Hirayama disease hd or juvenile muscular atrophy of distal upper extremity jmadue is a rare myelopathy, occurring predominantly in males. Marked lower cervical cord atrophy which is confirmed on post contrast flexion imaging to be due to hirayama disease. Hirayama s disease in 2003 and have now been going for 5 years or so. Intraspinal lesions eg, syringomyelia, syringobulbia, or tumor can present with symptoms that can mimic hirayama disease. 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.
Hirayama disease, also known as monomelic amyotrophy mma, is a rare cervical myelopathy that manifests itself as a selflimited. 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 clinical study of hirayama disease in taiwan, muscle and. Predominantly affecting male adolescents, it is characterized by progressive muscular weakness and atrophy of distal upper limbs, followed by spontaneous arrest within several years. 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. Additionally structural lesions, such as a ganglion cyst, selectively compressing the recurrent motor branch should be considered. Hirayama disease, also known as monomelic amyotrophy, is a relatively benign motor neuron disease, characterized by insidious muscle wasting and weakness, unilateral or asymmetric restricted to the distal upper limb, followed by a quiescent phase after onset in the second to third decades. Strict quarantine measures are used to prevent the disease from spreading to areas where it does not yet occur. 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. 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. These cases are usually diagnosed after carefully excluding other conditions. Jul 12, 2011 hirayama disease, also known as monomelic amyotrophy, is a relatively benign motor neuron disease, characterized by insidious muscle wasting and weakness, unilateral or asymmetric restricted to the distal upper limb, followed by a quiescent phase after onset in the second to third decades. Cervical laminectomy and micro resection of the posterior. Nov 06, 2014 what if everything you learned about cholesterol was a lie.
Predominantly affecting male adolescents, it is characterized by. Monomelic amyotrophy mma, is a rare motor neuron disease first described in 1959 in japan. It was identified for the first time in 1959 by hirayama et al. The full text of this article is available in pdf format. Typing is manageable, but i find small everyday tasks a pain. Plant diseases caused by bacteria narratives should be produced in covered, insectproof screen houses.
With increasing dispersion of these populations this condition is increasingly being encountered internationally. Hirayama disease article pdf available in ftr turkiye fiziksel tip ve rehabilitasyon dergisi 6. Hirayama disease in unilateral and bilateral forms3 case. It is characterized by progressive muscular weakness and atrophy of unilateral or asymmetrically bilateral distal upper limbs. Clinical and radiological profile of hirayama disease. The documents contained in this web site are presented for information purposes only. Lower motor neurons are cells that help communicate information from the brain to the muscles that are involved in movement skeletal muscles. In this chapter, we will discuss the smas related to mutations of the survival motor neuron smn gene, the nonsmn infantile forms of the disease, the rare childhood bulbar forms of motor neuron disease mnd, hirayama disease, kennedy disease, the distal smas, the scapuloperoneal forms of sma, and the uncommon sma phenotypes that occur in association with multisystem disorders tables 82. What if everything you learned about cholesterol was a lie. Assessing loneliness and other types of emotional distress among practicing physicians. Amyotrophic lateral sclerosis genetic and rare diseases. A mechanism of juvenile muscular atrophy localized in the hand and forearm hirayamas disease. Drinking water quality risk management risk management. Cervical spine mr imaging findings of patients with hirayama.
We report a case of an 18yearmale painter, who presented with gradually progressive, symmetrical bilateral weakness of hands and. Hirayama disease is a benign, selflimiting cervical myelopathy first brought to attention by hirayama in 1959. Mar 21, 2017 hirayama disease is a benign, selflimiting cervical myelopathy first brought to attention by hirayama in 1959. This work is licensed under a volum creative commons. T2weighted cervical spine mri of a patient with hirayama disease showing focal cord hyperintensity at c5c6 level. Monomelic amyotrophy mma, also known as hirayama disease, sobue disease, juvenile nonprogressive amyotrophy and juvenile asymmetric segmental spinal muscular atrophy jassma is an untreatable, focal motor neuron disease that primarily affects young 1525 year old males in india and japan. Hirayama disease is a slowly progressing benign motor neuron disease that affects the distal upper limb. T2weighted cervical spine mri of the same patient during neck flexion showing anterior displacement of the posterior dural wall with flattening and compression of the cord against the bodies of the vertebrae with prominent dorsal. May 11, 2017 hirayama disease, a type of cervical flexion myelopathy, is a rare neurological disease characterized by muscular atrophy of the forearms and hands. Monomelic amyotrophy mma is a rare disease that causes muscle weakness in the upper extremities. Hirayama s disease, also referred to as monomelic amyotrophy, mms, sobue disease, or juvenile nonprogressive amyotrophy, is an untreatable, focal, lower motor neuron disease. This clinical picture was called as juvenile spinal muscular atrophy, juvenile.
