Clinical and radiological profile of Hirayama disease: A flexion myelopathy due to tight cervical dural canal amenable to collar therapy
Clinical and radiological profile of Hirayama disease: A flexion myelopathy due to tight cervical dural canal amenable to collar therapy
Blog Article
Background: Hirayama disease (HD) is benign focal amyotrophy of the distal upper limbs, often misdiagnosed as motor neuron disease.Routine magnetic resonance imaging (MRI) is often reported normal.Objective: To study the clinicoradiological profile of hand wasting in young males.
Materials and Methods: Patients presenting with insidious-onset hand wasting from March 2008 to May 2011 were evaluated electrophysiologically.Cervical MRI in neutral position was done in 11 patients and flexion contrast imaging was done in 10 patients.Results: All patients were males less than 25 years of age, with median NippleToys age 23 years, except one patient who was 50 years old.
Duration of illness was 3 months to 3 years.All (100%) had oblique amyotrophy, four (36%) cold paresis, 10 (91%) minipolymyoclonus and three (27%) had fasciculations.Regional reflexes were variably absent.
Two patients (18%) had brisk reflexes of lower limbs with flexor plantars.Electromyography (EMG) showed chronic denervation in the C7-T1 myotomes.Neutral position MRI showed loss of cervical lordosis in 10/11 (91%), localized lower cervical cord atrophy in 9/11 (82%), asymmetric cord flattening in 11/11 (100%) and intramedullary hyperintensity in 2/11 (18%); flexion study showed loss of dural attachment, anterior displacement of dorsal dura, epidural flow voids in 9/10 (90%) and enhancing epidural crescent in 10/10 (100%).
Clinical profile, imaging and electrophysiological findings of the patient aged 50 years will be described in detail as presentation at this age is exceptional.Collar therapy slowed progression in most cases.Conclusion: Clinical features of HD corroborated well with electrophysiological diagnosis of anterior horn cell disease of lower cervical cord.
While Rolling Cases dynamic contrast MRI is characteristic, routine studies have a high predictive value for diagnosis.Prompt diagnosis is important to institute early collar therapy.