Gomathy, Saranya B;
Priyanka, Yamini;
Garg, Ajay;
Macken, William L;
Agarwal, Ayush;
Ahmed, Tanveer;
Bhatia, Rohit;
... Vishnu, Venugopalan Y; + view all
(2024)
Phenotypic, Electrophysiologic, and Imaging Spectrum of Hirayama Disease from Northern India.
Annals of Indian Academy of Neurology
, 27
(5)
pp. 524-529.
10.4103/aian.aian_348_24.
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Abstract
Background and Objectives: Cervical flexion-induced myelopathy, also known as Hirayama disease (HD), is a lower motor neuron disorder seen mainly in adolescents and young adults, affecting the C7–T1 myotomes, presenting as asymmetric weakness with wasting of one or both the distal upper limbs. We aimed to describe the clinical features, electrophysiology, and radiologic features of HD in a tertiary care institute in northern India. // Methods: One hundred and forty patients between 2017 and 2022 with clinical and imaging features consistent with HD were retrospectively reviewed from the All India Institute of Medical Sciences-Comprehensive Neuromuscular Diseases center database. // Results: Majority were males with the mean age of onset of illness being 17.8 years. The median duration of the symptoms was 3 (1.5–4) years. Sixty-nine (49%) patients had unilateral involvement, and the disease was actively progressing in 88 (63%) patients at presentation. Two families had history of HD in two (1.4%) siblings. Electromyography showed abnormal findings in the clinically involved limb in all the patients and in the clinically uninvolved limb in 17/50 (34%) patients. Flexion magnetic resonance imaging (MRI) demonstrated forward dural displacement in 134 (96%) patients and asymmetric cord flattening in 124 (88.5%) patients. Disability was graded as mild, moderate, and severe; 12 (13%) had severe disability. The majority were managed conservatively, and four underwent surgery for HD. // Conclusion: A high index of suspicion of HD needs to be kept in a young male presenting with distal upper limb weakness and atrophy. Dynamic MRI of the cervical spine in young adults presenting with hand wasting is inevitable. This disease needs to be managed aggressively and early to prevent serious dysfunction and loss of productivity.
| Type: | Article |
|---|---|
| Title: | Phenotypic, Electrophysiologic, and Imaging Spectrum of Hirayama Disease from Northern India |
| Location: | India |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.4103/aian.aian_348_24 |
| Publisher version: | http://dx.doi.org/10.4103/aian.aian_348_24 |
| Language: | English |
| Additional information: | This article is available under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike License (CC BY-NC-SA), https://creativecommons.org/licenses/by-nc-sa/4.0/deed.en, which permits non-commercial use, distribution and reproduction in any medium, provided the original work is properly cited. |
| Keywords: | Hirayama disease; dynamic MRI; electromyography; cervical collar |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10199860 |
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