epilepsy and learning difficulties

8/2/2019 Epilepsy and Learning Difficulties

1/50

Epilepsy and Learning Disability

Wessex Epilepsy Interest group24th June 2011


2/50

Learning difficulties and epilepsy

Major co-morbidity

Complex association

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??


3/50

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


4/50

Cerebral palsy

Risks of epilepsy:

4 limb involvement 50-94%

Hemiplegia 22-47%

Diplegia 16-27%

EEG abnormalities common in childrenwith CP +/- epilepsy


5/50

The epileptic encephalopathies

Definition:

A condition in which the epileptiform

abnormalities are believed to contribute

to progressive disturbance in cerebral

function


6/50

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.


7/50

West syndrome- prognosis

Prognosis is determined nearly exclusively

by the causative factors and their severity.

The spasms themselves and their responseto treatment may not have prognostic

significance

Panayiotopoulos


8/50

Epilepsy in Infancy study

Developmental abilities approximately 3 months afterdiagnosis are predictive of subsequent developmentalability at 12 months follow up.

Children with structural brain abnormalities have areduction in developmental cognitive score over time

Seizure status does not influence cognitive outcome 1year after diagnosis


9/50

LGS - prognosis

Poor outcome associated with:

Early age of onset

Symptomatic aetiology

Frequent tonic seizures

Repeated episodes of NCS

Consistently slow EEG background


10/50

Learning

difficulties

Aetiology

Seizures


11/50

Aetiology???????????

Dilemma regarding diagnosis.


12/50

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

Chromosome arraytesting..


13/50

Aetiology

Genetic

Metabolic

Structural

Neurocutaneous

Tumour


Mitochondrial

Biotinidase deficiency

Peroxisomal

Lysosomal

Creatine disorders

Purine disorders

Amino/organic acids

Vitamin responsiveepilepsies

.


14/50

Aetiology

Genetic

Metabolic

Structural

Neurocutaneous

Tumour


Perisylvian

syndromes

Periventricular

And nodular

heterotpia

Hemimegalencephaly


15/50

Aetiology

Genetic

Metabolic

Structural

Neurocutaneous

Tumour

http://img.webmd.com/dtmcms/live/webmd/consumer_assets/site_images/articles/health_tools/what_your_skin_says_about_your_health_slideshow/dermnet_rf_photo_of_tuberous_sclerosis.jpghttp://img.webmd.com/dtmcms/live/webmd/consumer_assets/site_images/articles/health_tools/what_your_skin_says_about_your_health_slideshow/dermnet_rf_photo_of_tuberous_sclerosis.jpg


16/50

Aetiology

Genetic

Metabolic

Structural

Neurocutaneous

Tumour



17/50

Aetiology

Genetic

Metabolic

Structural

Neurocutaneous

Tumour

http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


18/50

Aetiology

Genetic

Metabolic

Structural

Neurocutaneous

Tumour


NMDAR

VGK

GAD

Hashimoto encephalitis

Rassmussen encephalitis
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


19/50

Some syndromes.
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


20/50

Electrical Status Epilepticus in Slow wave sleep

NREM

REM
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


21/50

Electrical Status Epilepticus in

Slow wave sleep Triad of:

Continuous spike and slow wave activity in

NREM sleep

Seizures

Neuropsychological and motor impairment
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


22/50

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
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


23/50

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
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


24/50

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

Duration of ESES related to finalneuropsychological functioning
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


25/50

Motor effects of ESES

Ataxia

Dyspraxia

Hemiparesis
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


26/50

Treatment of ESES

Sodium valproate, LVT, LTG

Benzodiazepines

Steroids

Multiple subpial transection in LKS
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


27/50

Landau-Kleffner syndrome

Acquired epileptic aphasia

Onset age 2-8 years

M>F

Partly reversible epileptic encephalopathy

Cognitive/behavioural/neuropsychologicaldifficulties

+/-Seizures
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


28/50

LKS- linguistic difficulties

Auditory verbal agnosia

Unable to understand linguistic sounds

May progress to inability to make sense of non-

linguistic sounds Mistaken as deafness, mutism, autism

Affects other linguistic functions

Expressive speech

Perseverations

jargon speech

May become entirely mute

May be fluctuating in nature
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


