Pediatric Neurology - UT Southwestern

Susan Iannaccone, M.D.
Professor,
Director, Pediatric Nephrology
Faculty
Pediatric Neurology
2011 Academic Review
The Division of Pediatric Neurology is the only academic group in North Central Texas
providing consultations and multi-disciplinary care for children with all kinds of
neurologic disease. Internationally recognized faculty provide care at Children’s Medical
Center’s Dallas, Legacy and Southlake campuses and at Texas Scottish Rite Hospital for
Children (TSRHC).
Each member of the faculty provides special expertise in areas of focus including
epilepsy and epilepsy surgery, neuromuscular disorders, stroke, demyelinating disease,
autism, cerebral palsy and headache. Patients receive a sophisticated and integrated
approach to diagnosis including genetic testing, metabolic analysis and interpretation
of neuroimaging.
Our multidisciplinary clinics include, but are not limited to:
• Urgent epilepsy
• Ketogenic diet
• Myasthenia
• Childhood stroke
• Autism
• Transverse myelitis
• Neuroncology (directed by
Hematology/Oncology)
• Headache (directed by Pain
Management Team)
Our group includes 13 full-time faculty based at Children’s and two based at TSRHC. In addition, we have one part-time
faculty serving in Neuro-oncology and one volunteer faculty serving in a teaching capacity. There are a total of 10 advanced
practice providers: two physician assistants and eight nurse practitioners who work alongside our faculty to provide timely
and comprehensive care.
Susan Arnold, M.D.
Associate Professor
Michael Dowling, M.D., Ph.D.
Assistant Professor
Patricia Evans, M.D.
Associate Professor
Sailaja Golla, M.D.
Assistant Professor
Susan Iannaccone, M.D.
Professor, Division Director
Muna Kahn, M.D.
Assistant Professor
Saadat Kahn, M.D.
Assistant Professor
Fares Kokash, M.D.
Assistant Professor
Rana Said, M.D.
Assistant Professor
Deepa Sirsi, M.D.
Assistant Professor
William Zinser, M.D.
Associate Professor
In addition to our faculty, the division has:
• one psychometrician
• four academic administrative staff
• one program administrator-clinic
• one program administrator-epilepsy
• eleven nursing staff
• one case manager
• one social worker
• one interpreter
• seven research coordinators
• one dietitian
• four clinic techs/MA’s
• two front desk/call center support people
Dr. William Zinser retired in December 2011.
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Research Activities
Division faculty conduct a variety of neurologic bench/basic research and clinical studies, including clinical trials. Current
studies are focused in five areas:
• metabolic disorders of the nervous system
• neuromuscular disease clinical trials
• anti-epileptic drug trials
• clinical trials in pediatric stroke
• clinical studies in the autism spectrum
Faculty present results of their work at many national and international meetings and published over 30 articles in peerreviewed
journals in 2011. Dr. Iannaccone is a co-investigator for the NIH Network for Excellence in Neuroscience Clinical
Trials (NeuroNEXT) which started in October 2011. UT Southwestern Neurology is one of 25 sites to participate in this
study. NeuroNEXT is designed to increase the efficiency of clinical trials, facilitate patient recruitment and retention,
increase the quality of neuroscience clinical trials, and enable public-private partnerships. The network aims to share
expertise and infrastructure across diseases, leverage research resources at clinical sites and take advantage of clinical
research opportunities as they arise. This clinical research network will provide a robust, standardized, and accessible
infrastructure to facilitate rapid development and implementation of protocols in neurological disorders affecting adult
and/or pediatric populations. The network is designed to assure the broadest access to new therapies for patients by carrying
out trials coming from partnerships between NINDS and industry, foundations or academia.
Clinical Activities
The expertise of the faculty is focused on several sub-specialties: comprehensive epilepsy management including
seizure surgery; neuromuscular medicine clinics including myasthenia gravis, muscular dystrophy, Charcot Marie
Tooth and transitional care for adolescent muscle; cerebrovascular disease; neonatal intensive care follow-up; Zero-2-3
neurodevelopment clinic; rare disorders clinic; and neurodevelopmental disabilities clinics including autism, neurocognitive
delay, attention-deficit hyperactivity disorder/learning disabilities (ADHD/LD) and traumatic brain injury.
