Early-onset Hirayama disease in a female

SAGE Open Med Case Rep. 2017 Jan 1:5:2050313X16686710. doi: 10.1177/2050313X16686710. eCollection 2017.

Abstract

Objectives: Hirayama disease is a rare myelopathy, occurring predominantly in males with onset in the teens.

Methods and results: Here, we report a young female patient who developed the first signs of Hirayama disease at 10.5 years of age. Prior to onset, she had experienced a growth spurt and grew about 8 cm. The disease progressed over 3 years and the typical clinical, electrophysiological, and neuroimaging signs of Hirayama disease were found. After this period and achievement of her final height, no further progression was noticed.

Conclusions: This case highlights that pediatric neurologists should be aware of Hirayama disease, which can also occur in girls in early adolescence.

Keywords: Hirayama disease; magnetic resonance imaging; motor neuron disease; myelopathy; nerve conduction.

Publication types

  • Case Reports