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Original Article
Online Published: 11 Jan 2025
 


Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India

Subrata Halder, Ayon Ghorai, Shankar Prasad Nandi, Tanvir Ahmed, Pinaki Maiti, Gouranga Prasad Mondal, Ramesh Bhattacharyya.


Abstract
Hirayama disease (HD) is a focal Anterior Horn Cells (AHC) disease due to dynamic compression causing upper limb atrophy mainly young male. This is an observational retrospective study of 42 cases who attended Neurology Department from 2021 to 2023. In our study all are male, aged 12 to 42 years, majorly below 25 years. Most cases presented late on an average of 13.7 months. Significant number of bilateral presentations were seen in our study (47%). It is right hand predominant having both unilateral and bilateral cases. “Reverse split hand syndrome” observed in 90% cases and forearm weakness and atrophy significantly over medial aspect sparing Brachioradialis (93%). Polyminimyoclonus is a significant finding noted in 88% cases, Deep Tendon Reflexes (DTRs) are usually preserved except 14% cases in which triceps jerk is reduced. Nerve Conduction Study (NCS) shows > 50% reduction of Ulnar CMAP in affected side where Median Compound Muscle Action Potential (CMAP) is relatively preserved and Ulnar/Median CMAP ratio <0.5 but velocity and Sensory Nerve Action Potential (SNAP) are preserved. On Electromyography, neurogenic pattern was seen in all cases in C8-T1, 26(81%) C7, 4(12.5%) C5-6. MRI Cervical spine shows loss of lordosis, cord atrophy, focal demyelination. Flexion MRI detected anterior buckling of the posterior cervical dura with flow voids that not only restrict to cervical spine but may extend extensively. HD can be diagnosed clinically with the help of electrophysiology which is a cost-effective way of diagnosis and radiology only helps further confirmation.

Key words: Compound muscle action potential, Electromyography, Hirayama dis-ease, Nerve conduction study


 
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How to Cite this Article
Pubmed Style

Halder S, Ghorai A, Nandi SP, Ahmed T, Maiti P, Mondal GP, Bhattacharyya R. Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India. J Med Allied Sci. 2024; 14(2): 65-71. doi:10.5455/jmas.207652

Web Style

Halder S, Ghorai A, Nandi SP, Ahmed T, Maiti P, Mondal GP, Bhattacharyya R. Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India. https://jmas.in/?mno=207652 [Access: January 11, 2025]. doi:10.5455/jmas.207652

AMA (American Medical Association) Style

Halder S, Ghorai A, Nandi SP, Ahmed T, Maiti P, Mondal GP, Bhattacharyya R. Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India. J Med Allied Sci. 2024; 14(2): 65-71. doi:10.5455/jmas.207652


Vancouver/ICMJE Style

Halder S, Ghorai A, Nandi SP, Ahmed T, Maiti P, Mondal GP, Bhattacharyya R. Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India. J Med Allied Sci. (2024), [cited January 11, 2025]; 14(2): 65-71. doi:10.5455/jmas.207652


Harvard Style

Halder, S., Ghorai, . A., Nandi, . S. P., Ahmed, . T., Maiti, . P., Mondal, . G. P. & Bhattacharyya, . R. (2024) Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India. J Med Allied Sci, 14 (2), 65-71. doi:10.5455/jmas.207652


Turabian Style

Halder, Subrata, Ayon Ghorai, Shankar Prasad Nandi, Tanvir Ahmed, Pinaki Maiti, Gouranga Prasad Mondal, and Ramesh Bhattacharyya. 2024. Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India. Journal of Medical and Allied Sciences, 14 (2), 65-71. doi:10.5455/jmas.207652


Chicago Style

Halder, Subrata, Ayon Ghorai, Shankar Prasad Nandi, Tanvir Ahmed, Pinaki Maiti, Gouranga Prasad Mondal, and Ramesh Bhattacharyya. "Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India." Journal of Medical and Allied Sciences 14 (2024), 65-71. doi:10.5455/jmas.207652


MLA (The Modern Language Association) Style

Halder, Subrata, Ayon Ghorai, Shankar Prasad Nandi, Tanvir Ahmed, Pinaki Maiti, Gouranga Prasad Mondal, and Ramesh Bhattacharyya. "Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India." Journal of Medical and Allied Sciences 14.2 (2024), 65-71. Print. doi:10.5455/jmas.207652


APA (American Psychological Association) Style

Halder, S., Ghorai, . A., Nandi, . S. P., Ahmed, . T., Maiti, . P., Mondal, . G. P. & Bhattacharyya, . R. (2024) Correlation of clinical, electrophysiological and radiological features of Hirayama disease in a tertiary care hospital in Eastern India. Journal of Medical and Allied Sciences, 14 (2), 65-71. doi:10.5455/jmas.207652


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