Issues in Developmental Disabilities Epilepsy in the Intellectually and Developmentally Disabled

1. Issues in Developmental DisabilitiesEpilepsy in the Intellectually and Developmentally Disabled Lecture Presenter: Christopher M. Inglese, M.D. Regional Epilepsy Center St. Luke's Medical Center Milwaukee,Wisconsin

2. Video of Inglese

3. Epilepsy In The Multiply-Handicapped • Worldwide movement to de-institutionalize patients with MR • Improved seizure control, fewer side effects and less complicated regimens allow more successful placement in community

4. Intellectual and Developmental Disabilities Associated with Epilepsy • Cognitive • Motoric • Sensory • Attentional • Behavioral • Affective

5. Cognitive Mental Retardation • SMR • MMR • Learning Disabilities • Apraxias/Dyspraxias

6. Motoric Cerebral Palsy • Spastic • Extrapyramidal • Developmental Dyspraxias • Hypotonia • Weakness

7. Sensory • Hearing Loss • Visual Impairment • Sensory Integration Dysfunction

8. Attentional • ADHD -Combined Type, Inattentive Subtype • Primary Disorders of Vigilance • Secondary Disorders of Vigilance

9. Behavioral • Impulsivity • Hyperkinesis • Affective Storms • Episodic Dyscontrol • Self Injurious Behavior • Aggression

10. AffectiveMood Disorders • Anxiety • Depression • Bipolar, Cyclic mood disturbances • Thought Disorders

11. Autistic Spectrum Disorders • Aspergers • Hellers • Retts • Kanners (classical autism) • PDD NOS

12. Common Medical Comorbidities • Congenital malformations • Chromosomal Abnormalities • Genetic Disorders • Metabolic Disorders • Static Enephalopathis

13. Terminology & DefinitionsDiagnostic Criteria for Mental Retardation • IQ < 70 • Impairment in interpersonal relations, self-care, maturation • Onset before age 18 • DSM IV 37.90

14. Seizures The outward manifestations of the epilepsies can be purely subjective, experiential, imposed emotions.

15. Epilepsy A predisposition for unprovoked, recurrent seizures by a proximate identifiable cause.

16. Epileptic Syndromes Collections of signs, symptoms from a common cause which define recognizable patterns of disease.

17. The Classification of the Epilepsies There are many ways to classify the epilepsies or seizures

18. Classifications cont. • By Cause or Etiology • Idiopathic • Cryptogenic • Symptomatic

19. By Clinical Appearance

20. Partial Onset Generalized Onset By Electro-Clinical Characteristics* *Determined by the Anatomic Substrate of the Seizure Generator

21. Complete History Detailed physical/neuro exam Family History Routine blood work, toxic and metabolic screening, serum levels EEG (often requires sedation) Neuro-imaging (MRI preferred) Video-EEG monitoring Video-recording of events Diagnostic Evaluation

22. Why is Classification Important? • Basic Science and Clinical Scientists must have uniformity of definitions in heterogeneous conditions • “Apples to apples, oranges to oranges”

23. Classification Facilitates Research • Causal Mechanisms • Treatments • Outcomes • Predispositions

24. Partial Seizures Simple Partial Complex Partial Simple or Complex Partial which generalize Sensory Motor Autonomic International Classification of Epileptic Seizures

25. International Classification of Epileptic Seizures-Generalized • Absence (typical and atypical) • Myoclonic • Tonic • Clonic • Atonic-astatic

26. International Classification of Epileptic Seizures-Unclassified • Febrile Seizures • Reflex Epilepsies • Status Epilepticus

27. Classification of Epilepsy Syndromes • Idiopathic focal epilepsies • Familial focal epilepsies • Symptomatic and Cryptogenic focal epilepsies

28. Idiopathic Generalized Epilepsies • Reflex Epilepsies • Epileptic Encephalopathies • Progressive myoclonus epilepsies

29. Epidemiology and Statistics-Prevalence • Numerator-old and new cases • Denominator-population at risk

30. Epidedemiology (continued) • Prevalence of MMR IQ < 70 3.7-7.6 per 1000 • Prevalence of SMR IQ < 50 2.8-4.6 per 1000 • Prevalence of epilepsy 4.0-8.8 per 1000 • Prevalence of MR in childhood epilepsy 31-41%

