21 ST CENTURY TECHNOLOGY IN PEDIATRIC NEUROLOGIC DISORDERS PEDIATRIC NEUROLOGIC DISORDERS YOUR LEARNING EXPERIENCE LEARNING OBJECTIVES

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1 21 ST CENTURY TECHNOLOGY IN PEDIATRIC NEUROLOGIC DISORDERS Saturday, April 30, :00 am David J. Siegler, M.D. Board Certified American Board of Psychiatry and Neurology National Board of Physicians and Surgeons Child Neurology of Tulsa, P.C. Adjunct Clinical Professor, Pediatrics, OSU Assistant Clinical Professor, Pediatrics, OU-Tulsa Cell: ST CENTURY TECHNOLOGY IN PEDIATRIC NEUROLOGIC DISORDERS LEARNING OBJECTIVES Learn latest technological therapeutics for Treating Medically Intractable Partial Epilepsy Treating Medically Intractable Movement Disorders Learn How To Use Video Technology to Aid in Diagnosis of Pediatric Neurologic Disorders YOUR LEARNING EXPERIENCE Your experience, knowledge, interest and trust in the speaker The Speaker s: Experience, knowledge, interests Where I come from 1

2 2

3 Electrical Stimulation Dyskinesias/Movement D/O Laser Ablation Surgery Cellphone-Videos Electrical Stimulation Vagus Nerve Stimlation (VNS Therapy ) Nice Medscape overview on VNS Therapy at overview#a1 3

4 Electrical Stimulation Vagus Nerve Stimlation (VNS Therapy ) VNS: vs additional AED First implanted in l : Europe approved VNA; 1997 FDA approved: adjunct for partial epilepsy 12+ years of age Mechanism of action is unknown Now over 50,000 patients implanted Exclusively for refractory epilepsy patients Not a first line or even second line therapy. Has similar efficacy to AEDs with continued improvement in seizure reduction for up to 2 years. Medscape Overview at Electrical Stimulation Vagus Nerve Stimlation (VNS Therapy ) VNS: efficacy improves over time learning 22.5% (n=24) (6 months) 32% (n=20); (1 year) 42% (n=16) (2 years) 50.45% (n=11) (3 years) 52% (n=10) (4 years) 60% (n=8) (5 years) 61.25% (n=8) (6 years) 61.6% (n=6) (7 years) Arhan E. et al. Eur J Paediatr Neurol Jul;14(4): Electrical Stimulation Vagus Nerve Stimlation (VNS Therapy ) Pulse Generator Bipolar lead 4

5 Electrical Stimulation Vagus Nerve Stimlation (VNS Therapy ) Pulse Generator/Bipolar lead Programming Wand ELECTRICAL STIMULATION SEIZURE MANAGEMENT VAGUS NERVE STIMLATION (VNS THERAPY ) Anti-Epileptic Drugs (AEDs) Effective in inhibiting seizures: ~70% seizure free on meds Side Effects Fatigue, weight gain/loss, sleep disturbances, cognitive impairment, behavioral changes, mood changes (including suicidal ideation) Adverse Reactions: Drug eruption (SJS, TEN), hepatitis, pancreatitis, PCOD, agranulocytosis, aplastic anemia FDA Counseling patients Electrical Stimulation Vagus Nerve Stimlation (VNS Therapy ) VNS: vs additional AED When to advance therapy from oral to surgical VNS Therapy : safe, effective, improved quality of life vs AEDs Side Effects: hoarseness, coughing, dysphagia: resolves in time No idiosyncratic side effect ever reported MRI: can be done Compliance is guaranteed? Lost to F/U; Fractures Cost over 8 years (ave battery life) is less using a new AED Savings in hospital costs due to seizures Milby AH, Halpern CH, Baltuch GH Neurotherapeutics 2008 Jan;5(1): Ben-Menachem E, French JA. Epileptic Disord Sep;7 Suppl 1:S

6 Electrical Stimulation Vagus Nerve Stimlation (VNS Therapy ) Early vs Later use VNS Typically VNS used after years of intractable seizures Compared use before 5 years (N 120) with after 5 years (N 2785) of epilepsy Favorable results in early use population At 3 months of VNS Therapy Overall Seizure freedom increased from 5% to 15% Complex Partial Seizure freedom of 32% vs 17% Renfroe JB, Wheless JW Neurology 2002 Sep 24;59(6 Suppl 4):S Electrical Stimulation Vagus Nerve Stimlation (VNS Therapy ) Summary Not first or second line seizure therapy Earlier use more effective than later use: 2 years Barrier is initial cost and surgery Benefits: No cognitive side effects Improved QOL Compliance Continued improvement of seizure control over time DEEP BRAIN STIMULATION (DBS) FOR HYPERKINETIC DISORDERS 6

7 Deep Brain Stimulation (DBS) for Hyperkinetic Disorders Adult Deep Brain Stimulation (DBS) for Hyperkinetic Disorders Ryan 7

