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Hirayama Disease: Case report of a rare case in Brazil

Context: Hiravama’s disease (HD) is a rare neurological condition described by keizo Hirayama in 1959, in which growth of the content of the spinal canal displaces the posterior dural sac previously when flexing the neck at the level of C7 and T1. Thus, it generates ischemic damage in the cells of the anterior spine. In this context, we present a case report about the disease. Case report: Male, 13 years old, complaining of difficulty in grasping his right hand for 5 months and atrophy in his right upper limb for 1 month. On physical examination, the neurological changes found were: Decreased strength of the right upper limb distally, the deep hypoactive reflexes in both upper limbs. Magnetic resonance imaging of the flexed cervical spine showed stenosis of the C5 to T1 spinal canal, flow-voids prominence in the posterior epidural space (suggestive of venous engorgement), tapering and alteration of T2 signal in the C5-C6 medulla. These findings and along with the clinical history confirmed the diagnosis of HD. Conclusion: HD is a rare disease that needs to be known. Thus, the present study expands the database about the disease, which if diagnosed early, improves the patient’s prognosis.