Evidence map›Paper›PMID 40260871›Full record

ArticleAnnals of Indian Academy of Neurology2025

In-Depth Understanding of Hirayama Disease: Dural Detachment Beyond Cervical Spine.

Seena Vengalil, Vijaykumar Boddu, Karthik Kulanthaivelu, Dipti Baskar, Saraswati Nashi, Nupur Pruthi, Hemant Bhargav, Alok M Uppar, Chandrajit Prasad, Madhulika Kotra and 3 more

Abstract read
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Article in Annals of Indian Academy of Neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Hirayama disease in a young Indonesian male: a case report.Journal of spine surgery (Hong Kong) · 2026
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4 · The record

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5 · Who and what money

Authors and funding

13 authors.

Seena VengalilDepartment of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Vijaykumar BodduDepartment of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Karthik KulanthaiveluDepartment of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Dipti BaskarDepartment of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Saraswati NashiDepartment of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Nupur PruthiDepartment of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Hemant BhargavDepartment of Integrative Medicine, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Alok M UpparDepartment of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Chandrajit PrasadDepartment of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Madhulika KotraDepartment of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Kiran PolavarapuDepartment of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Veeramani Preethish-KumarDepartment of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Atchayaram NaliniDepartment of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

BACKGROUND AND

objectivesHirayama disease (HD) is a cervical flexion-induced compressive myelopathy. Typically, there is forward displacement and loss of attachment of dural sac to lamina at the cervical level during neck flexion. However, the extent of the dural detachment (DD) has not been studied carefully. We undertook this study to know the extent of DD in HD.

methodsWe conducted a retrospective study of HD patients evaluated from 2015 to 2023. Patients with DD extending beyond the cervical spine were selected, and their clinical and radiological features were studied.

resultsOne hundred and thirty-two (62.8%) patients were identified to have DD beyond the cervical spine in a cohort of 210 HD patients. The mean age at onset and duration were 18.09 ± 2.3 years (13-26) and 38.63 ± 39.9 months, respectively. Proximo-distal involvement was noted in 50% of patients, while 33% and 17% of patients had isolated distal and proximal involvement, respectively. Wasted legs were observed in three patients. Cord atrophy was present in 96.9% of patients, extending from C5 to C7. Epidural detachment and engorgement of posterior epidural venous plexus were evident in all. DD extended from C2 to D10 vertebral level. The cranial extent of DD was from C2 to C4 in 87% of cases, and the caudal extent was D1-D5 in 84% of cases, extending up to D10 in two cases.

conclusionsThe HD spectrum continues to evolve phenotypically and radiologically. The pathophysiological mechanisms and DD extend beyond the cervical spine in a large proportion of patients. This makes it important to cover a longer part of the spine during imaging. This may have implications on the management of patients, particularly those with isolated lower limb involvement.

Indexed as

dural detachmentflexion-induced myelopathyHirayama diseaseIndiamagnetic resonance imagingMonomelic amyotrophy

Identifiers

PMID40260871
PMCPMC12393858

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