Neurodevelopmental Disabilities Program
The Neurodevelopmental Disabilities Program (NDD) concludes its fourth year under the direction
of Dr. Patricia Evans at UT Southwestern. The program continues to experience significant growth
specifically with regard to clinical opportunities, research, education and community outreach. NDD
clinics are multi-disciplinary in structure and are specifically created to serve children with autism,
developmental delay, traumatic brain injuries and ADHD/LD issues. The program has expanded to
provide full-time NDD services to both the Childrne’s Dallas and Legacy campuses.
The NDD team serves the North Texas community at large. The team attends special needs children
on site at the Dallas-based Autistic Treatment Center on a quarterly basis, and works closely with Dr.
Carolyn Garver and her outstanding team of specialists. Drs. Evans and Morris speak throughout
the metropolis in various educational settings, including the Dallas ISD, the Dallas Theological
Seminary, and other specialty programs regarding children with special needs.
Dr. Patricia Evans
Associate Professor
Epilepsy Program
The Epilepsy Program, under the direction of Dr. Susan Arnold, includes a Designated Level 4
Epilepsy Center, the first and only Pediatric Epilepsy program in the Country to be certified by the
Joint Commission.
The program includes the Children’s Epilepsy Monitoring Unit (EMU) that contains eight beds
and providing 24/7 coverage for patients in the pediatric intensive care unit (PICU), the neonatal
intensive care unit (NICU) and the emergency department. The EMU team includes an attending,
support staff, and several third-year medical students on their required neurology rotation plus
rotating residents from adult neurology, psychiatry or other specialties.
Dr. Susan Arnold
Associate Professor
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Neuromuscular Clinic
Under the supervision of Dr. Susan Iannaccone the neuromuscular clinic
is the only full-time pediatric program in North Texas to be sponsored
by the Muscular Dystrophy Association (MDA). The aim of these
multidisciplinary clinics are to provide comprehensive health care for the
pediatric neuromuscular population. The multidisciplinary approach
fosters an environment of all-inclusive health care. Clinic is held four
days per week.
Stroke Clinic
Annual Ketogenic Diet Halloween Party
The Pediatric Stroke Clinic, established in 2003, is directed by Dr. Michael Dowling and holds two
half-day clinics per week. The patient population consists of children and young adults with arterial
ischemic stroke, cerebral venous sinus thrombosis, Sturge-Weber Syndrome, moya-moya or other
cerebrovascular disorders. Patients are referred from all parts of Texas, Oklahoma, New Mexico,
Arkansas, Louisiana and the United Kingdom.
Dr. Michael Dowling
Assistant Professor
The Multidisciplinary Stroke Clinic sees patients twice monthly for half days. Dr. Dowling
established this clinic in October 2007 and meets twice monthly with attendings from Pediatric
Hematology, Physical Medicine and Rehabilitation and Neuropsychology, as well as research nurses
for coordinated patient visits (approximately four patients per clinic). This serves as a unique clinical
and teaching program, with involvement of medical students, Pediatric Psychiatry fellows, Pediatric
Neurology fellows, Adult Neurology residents, and Physical Medicine and Rehabilitation fellows.
The Acute Stroke Team is on call 24 / 7 / 365 for any stroke or suspected stroke in a Children’s Dallas or Legacy campus
patient. Dr. Dowling provides emergent telephone consultation with in-person evaluation and treatment in the emergency
setting and follows the patients clinically alongside the neurology inpatient service.
Neurology Patient Visits
Epilepsy 4,574
General Neurology 683
Headache 935
Neuromuscular 1,010
Neurodevelopment 832
Rare Disorders 106
Research 162
Residents 519
Stroke/Sickle Cell 260
Demyelinating 268
Faculty 6,871
APPs 2,532
Total 9,403
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Education and Training
Our three-year training program for pediatric neurology is the only one in north Texas and is highly competitive. Our
residency for neurodevelopmental disabilities is one of only seven in the country. We graduate three pediatric neurologists
each year and offer subspecialty fellowships in epilepsy, neurophysiology, demyelinating disease, and neuromuscular
medicine for our graduates as well as candidates from other programs.
