Diagnosis and management of diabetic amyotrophy about 50% of patients with diabetes mellitus worldwide suffer from diabetic peripheral neuropathy dpn. Two young siblings are reported with monomelic amyotrophy who had proximal muscle weakness confined to one arm. Occupationbased hand therapy in a baker with monomelic. 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 hd, also interchangeably referred to as monomelic amyotrophy, has been more frequently recognised in the west only in the last two decades. Postradiation lower motor neuron syndrome presenting as. Descriptive terms such as brachial monomelic amyotrophy mma confined. Monomelic amyotrophy is a rare disorder marked by the progressive loss and degeneration of motor neurons. 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. Monomelic amyotrophy mma, also known as hirayama disease, is a sporadic juvenile muscular atrophy in the distal upper extremities, which predominantly affects the lower cervical cord e. A case presentation on a patient with the familial form of. The diagnostic criteria of benign monomelic amyotrophy.
It is characterized by weakness and wasting in a single limb, usually an arm and hand instead of a foot and leg. Proximal involvement of the arm and shoulder muscles is an unusual presentation that has been rarely reported in the literature. To which als amyotrophic lateral sclerosis and variants falling under the same umbrella as being classified as motor neuron disease mnd. Far more discussed is whether or not the name monomelic amyotrophy is the correct name, as there are a high percentage of cases where monomelic is misleading or inaccurate. This disorder usually develops in the late teens and early twenties with a male preponderance. Hirayama disease monomelic amyotrophy clinically confused. Monomelic amyotrophy symptoms, diagnosis, treatments and. And there is barely a whisper as to why this is so disproportionately found in asia. Monomelic amyotrophy information page national institute. A 28yearold white man presented with insidiousonset, slowly progressive, unilateral weakness and atrophy of his left. Feb 28, 2015 monomelic amyotrophy mma, also known as hirayama disease, is a sporadic juvenile muscular atrophy in the distal upper extremities, which predominantly affects the lower cervical cord e.
Introduction the patient presenting with weakness and wasting of one limb, usually the arm, can be a considerable diagnostic challenge for the neurologist. Previously thought to be restricted to japan and south asia, it is now clear that this condition does occasionally occur in europe and merits consideration. My husband diagnosed with monomelic amyotrophy recently. Most cases are sporadic, although a familial form has been reported. Bma is usually sporadic, it has an insidious onset and slow progression followed by stabilization in 24 years. It is characterized by weakness and wasting in a single limb, usually an arm and hand rather than a foot and leg. Monomelic amyotrophy synonyms, monomelic amyotrophy pronunciation, monomelic amyotrophy translation, english dictionary definition of monomelic amyotrophy. Monomelic amyotrophy of the upper limb or hirayama disease is mostly considered as an anterior horn disorder resulting from local ischemia, triggered by arterial compression from an anterior shifting of the posterior cervical dura upon neck flexion. Mma should be considered in differential diagnosis in patients presenting with amyotrophy involving lower and. While the spectre of the amyotrophic lateral sclerosis form of motor neuron disease alsmnd is often in the clinicians mind, there is also a significant differential diagnosis to be considered. Nascimento abstract a consecutive series of 21 patients with single limb atrophy monomelic amyotrophy is reported. Monomelic amyotrophy may also be known as benign focal amyotrophy, single limb atrophy, hirayama syndrome or sobue disease. Monomelic amyotrophy hirayama disease with upper motor.
Encyclopedia article about monomelic amyotrophy by the free dictionary. The trip database provides clinical publications about. Monomelic amyotrophy is a sporadic juvenileonset disease that presents with gradual onset of. 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. Monomelic amyotrophy is a rare, benign motor neuron disorder. This means that monomelic amyotrophy, or a subtype of monomelic amyotrophy, affects less than 200,000 people in the us population. Diabetic amyotrophy is a complication of diabetes mellitus.
