Issues in Developmental Disabilities Epilepsy in the Intellectually and Developmentally Disabled

1. Issues in Developmental DisabilitiesEpilepsy in the Intellectually and Developmentally Disabled Discussion with Christopher M. Inglese, M.D. and Susan Heighway, M.S., A.P.R.N.

2. Questions for Child Neurologist • Is the child having a seizure? • Children should be referred to someone with a good sense of what a seizure is and what it represents • Difficult to answer because there are many variations on the seizure theme

3. Q: Are there developmental/genetic diagnoses that signify need for neurological evaluation? • Tuberous Sclerosis, Neurofibromatosis, Trisomy 21, Down Syndrome, Fragile X – highly associated with seizures and epilepsy

4. Q: Are children with autism spectrum disorder at risk for seizures? • Children with autism at tremendous risk since autism is symptom of variety of brain insults • Idiopathic/primary autisms – still relatively high incidence of seizures • High incidence of behaviors that mimic seizures • Much overlap between seizures and behaviors

5. Q: What kinds of information related to seizures would be helpful for neurologist? • Caretakers/parents/teachers need to be the eyes and ears of clinician making diagnosis • Need for careful, precise, blow-by-blow description of ictus (event) – more important than EEG, MRI or CAT scan

6. Q: When should neurological opinion be sought for staring seizures? • Two kinds of staring seizures • 1) Absence seizure – abrupt onset and offset, particularly if there’s arrest of ongoing activity like walking or eating; usu. 10-20 seconds • 2) Complex partial seizure – characterized by fumbling with hands (automatisms), smacking lips, drooling or involuntary swallowing; usu. 1 minute to 90 seconds • Useful tools for parents/teachers – gentle shaking of shoulders, speaking to child, waving in front of eyes

7. Q: How often and for what purposes would it be useful for a child to be seen by a neurologist? • Many children stay on medication well beyond point where it is needed • Medications may negatively impact learning, mood, bone health • Good to get kids off medication as soon as it’s safe • If child is still at risk for seizures, good for neurologist to be involved given medical advances

8. Q: What kinds of things are important for the other team members related to seizure follow-up? • Teacher feedback regarding plateau-ing or regression in rate of learning is important • Teacher feedback regarding whether child is exhibiting signs of ADHD • Information from occupational therapist or physical therapist can be useful

9. Q: What kinds of challenges have you seen working with teams that you would like some assistance with? • A disinterested person accompanying the person with epilepsy • Some patients may bring in too much documentation that is counterproductive • In context of Trisomy-21, Dementia, and seizures involving disinhibited behaviors and aggression, lack of information may lead to overprescription of medications • Ideally, need to look at social context

10. Q: What are the major changes, improvements in care, and treatment of seizures over the past few decades? • 2 categories – 1) better understanding of epidemiologic aspects and 2) more user friendly medical treatments • Better medications with less side effects • Novel interventions – vagus nerve stimulation • Better sense of when to consider resecting part of the brain that’s causing seizures • May be important at times to use old treatment modalities such as (ketogenic) diets

11. Q: What are the major changes, improvements in care, and treatment of seizures over the past few decades? (2) • Dr. Matteson’s and Dr. Brody’s Studies – medical intractability (the point at which it’s futile to keep prescribing more medications) • Medications that don’t require blood being drawn or surveillance labs • Vagus nerve stimulation – a way of reducing seizure frequency, seizure duration, and recovery period; also helps mood & intractable depression in adults • Surgery – technological advances in imaging

12. Q: What are we (neurologists) not doing well? • Most parents want to hear that there isn’t a problem • Neurologists are in a difficult position of having to describe situation as is, provide a reality check • Neurologists need to communicate to parents that even though diagnostic tests may not reveal any serious conditions, there is still reason for concern; need to keep the door open