Abstract Details

Title The Practice of Pediatric EMG in the Molecular Era: State of the Science and Temporal Trends

Topic Clinical Neurophysiology

Presentation(s) P07 - (-)

Poster/Presentation
Number 186

Objective

Background BACKGROUND: Major advances in genetic analysis and neuroimaging have modified the traditional diagnostic approach for numerous neuromuscular disorders. The profile of patients and indications for EMG has thus changed over the past decade.

Design/Methods DESIGN/METHODS: We retrospectively surveyed all EMG reports from the EMG laboratory at Boston Children's Hospital from 2001 through 2011. Other medical records were cross-checked as needed. Information was collected on study numbers, age and gender, referring provider's subspecialty, indication, electrophysiologic diagnosis, and utility of the study. Descriptive statistics were generated depicting the temporal trends of these variables.

Results RESULTS: 2100 studies were performed in an 11 year period. The number of studies performed increased during that period (from [sim]160 studies/year to [sim]250 studies/year). There was a trend towards studying older children. Neurologists, including neuromuscular specialists, constituted the major referral pool (ranging from 64-85%) with the former shifting more to the latter, while orthopaedic referrals have been steadily increasing (>20% of referrals in the past 3 years). Polyneuropathy followed by mononeuropathy evaluations have been consistently the most common reasons for referral with myopathies and multiple specific or non-specific monomelic and polymelic neuromuscular indications ensuing. More than 50% of the studies were normal. Abnormal findings were most commonly polyneuropathies, followed by mononeuropathies. Nearly 50% of polyneuropathies were axonal, 35% were demyelinating and the rest were mixed. Peroneal and ulnar lesions constituted nearly 50% of mononeuropathies. Plexopathies and radiculopathies were infrequent. EMG provided meaningful information in 94% of the cases.

Conclusions CONCLUSIONS: Despite major discoveries in genetics and neuromuscular imaging, EMG has still a cardinal role to play in the diagnosis of pediatric neuromuscular disorders, although its practice paradigm is changing.

Authors/Disclosures
Ioannis Karakis, MD, FAAN (Emory University) PRESENTER	Dr. Karakis has received personal compensation for serving as an employee of Emory University School of Medicine. Dr. Karakis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Karakis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for GSK. Dr. Karakis has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Epitel. Dr. Karakis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ceribell. Dr. Karakis has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Hill Ward Henderson.
Wendy K. Liew, MD	No disclosure on file
H. R. Jones, Jr., MD (Lahey Clinic Medical Center)	No disclosure on file
Basil T. Darras, MD (Children'S Hosp Boston Harvard Med School)	The institution of Dr. Darras has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amicus. Dr. Darras has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Amicus. The institution of Dr. Darras has received research support from National Institutes of Health/National Institute of Neurological Disorders and Stroke,. The institution of Dr. Darras has received research support from Slaney Family Fund for SMA. The institution of Dr. Darras has received research support from Spinal Muscular Atrophy Foundation. The institution of Dr. Darras has received research support from CureSMA. The institution of Dr. Darras has received research support from Working on Walking Fund . The institution of Dr. Darras has received research support from CHERISH, CS2/CS12 . The institution of Dr. Darras has received research support from Biogen for CS11. The institution of Dr. Darras has received research support from AveXis. The institution of Dr. Darras has received research support from Sarepta Pharmaceuticals. The institution of Dr. Darras has received research support from PTC Therapeutics. The institution of Dr. Darras has received research support from Roche. The institution of Dr. Darras has received research support from Santhera. The institution of Dr. Darras has received research support from Scholar Rock. The institution of Dr. Darras has received research support from Fibrogen. The institution of Dr. Darras has received research support from Summit. Dr. Darras has received publishing royalties from a publication relating to health care. Dr. Darras has received publishing royalties from a publication relating to health care.
Peter B. Kang, MD, FAAN (University of Minnesota Medical School)	Dr. Kang has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Neurogene. Dr. Kang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lupin. Dr. Kang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ITF Therapeutics (Italfarmaco). An immediate family member of Dr. Kang has received personal compensation in the range of $0-$499 for serving as a Consultant for Life Sciences Consultants. Dr. Kang has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for MLAB Biosciences. Dr. Kang has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wiley. Dr. Kang has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for ��ɫ��. The institution of Dr. Kang has received research support from Xtraordinary Joy Foundation. The institution of an immediate family member of Dr. Kang has received research support from Sarepta Therapeutics. The institution of Dr. Kang has received research support from Centers for Disease Control and Prevention (CDC). The institution of Dr. Kang has received research support from National Institutes of Health (NIH). The institution of Dr. Kang has received research support from Food and Drug Administration. The institution of Dr. Kang has received research support from National Initiative for Cockayne Syndrome. The institution of Dr. Kang has received research support from Greg Marzolf Jr Foundation. The institution of Dr. Kang has received research support from Sarepta Therapeutics. The institution of Dr. Kang has received research support from Duchenne UK - PPMD. The institution of an immediate family member of Dr. Kang has received research support from Regenerative Medicine MN. The institution of an immediate family member of Dr. Kang has received research support from Greg Marzolf Jr Foundation. The institution of an immediate family member of Dr. Kang has received research support from NIH. The institution of an immediate family member of Dr. Kang has received research support from Viljem Julijan Association for Children with Rare Disease. The institution of Dr. Kang has received research support from Minnesota Partnership. Dr. Kang has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Kang has received intellectual property interests from a discovery or technology relating to health care. Dr. Kang has received publishing royalties from a publication relating to health care. Dr. Kang has a non-compensated relationship as a Medical Advisory Board with Speak Foundation that is relevant to AAN interests or activities. Dr. Kang has a non-compensated relationship as a President (Including President-Elect and Past President) with Child Neurology Society that is relevant to AAN interests or activities. Dr. Kang has a non-compensated relationship as a Board Member with Child Neurology Foundation that is relevant to AAN interests or activities.
Clemens Warnke (Klinik Fuer Neurologie, University of Duesseldorf)	The institution of Clemens Warnke has received research support from Novartis. The institution of Clemens Warnke has received research support from Alexion. The institution of Clemens Warnke has received research support from Sanofi-Genzyme. The institution of Clemens Warnke has received research support from Biogen. The institution of Clemens Warnke has received research support from Roche.

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