8 F DEEP BRAIN STIMULATION (DBS) FOR HYPERKINETIC DISORDERS FDA-Approved for Drug-Resistant Parkonisons Drug-Resistant Essential Tremor Drug-Resistant Obsessive Compulsive Disorder Dystonia: approved under an Humanitarian Device Exemption (HDE) for dystonia.* Deep Brain Stimulation (DBS) for Hyperkinetic Disorders Summary Very effective: regain QOL/Function Costly but likely cost effective (PD) in long run and if done earlier in course Potential permanent neurologic deficits Cook Children s Video Expose on a child with dyskinesia before and after DBS 8

9 1981 Nobel Prize in Physics Dr. Arthur Leonard Schawlow: (May 5, 1921 April 28, 1999) 9

10 MR-guided laser interstitial thermal therapy (MRgLITT) 10

11 MR-guided laser interstitial thermal therapy (MRgLITT) 1.6mm catheter through 3.2mm burr hole MR-guided laser interstitial thermal therapy (MRgLITT) Pediatric Neurology Used for brain lesions including: Tumors Symptomatic intractable epilepsy 30% epileptic patients do not respond to anti-epileptic meds Have tried at least 2 meds minimum (or Ketogenic diet) Focal or multifocal lesions causing seizures (epileptogenic foci) Lesions include Tumors Tuberous Sclerosis Cortical dysplasia Mesial temporal sclerosis MR-GUIDED LASER INTERSTITIAL THERMAL THERAPY (MRGLITT) Patel, et al, New Jersey (Rutgers), studies 102 pediatric patients 87: tumors; 10: epilepsy; 5: Chronic pain: cingulotomy LOS: mean: Median LOS for both the ICU and the hospital was 1 day/1 day Complications: 27 cases of morbidity 14 patients (13.7%) developed new deficits after the MRgLITT procedure 9 (64.3%) had complete resolution of deficits within 1 month 1 (7.1%) had partial resolution of symptoms within 1 month 2 (14.3%) no resolution of symptoms at the most recent follow-up 2 (14.3%) died without resolution of symptoms The 30-day readmission rate was 5.6% Patel P, Patel NV, Danish SF. J Neurosurgery Jan 1:

12 MR-guided laser interstitial thermal therapy (MRgLITT) Patel, et al, New Jersey (Rutgers), pediatric patient experience conclusion: MRgLITT, although minimally invasive, must be used with caution. Thermal damage to critical and eloquent structures can occur despite MRI guidance. There is a learning curve, after which the overall procedural complication rate is low Most patients can be discharged within 24 hours with a low readmission rate. Most complications are temporary. Patel P, Patel NV, Danish SF. J Neurosurgery Jan 1:1-8. MR-guided laser interstitial thermal therapy (MRgLITT) Initial Refractory Epilepsy Study supported by EFA s Epilepsy Therapy Project (ETP) Funded Texas Children s Hospital pilot study Wilfong, Pediatric Epileptologist 20 patients enrolled in Texas; presently over 200 nationally Tuberous Sclerosis (n=2) Mesial Temporal Sclerosis (n=2) Hypothalamic Hamartoma (n=12) Focal Cortical Dysplasia (n=4). MR-guided laser interstitial thermal therapy (MRgLITT) Hypothalamic hamartomas (HHs) causing intractable gelastic seizures Wilfong and Curry, TCH, treated with Visualase 12 (86%) seizure free by mean follow up of 9 months Asymptomatic subarachnoid bleed No permanent surgical complications, neurologic deficits, or neuroendocrine disturbances Conclusion: outcomes compare favorably vs open resection Wilfong AA 1, Curry DJ. Epilepsia Dec;54 Suppl 9:

13 MR-guided laser interstitial thermal therapy (MRgLITT) Benefits over tradition surgical resection Less invasive: small cranial opening (drill hole) vs craniotomy Less destructive to brain parenchyma (collateral damage) For more hard-to-reach lesions Less pain / more rapid post-surgical recover Raz, O et al: (2010) Curry, Gowda, McNichols and Wilfong (2012) MR-guided laser interstitial thermal therapy (MRgLITT) MR-guided laser interstitial thermal therapy (MRgLITT) afl4 13

14 Video In Pediatric Neurology Video In Pediatric Neurology Guidelines for Using Video in Diagnosis Ask parents to video for your review in office preferably Explain video is typically insufficient for diagnosis of epilepsy: Time-locked EEG is generally needed Obtain consent from parents/guardian if sharing/saving I don t save I don t share without overt documented consent HIPAA-compliant is best; avoid text-sharing Video In Pediatric Neurology Disorders Video is Helpful in Diagnosis Seizures Epileptic Non-epileptic Breath Holding Spells Sandifer s Syndrome (tonic posturing with GERD) Stereotypic Repetitive Behaviors Benign Sleep Myoclonus (in Infancy) Movement Disorders Tics/Tourette Syndrome Dystonia Dyskinesia Chorea 14

15 Video In Pediatric Neurology Video In Pediatric Neurology Video In Pediatric Neurology 15

16 Video In Pediatric Neurology 16

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