Having been tested for two months they concluded that is hirayama disease. Hirayama disease is a form of muscular dystrophy seen in 2nd3rd decade. The importance of flexion mri in hirayama disease with. Hirayama disease occurs mainly in young males between the ages of 15 and 25 years.
It is considered a benign motor neuron disorder with a stationary stage after a progressive course. In 2015 i have consulted the doctors at nimhans bangalore. Severe cervical flexion myelopathy with long tract signs. Introduction hirayama disease hd, also known as monomelic amyotrophy mma was first reported by hirayama et al. Participation eligibility participant eligibility includes age, gender, type and stage of disease, and previous treatments or health concerns. Hirayama disease is a benign focal amyotrophy of the distal upper limbs involving c7, c8, and t1 segmental myotomes with sparing of the brachioradialis and proximal muscles of the upper limb innervated by c56 myotomes. Hirayama disease, also known as monomelic amyotrophy or benign focal amyotrophy, is a rare disease affecting primarily males of southeast asian decent in their second to third decades.
Effect of neck flexion on f wave, somatosensory evoked. According to the first published autopsy report, 2 the cervical spinal cord lesions were not typical of a motor neuron disease, but rather suggested ischaemic necrosis in. Mar 14, 2018 hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord. Its symptoms usually appear about two years after adolescent growth spurt and is significantly more common in males average age of onset, 15 to 25yearold. Cervical spine mr imaging findings of patients with. Methods and results here, we report a young female patient who developed the first signs. The objective of the present study was to study the utility of mr imaging in young patients presenting with weakness and wasting of the. I have been suffering from hirayama disease since 2014. Eight hd patients and seven matched controls were subjected to. The objective of the present study was to study the utility of mr imaging in young patients presenting with weakness and wasting of the distal upper. It differs from the other motor neuron diseases because of its nonprogressive nature and pathological findings of focal ischemic changes in the anterior horn. We report clinical and magnetic resonance imaging findings in nine patients with hirayamas disease.
Clinical features of hirayama disease in mainland china. He was diagnosed with hirayama disease 9 years ago, while there. Magnetic resonance imaging in juvenile asymmetric segmental spinal muscular atrophy. Clinical dilemma solved by imaging shalabh jain, 1 siddharth yadav, 2 swarna gupta, 1 and ritu gupta 3 1 d e p a r t m e n to fr a d i o d i a g n o s i s,v m m ca n ds. Hirayama disease hd is a rare disease affecting primarily young men in the second to third decades of life.
When it is seen, a flexion mr study is warranted to prove this diagnosis. This study therefore evaluates the effect of neck flexion in hd using somatosensory evoked potentials seps, f waves, and magnetic resonance imaging mri. Amyotrophic lateral sclerosis is very unlikely to happen in your sons age, hirayama disease is a condition that may be diagnosed at that age, however, i need to ask you for more information about your sons condition such detailed description of symptoms and signs duration, distribution, relieving and worsening factors etc. Hirayamas disease hd, also known as monomelicamyotrophy of distal upper limb, is characterized by pure distal motor atrophy of the upper limbs, affecting young men, in the muscles that are innervated by c7, c8 and t1 segments 14. If an internal link intending to refer to a specific person led you to this page, you may wish to change that link by adding the persons given names to the link. Aug 06, 2011 hirayama disease occurs mainly in young males between the ages of 15 and 25 years.
Clinical and radiological profile of hirayama disease. What if everything you learned about cholesterol was a lie. Pure motor focal amyotrophy in distribution of c7,8,t1 spinal segments sporadic, men, second and early third decade. Hirayamas disease hd is the eponym which continues to be used to identify a rare condition frequently reported in asia, most in japan and india, and rarely referred among westerners.
