scholarly journals Juvenile Muscular Atrophy of a Unilateral Upper Extremity (Hirayama Disease) in a Patient with CHARGE Syndrome

2010 ◽  
Vol 1 (2) ◽  
pp. 91-94 ◽  
Author(s):  
T. Yagihashi ◽  
K. Hatori ◽  
K. Ishii ◽  
C. Torii ◽  
S. Momoshima ◽  
...  
Keyword(s):  
Upper Extremity ◽  
Muscular Atrophy ◽  
Charge Syndrome ◽  
Hirayama Disease ◽  
Juvenile Muscular Atrophy

96. Ionic mechanisms for cold paresis in juvenile muscular atrophy of distal upper extremity (Hirayama disease)

2009 ◽  
Vol 120 (5) ◽  
pp. e168-e169
Author(s):  
Setsu Sawai ◽  
Sonoko Misawa ◽  
Kazuaki Kanai ◽  
Sagiri Isose ◽  
Kazumoto Shibuya ◽  
...  
Keyword(s):  
Upper Extremity ◽  
Muscular Atrophy ◽  
Hirayama Disease ◽  
Ionic Mechanisms ◽  
Juvenile Muscular Atrophy

scholarly journals Juvenile Muscular Atrophy of the Proximal Upper Extremity as So-Called Proximal-Type Hirayama Disease: Case Report and Review of the Literature

2019 ◽  
Vol 11 (1) ◽  
pp. 106-111 ◽  
Author(s):  
Akira Yokote ◽  
Kousuke Fukuhara ◽  
Jun Tsugawa ◽  
Yoshio Tsuboi
Keyword(s):  
Upper Extremity ◽  
Muscular Atrophy ◽  
Distinct Type ◽  
Forward Displacement ◽  
Hirayama Disease ◽  
Disease Case ◽  
Upper Cervical ◽  
Cervical Magnetic Resonance Imaging ◽  
Juvenile Muscular Atrophy ◽  
Extradural Space

Hirayama disease is a distinct type of cervical myelopathy characterized by juvenile onset of unilateral muscular atrophy of a distal upper extremity. We report herein a case with Hirayama disease-like juvenile muscular atrophy involving proximal muscles in the upper extremities. In this case, in the flexion position of the neck, cervical magnetic resonance imaging revealed that the spinal cord was compressed by expansion of the posterior extradural space with forward displacement of the dura matter. These neuroimaging results are identical to those of Hirayama disease. However, the involved muscles in this case were the proximal muscles, unlike Hirayama disease. Five previous cases have displayed this rare subtype of Hirayama disease. The cause of the unique phenotype may be abnormal cervical column alignment, with upper cervical kyphosis producing a higher apex of the vertebral level in a cervical flexion position, resulting in mid-cervical segmental myelopathy.

Juvenile Muscular Atrophy of the Distal Upper Extremity (Hirayama Disease) in Two Lanky Look-Alike Brothers

2009 ◽  
Vol 15 (4) ◽  
pp. 220-222 ◽  
Author(s):  
Hiroyuki Kajikawa ◽  
Yasumasa Kokubo ◽  
Akira Taniguchi ◽  
Yutaka Naito ◽  
Shigeki Kuzuhara
Keyword(s):  
Upper Extremity ◽  
Muscular Atrophy ◽  
Hirayama Disease ◽  
Juvenile Muscular Atrophy

Altered axonal excitability properties in juvenile muscular atrophy of distal upper extremity (Hirayama disease)

2011 ◽  
Vol 122 (1) ◽  
pp. 205-209 ◽  
Author(s):  
Setsu Sawai ◽  
Sonoko Misawa ◽  
Kazuaki Kanai ◽  
Sagiri Isose ◽  
Kazumoto Shibuya ◽  
...  
Keyword(s):  
Upper Extremity ◽  
Muscular Atrophy ◽  
Hirayama Disease ◽  
Axonal Excitability ◽  
Juvenile Muscular Atrophy

scholarly journals Nosology of Juvenile Muscular Atrophy of Distal Upper Extremity: From Monomelic Amyotrophy to Hirayama Disease—Indian Perspective

2013 ◽  
Vol 2013 ◽  
pp. 1-12 ◽  
Author(s):  
Kaukab Maqbool Hassan ◽  
Hirdesh Sahni
Keyword(s):  
Upper Extremity ◽  
Muscular Atrophy ◽  
Original Description ◽  
Cervical Cord ◽  
Motor Neuron Diseases ◽  
Hirayama Disease ◽  
Characteristic Appearance ◽  
Contrast Magnetic Resonance ◽  
Cervical Flexion Myelopathy ◽  
Juvenile Muscular Atrophy

Since its original description by Keizo Hirayama in 1959, “juvenile muscular atrophy of the unilateral upper extremity” has been described under many nomenclatures from the east. Hirayama disease (HD), also interchangeably referred to as monomelic amyotrophy, has been more frequently recognised in the west only in the last two decades. HD presents in adolescence and young adulthood with insidious onset unilateral or bilateral asymmetric atrophy of hand and forearm with sparing of brachioradialis giving the characteristic appearance of oblique amyotrophy. Symmetrically bilateral disease has also been recognized. Believed to be a cervical flexion myelopathy, HD differs from motor neuron diseases because of its nonprogressive course and pathologic findings of chronic microcirculatory changes in the lower cervical cord. Electromyography shows features of acute and/or chronic denervation in C7, C8, and T1 myotomes in clinically affected limb and sometimes also in clinically unaffected contralateral limb. Dynamic forward displacement of dura in flexion causes asymmetric flattening of lower cervical cord. While dynamic contrast magnetic resonance imaging is diagnostic, routine study has high predictive value. There is a need to lump all the nomenclatures under the rubric of HD as prognosis in this condition is benign and prompt diagnosis is important to institute early collar therapy.

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