Dr. Rana Said, Program Director for the pediatric neurology residency and clerkship, leads the teaching program that
educates residents and medical students for inpatient and outpatient rotations, with the assistance of Program Coordinator,
Kellie Shaw. The program includes:
• two adult neurology residents for three four-week blocks each,
• seven UT Southwestern Internal Medicine residents for two months each,
• one to three adult and pediatric neurophysiology/epilepsy fellows, and
• one to three neuromuscular medicine fellows for one year each.
More than 200 medical student neurology clerkship participants rotate for two-week intervals on either inpatient or
outpatient rotations each year.
The faculty participates in up to eight neurology teaching conferences per week as well as a series of pediatric teaching
conferences on neurologic topics.
The Carrell-Krusen Neuromuscular Symposium is held annually in February. Dr. Susan Iannaccone is the course director for
this symposium designed for muscular dystrophy clinic directors, case managers, nurses and members of interdisciplinary
teams. The focus is on protocols for treatment and management of both common and unusual neuromuscular disorders,
identifying characteristic findings on muscle biopsies and EMG studies, and describing recent advances in basic and clinical
research in neuromuscular disorders.
Presentations
Michael M. Dowling, M.D., Ph.D., M.S.C.S.
• Ichord RN, Bastian R, Abraham L, Askalan R, Benedict S, Bernard T, Beslow L, deVeber G, Dowling M, Friedman M,
Fullerton H, Jordan L, Kirton A, Amlie-Lefond C, Licht D, Lo W, McClure C, Pavlakis S, Smith SE, Tan M, Jawad AF.
“Inter-observer Reliability of the Pediatric NIH Stroke Scale (PedNIHSS) in a Multicenter Study,” International Stroke
Conference, Los Angeles, CA, February 2011
• “PFO and Stroke in SCD,” Hematology Clinical Research Seminar, Cincinnati Children’s Hospital Medical Center,
University of Cincinnati College of Medicine, OH, June 2011
• “Sickle Cell in Focus,” Stroke and Cerebrovascular Complications of Sickle Cell Disease, Kings College, London, UK,
June 2011
• “Stroke in Children,” Texas Pediatric Society Annual Meeting Plenary Session, Sugarland, TX, September 2011
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• Dowling MM, Hynan L, Lo W, Licht D, McClure C, Yager J, Dlamini N, Kirkham F, deVeber G, Pavlakis S.
“International Pediatric Stroke Study: Stroke Associated with Cardiac Disorders,” World Congress of Neurology,
Marrakesh, Morocco, November 2011
• Quinn CT, Hoof PD, Dowling MM. “Cerebral Tissue Desaturation in Children with Sickle Cell Disease,” American
Hematology Society Annual Meeting, San Diego, CA, December 2011
Patricia Evans, M.D.
• “Reel Docs,” Film Series, Dr. Temple Grandin, Documentary on Autism, June 2011
• “Autistic Spectrum Disorder,” University of Texas Permian Basin, July 2011
Susan T. Iannaccone, M.D.
• “An Overview - Recent Advances in SMA and Other Pediatric Neuromuscular Diseases,” Texas Scottish Rite Hospital
for Children, Dallas, February 2011
• “Standard of Care for Duchenne Muscular Dystrophy - Recent Advances in SMA and Other Pediatric Neuromuscular
Diseases,” Northwestern University Medical Center, Chicago, IL, May 2011
Juan M. Pascual, M.D., Ph.D.
• 3 rd Symposium on Glucose Transporter Deficiency, New Orleans, LA, July 2011
Rana R. Said, M.D.
• “Neurological Disorders: Seizures, Headaches, Brain Tumors, Neurodegenerative and Neuromuscular Disorders,”
American Academy of Child & Adolescent Psychiatry (AACAP), Honolulu, Hawaii, March 2011
• “Epilepsy Management: In the Office and ER,” Children’s Medical Center at Legacy, November 2011
• “Early Surgical Treatment of Infants with Catastrophic Epilepsy Symptomatic to Cortical Dysplasias,” American
Academy of Neurology Annual Meeting, Honolulu, Hawaii, April 2011
Awards and Honors
Susan Arnold, M.D.
