• Users Online: 796
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Contacts Login 


 
 Table of Contents  
IMAGES IN MEDICINE
Year : 2016  |  Volume : 29  |  Issue : 5  |  Page : 311

Hirayama disease


Department of General Medicine, Academy of Medical Sciences, Pariyaram, Kannur, Kerala, India

Date of Web Publication6-Jan-2017

Correspondence Address:
A P Shibeesh
Department of General Medicine, Academy of Medical Sciences, Pariyaram, Kannur, Kerala
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


PMID: 28098093

Rights and Permissions

How to cite this article:
Valliyot B, Sarosh Kumar K K, Beevi K, Sarin S M, Shibeesh A P. Hirayama disease . Natl Med J India 2016;29:311

How to cite this URL:
Valliyot B, Sarosh Kumar K K, Beevi K, Sarin S M, Shibeesh A P. Hirayama disease . Natl Med J India [serial online] 2016 [cited 2021 Jul 28];29:311. Available from: http://www.nmji.in/text.asp?2016/29/5/311/197830

A 70-year-old man presented with a history of weakness, wasting and deformities affecting both hands and forearms starting 40 years ago. There was no associated history of trauma, neck pain or loss of sensation in either upper limb. Neurological examination revealed flexion deformities of both hands ([Figure 1]) with weakness and atrophy of the thenar, hypothenar, interossei and forearm muscles along with fasciculations and an action tremor affecting both upper limbs. Magnetic resonance imaging (MRI; [Figure 2]) revealed focal atrophic changes in the spinal cord at the level of the 4th to 7th cervical vertebrae, with an abnormal signal in the region of the anterior horn cells suggestive of Hirayama disease.
Figure 1. Hands of the patient showing bilateral flexion deformities and wasting of muscles of the forearm

Click here to view
Figure 2. MRI showing thinning of the cervical spinal cord with signal changes in the region of the anterior horn cells

Click here to view


Hirayama disease typically affects males in the second and third decades with initial progression of weakness and wasting of the distal upper limbs followed by spontaneous arrest of progression after a few years; this distinguishes it from motor neuron disease, which is relentlessly progressive. Syringomyelia, spinal cord tumours, multifocal motor neuropathy and toxic neuropathies are important differential diagnoses. Early in the course of the disease imaging reveals flattening of the spinal cord in routine non-flexion MRI images. In later stages, there may be focal spinal cord atrophy. [1] In the absence of specific treatment, use of a cervical collar and physiotherapy were advised for our patient.

 
  References Top

1.
Chen CJ, Chen CM, Wu CL, Ro LS, Chen ST, Lee TH. Hirayama disease: MR diagnosis. AJNR Am J Neuroradiol 1998; 19: 365-8.  Back to cited text no. 1
    


    Figures

  [Figure 1], [Figure 2]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
References
Article Figures

 Article Access Statistics
    Viewed1184    
    Printed49    
    Emailed0    
    PDF Downloaded541    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]