HIRAYAMA DISEASE: ABOUT TWO CASES

OBJECTIVE:

Report of two clinical cases of Hirayama Disease, a rare pediatric myelopathy associated with cervical flexion.

MATERIAL AND METHOD:

Retrospective and descriptive study of the clinical history of two patients with Hirayama Disease

RESULTS:

Patient 1: Seventeen-year-old patient presenting 14-month history of progressive muscle weakness of the right upper limb. Physical examination revealed forearm and hand muscle weakness on flexion-extension, associated with muscle wasting.

Patient 2: Sixteen-year-old presenting involuntary movements in both hands on neck flexion associated with associated paresthesia. Physical examination revealed mild left biceps atrophy and bilateral wasting of hypothenar eminence muscles.

In both cases, diagnosis was confirmed with functional MRI, which showed forward displacement of the spinal cord and widening of the posterior epidural space on neck flexion (Figure 1 and 2).

CONCLUSION:

Recognition of this pathology is key, to include functional MRI imaging of the cervical spine in patient workup, with flexion-extension of the neck, to confirm diagnosis. Reaching an accurate diagnosis is important both for timely treatment and to avoid clinical progression.
Keywords: Hirayama disease, myelopathy, functional MRI.