Clinical and radiological profile of Hirayama disease: A flexion myelopathy due to tight cervical dural canal amenable to collar therapy.

Annals of Indian Academy of Neurology
Kaukab Maqbool HassanAtul Jha

Abstract

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. To study the clinicoradiological profile of hand wasting in young males. 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. All patients were males less than 25 years of age, with median 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 displaceme...Continue Reading

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Citations

Nov 30, 2013·Journal of Neurosurgery. Spine·Igor ParedesJuan José Rivas
Dec 17, 2015·Journal of the Neurological Sciences·Satish KhadilkarChetan Chaudhari
Mar 18, 2016·Journal of Medical Case Reports·Eman Al-GhawiMohamed Binfalah
Apr 7, 2016·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Veeramani Preethish-KumarAtchayaram Nalini
Mar 15, 2015·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Emma FosterAron Hill
Mar 20, 2018·PloS One·Candice Brinkmeyer-LangfordJoe N Kornegay
Apr 4, 2018·Continuum : Lifelong Learning in Neurology·Amro Maher Stino, Samantha J LoRusso
Oct 6, 2015·Asian Spine Journal·Cecilia QuarracinoAndrés M Villa
Jan 18, 2017·Case Reports in Neurological Medicine·S Anuradha, Vanlalmalsawmdawngliana Fanai
Mar 8, 2021·Neurophysiologie clinique = Clinical neurophysiology·Chaojun ZhengJianyuan Jiang

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