Epilepsy in the Primary School Aged Child

Transcription

1 Epilepsy in Primary School Aged Child Deepak Gill Department of Neurology and Neurosurgery The Children s Hospital at Westmead CHERI Research Forum 15 July 2005

2 Overview The School Age Child and Epilepsy Diagnosis of Epilepsy Identification of Epilepsy Syndrome Rational Treatment Options Side-Effects of Anti-epileptic medications

3 The Child at The Centre of Epilepsy Care Paediatrician/ Neurologist Child School Parent

4 The Child at The Centre of Epilepsy Care Paediatrician/ Neurologist Child Acute Seizure/ Safety School Parent Learning/ Cognition Social Difficulties

5 The Child at The Centre of Epilepsy Care Paediatrician/ Neurologist Eye witness account of Seizure Child Observation of seizure frequency School Parent Observation of cognitive abilities

6 The Child at The Centre of Epilepsy Care Paediatrician/ Neurologist Neurology Nurse/Epilepsy Support Group Child Acute Seizure/ Safety School Parent Learning/ Cognition Social Difficulties

7 The Child at The Centre of Epilepsy Care Neurology Nurse/Epilepsy Support Group Paediatrician/ Neurologist Child School Parent

8 The Child at The Centre of Epilepsy Care Paediatrician/ Neurologist Neurology Nurse/Epilepsy Support Group Epilepsy Education School Child Parent

9 Definitions Seizure An Episode of transient alteration of cerebral function Epileptic Seizure- onset is electrochemical (cf anoxic, traumatic etc) Epilepsy Recurrent Epileptic Seizures Epileptic disorders are similar in that seizures predominate clinical picture; however symptom of a seizure may be due to very diverse aetiologies

10 Age-specific incidence of epilepsy in industrialized countries (Hauser 1998)

11 The Diagnosis of Epilepsy Epilepsy is a clinical diagnosis Partial (focal) v Generalised Idiopathic v Symptomatic Prevalence 0.5-1% 70% Well controlled or no medication 30 % Difficult to Control epilepsy

12 Concept of Epilepsy Syndromes Clusters of Signs and Symptoms customarily occurring toger. A syndrome may have several causes and different outcomes (!) Level of specificity and precision uneven To serve as a basis of classification, a syndrome should be well-characterised, sufficiently agreed upon and defined in a sufficient number of patients Aicardi 2004

13 Paediatric Epilepsy syndromes Benign 0y 2y 3y 8y 10y 18y Cognitive morbidity

14 Paediatric Epilepsy syndromes Benign Neonatal Seizures Benign Benign Infantile Seizures Cognitive morbidity 0y 2y 3y 8y 10y 18y West Syndrome Dravet Syndrome Migratory seizures of Infancy Ohtahara Syndrome Early Myoclonic Encephalopathy

15 Paediatric Epilepsy syndromes Focal Generalised Focal and Generalised Benign Neonatal Seizures Absence Epilepsy Benign Benign Infantile Seizures BFEC Juvenile Myoclonic Epilepsy Cognitive morbidity 0y 2y 3y 8y 10y 18y West Syndrome Lennox-Gastaut Dravet Syndrome CSWS Myoclonic Astatic epilepsy Migratory seizures of Infancy Ohtahara Syndrome Early Myoclonic Encephalopathy

16 Paediatric Epilepsy syndromes Focal Generalised Focal and Generalised Benign Neonatal Seizures Absence Epilepsy Benign Benign Infantile Seizures BFEC Juvenile Myoclonic Epilepsy Cognitive morbidity 0y 2y 3y 8y 10y 18y West Syndrome Lennox-Gastaut Dravet Syndrome CSWS Myoclonic Astatic epilepsy Migratory seizures of Infancy Ohtahara Syndrome Early Myoclonic Encephalopathy Modified from Hirsch 2004

17 Epilepsy Syndromes and EEG EEG may be encephalopathic in severe disorders Abnormal EEG may be seen in benign disorders.?correlation with cognition Some severe disorders eg Dravet syndrome (severe myoclonic epilepsy of infancy) may have normal EEG

18 Paediatric Epilepsy syndromes Focal Generalised Focal and Generalised Benign Neonatal Seizures Absence Epilepsy Benign Benign Infantile Seizures BFEC Juvenile Myoclonic Epilepsy Cognitive morbidity 0y 2y 3y 8y 10y 18y West Syndrome Lennox-Gastaut Dravet Syndrome CSWS Myoclonic Astatic epilepsy Migratory seizures of Infancy Ohtahara Syndrome Early Myoclonic Encephalopathy

19 Paediatric Epilepsy syndromes Focal Generalised Focal and Generalised Benign Neonatal Seizures Absence Epilepsy Benign Benign Infantile BFEC Focal - Lesional Epilepsy Juvenile Myoclonic Epielpsy Seizures Cognitive morbidity 0y 2y 3y 8y 10y West Syndrome Lennox-Gastaut Dravet Syndrome CSWS Myoclonic Astatic epilepsy Migratory seizures of Infancy Ohtahara Syndrome Early Myoclonic Encephalopathy Focal - Lesional Epilepsy 18y