31. Epidedemiology (continued) • MMR and epilepsy 8-18% • SMR and Epilepsy 30-36% • Prevalence of Epilepsy in Swedish study of 6-13 year olds – 2 per 1000 (98 of 48,873)

32. The risk of Epilepsy increases 30 fold when associated with: • TBI • CP • MR • The risk is 5-15% higher with previous meningitis or encephalitis • Hauser and Nelson CP or MR 11% w/ epilepsy-Both CP/MR 48% with Epilepsy

33. Disease stigma Autonomy Driving restrictions Impact of seizures on memory Impact of treatment on mood, memory motivation to learn Occupational restrictions Discrimination Impact on learning of ictus, interictal state, postical state Epilepsy can be a disabling condition in and of itself

34. Cognitive Neuromotor Sensory Attentional Behavioral self regulatory Affect and mood Epilepsy Can tremendously potentiate the impact of a disability when added to co-existing challenges, comorbidities

35. Is it Epilepsy? Both epileptic and non-epileptic seizures? Are seizures caused exclusively by controllable medical conditions? Cardiac? Hemodynamic-vascular? Iatrogenic? Endocrenologic? Metabolic? General Principles of Management-Diagnostic

36. General Principles of Treatment:Is Treatment Necessary? • Febrile Fits • BRE • Select appropriate drug for seizure type or syndrome • Avoid seizure exacerbating drugs • Select drug that may target other issues of importance to patient • Migraine, mood, sleep, weight, sex

37. Generalized Principals of Treatment (continued) • Discontinue meds whenever possible • Consensus with client regarding treatment or discontinuation

38. Salient Nonepileptic Disorders at Different Ages: Age 0-2 months • Tremor • Dyskenesias associated =BPD • Benign neonatal myoclonus • Sleep myoclonus • Apnea

39. Salient Nonepileptic Disorders at Different Ages: Age 2-18 months • Paroxysmal torticollis • Opsoclonus-myoclonus syndrome • Sandiffers syndrome • Jactatio capitis • Masturbation • Paroxysmal choreo-athetosis • GERD

40. Salient Nonepileptic Disorders at Different Ages: Age 18 months - 5 yrs. • Disorder • Pavor nocturnus • Benign positional vertigo • Nodding puppet syndrome • Enuresis nocturnus • Familial dystonia-chorea • Athetosis

41. Salient Nonepileptic Disorders at Different Ages: 5-12 yrs. & beyond • Tics • Complicated migraine • ADHD inattentive type • Parasomnias • Vertebro basilar migraine • Syncope • Hyperventilation syndrome • Panic attacks • Affective storms-rage • Obstructive apnea

42. General Principles of Treatment • Avoid polytherapy whenever possible • Why? • Efficacy-studies have shown that 60% of people with IDD and Epilepsy can be controlled with one drug

43. Tolerability • Sedation increases with burden of superfluous drugs • Phamacodynamic effects, can't be measured • Avoid drugs that may worsen comorbid diseases • VPA, CBZ, Wt. Gain, obesity, diabetes, joint disease

44. Newer Drugs? • There is no evidence that newer drugs are significantly more effective • Distinguished by • Less significant AE's • Ease of administration • Reduced need for surveillance labs, level monitoring • Potential to be useful for comorbidities.

45. Refractory Epilepsy • There is no consensus regarding the definition of Intractable Seizures. Seizures which persist despite appropriate therapy. • Persistent seizures in spite of adequate trials of 2 or more first and second line drugs dosed to maximally tolerated levels within an acceptable therapeutic range.

46. Types of Intractable Seizures • True intractable epilepsy • Pseudo intractable

47. Medically and Surgically Intractable Epilepsy • Not accessible for resective surgery • Failure of resection surgery • Palliative surgery not applicable • Failure of palliative surgery

48. Favorable Factors for Seizure Remission-Clinical • Normal intellectual development • Normal neurological exam • Absence of any clinical or imaging evidence of brain damage

49. Favorable Factors for Seizure Remission-Seizure related • Age of onset of Epilepsy > 2 • Only one type of seizure • Low frequency of seizures • No tonic-atonic-astatic seizures • Rapid remission with first drug • Brief period of poor control • No episodes of SE • A benign syndromic diagnosis

50. Favorable Factors for Seizure Remission-EEG related • Normal EEG at onset of RX • Rapid improvement, normalization of EEG • Normal background features on EEG • No slowing or slow spike waves