What is Monomelic Amyotrophy?

What is Monomelic Amyotrophy?

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. It is characterized by weakness and wasting in a single limb, usually an arm and hand rather than a foot and leg.

What causes Hirayama disease?

The condition is caused by a tight dural sac in the cervical canal that leads to chronic ischemic changes to the anterior horn cells. Although commonly considered a non-progressive and self-limiting disease, this has been noted to be a source of significant disability for some affected individuals.

What type of disease is spinal muscular atrophy?

Spinal muscular atrophy (SMA) is a genetic (inherited) neuromuscular disease that causes muscles to become weak and waste away. People with SMA lose a specific type of nerve cell in the spinal cord (called motor neurons) that control muscle movement.

How is Hirayama disease diagnosed?

The key to diagnose this disease is based on the typical clinical features and dynamic MRI study when the neck is flexed. MR studies in flexion show not only the anterior displacement of the posterior wall but also a well-enhanced crescent-shaped lesion in the posterior epidural space of the lower cervical canal.

How is Amyotrophy diagnosis?

How is Diabetic Amyotrophy diagnosed? Electrodiagnostic testing can be useful to definitively diagnose this condition. Nerve conduction studies and needle electromyography are often necessary to diagnose this condition and rule out other problems.

What is anterior horn cell disease?

Anterior horn disease is one of a number of medical disorders affecting the anterior horn of the spinal cord. Anterior horn diseases include spinal muscular atrophy, poliomyelitis and amyotrophic lateral sclerosis.

When does Hirayama stop?

As “Hirayama disease” is considered a self-limited disease and often stops progressing after 1–5 years of onset, the mainstay of treatment consists of preventing neck flexion using a cervical collar to halt further progression.

What’s the difference between atrophy and dystrophy?

atrophy: To wither or waste away. dystrophy: A wasting of body tissues, of genetic origin or due to inadequate or defective nutrition. sarcopenia: Age-related loss of skeletal muscle, resulting in frailty.

How is spinal atrophy treated?

Spinal Muscular Atrophy Treatment. The FDA has approved three medications to treat SMA: nusinersen (Spinraza), onasemnogene abeparvovec-xioi (Zolgensma) and risdiplam (Evrysdi). Both are forms of gene therapy that affect the genes involved in SMA.

What is the treatment for Amyotrophy?

How is Diabetic Amyotrophy treated? This condition frequently will improve with time and the body will heal itself. Physical therapy and strict observation of blood glucose levels are recommended to help diabetic amyotrophy. Medications may help with the pain.

What do you need to know about monomelic amyotrophy?

View Full Treatment Information. Definition. 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.

What is the treatment for monomelic amyotrophy ( MMA )?

Treatment consists of muscle strengthening exercises and training in hand coordination 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.

When was monomelic amyotrophy first described in Japan?

Monomelic amyotrophy (MMA), is a motor neuron disease first described in 1959 in Japan.