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?

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.

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 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.

Is Hirayama disease a disability?

The person affected by the disease experiences a certain level of disability due to the disease. On rare occasion, the weakness associated with Hirayama’s disease may progress to the person’s opposite limb.

How is SMA caused?

Most kinds of SMA are caused by a problem with a gene called the SMN1 gene. The gene does not make enough of a protein needed for the motor neurons to work normally. The motor neurons break down and can’t send signals to the muscles. A child with SMA gets one copy of the SMN1 gene from each parent.

What is another name for Kennedy’s disease?

Kennedy’s disease is also known as X-linked spinal bulbar muscular atrophy (SBMA). There is no cure yet, and treatment can only ease some of the symptoms.

What is the treatment for Kennedy’s disease?

Currently there is no known cure for Kennedy’s disease. Treatment is symptomatic and supportive. Physical therapy and rehabilitation to slow muscle weakness and atrophy may prove helpful. Kennedy’s disease is an inherited motor neuron disease that affects males.