Lower motor neurons are cells that help communicate information from the brain to the muscles that are. This work is licensed under a volum creative commons attribution. Hirayamas disease, also referred to as monomelic amyotrophy, mms, sobue disease, or juvenile nonprogressive amyotrophy, is an untreatable, focal, lower motor neuron disease. He was diagnosed with hirayama disease 9 years ago, while there was no further progression of the muscle weakness afterward. Global burden of disease global burden of disease gbd analysis provides comprehensive and comparable assessment of mortality and loss of health due to diseases, injuries and risk factors for all regions of the world. Imaging features in hirayama disease sonwalkar ha, shah rs. 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. Feb 20, 2018 t2weighted cervical spine mri of a patient with hirayama disease showing focal cord hyperintensity at c5c6 level. During the past half century, researches have established this disease as a new entity that differs from motor neuron disease because of spinal cord compression by the posterior dural sac during neck flexion. Hirayama disease is a rare neurological entity which can present in any setting emergency departments included. Jan 20, 2015 hirayama k 1991 nonprogressive juvenile spinal muscular atrophy of the distal upper limb hirayamas disease. Hirayamas disease hd, is a benign, selflimited, motor neuron disease, characterized by asymmetric weakness and atrophy of one or both distal upper extremities.
Hirayama disease, also known as juvenile nonprogressive cervical amyotrophy or monomelic amyotrophy is a rare focal motor neuron disease that primarily affects young asian males 1525 years old. 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. Amyotrophic lateral sclerosis als, also referred to as lou gehrigs disease, is a progressive motor neuron disease which leads to problems with muscle control and movement. Severe cervical flexion myelopathy with long tract signs. Later, these clinical findings were identified in detail in 38 patients by. References improving outcomes in diabetic kidney disease. Sep 27, 2018 amyotrophic lateral sclerosis als, also referred to as lou gehrigs disease, is a progressive motor neuron disease which leads to problems with muscle control and movement. This disease classically presents with bilateral forearm and hand muscle wasting. A diagnosis of hirayama disease was made, and the use of a cervical collar. Case fatality ratios, transmission routes, incubation times, etc. 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. If you would like a large, unwatermarked image for your web page or. Hirayama disease is a rare cervical myelopathy predominantly affecting young adults andmainly found in asia. It differs from the other motor neuron diseases because of its nonprogressive nature and pathological findings of focal ischemic changes in the anterior horn.
Hirayama disease turkish journal of physical medicine and. Hirayama disease is a nonprogressive asymmetric juvenile muscular atrophy involving c7t1 myotomes leading to weakness and atrophy of. Hirayama disease hd is benign focal amyotrophy of the. Feb 08, 2018 monomelic amyotrophy mma is a rare disease that causes muscle weakness in the upper extremities. Nonprogressive juvenile spinal muscular atrophy of. Unfortunately it affects both my hands, left much worse than the right, so that i also cannot play video games anymore hahah. It is usually sporadic, it has an insidious onset and there is a slow progression followed by stabilization in 24 years.
It is considered a benign motor neuron disorder with a stationary stage after a progressive course. This benign focal cervical poliopathy is believed to be caused by forward displacement of the posterior cervical dural sac. It results in a pure motor distal lesion of the upper limbs with slow progression. Hirayama disease juvenile muscular atrophy of distal upper extremity is a cervical myelopathy predominantly affecting adolescent males. 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. Monomelic amyotrophy ma is a rare benign lower motor neuron disorder characterized by. Hirayama, cervical myelopathy, mri in flexion, upper limb deficit. It is primarily seen in young males of indian or asian descent. Hirayama disease hd, also known as monomelic amyotrophy ma, is a rare motor neuron disorder that affects one upper extremity 1. 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. Lower trunk or medial cord brachial plexopathy true neurogenic thoracic outlet syndrome ntos is a.
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. Surgical treatment is controversial for several reasons. Dynamic magnetic resonance imaging mri, which allows the diagnosis to be made, shows a typical appearance of anterior compression of the cervical spinal cord associated with enlargement. If you would like a large, unwatermarked image for your web page or blog, please purchase the appropriate license. Most studies of hd have been conducted in asia, particularly japan. Hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord.
Hirayama disease, a type of cervical flexion myelopathy, is a rare neurological disease characterized by muscular atrophy of the forearms and hands. Hirayamas disease in 2003 and have now been going for 5 years or so. Hiroshi hirayama dmd 745 boylston street suite 406 boston, ma 02116 t. In conclusion, this case showcases how cervical mri, with neck flexed, can pick up dynamic cord compression and contribute to the diagnosis of hirayama disease. 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. You may not embed one of our images on your web page without a link back to our site. Although hirayama disease is a selflimiting disorder, early diagnosis is necessary because a cervical collar, by preventing neck flexion, may arrest the progression of the disorder. A rare clinical entity salman mansoor, kevin murphy, mohammad hijaz adenan, eimear joyce, orla hardiman, michael hennessey, siobhan kelly sajem. Nonprogressive juvenile spinal muscular atrophy of the. A 29yearold man visited the hospital with a 1year history of weakened left proximal upper limb. However, focal compression of the ventral nerve roots cannot be entirely. Spinal muscular atrophies neuromuscular disorders, 2e.