Dynamic flexion mri showed the cervical cord to be displaced anteriorly and compressed over the posterior surface of the c 56 vertebral bodies with a prominent crescent shaped epidural mass with flow voids c. The forward displacement of the posterior dura of the lower cervical dural canal during neck flexion has been postulated to lead to lower cervical cord. Hirayama disease is a monomyelic variant of motor neuron disease mnd and has distinctive features of male predominance, asymmetric involvement of upper extremities with a self limiting course. Hirayamas disease affects mainly younger males who are between the ages of fifteen and twentyfive years of age, mostly in the nations of india and japan. Hirayama disease, also known as juvenile muscular atrophy of distal upper extremity, is a benign, uncommon disease predominantly seen in young men of southeast asian descent1 it often presents with insidious onset of muscular atrophy of the hands and forearms. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Hirayama disease hd, also known as juvenile muscular atrophy of the distal upper extremity, was first reported by hirayama et al. Hirayamas disease is a benign juvenile form of focal amyotrophy affecting the upper limbs. It differs from the other motor neuron diseases because of its nonprogressive nature and pathological findings of focal ischemic changes in the anterior horn cells of the localized lower. Although the cause of cervical myelopathy remains unclear, neuropathologic and neuroradiologic. Previous studies have suggested that the disorder is a neck flexion induced cervical myelopathy. However, utility of dti in evaluating the integrity of the spinal. Specifically, monomelic amyotrophy causes weakness and loss of muscle mass in the arms. It is characterized by progressive muscular weakness and atrophy of unilateral or asymmetrically bilateral distal upper limbs.
It is primarily seen in young males of indian or asian descent. Imaging features in hirayama disease, sonwalkar ha, shah rs. Nov 14, 2012 hirayama disease mri an 18 yo male with 4 year history of slowly progressive weakness of forearms and hand marked on right side. Hirayama disease juvenile muscular atrophy of distal upper extremity is a cervical myelopathy. Hirayama disease, also known as juvenile muscular atrophy of distal upper extremity, is a benign, uncommon disease predominantly seen in young men of southeast asian descent1 it often presents with insidious onset of muscular atrophy of the hands and forearms, and generally spares the brachioradialis1. Monomelic amyotrophy mma, is a rare motor neuron disease first described in 1959 in japan. Hirayama s disease hd is the eponym which continues to be used to identify a rare condition frequently reported in asia, most in japan and india, and rarely referred among westerners. Objectives hirayama disease is a rare myelopathy, occurring predominantly in males with onset in the teens.
Jul 05, 2017 the symptoms of hirayama s disease as a whole, like the muscular atrophy associated with the disease, progress for a period of time, reaching a plateau, and then remain stable for many years after that point. Oct 16, 2014 hirayama disease hd, amyotrophic lateral sclerosis als or cervical spondylotic amyotrophy csa may result in atrophy of intrinsic hand and forearm muscles. The most widely accepted hypothesis on the aetiopathogenesis of hirayama disease postulates that the disease may arise from discrepancies between the growth of the spinal column, spinal cord, and dura mater. Contrast mri may be done to demonstrate findings better but is not essential. Mar 01, 2004 forward shifting of posterior dural sac during flexion cervical magnetic resonance imaging in hirayama disease. The dynamic flexion mri findings confirmed the clinical diagnosis of hirayama disease. Imaging features in hirayama disease sonwalkar ha, shah rs. Dynamic post contrast mri evaluation of cervicothoracic spine is a helpful method in arriving at the correct diagnosis of hirayama disease and should be an essential part of the protocol in cases with high suspicion of motor neuron disease. Hirayama is a rare disease of the young where early diagnosis and treatment prevents the progression of disease. Hirayama disease was first defined in japanese in 1959, and in english in 1963, 1 as unilateral focal amyotrophy of one of the upper limbs. Neurologic examination revealed atrophic changes in thenar, hypothenar muscles, interossei of the hands, muscles of forearm more on right side. Hirayama disease with proximal involvement hirayama disease is a slowly progressing benign motor neuron disease that affects the distal upper limb. Hirayama s disease affects mainly younger males who are between the ages of fifteen and twentyfive years of age, mostly in the nations of india and japan. The purpose of this study is to determine the longterm progression and outcomes in patients who have the diagnosis of hirayama disease.
It is thought to be due to disproportionate growth between canal contents and bony spinal canal resulting in a lax dura in extension and vice versa. An initial study on normal subjects compared to patients with hirayama disease european journal of radiology, vol. Predominantly affecting male adolescents, it is characterized by progressive muscular weakness and atrophy of distal upper limbs, followed by spontaneous arrest within several years. This benign focal cervical poliopathy is believed to be caused by forward displacement of the posterior cervical dural sac. We report a case of an 18yearmale painter, who presented with gradually progressive, symmetrical bilateral weakness of hands and.
