Abstract Details

Title Hirayama Disease in Children from North America

Topic Child Neurology/Developmental Neurobiology

Presentation(s) P03 - (-)

Poster/Presentation
Number 001

Objective

Background BACKGROUND: Hirayama disease has been mainly reported from Asia, with only a few cases from western hemisphere particularly North America. In this study we highlighted our experience with Hirayama disease in non-Asian children from North America.

Design/Methods DESIGN/METHODS: Retrospective chart review of patients < 18 years, diagnosed with Hirayama Disease from a single center over the last 10 years.

Results RESULTS: Six children (4 males), age 15.1 ± 1.2 years, diagnosed with Hirayama Disease.All children were Caucasian. Symptom onset: 3 months-3 years prior to presentation. Referral diagnoses: tremors (2), median and ulnar neuropathies (1), ulnar neuropathy (1), neuralgic amyotrophy (1), and anterior horn cell disease (1). All had unilateral or bilateral asymmetric distal upper extremity weakness. "Oblique amyotrophy" and "Cold paresis" were noted in 5 each. Two had only subjective sensory symptoms. On electromyography (EMG), acute-on-chronic denervation was most frequently noted in C8-T1 myotomes followed by C7 myotome in both upper limbs (even if one limb was symptomatic). C5, C6 segments were spared. Cervical MRI findings were: normal (3), cervical cord atrophy without signal changes (1), engorgement of posterior epidural venous plexus on neck-flexion (1), and atrophic cervical cord with T2W hyperintensities (1). Symptoms progressed over a mean of 16.5 months. Treatment consisted of placement of cervical collar; follow-up (mean 15.6 months): progression stopped in all with some improvement of hand function in 1.

Conclusions CONCLUSIONS: Hirayama Disease can rarely affect Caucasian children from North America. Heightened awareness of this entity among pediatric neurologists will lead to early diagnosis and intervention thus avoiding unnecessary investigations.

Authors/Disclosures
Partha S. Ghosh, MD, FAAN (Boston Children'S Hospital) PRESENTER	Dr. Ghosh has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sarepta. Dr. Ghosh has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen.
Manikum Moodley, MD, FAAN	Dr. Moodley has nothing to disclose.
Neil R. Friedman, MD, MBChB (Phoenix Children's Hospital)	No disclosure on file
A. D. Rothner, MD (Cleveland Clinic Foundation)	No disclosure on file
Debabrata Ghosh, MD	No disclosure on file
	No disclosure on file

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