Session Time: 12:30pm-2:00pm
Location: Research Hub - Kiosk 1
Disclosures: Matthew Sherrier, MD: Nothing to disclose
Case Description: A healthy 16-year-old male was playing hockey when he suddenly experienced rapid onset of weakness in his left hand, which spread to his right hand over the course of a few hours without numbness, tingling, or radicular pain. There was no recent trauma or unusual event prior to symptom onset. In the ED, imaging showed a small syrinx at C6-C7, which neurosurgery did not feel corresponded to his degree of symptomatology. No fractures or instability were noted and he was discharged. His hand weakness persisted, prompting further evaluation.
Setting: Outpatient EMG clinic
Patient: 16-year-old male with no significant past medical or family history.
Assessment/Results: Repeat MRI revealed bilateral anterior horn gray matter T2 hyperdensities at C3-C4 and C6-C7 and a deep ventral cleft vs partial diastematomyelia at C6-C7. Rheumatologic and infectious disease evaluation was unremarkable. NCS/EMG demonstrated evidence of bilateral active denervation in muscles innervated by the C8-T1 nerve roots. Sensory responses were unremarkable. Based on these findings in the absence of trauma, the diagnosis was felt to be Hirayama Disease.
Discussion: Less than 1000 cases of Hirayama Disease have been reported. Males in their teens or twenties are primarily affected. Clinical signs include muscular weakness of the hands and forearms with preservation of sensory function. Diagnosis is based on clinical suspicion correlated with MRI findings. EMG shows denervation of affected muscles. In the majority of cases, the disease stabilizes within 5 years of symptom onset. The patient’s congenital deep ventral cleft vs partial diastematomyelia in combination with years of sub-acute repetitive trauma from hockey predisposed him to local ischemia in his anterior horn cells, resulting in development of Hirayama Disease.
Conclusion: The patient experienced clinical improvement in hand strength but remains significantly below his baseline strength. He was given a lifetime ban on participation in contact sports given his spinal anatomic variant.
Level of Evidence: Level V
To cite this abstract in AMA style:Sherrier M, Miknevich M. A Curious Case of Acute Onset Bilateral Hand Weakness in a Youth Hockey Player: A Case Report [abstract]. PM R. 2019; 11(S2)(suppl 2). https://pmrjabstracts.org/abstract/a-curious-case-of-acute-onset-bilateral-hand-weakness-in-a-youth-hockey-player-a-case-report/. Accessed December 9, 2023.
« Back to AAPM&R Annual Assembly 2019
PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/a-curious-case-of-acute-onset-bilateral-hand-weakness-in-a-youth-hockey-player-a-case-report/