Do longer necks predispose to Hirayama disease? A comparison with mimics and controls

Khadilkar, Satish V; Patel, Bhagyadhan A.; Bhutada, Ashish; Chaudhari, Chetan

doi:10.1016/j.jns.2015.11.005

Cited by 13 publications

(14 citation statements)

References 8 publications

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“…In recently published studies, Khadilkar et al reported that their patients had longer necks than did normal patients (49). Li et al found that the imbalance between the strengths of flexor and extensor muscles that was found in HD patients during cervical flexion resulted in dynamic instability and showed that it was a risk factor for spinal cord atrophy.…”

Section: Pathogenesismentioning

confidence: 99%

Update on the Pathogenesis, Clinical Diagnosis, and Treatment of Hirayama Disease

Wang

Tian

et al. 2022

Front. Neurol.

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Hirayama disease (HD) is characterized by the juvenile onset of unilateral or asymmetric weakness and amyotrophy of the hand and ulnar forearm and is most common in males in Asia. A perception of compliance with previous standards of diagnosis and treatment appears to be challenged, so the review is to update on HD. First, based on existing theory, the factors related to HD includes, (1) cervical cord compression during cervical flexion, (2) immunological factors, and (3) other musculoskeletal dynamic factors. Then, we review the clinical manifestations: typically, (1) distal weakness and wasting in one or both upper extremities, (2) insidious onset and initial progression for 3–5 years, (3) coarse tremors in the fingers, (4) cold paralysis, and (5) absence of objective sensory loss; and atypically, (1) positive pyramidal signs, (2) atrophy of the muscles of the proximal upper extremity, (3) long progression, and (4) sensory deficits. Next, updated manifestations of imaging are reviewed, (1) asymmetric spinal cord flattening, and localized lower cervical spinal cord atrophy, (2) loss of attachment between the posterior dural sac and the subjacent lamina, (3) forward displacement of the posterior wall of the cervical dural sac, (4) intramedullary high signal intensity in the anterior horn cells on T2-weighted imaging, and (5) straight alignment or kyphosis of cervical spine. Thus, the main manifestations of eletrophysiological examinations in HD include segmental neurogenic damages of anterior horn cells or anterior roots of the spinal nerve located in the lower cervical spinal cord, without disorder of the sensory nerves. In addition, definite HD needs three-dimensional diagnostic framework above, while probable HD needs to exclude other diseases via “clinical manifestations” and “electrophysiological examinations”. Finally, the main purpose of treatment is to avoid neck flexion. Cervical collar is the first-line treatment for HD, while several surgical methods are available and have achieved satisfactory results. This review aimed to improve the awareness of HD in clinicians to enable early diagnosis and treatment, which will enable patients to achieve a better prognosis.

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Section: Pathogenesismentioning

confidence: 99%

Update on the Pathogenesis, Clinical Diagnosis, and Treatment of Hirayama Disease

Wang

Tian

et al. 2022

Front. Neurol.

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“…18 19 Another theory suggests an overstretch of the dura on flexion occurring due to disparity between the growth of the vertebral column and the spinal content. 20 Since males tend to have faster rates of vertebral column growth than females, this disproportionate-growth theory can also explain the male preponderance seen in HD. 20 …”

Section: Discussionmentioning

confidence: 99%

“… 20 Since males tend to have faster rates of vertebral column growth than females, this disproportionate-growth theory can also explain the male preponderance seen in HD. 20 …”

Section: Discussionmentioning

confidence: 99%

“…18,19 Another theory suggests an overstretch of the dura on flexion occurring due to disparity between the growth of the vertebral column and the spinal content. 20 Since males tend to have faster rates of vertebral column growth than females, this disproportionate-growth theory can also explain the male preponderance seen in HD. 20 Non dural-based theories of HD include compression by anteriorly located osteophytic bars, 17 flexion-induced venous stagnation, 16,21,22 and underlying instability.…”

Section: Pathogenesis Of Hd-theories and Substratesmentioning

confidence: 99%

“…20 Since males tend to have faster rates of vertebral column growth than females, this disproportionate-growth theory can also explain the male preponderance seen in HD. 20 Non dural-based theories of HD include compression by anteriorly located osteophytic bars, 17 flexion-induced venous stagnation, 16,21,22 and underlying instability. 6,23 The genesis of the latter hypothesis is related to the effectiveness of collar wear and reports of abnormal alignment 24 and range of neck flexion 25 rather than any definite radiological evidence of instability.…”

Section: Pathogenesis Of Hd-theories and Substratesmentioning

confidence: 99%

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Outcomes after Cervical Duraplasty for Monomelic Amyotrophy (Hirayama Disease): Results of a Case–Control Study of 60 Patients

Thakar

Arun

Rajagopal

et al. 2021

JNRP

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Background The optimal management and the role of surgery in monomelic amyotrophy, also known as Hirayama disease (HD), remain controversial. In the largest series of patients with HD managed by cervical duraplasty (CD), this study compares the outcomes between conservative and surgical management. Methods A retrospective case–control study was performed on 60 patients with HD. The cases consisted of 30 patients who underwent CD, and 30 age- and sex-matched controls who managed with long-term collar wear. Clinical improvement was recorded using two patient-reported outcome measures—the Odom's scale and a self-rated hand grip strength score. Median and ulnar compound muscle action potential (CMAP) amplitudes and various magnetic resonance imaging parameters were recorded. Results None of the controls experienced clinical improvement at follow-up; 90% remained status quo at 24.33 ± 9.38 months. Seventy-six percent of the operated patients demonstrated clinical improvement at 29.61 ± 12.78 months, and a majority of them reported moderately better grip strength. The preoperative anteroposterior diameter (APD) of the surgical group improved from 0.74 ± 0.13 to 0.86 ± 0.21 (p = 0.01), and the cord demonstrated a significant decrease in forward migration on flexion (0.20 ± 0.11 and 0.03 ± 0.07 preoperatively and at follow-up, respectively, p < 0.0001). Patients with clinical improvement demonstrated significantly better mean APD and median and ulnar CMAP values than those who did not improve (p < 0.0001). The mean cervical alignment did not change significantly at follow-up (p = 0.13). Conclusions This study shows that CD can significantly alter the clinical course of patients with progressive symptoms of HD. This approach successfully arrests the forward migration of the cervical cord on flexion and promotes morphological recovery of the cord. A majority of the patients undergoing CD demonstrate clinical and electrophysiological improvement at mid-term follow-up.

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Hirayama Disease and Other Focal Amyotrophies

Khadilkar

Yadav²,

Patel³

2017

Neuromuscular Disorders

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Do longer necks predispose to Hirayama disease? A comparison with mimics and controls

Cited by 13 publications

References 8 publications

Update on the Pathogenesis, Clinical Diagnosis, and Treatment of Hirayama Disease

Update on the Pathogenesis, Clinical Diagnosis, and Treatment of Hirayama Disease

Outcomes after Cervical Duraplasty for Monomelic Amyotrophy (Hirayama Disease): Results of a Case–Control Study of 60 Patients

Hirayama Disease and Other Focal Amyotrophies

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