONLY occurs in childhood
3 stages
Initial seizures, often nocturnal and focal
Benign and symptomatic aetiology
ESES starts 1-2 years after initial seizure Remission months-7 years from onset
Seizures in ESES
Habitual seizures and new seizures emerge.. Hemi-clonic , GTCS [mostly nocturnal] typical/atypical absences, atonic, myoclonus, NCSE
Most children have numerous, frequent seizures
Negative myoclonus
Neuropsychology of ESES
Dramatic decline in cognitive abilities Deficit dependant on spike localisation
Frontal
Executive functioning, language, behaviour
Opercular
Drooling, dysarthria, speech arrest
Temporal
Linguistic difficulties
Hemiparesis
Treatment of ESES
Sodium valproate, LVT, LTG
Benzodiazepines Steroids Multiple subpial transection in LKS
Landau-Kleffner syndrome
Acquired epileptic aphasia
Onset age 2-8 years M>F
LKS- cognitive/behavioural
Cognitive and behavioural difficulties common ADHD/ hyperactivity Rarely psychosis Variable
LKS- seizures
In 75% Infrequent, usually well controlled Various seizure types
GTCS Focal seizure Atonic
LKS- pathophysiology
Functional epileptogenic lesion in eloquent speech area MRI usually normal Occur at a critical age of brain development Aggressive epileptic activity in the dominant temporal lobe detrimental to establishment of appropriate neuronal connections
LKS
?
Language impairment
BECTS LKS
LKS - EEG
Posterior temporal foci +/- multifocal sharp/spikes, bisynchronous activity
Markedly facilitated by NREM sleep
LKS - prognosis
EEG abnormalities and seizures age dependant Remit by 15 years Cognitive and language improves as EEG normalises Influenced by duration of ESES and age of onset 10-20% achieve complete normalisation
Autism-epilepsy connection
Prevalence of epilepsy in children with autism 5-38% Bimodal incidence
Infancy - 5years 2nd peak in adolescence [>10y]
EEG in autism
EEG abnormalities frequently seen in the absence of clinical seizures
18.9% in a study of 106 people with autism 331 yrs of age 21-68% have epileptiform sleep EEG
Usually focal spikes, may be multifocal Abnormalities frequently similar to those seen in benign focal epilepsies of childhood
Autism an end result of epileptic encephalopathy ie, West syndrome, LGS, Dravet syndrome
Epilepsy early in brain development affects neuronal networks
Rett syndrome
Background EEG becomes epileptiform Epileptic and non-epileptic events
Stereotypies Hyperventilation Dystonia
Genetics:
MECP2
80% of cases
CDKL5
Early onset seizures, infantile spasms, tonic and focal seizures
Behaviour Psychological
Sleep
Both uncomplicated and complicated epilepsy groups have increase in emotional and behavioural disorders Only the complicated group was associated with significant increase rate of hyperactive and pervasive developmental disorders
Adolescence
Social stigma
Self Esteem
Sleep disorders are more common in children with epilepsy [stores et al, 1998]
79 children with epilepsy, 73 controls
Summary
Relationship between epilepsy and learning disability complex and reciprocal Some epileptic encephalopathies may be treatable Management requires a structured and holistic approach
Questions???