Monomelic amyotrophy symptoms pdf

It is characterized by weakness and wasting in a single limb, usually an arm and hand rather than a foot and leg. In rare cases, symptoms of monomelic amyotrophy may spread to the opposite arm or may start worsening again after age 40yearsold. Pdf monomelic amyotrophy mma, also known as hirayama disease, is a sporadic juvenile muscular atrophy in the distal upper extremities. This disorder usually develops in the late teens and early twenties with a male preponderance. Monomelic amyotrophy with proximal upper limb involvement. The list of signs and symptoms mentioned in various sources for monomelic amyotrophy includes the 11 symptoms listed below. Diabetic amyotrophy or diabetic proximal neuropathy is a lumbosacral plexopathy of diabetic patients with presenting symptoms of weak thigh muscles and severe pain in the thighs, back, or both. This disease is characterized by muscle wasting and weakness, affecting predominantly the lower cervical myotomes. Monomelic amyotrophy mma is a benign motor neuron disease with bilateral muscular atrophy in asymmetry and abnormal in the. Nascimento abstract a consecutive series of 21 patients with single limb atrophy monomelic amyotrophy is reported. Monomelic amyotrophy hirayama disease with upper motor.

Hirayama disease turkish journal of physical medicine and. Monomelic amyotrophy ma is a rare benign lower motor neuron disorder characterized by muscular. Pdf monomelic amyotrophy hirayama disease with upper. 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 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. Most cases are sporadic, although a familial form has been reported. Specifically, monomelic amyotrophy causes weakness and loss of muscle mass in the arms. The quadriceps, adductor, and iliopsoas muscles are weak 100% of the time and the glutei and hamstrings 50% of the time all are proximal muscles. Monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons, affecting predominantly the hand and forearm muscles. 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. Benign monomelic amyotrophy with lower limb involvement in an adult. Monomelic amyotrophy genetic and rare diseases information. Clinical findings, emg characteristics and differential diagnosis pdf.

Lower motor neurons are cells that help communicate information from the brain to the muscles that are involved in movement skeletal muscles. Monomelic amyotrophy mma is a rare disease that causes muscle weakness in the upper extremities. A 28yearold white man presented with insidiousonset, slowly progressive, unilateral weakness and atrophy of his. Sixteen had lower limb atrophy and five had upper limb involvement. Monomelic amyotrophy mma, is a rare motor neuron disease first described in 1959 in japan. Previously thought to be restricted to japan and south asia, it is now clear that this condition does occasionally. Monomelic amyotrophy is a rare restricted form of motor neuron disease. Monomelic amyotrophy mma is a benign motor neuron disease characterized by neurogenic amyotrophy, which usually affects one of the upper or lower extremities. Monomelic amyotrophy ma, also known as hirayama disease, is a rare, benign lower motor neuron disease. When bma is restricted to the distal aspect of the upper limb it is called hirayama disease4,5. Monomelic amyotrophy information page national institute. Other rare symptoms of the disease may include worsening weakness in cold temperatures cold paresis, muscle cramps, having cold hands, and tremors.