Pediatric Epilepsy Care in Milwaukee

Transcription

1 Pediatric Epilepsy Care in Milwaukee Priya Monrad, MD Assistant Professor, Pediatric Neurology and Epilepsy Children s Hospital of Wisconsin

2 Disclosures I have no relevant financial relationships to disclose.

3 Objectives Definitions and concepts in epilepsy Workup for first and recurrent seizures Medical and surgical management of epilepsy The concept of refractory epilepsy. What is the benefit of treating pediatric epilepsy? Epilepsy services available at the Children s Hospital of Wisconsin

4 Definitions and Concepts

5 What is Epilepsy? One isolated seizure is not epilepsy. Provoked seizures are not epilepsy (fever, hypoglycemia, stroke etc.) However, a provoked seizure can lead to the development of epilepsy.

6 What is Epilepsy (seizure disorder)? Two or more unprovoked seizures. One seizure could mean epilepsy in the right setting: abnormal EEG epileptogenic structural lesion family history of seizures/epilepsy

7 Types of epilepsy Focal Generalized

8 What is the risk of seizure recurrence after presenting with seizures? (Lizana 2000) Recurrence rate after: After first seizure After >1 seizure 6 months 37% 58% 1 year 45% 65% 2 years 57% 74% 3 years 57% 74% 4 years 61% 78% 5 years 65% 79%

9 What is the risk of seizure recurrence after presenting with seizures? Risk factors for recurrence include: (Lizana, 2000) Age <3 or >10 years Symptomatic cause (97% recurrence at 2 yrs) Idiopathic/cryptogenic with: Abnormal EEG Seizures during sleep

10 Workup

11 Some historical details suspicious for seizure Aura: a warning (visual, auditory, auditory, psychic etc.), which is actually part of the seizure. Asymmetric shaking Not every motor seizure is a GTCS! Not every staring seizure is an absence seizure (generalized epilepsy)

12 Workup of a first seizure History taking, patient first if possible, then witness Cell phone videos can be helpful! Birth history and development matter Physical exam Routine EEG with sleep recording (sleep deprivation) MRI brain (3T epilepsy protocol if possible)

13 Workup of a first seizure Referral to neurology, especially with: Focal features Family history Status epilepticus (seizure over 10 minutes) Abnormal exam Associated developmental delay or dysmorphism Abnormal EEG or MRI

14 When to consider genetic testing? Refractory epilepsy of unclear origin Developmental delay Dysmorphic features Associated movement disorders. Suspicion of a neuro-cutaneous disorder Metabolic abnormalities Strong family history of epilepsy

15 Genetic Testing Several panels exist and constantly change with new discoveries Infantile epilepsy Childhood epilepsy Progressive myoclonic epilepsy Rett/Angelman Syndrome Brain malformation panel Whole Exome/Genome

16 Medical Management

17

18 What is refractory epilepsy? Epilepsy not controlled by 2 or more AEDs Appropriate for the epilepsy (focal, generalized) Maximal dose vs emergence of side effects 70% responders vs 30% non-responders

19 What is refractory epilepsy?

20 Non Medical Management

21 Ketogenic Diet High fat intake, low carb and protein Administered by ketogenic dietician Fats as the main source of energy, leading to ketosis ~ 50% seizure freedom in ~ 50% of patients.

22 Ketogenic Diet Has Specific Uses Glucose transporter type 1 (GLUT1) deficiency Pyruvate dehydrogenase deficiency Refractory idiopathic generalized epilepsy Progressive myoclonic epilepsy Lennox Gastaut syndrome, other epileptic encephalopathies

23 Surgical treatment of Epilepsy Refractory focal epilepsy Resection/disconnection of the epileptogenic zone, the pacemaker of seizures

24

25

26

27

28

29

30

31 Why Bother To Detect and Treat Seizures? 1 in 26 people will develop epilepsy at some time in their life 10% of all seizures occur in children under the age of 3 Common comorbidities include cognitive impairment, depression, anxiety, attention deficits, and migraine Delay in diagnosis of seizures is associated with point drop in IQ at 8-year followup (Berg, 2014) Seizures interfere with school attendance and social relationships

32 Seizures are associated with altered brain development Prospective study of 38 children (8-18y) with no predisposing factors and normal MRIs with recent diagnosis of epilepsy compared with healthy firstcousin controls (Herman 2010) Decrease in total white matter volume growth in children with epilepsy, especially in frontal lobes

33 Detection of Seizures Affects ICU Treatment and Prognostication In a retrospective review of 550 PICU patients receiving continuous EEG during their ICU stay (Abend, 2013) 165 (30%) had recorded electrographic seizures without any clinical symptoms Of these, 61 (30%) were in electrographic status epilepticus, associated with higher mortality EEG monitoring led to treatment changes in 59% of monitored PICU patients (Abend, 2011)

34 Detection and Treatment of Seizures May Improve ICU Outcomes Seizure duration over 12 minutes per hour in monitored PICU/CICU is associated with significant increases in morbidity/mortality (Payne, 2014) Poor outcomes were associated with average seizure burden of 10 min/hr; good outcomes with average of 1 min/hr Similar observations in NICU babies Potential for neurodevelopmental outcome-altering interventions

35 Our new inpatient and rehabilitation unit

36 3D Image of Neuroscience Center

37 What Services We Offer Inpatient video EEG monitoring Ambulatory (at-home) EEGs Routine (30-60 minute) outpatient EEGs 3T MRI, fmri, MEG, PET, Wada testing Pediatric Neuropsychology Surgical pre-evaluation and invasive monitoring (grids, stereo EEG, cortical mapping) Ketogenic Diet (with dieticians)

38 Contact Information Pediatric Neurology Outpatient Clinics (414) Physician Referral and Consultation: (800)

39 Thank You!