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Landau Kleffner Syndrome Learning and Behaviour

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Title: Landau Kleffner Syndrome Learning and Behaviour


1
Landau Kleffner SyndromeLearning and Behaviour
  • Neurodisability Service, GOSH

2
Outline
  • Epilepsy
  • LKS
  • Assessment and Intervention

3
Epilepsy
  • Electrical storm
  • Noise
  • Causes interference

4
How Common?
  • Learning disability and Epilepsy

5
  • 30 with epilepsy have a LD
  • 30 with a severe LD have epilepsy
  • 3-10 with mild/moderate LD have epilepsy
  • lt0.5 without LD have epilepsy

6
Risk Factors for Learning difficulties
7
Cognitive Profiles
  • Pervasive / Specific deficits
  • Attention (Williams 96)
  • Memory
  • Motor / perceptual skills (Parsons 71)
  • Executive function

8
Learning difficulties
  • Even if IQ is controlled for, those with epilepsy
    experience more academic problems and
    underachievement

9
LKS
  • Mainly affects language abilities
  • Other higher functions may be involved
    (attention, social interaction, behaviour, motor)
  • Non-verbal cognitive skills are relatively spared
  • Impairments - related to seizure activity -
    may recover

10
Language at presentation
Comprehension
Partial loss of understanding of speech (9/26)
Loss of understanding of speech preserved
understanding of other sounds (8/26)
Loss of understanding of speech and environmental
sounds (9/26)
Speech
Partial loss of speech (12/29)
Complete loss of spoken language (17/29)
GOSH 2006
11
At Assessment
12
Associated impairments
13
Fluctuation
  • Not typical of other learning difficulties
  • Unpredictable
  • May lead to wrong assumptions

14
Contributors to Fluctuation
  • Seizure activity
  • Night time (e.g. ESES)
  • Clinical (e.g. absences)
  • Peri and post Sz disturbance
  • Sleep disturbance
  • Drug related side effects
  • Fear / anxiety

15
Strategies
  • Flexible approach
  • Windows of opportunity
  • Over-teaching

16
School response
  • Obtain developmental profiles
  • Modified curriculum
  • Adapted teaching strategies
  • Good communication
  • Staff knowledge and training

17
How Common?
  • Behavioural difficulties and Epilepsy

18
Isle of Wight Study(Rutter et al., 1970)
  • Behaviour problems in children
  • 6.6 general population
  • 11.6 physical disability (not brain)
  • 28.6 uncomplicated seizures
  • 37.5 brain damage
  • 58.3 seizures and brain damage

19
Increased incidence of behaviour problems seen in
  • New onset epilepsy
  • 6mns prior to 1st seizure

20
Risk Factors for Behavioural problems
21
Antiepileptic drugs
  • Common parental concern
  • More likely if more than one drug
  • Effect is not always negative
  • Behavioural disturbance mimics ADHD
  • Phenobarbitone Benzodiazepines Gabapentin
  • Vigabatrin
  • irritability, agitation, aggression, psychosis,
    depression
  • Topiramate
  • increase negative behaviours
  • Lamotrigine
  • - Sleep disturbance

22
Behavioural syndromes
  • ADHD
  • Autistic Spectrum Disorder

23
Assessment of behaviour in children with epilepsy
  • Vital
  • Multi-disciplinary
  • Cant interpret effectively w/o knowledge of full
    medical history, cognitive level etc
  • Expertise
  • Disentangling organic and functional aspects can
    be very difficult

24
Influences on Behaviour
Intrinsic Factors EPILEPSY Age Sex Temperament
Genes Cognitive factors Lang/communication Develop
mental or psychiatric disorder
External Factors Parenting Bullying Child
Abuse Home situation School situation Siblings
Behaviour
Psychological Factors Attachment Relationship
s Self-esteem Anxiety
25
Information Gathering
  • Identify the behaviour
  • Key questions what/where/when
  • Methods for information gathering
  • Interview, observation, other sources
  • Clinical screening instruments
  • What to look for
  • cognitive level history/duration temporal
    relationships medication changes parenting

26
Assessment Formulation
  • ABCs
  • Standardised measures
  • Formulation
  • Plan appropriate intervention

27
Intervention
  • Disruptive disorders
  • CD ODD most evidence for behavioural
    treatments
  • ADHD may respond to methylphenidate
  • Affective disorders
  • Depression
  • Anxiety disorders (GAD, fears/phobias, OCD)

28
Epilepsy Learning - Behaviour
  • Cognitive, emotional and behavioural problems
    are an integral component of the disability of
    epilepsy
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