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European Spine Journal (2018) 27:1207–1208
https://doi.org/10.1007/s00586-018-5610-4
REVIEWER’S COMMENT
Expert’s Comment concerning Grand Rounds case entitled “Hirayama
disease” by D. C. Kieser, P. J. Cox, S. C. J. Kieser (Eur Spine J; 2018:
doi:10.1007/s00586‑018‑5545‑9)
Takeo Furuya
1
Received: 2 April 2018 / Accepted: 12 April 2018 / Published online: 20 April 2018
© Springer-Verlag GmbH Germany, part of Springer Nature 2018
This is a well-written case report and literature review
describing Hirayama disease in a 21-year-old man with pro-
gressive weakness of the hand and forearm [1]. The paper
highlights many of the important aspects of performing the
diagnosis by neurological examination and imaging studies.
It shows a representative photographic image of the forearms
of the patient and describes the characteristic sign of Hiray-
ama disease, which is an oblique border of muscular atro-
phy on both the volar and dorsal forearms, a finding widely
known as ‘oblique atrophy’. The paper also describes the
importance of dynamic imaging followed by MRI with flex-
ion of the cervical spine and CT myelography with the neck
in the neutral position and flexion position.
I will not discuss the many specific points mentioned in
the "Grand Rounds" portion of the paper, except to make
specific comments about the treatment of Hirayama disease.
Conservative therapy using the soft collar is emphasized in
this paper. As the authors discussed in the paper, there is no
doubt that conservative management is the first-line treat-
ment of choice, and that it has shown excellent results with
a successful arrest of disease progression in many patients
and occasional reversal of signs and symptoms. I would like
to add information regarding the paper [2] that the authors
cited (reference 5 in this report). Tashiro et al. reported the
positive effects of cervical collar immobilization. They also
reported the efficacy of surgical treatment with various sur-
gical techniques in 41 cases to restrict the forward flexion
of the cervical spine or to permit the dura mater to relax.
They concluded that all these techniques showed favorable
therapeutic effects.
The discussion of the surgical indications and the surgi-
cal outcomes is limited in the paper. The efficacy of surgical
treatment has been reported in previous articles [3–9]. The
purpose of surgical intervention is to prevent the progres-
sion of neurological signs and symptoms. Readers should
be informed that there are patients with this disease who
progress to irreversible deficits. Because of such cases, we
should bear in mind the limitations of conservative treatment
and consider performing surgical treatment and discontinu-
ing conservative therapy.
This case study and Grand Rounds underscores the
importance of differential diagnosis in young patients with
muscular weakness and atrophy of muscles of the forearms.
It contributes to closing the gap between our current level of
knowledge and a comprehensive understanding of Hirayama
disease.
Compliance with ethical standards
Conflict of interest The author declares that he has no conflict of inter-
est.
* Takeo Furuya
furuya-takeo@chiba-u.jp
1
Spine Division, Department of Orthopaedics, Chiba
University Graduate School of Medicine, 1-8-1 Inohana,
Chuo-ku, Chiba 260-8670, Japan
@Phil_Robichaud
@deepthiw
@JoseServera