Hirayama Disease in Children From North America

Abstract

Hirayama disease has been mainly reported from Asia; only a few cases are from the Western hemisphere, particularly North America. This is a retrospective chart review of patients < 18 years, diagnosed with Hirayama disease from a single center over 10 years. We diagnosed 6 children (4 boys), 15.1 ± 1.2 years of age. Symptom onset was 3 months to 3 years before presentation. All had unilateral or bilateral asymmetric distal upper extremity weakness without objective sensory loss. Oblique amyotrophy and cold paresis were noted in 5. On electromyography, acute-on-chronic denervation was most frequently noted in cervical-8 (C8) and thoracic-1 (T1) myotomes followed by cervical-7 (C7) myotome in both upper limbs, sparing C5-C6 myotomes. Cervical magnetic resonance imaging (MRI) was abnormal in 3. Symptoms progressed over a mean of 16.5 months. Treatment consisted of placement of cervical collar. Heightened awareness of this entity among pediatric neurologists in North America will lead to early diagnosis and intervention, avoiding unnecessary investigations.

A Proposal for the Definition of Hirayama Disease and Monomelic Amyotrophy

“…1 The authors reported 6 patients in this article. Symptom onset was 3 months to 3 years before presentation.…”

Hirayama disease: a frequently undiagnosed condition with simple inexpensive treatment

“…This was initially thought to have some ethnic or regional significance 1–4. However, this condition is not limited to Asian population, as cases have been reported even from Europe and America 5 6. The exact data regarding the epidemiology of this condition are scarce.…”

Hirayama Disease Involving Bilateral Distal Upper Limbs of a Student: A Case Report

“…[2,3] It seems to be caused by dynamic compression of the lower cervical cord resulting from sustained or repeated neck flexion. [2,4,5] Its relatively benign course and characteristic appearance of oblique amyotrophy differentiate it from motor neuron disease. [2,4] As the Dynamic MRI (Neutral and flexed) of cervical spine is the mainstay for confirming clinical diagnosis, it is frequently underdiagnosed.…”

“…[2,4,5] Its relatively benign course and characteristic appearance of oblique amyotrophy differentiate it from motor neuron disease. [2,4] As the Dynamic MRI (Neutral and flexed) of cervical spine is the mainstay for confirming clinical diagnosis, it is frequently underdiagnosed. [4][5][6] Name & Address of Corresponding Author Rachit Gulati Post Graduate Trainee, Department of Physical Medicine and Rehabilitation, R.G.…”

“…[2,4] As the Dynamic MRI (Neutral and flexed) of cervical spine is the mainstay for confirming clinical diagnosis, it is frequently underdiagnosed. [4][5][6] Name & Address of Corresponding Author Rachit Gulati Post Graduate Trainee, Department of Physical Medicine and Rehabilitation, R.G. Kar Medical College, Kolkata, West Bengal (India).…”