20 Focal Epilepsies- Epilepsy Syndromes and MRI The site of lesion may be predictive of cognitive impairment Mesial temporal lobe epilepsy Occipital Lobe Epilepsy Frontal lobe epilepsy

21 Focal Epilepsies- Epilepsy Syndromes and MRI The site of lesion may be predictive of cognitive impairment Mesial temporal lobe epilepsy Occipital Lobe Epilepsy Frontal lobe epilepsy

22 Focal Epilepsies- Epilepsy Syndromes and MRI The site of lesion may be predictive of cognitive impairment Mesial temporal lobe epilepsy Occipital Lobe Epilepsy Frontal lobe epilepsy

23 Focal Epilepsies- Epilepsy Syndromes and MRI The site of lesion may be predictive of cognitive impairment Mesial temporal lobe epilepsy Occipital Lobe Epilepsy Frontal lobe epilepsy

24 Cognition and Focal (Lesional) Epilepsy Site of Lesion Nature of lesion Localised or diffuse Bilateral or Unilateral Chronicity of epilepsy Chronic focal epilepsy generalised epilepsy EEG may become encephalopathic High risk of cognitive impairment Role of Early Surgical Intervention in Focal Epilepsies?

25 Cognition and Focal (Lesional) Epilepsy Site of Lesion Nature of lesion Localised or diffuse Bilateral or Unilateral Chronicity of epilepsy Chronic focal epilepsy generalised epilepsy EEG may become encephalopathic High risk of cognitive impairment Role of Early Surgical Intervention in Focal Epilepsies?

26 The Child at The Centre of Epilepsy Care Paediatrician/ Neurologist Child Acute Seizure/ Safety School Parent Learning/ Cognition Social Difficulties

27 Cognition and Behaviour Complex and multifactorial Epilepsy more frequent in intellectually disabled (ID) children Not all children with epilepsy have cognitive deficits Good data is scarce Many studies have high rates of ID ID related to severity of epilepsy Short seizures no effect on gross development

28 Cognitive Impairments Attention Memory Mental Speed Language Executive Social Exacerbated by Increased Seizure frequency Multiple anti-epileptic medication

29 Behavioural Impairments Fatigue Depression Anxiety Psychosis

30 Cognition and Behaviour Idiopathic Epilepsies - most children normal or superior intelligence 98 with non-lesional epilepsy did less well than 96 asthma pts in all domains 1 51 children v 48 controls, 48h after dx, 3m, 12 m; no persistent attention deficits, no detremental effect of AED; related to prior school or behav diffic, maladaptive reaction from parents Complex Partial Seizures inferior to controls in seven cognitive domains 2 Early age onset-- related to cognitive deficit Seizure frequency -- related to behavioural difficulties 1 Austin et al, Dev Med Child Neurol Oostromet al Epilepsia Schoenfeld et al, Dev Med & Child Neurol 1999

31 Cognition and Behaviour Decline Risk factors Multiple short seizures - transient cognitive impairment (TCI). Untreated absence epilepsy, atypical absences Syndromes of early childhood West Syndrome Lennox-Gastaut syndrome SMEI / Dravet Syndrome Continuous Discharges on EEG Landau-Kleffner, ESES, Frontal ESES?BFEC

32 Cognition and Behaviour Anti-Epileptic Drugs (AED s) Few good studies Combination rapy, retrospective, controls, small sample size, Global IQ (insensitive for subtle changes) Many inconclusive Not all behavioural disturbances are due to AED s Older AED;- Phenobarbitone, older benzodiazepines greatest risk No consistent difference between carbamazepine, phenytoin, valproate

33 Paediatric Epilepsy syndromes Focal Generalised Focal and Generalised Benign Neonatal Seizures Absence Epilepsy Benign Benign Infantile Seizures BFEC Juvenile Myoclonic Epilepsy Cognitive morbidity 0y 2y 3y 8y 10y 18y West Syndrome Lennox-Gastaut Dravet Syndrome CSWS Myoclonic Astatic epilepsy Migratory seizures of Infancy Ohtahara Syndrome Early Myoclonic Encephalopathy

34 Newer Anti-Epileptic Drugs (AED s) Clobazam Lamotrigine - increased alertness, aggression Topiramate - emotional lability, fatigue, word-finding, despite improved seizure control 1. Levetiracetam - Numbers too few Aggression, agitation, sedation 1 Elterman RD, Neurology 1999

35 The Child at The Centre of Epilepsy Care Paediatrician/ Neurologist Child Acute Seizure/ Safety School Parent Learning/ Cognition Social Difficulties

36 Social Difficulties Over-protection Low Self-esteem Peer-peer relationships Exclusion Neurological disorder Poor non-verbal ability

37 The Child at The Centre of Epilepsy Care Review diagnosis Paediatrician/ Neurologist?Epilepsy Syndrome?Intractable Epileptic v Non Epileptic Child School Parent

38 Conclusions Epilepsy is associated with co morbidities of cognitive and behavioral difficulties Epileptic syndromes group toger children with similar clinical (?and cognitive) characteristics Rational drug rapy based on epilepsy syndromes may reduce cognitive morbidity Early intervention with surgery may be beneficial in early childhood in children with discrete lesions Furr studies are needed to assess cognitive effects of AED s in children