• Best Pediatric Specialists in Dallas, D Magazine
• Top US Doctors, U.S. News & World Report
Michael Dowling, M.D., Ph.D., M.S.C.S.
• Best Pediatric Specialists in Dallas, D Magazine
Susan Iannaccone, M.D.
• Best Pediatric Specialists in Dallas, D Magazine
• Texas Super Doctors, Texas Monthly
• Top US Doctors, U.S. News & World Report
Rana Said, M.D.
• Best Pediatric Specialists in Dallas, D Magazine
• Child Neurology Residents’ Program Director Appreciation Award
Publications
Articles
1. Castro D, Iannaccone ST, Gilbreath H, et al. Juvenile myasthenia gravis: a twenty year experience (33rd Annual
Carrell-Krusen Neuromuscular Symposium, Texas Scottish Rite Hospital for Children, Dallas, Texas, February 17-18,
2011). J Clin Neuromuscular Disease 2011;12:17:173-95.
2. Choi C, Ganji SK, DeBerardinis RJ, Dimitrov IE, Pascual JM, et al. Measurement of glycine in the human brain in
vivo by 1H-MRS at 3 T: application in brain tumors. Magn Reson Med;66:609-18.
3. Derisavifard S, Castro C, Guleserian K, Iannaccone ST, Andersen M, Greene M. Juvenile myasthenia gravis: a twentyyear
experience. Neurology 2011;76:A94 (Suppl 4).
4. Gilbreath H, Iannaccone ST, Bonnemann C. Variable phenotypes in Ullrich congenital muscular dystrophy (33rd
Annual Carrell-Krusen Neuromuscular Symposium, Texas Scottish Rite Hospital for Children, Dallas, Texas,
February 17-18, 2011). J Clin Neuromuscular Disease 2011;12:7:173-95.
5. Ichord RN, Bastian R, Abraham L, Askalan R, Benedict S, Bernard TJ, Beslow L, Deveber G, Dowling M, et al. Interrater
reliability of the Pediatric National Institutes of Health Stroke Scale (PedNIHSS) in a multicenter study. Stroke;42:613-7.
6. Kaufmann P, Engelstad K, Wei Y, Kulikova R, Oskoui M, Sproule DM, Battista V, Koenigsberger DY, Pascual JM, et
al. Natural history of MELAS associated with mitochondrial DNA m.3243A>G genotype. Neurology;77:1965-71.
7. Marin-Valencia I, Good LB, Ma Q, Jeffrey FM, Malloy CR, Pascual JM. High-resolution detection of (1)(3)C
multiplets from the conscious mouse brain by ex vivo NMR spectroscopy. J Neurosci Methods;203:50-5.
8. Montgomery M, Joyce C, Iannaccone ST, et al. Utilizing a national continuing medical education (CME) series to
highlight emerging therapeutic opportunities in spinal muscular atrophy (SMA). Neurology 2011;76:A94 (Suppl 4).
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9. Pascual JM. Animal models of the human mind: Is there anything like being autistic Neurosci Lett;505:59-60.
10. Pascual JM. Synaptic transporters are deceived if they think themselves free. Neurosci Lett;487:1-2.
11. Perez-Duenas B, Ormazabal A, Toma C, Torrico B, Cormand B, Serrano M, Sierra C, De Grandis E, Marfa MP,
Garcia-Cazorla A, Campistol J, Pascual JM, Artuch R. Cerebral folate deficiency syndromes in childhood: clinical,
analytical, and etiologic aspects. Arch Neurol;68:615-21.
12. Rogers ZR, Wang WC, Luo Z, Iyer RV, Shalaby-Rana E, Dertinger SD, Shulkin BL, Miller JH, Files B, Lane PA,
Thompson BW, Miller ST, Ware RE, BABY HUG Investigators (including Dowling M). Biomarkers of splenic function
in infants with sickle cell anemia: baseline data from the BABY HUG Trial. Blood;117:2614-7.
