monomelic amyotrophy

Hirayama Disease (HD) is a focal motor neuron disorder generally affecting young adults with a male predominance who experience weakness and atrophy in distal upper extremity muscles in an asymmetric or unilateral pattern. Progression is insidious though significant weakness occurs during a progressive phase of the disease over 2-5 years. The long-term outcome of HD is not as well-known and, thus, this study presents self-reported outcomes from HD patients years after a diagnosis. Thirty HD patients reported quality of life (QOL) and other functional outcome measures after a mean of just over 11 years from diagnosis...

Hirayama disease is a rare neurological disorder, characterized by muscular atrophy of the distal upper extremities. The occurrence of spastic quadriparesis and autonomic dysfunction is rarely reported and has important perioperative considerations during cervical spine surgery for the treatment of this disorder. The role of the anesthesiologist is vital in the thorough assessment of the patient for the involvement of the pyramidal tract, autonomic dysfunction, gastroparesis, hyperreactive airway disease, and documentation of neurological deficits...

BACKGROUND AND PURPOSE: The purpose of this study was to describe the clinical presentation and features in electrodiagnostic and imaging investigations of young South Korean males diagnosed with Hirayama disease (HD). METHODS: We reviewed the electronic medical records of South Korean enlisted soldiers who were diagnosed with HD and discharged from military service during 2011-2021. We investigated the clinical characteristics and results of electrodiagnostic and magnetic resonance imaging (MRI) investigations...

Juvenile monomelic amyotrophy (JMA), also known as Hirayama's disease, is a rare cervical myelopathy that predominantly affects young Asian males. It is characterized by degeneration of anterior horn cells due to compression by the redundant dural sac. This study presents an atypical case of a 23-year-old Indian male who exhibited uncommon symptoms of JMA. The patient displayed progressive weakness and atrophy in the left forearm, including the usually spared brachioradialis muscle. Electrophysiological tests and MRI scans solidified the diagnosis of Hirayama's disease...

BACKGROUND: Hirayama disease (HD), is a cervical compressive myelopathy. Anterior cervical discectomy and fusion (ACDF) is identified as best surgical approach. We evaluated surgical outcome and factors influencing ACDF in HD. METHODS: Between 2015 and 2019, 126 HD patients underwent ACDF. Contrast MRI of cervical spine in full flexion was done. Clinical examination and pre / post operative assessment of hand function using Fugl-Meyer, Jebsen-Taylor hand function test and handheld dynamometry were done at 3 monthly intervals for one year...

Monomelic amyotrophy, also known as Hirayama disease, is a rare neurological disorder characterized by focal and latent onset of upper limb weakness and atrophy in the absence of sensory deficits, bulbar or pyramidal signs. It usually occurs in young patients. The disease usually begins unnoticeably and progresses slowly, and can manifest itself as unilateral or asymmetrical weakness, as well as atrophy of the distal upper limb. Sensory disturbances, reflex changes and signs of lesions of lower motor neurons are rare...

RATIONALE: Monomelic amyotrophy is a rare form of motor neuron disease in which the neurogenic atrophy is restricted to 1 limb, mostly the distal part of the arm. The disease most often occurs in Asia, especially in Japan and India, while in European countries, this disease is rarely recognized. Registration and publication of new cases of this disease aims to increase the awareness of clinicians about the existence of this disease in European countries, and with the aim of easier recognition and faster diagnosis of this essentially benign disorder...

BACKGROUND AND PURPOSE: All young males in South Korea must undergo a physical examination for their participation in military service. We aimed to determine the prevalence rate (PR) of various neuromuscular diseases in young South Korean males using the data of exempted patients and soldiers. METHODS: The number of males exempted based on specific items of physical examination corresponding to neuromuscular disease during 2011-2020 were obtained from the records of the Military Manpower Administration...

Hirayama disease, or brachial monomelic amyotrophy, is not a common neurological disease characterized by unilateral or asymmetric bilateral lower motor weakness of distal upper limbs. The basic pathophysiology is compression of the dural sac and spinal cord during flexion of the neck. A case of a 21-year-old male presented with chief complaints of tremors in both hands (right more than left) with gradually progressive weakness of the right hand and forearm. Electromyography (EMG), nerve conduction velocity (NCV), and magnetic resonance imaging (MRI) neck in flexion showed focal atrophy of lower cervical myotomes and confirmed the diagnosis of monomelic amyotrophy...

