Monomelic amyotrophy (hirayama disease) with upper motor neuron signs: a case report

Annals of Rehabilitation Medicine
Seung Don YooYoo Jin Han

Abstract

Monomelic amyotrophy (MMA), also known as Hirayama disease, is a sporadic juvenile muscular atrophy in the distal upper extremities. This disorder rarely involves proximal upper extremities and presents minimal sensory symptoms with no upper motor neuron (UMN) signs. It is caused by anterior displacement of the posterior dural sac and compression of the cervical cord during neck flexion. An 18-year-old boy visited our clinic with a 5-year history of left upper extremity pain and slowly progressive weakness affecting the left shoulder. Atrophy was present in the left supraspinatus and infraspinatus. On neurological examination, positive UMN signs were evident in both upper and lower extremities. Electrodiagnostic study showed root lesion involving the fifth to seventh cervical segment of the cord with chronic and ongoing denervation in the fifth and sixth cervical segment innervated muscles. Cervical magnetic resonance imaging (MRI) showed asymmetric cord atrophy apparent in the left side and intramedullary high signal intensity along the fourth to sixth cervical vertebral levels. With neck flexion, cervical MRI revealed anterior displacement of posterior dural sac, which results in the cord compression of those segments. The me...Continue Reading

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Citations

Dec 17, 2015·Journal of the Neurological Sciences·Deepti VibhaS Vivekanandhan
Jan 18, 2017·Case Reports in Neurological Medicine·S Anuradha, Vanlalmalsawmdawngliana Fanai
Oct 5, 2018·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Anna RosliakovaMariya Zakharova
Feb 25, 2019·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Sezin Alpaydın BasloDilek Ataklı
Apr 8, 2020·Frontiers in Neurology·Annibale AntonioniEnrico Granieri
Jan 29, 2021·Rinshō shinkeigaku = Clinical neurology·Uka TsuzukiOsamu Kawakami
Jul 23, 2020·Indian Dermatology Online Journal·Dharmagat BhattaraiManphool Singhal

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