Juvenile muscular atrophy of the distal upper extremity (hirayama disease) in two lanky look-alike brothers.
BACKGROUND
Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is a sporadic disorder, but very rarely occurs in a familial pattern.


OBJECTIVE
To describe the clinical features of Hirayama disease in 2 brothers, especially the look-alike anatomic physiques and magnetic resonance imaging (MRI) findings.


METHODS
Case report.


METHODS
Two physically look-alike young brothers with Hirayama disease.


RESULTS
Clinical symptoms of the present brothers were consistent with those of sporadic cases. Their lanky physique, clinical features, and MRI findings looked alike to each other.


CONCLUSIONS
The pathogenesis of familial Hirayama disease is not well understood. The present brothers looked alike in terms of their physical frame and clinical symptoms. These findings suggest that similar mechanical damage against the cervical spinal cord by look-alike anatomic frame of the neck could be an important factor of familial Hirayama disease in the present brothers.
Kajikawa
Hiroyuki
H
Department of Neurology, Mie University Graduate School of Medicine, Tsu-City, Mie Prefecture, Japan. hkajikawa@sch.miekosei.or.jp
Kokubo
Yasumasa
Y
Taniguchi
Akira
A
Naito
Yutaka
Y
Kuzuhara
Shigeki
S
D000293:Adolescent; D000367:Age Factors; D017668:Age of Onset; D002574:Cervical Vertebrae / Q000473:pathology; D018450:Disease Progression; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006225:Hand / Q000294:innervation / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D040582:Inheritance Patterns / Q000235:genetics; D008279:Magnetic Resonance Imaging; D008297:Male; D018908:Muscle Weakness / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D018482:Muscle, Skeletal / Q000294:innervation / Q000473:pathology* / Q000503:physiopathology; D009133:Muscular Atrophy / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D010641:Phenotype; D035781:Siblings; D013008:Somatotypes / Q000235:genetics; D013116:Spinal Cord / Q000473:pathology / Q000503:physiopathology; D013117:Spinal Cord Compression / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D055815:Young Adult