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abstract: 'Hirayama disease is a monomyelic variant of motor neuron disease (MND) and has distinctive features of male predominance, asymmetric involvement of upper extremities with a self limiting course. Flexion MRI (magnetic resonance imaging) forms the main stay for diagnosis of this condition. Here we report such an unusual case of Hirayama disease in a male patient of 20 years who presented with weakness and atrophy in right upper limb. Careful clinical examination will help to use the flexion MRI studies for the diagnosis of this condition as done in our case.'
altloc:
  - http://www.ojhas.org/issue47/2013-3-16.html
chapter: ~
commentary: ~
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conference: ~
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contact_email: ~
creators_id:
  - ~
  - ~
  - ~
  - nmsbabu18@yahoo.com
  - ~
creators_name:
  - family: Mahajan
    given: SK
    honourific: ''
    lineage: ''
  - family: Kaushik
    given: M
    honourific: ''
    lineage: ''
  - family: Raina
    given: R
    honourific: ''
    lineage: ''
  - family: Sharath Babu
    given: NM
    honourific: ''
    lineage: ''
  - family: Raghav
    given: S
    honourific: ''
    lineage: ''
date: 2013-11-15
date_type: published
datestamp: 2015-02-21 14:35:19
department: ~
dir: disk0/00/00/97/03
edit_lock_since: ~
edit_lock_until: 0
edit_lock_user: ~
editors_id:
  - Kakkilaya BS
editors_name:
  - family: Kakkilaya
    given: Srinivas
    honourific: Dr
    lineage: ''
eprint_status: archive
eprintid: 9703
fileinfo: /9703/1.hassmallThumbnailVersion/2013-3-16.pdf;/9703/1/2013-3-16.pdf
full_text_status: public
importid: ~
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ispublished: pub
issn: ~
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item_issues_reported_by: []
item_issues_resolved_by: []
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keywords: Hirayama disease; Motor neuron disease; Flexion MRI; Monomyelic
lastmod: 2015-04-20 11:44:35
latitude: ~
longitude: ~
metadata_visibility: show
note: ~
number: 3(16)
pagerange: ~
pubdom: TRUE
publication: Online Journal of Health and Allied Sciences
publisher: Kakkilaya BS
refereed: TRUE
referencetext: "1.\tChan YW, Kay R, Schwartz MS. Juvenile distal spinal muscular atrophy of upper extremities in Chinese males: A single fiber electromyographic study of arms and legs. J Neurol Neurosurg Psychiatry 1991; 54:165-166.\r\n2.\tNascimento OJ, Freitas MR. Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease): a clinical variant of the benign monomelic amyotrophy. Arq Neuropsiquiatr 2000;58:814-819.\r\n3.\tKikuchi S, Tashiro K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease). In Jones HR Jr., De Vivo DC, Darras BT (eds), Neuromuscular Disorders of Infancy, Childhood and Adolescence-A clinician's approach. Butterworth-Heinemann; UK 2003; pp 167-181.\r\n4.\tWilliams PL, Warwick R, Dyson M, Bannister LH. Gray’s Anatomy. 37th ed. London, England: Churchill Livingstone; 1989. pp 1086–1092.\r\n5.\tBland JH. Basic anatomy. In: Bland JH, ed. Disorders of the Cervical Spine: Diagnosis and Medical Management. 2nd ed. Philadelphia, Pa: Saunders; 1994. pp 41–70.\r\n6.\tHirayama K. Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama’s disease). In: De Jong JMBV, ed. Handbook of Clinical Neurology. Amsterdam, the Netherlands: Elsevier. 1991;15:107–120.\r\n7.\tMukai E, Matsuo T, Muto T, Takahashi A, Sobue I. Magnetic resonance imaging of juvenile-type distal and segmental muscular atrophy of upper extremities. Clin Neurol (Tokyo) 1987;27:99–107.\r\n8.\tKikuchi S, Tashiro K, Kitagawa K, Iwasaki Y, Abe H. A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama’s disease): flexion myelopathy with tight dural canal in flexion [in Japanese]. Clin Neurol (Tokyo) 1987;27:412–419."
relation_type: []
relation_uri: []
reportno: ~
rev_number: 10
series: ~
source: ~
status_changed: 2015-02-21 14:35:19
subjects:
  - OJHAS
succeeds: ~
suggestions: ~
sword_depositor: ~
sword_slug: ~
thesistype: ~
title: 'Hirayama Disease - A Variant of Motor Neuron Disease and Role of Flexion MRI in Diagnosis'
type: journale
userid: 4338
volume: 12
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