{"id":"84d113aa-9d91-472c-abf3-324e0caa6325","slug":"somatosensory-evoked-potentials-and-hirayama-disease","title":"Somatosensory evoked potentials and Hirayama disease","authors":["Adeel Shakil Zubair","Brian Crum"],"abstract":"Background: Hirayama disease (HD) is characterized by slowly progressive, asymmetric distal amyotrophy in the upper limbs, typically presenting in juvenile years with muscle weakness and atrophy. The etiology remains unclear, but one theory suggests cervical dural canal compression during neck flexion. Somatosensory evoked potentials (SSEPs) have been considered as a potential electrophysiologic measure for this condition. Summary: In our report, we present a series of six male patients diagnosed with HD between 2004 and 2012, all of whom underwent ulnar SSEP studies that returned normal results. This adds to the evidence that SSEPs may not serve as a reliable electrophysiologic marker for HD. Previous studies have shown variability in SSEP results, indicating the need for further research to explore the utility of SSEPs in this patient population. Conclusion: The findings from our cohort support the notion that SSEPs may not be a definitive electrophysiologic marker for Hirayama disease. The variability in published data suggests the possibility of different phenotypes of HD, emphasizing the necessity for larger studies to better understand the condition. Keywords: Hirayama disease, somatosensory evoked potentials, SSEP, upper limb amyotrophy, cervical dural canal, electrophysiologic marker","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/placeholders/specialty/neuroanatomy-and-neurophysiology.png","publishDate":"2021-04-26T00:00:00.000Z","doi":"10.25259/SNI_88_2021","categories":["Neuroanatomy and Neurophysiology","Letter to the Editor"],"fullTextUrl":"https://surgicalneurologyint.com/api/articles/84d113aa-9d91-472c-abf3-324e0caa6325/pdf"}