Hirayama Disease: An Important Cause of Focal Hand Weakness in Young Adults

Patients with progressive hand weakness may be seen in ambulatory medical clinics or in emergency rooms due to direct effects on activities of daily living or inadvertent injury associated with overuse or attempts to maintain normal function. It is important to recognize potential cause(s) and perform appropriate diagnostic tests and referrals that aid guide appropriate treatment that may lead to good outcomes. Hirayama disease is an underrecognized disorder in young adults due to an asymmetric growth-associated cervical spinal cord compression injury. Awareness of this disorder by internists, emergency room physicians, and radiologists would prevent unnecessary tests and interventions that may contribute to disease progression by delaying appropriate treatments or treating inappropriately, with consequential effects on outcomes. In this article, we describe 3 Hirayama disease cases from a single tertiary care institution and demonstrate how delayed diagnosis affected outcomes in 2 patients and early recognition facilitated improved outcomes in a patient.