Landau Kleffner Syndrome Learning and Behaviour

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Landau Kleffner SyndromeLearning and Behaviour

• Neurodisability Service, GOSH

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Outline

• Epilepsy
• LKS
• Assessment and Intervention

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Epilepsy

• Electrical storm
• Noise
• Causes interference

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How Common?

• Learning disability and Epilepsy

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• 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

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Risk Factors for Learning difficulties
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Cognitive Profiles

• Pervasive / Specific deficits
• Attention (Williams 96)
• Memory
• Motor / perceptual skills (Parsons 71)
• Executive function

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Learning difficulties

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

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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

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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
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At Assessment
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Associated impairments
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Fluctuation

• Not typical of other learning difficulties
• Unpredictable
• May lead to wrong assumptions

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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

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Strategies

• Flexible approach
• Windows of opportunity
• Over-teaching

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School response

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

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How Common?

• Behavioural difficulties and Epilepsy

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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

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Increased incidence of behaviour problems seen in

• New onset epilepsy
• 6mns prior to 1st seizure

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Risk Factors for Behavioural problems
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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

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Behavioural syndromes

• ADHD
• Autistic Spectrum Disorder

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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

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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
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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

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Assessment Formulation

• ABCs
• Standardised measures
• Formulation
• Plan appropriate intervention

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Intervention

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

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Epilepsy Learning - Behaviour

• Cognitive, emotional and behavioural problems
  are an integral component of the disability of
  epilepsy