Anterior displacement of the spinal cord on flexion views in a patient with unilateral arm weakness

Sim, Kenneth; Gaillard, Frank; Day, Timothy

doi:10.1016/j.jocn.2013.06.028

Cited by 4 publications

(3 citation statements)

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“…During the last two decades, more than 900 patients with Hirayama disease have been included in clinical studies conducted in mainland China (19)(20)(21)(22)(23). There have been reports of increasing numbers of cases in other countries and regions including India, North America, Europe, and Australia (3,(24)(25)(26)(27). Inadequate understanding of the disease by pediatric neurologists in North America may decrease the rate of diagnosis of HD, and there is no doubt that the number of HD patients has increased (26).…”

Section: Epidemiologymentioning

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: Epidemiologymentioning

confidence: 99%

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

Wang

Tian

et al. 2022

Front. Neurol.

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“…Even though Hirayama disease is mainly seen in Asia, a few cases have been reported in the Western hemisphere as well. Increased awareness may lead to early diagnosis and intervention in North America [ 19 - 21 ].…”

Section: Discussionmentioning

confidence: 99%

Cervical Spine Magnetic Resonance Imaging Findings in Hirayama Disease

Kalekar¹,

Prabhu²,

Suhas³

2023

Cureus

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“…The strength of posterior cervical extensors and cervical sagittal alignment were closely related, indicating that the weakness of posterior cervical extensors might predispose the cervical spine of patients with HD to a less stable situation (22). A total of eighteen case reports, including 19 patients with HD with proximal involvement, were reported in English studies, as shown in Table 4 (3)(4)(5)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37). A number of these patients were affected in the proximal and distal muscles of the upper limb.…”

Section: Discussionmentioning

confidence: 99%

The radiological and electrophysiological characteristics of Hirayama disease with proximal involvement: A retrospective study

Wang

Tian

et al. 2022

Front. Neurol.

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PurposeHirayama disease (HD) has been largely believed to affect only distal muscles. However, the proximal upper extremities have been affected in some cases, which can be confused with motor neuron diseases.MethodsBaseline data, deep tendon reflex, Hoffmann sign, cervical curvature, sagittal Cobb angle, atrophied spinal cord, high signal intensity, loss of attachment, and affected muscles and segments on electromyography (EMG) were retrospectively obtained and compared between patients with HD with proximal involvement and patients with simple distal HD in one center from September 2007 to April 2022.ResultsIn this study, fifteen patients with proximal HD and 30 patients with simple distal HD were included. The proximal group had a larger proportion of patients with decreased biceps reflex, decreased triceps reflex, brisk or hyperactive knee reflex, positive Hoffmann sign, and cervical kyphosis. The curvatures of the upper part of the cervical spine (C2-4) were lost to a greater degree in the proximal group. More affected segments were observed on magnetic resonance imaging (MRI) and electromyography in the proximal group.ConclusionThe injured segments were longer and the upper curvature of the cervical spine was poorer in patients with HD with proximal involvement. These findings indicated that proximal involvement may indicate more serious HD.

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Anterior displacement of the spinal cord on flexion views in a patient with unilateral arm weakness

Cited by 4 publications

References 0 publications

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

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

Cervical Spine Magnetic Resonance Imaging Findings in Hirayama Disease

The radiological and electrophysiological characteristics of Hirayama disease with proximal involvement: A retrospective study

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