Then you can start reading kindle books on your smartphone, tablet, or computer no kindle device required. Monomelic amyotrophy with late progression sciencedirect. Juvenile muscular atrophy of unilateral upper extremity. Clinical findings, emg characteristics and differential diagnosis pdf. Furthermore, signs and symptoms of monomelic amyotrophy may vary on an individual basis for each patient. Lower motor neurons are cells that help communicate information from the brain to the muscles that are involved in movement skeletal muscles. The study of monomelic amyotrophy has been mentioned in research publications which can be found using our bioinformatics tool below. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis.
The characteristic clinical features were insidious onset in the second and third decades, male preponderance, sporadic occurrence, wasting and weakness confined to. Motor differential diagnosis hereditary amyotrophic lateral sclerosis. Sixteen had lower limb atrophy and five had upper limb involvement. A 28yearold white man presented with insidiousonset, slowly progressive, unilateral weakness and atrophy of his.
Specifically, monomelic amyotrophy causes weakness and loss of muscle mass in the arms. We present the case of a 33yearold woman with benign sporadic monomelic amyotrophy of the distal part of the arm, called hirayama disease. Monmelic amyotrophy hirayamas dissease practical neurology. Electrophysiologic studies are suggestive of localized chronic anterior horn cell disease. Monomelic amyotrophy mma is a benign motor neuron disease characterized by. Monomelic definition at, a free online dictionary with pronunciation, synonyms and translation. Monomelic amyotrophy of lower limb is a clinically localized variant of spinal muscular atrophy with a particularly benign course. Thirteen had upperlimb and ten had lowerlimb involvement. Please tell us where you read or heard it including the quote, if possible. Monomelic amyotrophy mma is a rare disease of the nerves that control voluntary movements of the limbs. Diabetic amyotrophy is characterized by relatively rapid, progressive asymmetrical weakness and pain in the muscles in the proximal lower extremities. Hirayama disease hd is a rare motor neuron disorder that involves a single upper extremity. From 1977 through 1981, we examined 23 patients with singlelimb atrophy.
Monomelic amyotrophy mma is a rare disease that causes muscle weakness in the upper extremities. Previously thought to be restricted to japan and south asia, it is now clear that this condition does. Longterm followup of monomelic amyotrophy of the upper. Electromyography showed denervated patterns in the extensor. Specifically, monomelic amyotrophy causes weakness and loss of muscle mass in the arms and fingers. Mma a pure movement disorder characterized by degeneration and death of motor neurons, resulting in atrophy of a limb, typically one arm.
Pdf monomelic amyotrophy with proximal upper limb involvement. Monomelic amyotrophy ma is a rare condition in which neurogenic amyotrophy is restricted to an upper or lower limb. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. Enter your mobile number or email address below and well send you a link to download the free kindle app. Benign monomelic amyotrophy bma is a rare condition in which neurogenic amyotrophy is restricted either to the upper or to the lower limb1,2. Pdf monomelic amyotrophy in siblings haluk topaloglu. Clinical features included forearm amyotrophy sparing the brachioradialis muscle, cold paresis and causalgia.
The symptoms of mma usually progress slowly for one to two years before reaching a plateau and then remain stable for many years. Disease bioinformatics research of monomelic amyotrophy has been linked to muscular atrophy, atrophy, weakness, spinal muscular atrophies of childhood, flexed fetal attitude. We use cookies to offer you a better experience, personalize content, tailor advertising, provide social media features, and better understand the use of our services. Monomelic amyotrophy mma is a benign motor neuron disease with. Monomelic amyotrophy article about monomelic amyotrophy. The official parents sourcebook on monomelic amyotrophy. Mar 27, 2019 monomelic amyotrophy mma is characterized by progressive degeneration and loss of motor neurons, the nerve cells in the brain and spinal cord that are responsible for controlling voluntary muscles. In two patients, involvement was clinically limited to one leg, with a subacute course followed by plateau in the first case and with progressive worsening in the second one. Longterm followup of monomelic amyotrophy of the upper limb.
