Spinal angiography and epidural venography in juvenile muscular atrophy of the distal arm “Hirayama disease”

2009 ◽  
Vol 40 (2) ◽  
pp. 206-212 ◽  
Author(s):  
Bakri Elsheikh ◽  
John T. Kissel ◽  
Gregory Christoforidis ◽  
Matthew Wicklund ◽  
Dimitri T. Kehagias ◽  
...  
Keyword(s):  
Muscular Atrophy ◽  
Spinal Angiography ◽  
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

scholarly journals Bimelic Hirayama Disease: Clinical Dilemma Solved by Imaging

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Shalabh Jain ◽  
Siddharth Yadav ◽  
Swarna Gupta ◽  
Ritu Gupta
Keyword(s):  
Muscular Atrophy ◽  
Definitive Diagnosis ◽  
Muscular Weakness ◽  
Neuron Type ◽  
Imaging Studies ◽  
Provisional Diagnosis ◽  
Hirayama Disease ◽  
Clinical Dilemma ◽  
Juvenile Muscular Atrophy ◽  
Heavy Metal Intoxication

Hirayama disease (juvenile muscular atrophy of distal upper extremity) is a cervical myelopathy predominantly affecting adolescent males. It is characterized by progressive muscular weakness and atrophy of unilateral or asymmetrically bilateral distal upper limbs. We report a case of an 18-year-male painter, who presented with gradually progressive, symmetrical bilateral weakness of hands and forearm for the last two years. On the basis of clinical examination, a provisional diagnosis of lower motor neuron type of symmetrical distal weakness due to heavy metal intoxication was kept. However, imaging studies helped in making a definitive diagnosis of Hirayama disease. The patient was advised cervical collar, and there was no progression in symptoms after six months of followup. Due to the rarity of bilateral symmetrical involvement in Hirayama disease, it remains obscured or unsuspected clinically, and MRI plays a pivotal role in diagnosis.

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
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