29/50

LKS- cognitive/behavioural

Cognitive and behavioural difficulties

common

ADHD/ hyperactivity

Rarely psychosis

Variable


30/50

LKS- seizures

In 75%

Infrequent, usually well controlled

Various seizure typesGTCS

Focal seizure

Atonic Often nocturnal

Remit before age 15y
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


31/50

LKS- pathophysiology

Functional epileptogenic lesion in eloquentspeech area

MRI usually normal

Occur at a critical age of brain development

Aggressive epileptic activity in the

dominant temporal lobe detrimental toestablishment of appropriate neuronalconnections
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


32/50

LKS

BECTS LKS

?Language impairment
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


33/50

LKS - EEG

Posterior temporal foci +/- multifocal

sharp/spikes, bisynchronous activity

Markedly facilitated by NREM sleep

ESES at some point in most cases
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


34/50

LKS - prognosis

EEG abnormalities and seizures agedependant

Remit by 15 years Cognitive and language improves as EEG

normalises

Influenced by duration of ESES and age ofonset

10-20% achieve complete normalisation


35/50

Autism-epilepsy connection

Prevalence of epilepsy in children withautism 5-38%

Bimodal incidenceInfancy - 5years

2nd peak in adolescence [>10y]

Risk of epilepsy higher in children withsevere LD

Cumulative probability of epilepsy 8% at 10 yrsnormal cognition, 27% at 10 years if SLD

Epilepsy persists into adult life


36/50

EEG in autism

EEG abnormalities frequently seen in theabsence of clinical seizures

18.9% in a study of 106 people with autism 3-31 yrs of age

21-68% have epileptiform sleep EEG

Usually focal spikes, may be multifocal

Abnormalities frequently similar to thoseseen in benign focal epilepsies of childhood
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


37/50

Autism and epilepsy..

? Autism and epilepsy both different clinical

manifestations of the same brain pathology

[fragile X, TSC] ? Temporal lobe dysfunction

Higher rates of autism in TLE

Autism an end result of epileptic encephalopathy

ie, West syndrome, LGS, Dravet syndrome

Epilepsy early in brain development affects neuronal

networks
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


38/50

Should we do an EEG in children

with autistic regression?

No evidence that epilepsy is a risk factor for

autistic regression

LKS/ESES may be misdiagnosed as autism

but phenotype distinctly different
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


39/50

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
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


40/50

The child with severe learning

disability and epilepsy

Is it epilepsy???

? Mixed epileptic and non-epileptic events

Staring spells

Stiffenings

Stereotypies

NEAD


41/50


disability and epilepsy What are the seizure types?

What is the epilepsy syndrome?

Correct AED

Awareness of AEDs that may exacerbate

behavioural problems

LVTAvoid polypharmacy

? Non-medical treatment options..
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


42/50


disability and epilepsy

What is the aetiology?

? Focal brain lesion

? Epilepsy surgery an option

LD is NOT a contraindication for surgery


43/50


disability and epilepsy What are the co-morbidities?

Behaviour

Psychological

Sleep


44/50

The co-morbidity of childhood

epilepsy The Isle of Wight study, Rutter et al, 1970

Rate of psychiatric disorder

In the general population: 7%Children with physical disorder outside the

CNS: 12%

Children with idiopathic seizures: 29%

Children with structural brain abnormalities:38%

Children with seizures AND structural brainabnormalities: 58%

A population survey of mental health


45/50

A population survey of mental health

problems in children with epilepsy[Goodman et al, Dev Med and Child Neurol, 2003]

Rates of DSM IV psychiatric disorder37% epilepsy

11% diabetes

9% in 10202 control children Both uncomplicated and complicated

epilepsy groups have increase in emotionaland behavioural disorders

Only the complicated group was associatedwith significant increase rate ofhyperactive and pervasive developmental

disorders

Family andAdolescence
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


46/50

Psychopathology

In childhood epilepsy

Social

stigma

Self

Esteem

AEDs

Seizure

type

Seizurecontrol

StructuralBrain

abnormalitySleep

Education

Polypharmacy

Family and

Social factors

Adolescence

Learning

disability

Sl di d
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


47/50

Sleep disorders are more

common in children with

epilepsy [stores et al, 1998] 79 children with epilepsy, 73 controls

Higher rates in children with epilepsy of:

Poor quality sleep

Anxieties regarding sleep

Sleep disordered breathing

Short duration sleep

?contributor to the learning and behavioural

difficulties
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.htmlhttp://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


48/50

Summary

Relationship between epilepsy and learningdisability complex and reciprocal

Some epileptic encephalopathies may betreatable

Management requires a structured andholistic approach


49/50

Questions???
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html


50/50
http://www.lib.uiowa.edu/hardin/md/dermnet/herpessimplex21.html

epilepsy and learning difficulties

Documents