• World Neurosurg · Mar 2024

    Monomelic Amyotrophy / Hirayama Disease: Surgical Outcome in a Large Cohort of Indian Patients.

    • Seena Vengalil, Nupur Pruthi, Dhananjay Bhat, UpparAlok MohanAMDepartment of Neurosurgery, National Institute of Mental Health and Neuro Sciences, Bengaluru, Karnataka, India., Kiran Polavarapu, Veeramani Preethish-Kumar, Saraswati Nashi, Srinithya Rajesh, Nangamangalam Srinivasan Aswini, Bidyut Prava Behera, Govindan Dhanasekaran Vandhiyadevan, Chandrajit Prasad, Dipti Baskar, Karthik Kulanthaivelu, Akshaya Saravanan, Thennarasu Kandavel, Vikas Nishadham, Akshata Huddar, Gopikrishnan Unnikrishnan, Aneesha Thomas, Muddasu Suhasini Keerthipriya, SankaSai BhargavaSBDepartment of Neurology, National Institute of Mental Health and Neuro Sciences, Bengaluru, Karnataka, India., Nisha Manjunath, Ravi Kiran Valasani, Mainak Bardhan, and Atchayaram Nalini.
    • Department of Neurology, National Institute of Mental Health and Neuro Sciences, Bengaluru, Karnataka, India.
    • World Neurosurg. 2024 Mar 1; 183: e88e97e88-e97.

    BackgroundHirayama disease (HD) is a cervical compressive myelopathy. Anterior cervical discectomy and fusion (ACDF) is identified as the best surgical approach. We evaluated surgical outcomes and factors influencing ACDF in HD.MethodsBetween 2015 and 2019, 126 patients with HD underwent ACDF. Contrast magnetic resonance imaging of the cervical spine in full flexion was performed. Clinical examination and preoperative/postoperative assessment of hand function using Fugl-Meyer assessment, Jebsen-Taylor hand function test, and handheld dynamometry were performed at 3-monthly intervals for 1 year. Surgical outcomes were assessed as per the Odom criteria and Hirayama outcome questionnaire.ResultsAge at onset and duration of illness were 12-31 years (mean, 18 ± 2.7) and 1-96 months (32.7 ± 24.4), respectively. All patients had progressive weakness and wasting of the affected limb. Cord atrophy was seen in 97.1%, with epidural detachment and engorgement of the posterior epidural venous plexus in all. All patients underwent ACDF. Of these patients, 54% had an excellent/good outcome and 39% had a satisfactory outcome as per the Odom scale at last follow-up (mean, 44.9 ± 16.5 months) after surgery. Handheld dynamometry showed improvement from preoperative values to 1 year follow-up. Duration of illness and age at onset had a negative correlation and the preoperative Fugl-Meyer score had a positive correlation with improvement.ConclusionsACDF resulted in remarkable improvement or stabilization in neurologic deficits in many patients with HD. Because motor disability ensues over time, early surgical intervention during the progressive phase is advocated.Copyright © 2023 Elsevier Inc. All rights reserved.

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