UNLABELLED: Hirayama disease (HD) is juvenile monomelic amyotrophy of the distal upper limb first described by Hirayama in 1959 AD. HD is a benign condition with chronic microcirculatory changes. The hallmark of HD is necrosis of the anterior horns of the distal cervical spine. MATERIALS AND METHODS: Eighteen patients were assessed for clinical and radiological Hirayama disease. Clinical criteria included insidious onset nonprogressive chronic upper limb weakness and atrophy in teens or early twenties without sensory deficits and coarse tremors...

BACKGROUND: Hirayama disease (HD) is a rare, nonfamilial neuromuscular disease causing cervical myelopathy and deformity, most commonly effecting pubertal Asian males. Patients whose nonoperative treatment fails and who cannot tolerate long-term cervical immobilization, experience relapse after arrest of symptoms, or present with severe features warrant surgical treatment. Here, the authors present an unusual case of HD that resulted in rapid progression of severe cervical kyphosis and discuss surgical management strategies...

Hirayama disease, also known as monomelic amyotrophy, usually affects young males who initially experience increasing muscle weakness and atrophy of the distal upper limb before experiencing a sudden plateauing of symptom progression a few years later. It is a form of cervical myelopathy characterized by self-limiting, asymmetrical lower motor weakness of the upper limbs affecting the hands and forearms. This condition is brought on by the cervical dural sac and spinal cord being abnormally displaced forward during neck flexion, which causes the anterior horn cells to atrophy...

Hirayama disease is a rare disease of the anterior horn motor neuron caused by compression of the cervical spinal cord when the neck is flexed. Cervical myelopathy may accompany the disease. It is characterized by symmetrical or asymmetrical muscle weakness and atrophy of muscles innervated by lower cervical and upper thoracic motor neurons. We recorded two male cases of Hirayama disease between the ages of 15 and 21 based on magnetic resonance imaging (MRI) features obtained from the cervical neutral state and from the flexion position which appeared in the right upper extremity...

BACKGROUND: Hirayama disease or juvenile-onset monomelic amyotrophy is a clinical syndrome that disproportionately affects young males. Standard of care revolves around conservative management, but some patients experience disease progression that may benefit from surgical intervention. METHODS: Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a systematic review of previous reports of surgical treatment for Hirayama disease was performed...

INTRODUCTION AND IMPORTANCE: Hirayama disease is a rare benign motor neuron disorder that involves a single upper limb. It affects the lower cervical myotomes and presents clinically by muscle wasting and weakness. CASE PRESENTATION: We report the case of a 17-year-old female who presented with a four weeks history of progressive weakness of the left upper limb. The blood investigations and image reported unremarkable findings. Patient improved on physiotherapy...

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Background  Monomelic amyotrophy (Hirayama disease) has been established to have accompanied biomechanical abnormalities such as flexion hypermobility and sagittal imbalance. Paraspinal muscles, the major contributor to cervical biomechanics, have, however, not been comprehensively evaluated in the disease. The objective of this study was to compare the morphology of the subaxial cervical paraspinal musculature in patients with and without Hirayama disease. Materials and Methods  A retrospective case-control study of 64 patients with Hirayama disease and 64 age- and sex-matched controls was performed ...

BACKGROUND: Monomelic amyotrophy (MMA) is a benign, rare, sporadic disorder of adolescent and young adults with male predominance, where neurogenic amyotrophy is restricted to an upper or lower limb. It is a variant of lower motor neuron disorder with insidious onset and slow progression for 2-4 years. Paucity of cohort studies as well as relative unawareness among physicians in eastern India stimulated us to do this work. MATERIAL AND METHODS: Prospective observational study involving 140 cases of MMA from 2012 to 2016, conducted at S...

No abstract text is available yet for this article.

Hirayama disease is a motor neuron disease predominantly affecting adolescent males. The identifying feature of Hirayama disease is unilateral forearm and intrinsic muscle weakness that spares the brachioradialis, termed "oblique atrophy." Hirayama disease progresses slowly over several years, followed by an abrupt arrest. The pathognomonic finding is the anterior displacement of the cervical spinal cord with the detachment of the posterior dura. Systematic clinical evaluation and appropriate diagnostic studies are crucial to rule out a variety of compressive, immune-mediated, and genetic disorders...