Hirayama disease juvenile muscular atrophy of distal upper extremity is a cervical myelopathy predominantly affecting adolescent males. It is important to differentiate these diseases because hd is caused by a benign focal lesion that. Nov 03, 2015 amyotrophic lateral sclerosis is very unlikely to happen in your sons age, hirayama disease is a condition that may be diagnosed at that age, however, i need to ask you for more information about your sons condition such detailed description of symptoms and signs duration, distribution, relieving and worsening factors etc. It is usually sporadic, it has an insidious onset and there is a slow progression followed by stabilization in 24 years. Cervical imaging of seven patients revealed spinal cord changes consisting of focal atrophy and foci. A high index of suspicion is required when imaging the spine in neutral position and can be confirmed with dynamic mri in neck flexion and use of new sequences like t2space which should be an essential part of mri protocol. A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of hirayama disease. Flexion myelopathy is one of the suggested mechanism for hirayama disease hd but simultaneous radiological and neurophysiological evaluation is lacking. Predominant proximal upper extremity involvement in. Hirayama disease is a nonprogressive asymmetric juvenile muscular atrophy involving c7t1 myotomes leading to weakness and atrophy of intrinsic muscles of hand and forearm with relative sparing of the brachioradialis muscle. Flexion mri magnetic resonance imaging forms the main stay for diagnosis of this condition. Imaging features in hirayama disease hemant a sonwalkar 1, rakesh s shah 2, firosh k khan 2, arun k gupta 1, narendra k bodhey 1, surjith vottath 1, sukalyan purkayastha 1 1 department of imaging sciences and interventional radiology, sree chitra tirunal institute for medical sciences and technology, trivandrum, kerala, india 2 department of neurology, sree chitra tirunal institute for medical. Hirayama disease in unilateral and bilateral forms3 case.
Mr diagnosis article pdf available in american journal of neuroradiology 192. Nonprogressive juvenile spinal muscular atrophy of the. Predominant proximal upper extremity involvement in hirayama. Hirayama disease, also termed nonprogressive juvenile spinal muscular atrophy of the distal upper limbs, is a kind of cervical myelopathy related to flexion movements of the neck 16. Most studies of hd have been conducted in asia, particularly japan. Feb 08, 2018 monomelic amyotrophy mma is a rare disease that causes muscle weakness in the upper extremities. The clinical features include insidious onset, predominantly unilateral upper extremity weakness and atrophy, cold paresis, and no sensory or pyramidal tract involvement. With increasing dispersion of these populations this condition is increasingly being encountered internationally. Cervical spine mr imaging findings of patients with hirayama disease in north america. The symptoms of hirayamas disease as a whole, like the muscular atrophy associated with the disease, progress for a period of time, reaching a plateau, and then remain stable for many years after that point. Muscular weakness and wasting of hand and forearm oblique amyotrophy. Hirayama disease mri an 18 yo male with 4 year history of slowly progressive weakness of forearms and hand marked on right side. Mr imaging of the cervical cord in juvenile amyotrophy of distal upper extremity. Hirayama disease, also known as monomelic amyotrophy mma, is a rare cervical myelopathy that manifests itself as a selflimited, asymmetrical, slowly progressive atrophic weakness of the forearms and hands predominantly in young males.
This work is licensed under a volum creative commons. Hirayama disease, also termed nonprogressive juvenile spinal muscular atrophy of the distal upper limbs, is a type of cervical myelopathy related to flexion movements of the neck. A 29yearold man visited the hospital with a 1year history of weakened left proximal upper limb. Role of dynamic mri study in hirayama disease parihar a. Sun chi, wang hongli, jiang jianyuan department of orthopaedics, huashan hospital, fudan university, shanghai 200040, china. Consequently, hd is often misdiagnosed as als or csa. Hirayamas disease hd, is a benign, selflimited, motor neuron disease, characterized by asymmetric weakness and atrophy of one or both distal upper extremities. The incidence of hd is low, and it is rarely encountered in the clinical setting. Magnetic resonance imaging of muscle injury and atrophy in glycolytic myopathies. Imaging features in hirayama disease, sonwalkar ha, shah. Hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord. Monomelic amyotrophy mma is a rare disease that causes muscle weakness in the upper extremities. Hirayama disease, motor neuron disease, electromyography, late onset.
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