Hirayama’s disease: an Italian single center experience and review of the literature

Vitale, Valerio; Caranci, Ferdinando; Pisciotta, Chiara; Manganelli, Fiore; Briganti, Francesco; Santoro, L.; Brunetti, Arturo

doi:10.21037/qims.2016.07.08

Cited by 23 publications

(24 citation statements)

References 48 publications

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“…[14][15][16][17] Some authors also proposed atopy and elevated serum immunoglobulin E levels as participating factors. 18 Tanaka et al 19 observed the intrathecal upregulation of proinflammatory T-helper-1 cytokines/ chemokines, such as interferon-␥ and macrophage inflammatory proteins-1 ␤ chemokine, in the CSF of patients with HD, indicating the possible involvement of intrathecal immunologic processes in this condition. In 2010, Ciceri et al 20 proposed that venous congestion in flexion might play an additional role in determining spinal cord ischemic changes.…”

Section: Discussionmentioning

confidence: 99%

The Importance of Flexion MRI in Hirayama Disease with Special Reference to Laminodural Space Measurements

Boruah¹,

Prakash

Gogoi

et al. 2018

AJNR Am J Neuroradiol

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Section: Discussionmentioning

confidence: 99%

The Importance of Flexion MRI in Hirayama Disease with Special Reference to Laminodural Space Measurements

Boruah¹,

Prakash

Gogoi

et al. 2018

AJNR Am J Neuroradiol

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“…Vitale et al [ 17 ] suggested an MRI protocol including: sagittal T1-weighted and T2-weighted sequences and axial T2 or T2*-weighted sequence in neutral position; sagittal T2-weighted sequences and axial T2 or T2*-weighted sequence in a neck-flexion of 25–35 degrees in addition to sagittal T1-weighted sequences in neck-flexion before and after gadolinium intravenous administration. Snake-eyes appearance, a radiological finding described as a symmetrical bilateral small high-signal-intensity lesion on axial T2-weighted MRI, appears during the late stage of “Hirayama disease” and is proposed as an indicator of irreversible damage and poor prognosis [ 18 ].…”

Section: Discussionmentioning

confidence: 99%

Monomelic Amyotrophy (Hirayama Disease): A Rare Case Report and Literature Review

2020

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Hirayama disease is a rare neurological entity that is characterized by initial progressive muscular wasting and weakness of the distal upper limb in young men, followed by a spontaneous arrest within several years. The disease is believed to be a result of forward displacement of the cervical dural sac and spinal cord induced by neck flexion. It is commonly seen in Asia and rarely encountered in the Middle East countries. We report a rare case of a 20-year-old Kuwaiti patient presenting with a 10-month duration of gradual left upper limb weakness and wasting. We describe his electrophysiological and radiological findings that confirmed the diagnosis, and conducted a literature review. Hirayama disease is rarely encountered in clinical settings and should be suspected in male patients presenting with unilateral or asymmetrical bilateral lower motor weakness of hands and forearms. It is a benign entity, and cervical collar is usually the only treatment needed in most cases.

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“…Fusion procedures, anterior or posterior, arrest segmental movement, and prevent the dural migration and cord compression during flexion. Duraplasty after laminectomy or laminoplasty, 21,[36][37][38][39][40] on the other hand, directly addresses the pathology of the tight dural canal. An added advantage of a posterior procedure is that it also addresses the flexion-induced dilatation of the epidural venous plexus seen in HD.…”

Section: Surgical Options In Hdmentioning

confidence: 99%

“…A literature search revealed that while over 300 cases of HD 6,8,26 were managed with anterior or posterior fusion, only 16 patients were treated with duraplasty without fusion (►Table 5). 16,21,[36][37][38][39][40] Ours is the first large series that critically evaluates CD as a surgical option in HD.…”

Section: Selection Of Surgical Proceduresmentioning

confidence: 99%

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’s disease: an Italian single center experience and review of the literature

Cited by 23 publications

References 48 publications

The Importance of Flexion MRI in Hirayama Disease with Special Reference to Laminodural Space Measurements

The Importance of Flexion MRI in Hirayama Disease with Special Reference to Laminodural Space Measurements

Monomelic Amyotrophy (Hirayama Disease): A Rare Case Report and Literature Review

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

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