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Hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord. It is primarily seen in young males of Indian or Asian descent. With increasing dispersion of these populations this condition is increasingly being encountered internationally. This grand round reviews this rare but increasingly recognized condition.
Materials and methods
We present a classic case of a young Indian male with progressive hand and forearm weakness. We discuss the typical clinical presentation, appropriate investigations and management of this condition.
Our patient presented with oblique amyotrophy and underwent a diagnostic flexion MRI scan which revealed anterior translation of the posterior dura with compression of the anterior horns of the lower cervical cord. He has been successfully treated in a cervical collar.
This case illustrates the typical presentation, diagnostic investigations and treatment of Hirayama syndrome. It is hoped that this review will alert clinicians of this condition and optimize the management of affected individuals.
KeywordsHirayama Oblique amyotrophy Atrophy Juvenile spinal muscular atrophy Juvenile muscular atrophy Monomelic amyotrophy Asymmetric segmental spinal muscular atrophy
Glynny Kieser for her editorial input.
Compliance with ethical standards
Conflict of interest
None of the authors have any potential conflict of interest.
- 2.Hirayama K, Toyokura Y, Tsubaki T (1959) Juvenile muscular atrophy of unilateral upper extremity; a new clinical entity. Psychiatr Neurol Jpn 61:2190–2197Google Scholar
- 7.Tokumaru Y, Hirayama K (1989) Anterior shift of posterior lower cervical dura mater in patients with juvenile muscular atrophy of unilateral upper extremity. Clin Neurol 29:1237–1243Google Scholar
- 8.Kano M (1998) Electrophysiology of Hirayama disease. Neurol Med 48:324–328Google Scholar
- 9.Kuwabara S, Nakajima M, Hattori T et al (1999) Electrophysiology of juvenile muscular atrophy of unilateral upper limb (Hirayama’s disease). Clin Neurol 39:508–512Google Scholar
- 10.Kijimi M, Nakajima M, Hattori T et al (1996) Electrophysiological investigation of cold paraparesis. Clin Neurol 36:1452Google Scholar
- 11.Suyama C, Suzuki T, Sugimoto Y et al (1994) Efficacy of short-latency somatosensory evoked potential (S-SEP) in cases of Hirayama disease. Igaku Kensa 43:1717–1723Google Scholar
- 12.Shizukawa H, Imai T, Kobayashi N et al (1994) Cervical flexion-induced changes of motor evoked potentials by transcranial magnetic stimulation in a patient with Hirayama disease: juvenile muscular atrophy of unilateral upper extremity. Clin Neurol 34:500–504Google Scholar
- 13.Saito M, Hirayam K, Narabayashi H et al (1978) Juvenile muscular atrophy localized to the hand and forearm: histochemical and ultrastructural studies of biopsied muscles in 10 cases. Clin Neurol 18:247–253Google Scholar
- 14.Yamazaki M, Hirayama K (1990) Queckenstedt test: pressure pattern analysis in spinal subarachnoid block. Clin Neurol 30:247–253Google Scholar
- 15.Hirayama K, Tomonaga M, Kitano K et al (1985) The first autopsy case of juvenile muscular atrophy of unilateral upper extremity. Neurol Med 22:85–88Google Scholar
- 19.Tokumaru Y, Hirayama K (1992) A cervical collar therapy for nonprogressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama’s disease). Clin Neurol 32:1102–1106Google Scholar