This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
Abstract
Background: Hirayama disease (brachial monomelic amyotrophy) is a unilateral or grossly asymmetric bilateral disease. Bilaterally symmetric involvement has never been described.
Methods: Based on cardinal clinical and MRI criteria, a total of 106 patients with Hirayama disease from two tertiary care hospitals of North India seen between 1992 and 2008 formed the basis of this study. All those found to have bilaterally symmetric involvement on clinical and electrophysiologic basis were evaluated for clinical, electrophysiologic, and MRI correlates other than those required for the diagnosis.
Results: Eleven patients, who constituted around 10% of all the patients with Hirayama disease, were found to have bilaterally symmetric involvement. Nine of them had a history of unilateral onset. The important characteristics of this type of presentation included severe weakness and wasting in C7, C8, and T1 myotomes that frequently spilled over to C6 segment, predominant autonomic dysfunction in distal upper extremities in the form of cold paresis, cold skin, excessive sweating, and hair loss over the dorsum of the hands, and a very prominent bilateral minipolymyoclonus. MRI during complete flexion of neck showed severe flattening of lower cervical spinal cord against C5-C6 vertebral bodies and development of a crescent-shaped enhancing epidural space extending from C4 to T2 spine.
Conclusion: Bilaterally symmetric Hirayama disease is a severe form of a classic disease which remains undiagnosed due to a common notion that it is a unilateral or grossly asymmetric disease. This description calls for review of the term “brachial monomelic amyotrophy” described to denote this disease.
Glossary
- ADM=
- abductor digiti minimi;
- ALS=
- amyotrophic lateral sclerosis;
- APB=
- abductor pollicis brevis;
- CMAP=
- compound motor action potential;
- EDC=
- extensor digitorum communis;
- JASSMA=
- juvenile asymmetric segmental spinal muscular atrophy;
- LMN=
- lower motor neuron;
- NCV=
- nerve conduction velocity.
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
Letters: Rapid online correspondence
- Bilaterally symmetric form of Hirayama disease
- Reply from the author
REQUIREMENTS
If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
You May Also be Interested in
Dr. Jessica Ailani and Dr. Zubair Ahmed