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Abstract Details

Immune-mediated Neuropathy with Tri-sulfated Heparin Disaccharide or Fibroblast Growth Factor Receptor 3 Antibodies in Pediatric Patients: A Case Series
Neuromuscular and Clinical Neurophysiology (EMG)
P2 - Poster Session 2 (2:45 PM-3:45 PM)
094

The goal of this case series is to illustrate the clinical presentation and treatment in 2 patients with novel antibodies to TS-HDS and FGFR-3.

Novel antibodies to tri-sulfated heparin disaccharide (TS-HDS) and fibroblast growth factor receptor 3 (FGFR-3) have been identified in cryptogenic neuropathy cases. There is limited information regarding these antibodies in pediatric patients. 

Two pediatric patients with acquired neuropathy were identified to have antibodies against TS-HDS or FGFR-3. Each chart was reviewed for presentation, diagnosis, intervention, and outcome.

Case 1: 15-year-old female with juvenile myoclonic epilepsy presented with back pain, bilateral hand and leg paresthesia, and shuffling gait. Exam notable for diminished sensation in the extremities, absent lower extremity reflexes, and sensory ataxia. Testing was positive for IgM against TS-HDS antibodies (31,000; normal is < 12,000).

Case 2: 12-year-old male with ADHD presented with worsening leg pain, bilateral foot paresthesia, and hand twitching. Exam notable for intact strength, sensation, reflexes, and gait. Testing was positive for IgG against FGFR-3 antibodies (4,600; normal is < 3,000).

Both patients were found to have axonal neuropathy on EMG. Treatment was initiated with monthly intravenous immunoglobulin (IVIG). There is subjective and objective improvement in one patient with monthly IVIG treatment.

There is limited data for treatment of immune-mediated neuropathy in pediatric patients. Further investigation is needed to determine the role of these antibodies in pediatric cases and whether IVIG will be beneficial.

Authors/Disclosures
Tim Nguyen, MD (Cincinnati Children's Hospital Medical Center)
PRESENTER
Dr. Nguyen has nothing to disclose.
Alexander Zygmunt, MD (Cincinnati Children'S Hospital Medical Center) The institution of Dr. Zygmunt has received research support from NS-Pharma. The institution of Dr. Zygmunt has received research support from Edgewise Therapeutics.
Elizabeth V. Wilson, MD (Cincinnati Children's Hospital) Dr. Wilson has received research support from Department of Defense. Dr. Wilson has received research support from NIH/NINDS.