Monomelic amyotrophy definition of monomelic amyotrophy by. Monomelic amyotrophy genetic and rare diseases information. Monomelic definition of monomelic by medical dictionary. Only rarely are the legs involved and pyramidal findings are uncommon. Monomelic amyotrophy definition of monomelic amyotrophy. Monomelic amyotrophy nosology hirayama disease course. Monomelic amyotrophy mma is characterized by progressive degeneration and loss of motor neurons, the nerve cells in the brain and spinal cord that are responsible for controlling voluntary muscles. A case presentation on a patient with the familial form of monomelic amyotrophy. Mma should be considered in differential diagnosis in patients presenting with amyotrophy involving lower and upper extremity behind other diagnosis. Hirayama disease is a rare neurological condition also known as monomelic amyotrophy mma. Juvenile onset spinal muscular atrophy of the distal upper extremities. Our muscles are contracting and relaxing due to continuous signals that are given by the motor neurons located in the brain and spinal cord. Pdf background monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons. Monomelic amyotrophy with proximal upper limb involvement.
What is the prognosis for monomelic amyotrophy mma. Monomelic amyotrophy information page national institute of. Mma is usually restricted to the upper limb, lower limb involvement is rare. Affected individuals suffer from painful, progressive damage to the lumbosacral plexus and consequent neurological deficits. The most common form of dpn is a symmetrical, predominantly sensory, polyneuropathy with distal onset and slow proximal progression. Monomelic amyotrophy treatment with stem cells swiss medica. Benign monomelic amyotrophy with proximal upper limb.
Monomelic amyotrophy is a rare form of motor neuron disease usually presenting as painless asymmetric weakness and atrophy in the distal upper. Hirayama disease monomelic amyotrophy clinically confused for carpal tunnel syndrome halil ay neurology department of medical faculty, harran university, sanliurfa, turkey abstract. Monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons, affecting predominantly the hand and forearm muscles. Source national institutes of health nih ophanet, a consortium of european partners, currently defines a condition rare when it affects 1 person per 2,000. However, such a dural shifting is not universally seen. It is characterized by weakness and wasting in a single limb, usually an. Nosology of juvenile muscular atrophy of distal upper. Since its original description by keizo hirayama in 1959, juvenile muscular atrophy of the unilateral upper extremity has been described under many nomenclatures from the east. It is a type of cervical myelopathy, which involves the anterior horn cells and affects the distal upper extremities. Monomelic medical definition merriamwebster medical. Monomelic amyotrophy article about monomelic amyotrophy by.
Monomelic amyotrophy mma, is a rare motor neuron disease first described in 1959 in japan. It is clinically characterized by weakness and atrophy of the muscles of the hand and forearm. Monomelic medical definition merriamwebster medical dictionary. Benign monomelic amyotrophy with lower limb involvement in an adult. A revised and updated directory for the internet age. Abstract a consecutive series of 21 patients with single limb atrophy monomelic amyotrophy is reported. The characteristic clinical features were insidious onset in the second and third decades, male preponderance, sporadic occurrence, wasting and weakness confined to one limb, and absence of involvement of the cranial nerves, cerebrum, brain stem, and sensory. Monomelic amyotrophy or juvenile nonprogressive amyotrophy of the upper limb is rarely encountered in the general neurology clinics but it may be under diagnosed. Monomelic amyotrophy is a rare restricted form of motor neuron disease. Pdf monomelic amyotrophy mma is a benign motor neuron disorder. It is selflimited, asymmetrical lower motor weakness of hands and forearms.
May 02, 2016 diabetic amyotrophy is believed to result from a multifocal immunemediated microvasculitis, ie an immune abnormality involving vasculitic changes, microvascular insufficiency and ischaemia followed by axonal degeneration and demyelination. The condition is hypothesized to occur due to an asymmetric. Monomelic amyotrophy als amyotrophic lateral sclerosis. Feb 08, 2018 monomelic amyotrophy mma is a rare disease that causes muscle weakness in the upper extremities. Article information, pdf download for occupationbased hand. Benign focal amyotrophy or monomelic amyotrophy jama network. Monomelic amyotrophy developed 16 months, nine and 12 years after irradiation of the lumbosacral spinal cord for seminoma in one patient and for hodgkins disease in two others. Mar 17, 2016 monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons, affecting predominantly the hand and forearm muscles. Monomelic amyotrophy mma is a benign motor neuron disorder. A total of 190 patients were diagnosed to have mma in our institute over the last 27 years and this is the first case of familial mma.
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