J Korean Acad Rehabil Med.  2010 Dec;34(6):701-709.

Clinical Characteristics, Electrodiagnostic, and Imaging Findings of Atypical Forms of Motor Neuron Disease

• Huh JP
• Sung DH
• Jo JM
• Yoo JS
• Kim BJ

Abstract

OBJECTIVE
To describe the clinical characteristics, electrodiagnostic, and imaging findings of Hirayama disease (HD), late onset monomelic amyotrophy (LMA), and brachial amyotrophic diplegia (BAD). METHOD: A retrospective analysis of the medical records, electrodiagnostic, and imaging findings of 12 patients (4 HD, 2 LMA, 6 BAD) was done. For patients whose last clinic follow-up exceeded 6 months a telephone survey was done to see if there were any symptom changes.
RESULTS
The clinical, electrodiagnostic, and imaging findings of the HD and BAD patients were similar to previous studies. Except for a later onset, age disease duration was too short to distinguish LMA from HD or other motor neuron diseases. One patient in the BAD group progressed to amyotrophic lateral sclerosis (ALS) and another died due to undetermined respiratory failure. These two patients showed abnormalities in their lower extremities, thoracic paraspinal, and craniocervical muscles on needle electromyography. Except for another patient, none of the other three patients showed abnormalities in their lower extremities, thoracic paraspinals, or craniocervical muscles on needle electromyography.
CONCLUSION
HD and BAD can be considered as separate disease entities. However, a longer follow-up period than previously recommended is necessary to differentiate BAD from ALS. Follow-up period was too short to determine whether LMA can also be considered as a separate disease entity.

Keyword

Hirayama disease; Brachial amyotrophic diplegia; Late onset monomelic amyotrophy

MeSH Terms

Amyotrophic Lateral Sclerosis
Electromyography
Follow-Up Studies
Humans
Lower Extremity
Medical Records
Motor Neuron Disease
Motor Neurons
Muscles
Needles
Respiratory Insufficiency
Retrospective Studies
Spinal Muscular Atrophies of Childhood
Telephone
Spinal Muscular Atrophies of Childhood