13. Scharner J, Brown CA, Bower M, Iannaccone ST, et al. Novel LMNA mutations in patients with Emery-Dreifuss
muscular dystrophy and functional characterization of four LMNA mutations. Hum Mutat;32:152-67.
14. Sirsi D. Is intranasal midazolam better than rectal diazepam for home management of acute seizures Arch
Neurol;68:120-1.
15. Tang W, Gilbreath H, Burns D, Iannaccone ST. Heat or exercise-induced rhabdomyolysis in ryanodine receptor
channelopathy (33rd Annual Carrell-Krusen Neuromuscular Symposium, Texas Scottish Rite Hospital for Children,
Dallas, Texas, February 17-18, 2011). J Clin Neuromuscular Disease 2011;12:13:173-95.
16. Wadman RI, Bosboom WM, van den Berg LH, Wokke JH, Iannaccone ST, Vrancken AF. Drug treatment for spinal
muscular atrophy types II and III. Cochrane Database Syst Rev:CD006282.
17. Wadman RI, Bosboom WM, van den Berg LH, Wokke JH, Iannaccone ST, Vrancken AF. Drug treatment for spinal
muscular atrophy type I. Cochrane Database Syst Rev:CD006281.
18. Waugh J, Plumb P, Rollins N, Dowling MM. Prolonged direct catheter thrombolysis of cerebral venous sinus
thrombosis in children: a case series. J Child Neurol;27:337-45.
19. Zimmerman A, Clemens PR, Tesi-Rocha C, Connolly A, Iannaccone ST, et al. Liquid formulation of pentoxifylline is
a poorly tolerated treatment for duchenne dystrophy. Muscle Nerve;44:170-3.
Book Sections
1. Evans P. End-of-life-care for the neurologically impaired child. In: William,
McGuire, Rizzo, eds. Practical Ethics in Clinical Neurology. Philadelphia, PA:
American Academy of Neurology, LIppincott, Wilkins and Williams; 2011.
2. Evans P. Informed assent and refusal for children and adolescents. In: William,
McGuire, Rizzo, eds. Practical Ethics in Clinical Neurology. Philadelphia, PA:
American Academy of Neurology, LIppincott, Wilkins and Williams; 2011.
3. Evans P. Why, O God A Biblical theology of suffering and disability. In: Water L,
ed. Practicing Inclusivity for Children with Special Needs. Wheaton, IL: Good News
and Crossways Publishers; 2011.
4. Pascual JM. Disorders of muscle excitability. In: Brady ST, Siegel GJ, Albers RW,
Price DL, eds. Basic Neurochemistry. 8th ed: Elsevier; 2011.
5. Pascual JM, DiMauro S. Disorders of the Krebs cycle and of pyruvate metabolism and
transport. In: Rudolph CD, Rudolph AM, Lister G, First LR, Gershon AA, eds. Rudolph’s
Pediatrics, 22nd edition. New York, NY: McGraw Hill; 2011.
Grants
Sponsored Clinical Trials
Susan Arnold, M.D.
Safety and Effectiveness of Open-Label Clobazam in Subjects with Lennox-Gastuat Syndrome
Ovation Pharmaceuticals, OV-1004
Random, Double-Blind, Placebo-Controled, Parallel-Group Study with Open-Label Ext. Phase to Evaluate Effect of
Perampanel (E2007) on Cognition, Growth, Safety, Tolerability and Pharmocokinetics when Administered as Adjunctive
Therapy in Adolescents (12 to

Double Blind Placebo Controlled Dose-Escalation Study of E2007 (perampanel) Given as Adjunctive Therapy in Subjects
with Refractory Partial Seizures
Eisai Medical Research, Inc., E2007-G000-305
Susan Iannaccone, M.D.
Open-Label Safety Study for Previously Treated Ataluren (PTC124) Patients with Nonsense Mutation Dystrophinopathy
PTC Therapeutics, Inc., PTC124-GD-016-DMD
Phase 2b Study of PTC124 in Subjects with Nonsense-Mutation-Mediated Duchenne and Becker Muscular Dystrophy
PTC Therapeutics, Inc., PTC124-GD-007(e)-DMD
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