Prognosis and Life Expectancy of NMOSD

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune inflammatory disease of the central nervous system (eye nerves, spinal cord, and brain). In autoimmune diseases, the immune system cannot tell the difference between healthy cells and invaders like viruses, fungi, or bacteria. Because it cannot tell the difference, the body begins to attack and damage healthy cells.¹

NMOSD is a chronic disease, meaning it lasts for a long time or never completely goes away. There is no cure for NMOSD, but there is treatment.¹

Being diagnosed with a rare disease like NMOSD will leave you with unanswered questions about the future. How will the disease develop over time? Will it worsen or get better? What will be my quality of life? What is the average life expectancy with NMOSD?

How will NMOSD develop over time?

The chance of recurrence after a first attack is greater than 90 percent. Though outcomes vary widely among people with NMOSD, attacks can be devastating. When NMOSD is left untreated, early and severe disability is common.^2,3

Disability from NMOSD may include:^2,3

• Vision loss or blindness
• Problems moving, walking, or standing
• Complete paralysis
• Bladder problems

Will the disease worsen or get better?

Attacks or relapses of NMOSD may be more or less severe over time. Relapses are highly variable, can last at least a month, and can include paralysis or blindness. New damage to the brain and spinal cord during these episodes can worsen disability and may or may not be reversible.^1-3

Some factors can predict a worse outcome in NMOSD, including:²

• The number of relapses within the first 2 years
• The severity of the first attack
• Age at onset of disease
• Other autoimmune diseases

The impact on quality of life

Disease outcomes vary widely among people with NMOSD. Many people need help walking over time. Some need a wheelchair. Vision loss and complete blindness may occur. Others may also need help with breathing and require the use of a ventilator. Early diagnosis and treatment of NMOSD appears to improve long-term quality of life.^1-3

Race and NMOSD

A 2018 study published by the American Academy of Neurology included 427 people with NMOSD. Those of African descent made up only 40 percent of the testing group but 90 percent of those who died. Other studies have also suggested racial differences in outcomes of NMOSD. But the greatest determiner of outcome was early and effective treatment. The reasons behind racial differences are not yet known.⁴

Will I die from this disease?

New studies have shown that life expectancy for those with NMOSD may be longer in recent years because of advances in treatment. Death from NMOSD varies widely from person to person and is based on factors such as:⁵

• Delayed diagnosis or misdiagnosis
• Presence of other diseases or conditions
• Choice of treatment

Deaths from NMOSD usually occur because of complications from the disease such as:^4-7

• Severe brain stem or upper spine nerve and brain cell damage – This can lead to respiratory failure when your brain can no longer tell your lungs to breathe.
• Pneumonia (lung or airway infection) – This may occur when food, saliva, liquid, or vomit is breathed into the airways instead of being swallowed. It also can occur when your cough is not strong enough to clear the airways effectively.

About 1 in 3 people with untreated NMOSD die within 5 years of their first attack. Treatment dramatically improves these outcomes. When treated, the 5-year survival rate for monophasic (single attack) NMOSD is 90 percent. The survival rate for relapsing NMOSD (more than 1 attack) is nearly 70 percent when it is treated.^5,8