Neurosarcoidosis

Neurosarcoidosis is a condition that causes inflammation and abnormal cell clusters (granulomas) to form in your nervous system. Common symptoms include weakness or drooping of one side of your face, problems with vision, chronic headaches and difficulty walking. Symptoms vary from person to person and range in severity.

Neurosarcoidosis is a type of sarcoidosis. This chronic (long-term) inflammatory condition can affect many organs, but it most often affects your lungs.

Sarcoidosis affects an estimated 60 people per 100,000 in the United States. Neurosarcoidosis affects 5% to 15% of people diagnosed with sarcoidosis.

Where do neurosarcoidosis granulomas form?

Neurosarcoidosis most commonly affects the cranial nerves, meninges or lining of your brain and spine. It can also invade your brain or spinal tissue.

Neurosarcoidosis symptoms

Neurosarcoidosis symptoms vary based on where inflammation happens in your nervous system. It also depends on where granulomas form. Brain and cranial nerve symptoms may include:

• Bowel and bladder symptoms
• Confusion
• Headaches
• Motor symptoms (weakness in your arms and legs or problems with walking)
• Sensory symptoms (numbness and tingling in your arms and legs)

If neurosarcoidosis affects your pituitary gland (a hormone-producing component in your brain), you may have the following symptoms:

• Fatigue
• Excessive thirst
• Changes to your weight
• Frequent urination
• Irregular periods

Neurosarcoidosis causes

Researchers believe an overactive immune system causes neurosarcoidosis. The reason why your immune system overreacts isn’t well understood. Your immune system fights off threats to your body to keep you healthy, like bacteria or viruses. When it detects a threat, it causes inflammation. In the case of neurosarcoidosis, that inflammation targets your nervous system.

Risk factors

Neurosarcoidosis can affect anyone. You may be more at risk of developing neurosarcoidosis if you:

• Have sarcoidosis
• Are female
• Have African or Northern European ancestry
• Are in your mid-40s
• Have someone in your biological family diagnosed with sarcoidosis
• Have a gene change (mutation) that affects immune system response
• Have certain environmental exposures

It’s about three times more common in Black adults than in white adults. The condition rarely affects children.

Complications of neurosarcoidosis

This condition can cause worsening or even permanent loss of neurological function (the way your brain, spinal cord and nerves work). The most bothersome symptoms are:

• Bowel and bladder dysfunction
• Depression
• Fatigue
• Loss of vision
• Loss of hearing
• Pain
• Problems with arm function (loss of coordination and weakness)
• Problems with leg function (difficulty walking)
• Thinking (cognition) challenges

How doctors diagnose neurosarcoidosis

A healthcare provider will perform a complete medical history and neurological exam. Testing helps confirm the diagnosis and rule out other possible causes of your symptoms.

Your provider will also check for symptoms of sarcoidosis in other parts of your body, like swollen lymph nodes, a rash or joint pain. This can help identify a good biopsy site, which is one of the best ways to confirm the diagnosis.

Sometimes, the nervous system is the first part of your body affected by sarcoidosis. So, you may not notice other symptoms yet.

What tests diagnose neurosarcoidosis?

Your healthcare provider may offer tests to rule out conditions with similar symptoms. While there isn’t a single test to diagnose this condition, the following may be helpful:

• An MRI of your brain and spinal cord
• A lumbar puncture to rule out other causes of your symptoms, like infection
• An EMG/NCS if there’s concern for nerve or muscle involvement
• A CT scan of your chest
• A lung or nerve biopsy

Your provider may recommend other tests as needed.

Neurosarcoidosis treatment

There’s no cure for neurosarcoidosis. But treatment may help control inflammation, prevent new or worsening damage, and give your body a chance to heal. Other treatments are available to help manage long-term symptoms that may continue even after inflammation improves.

Treatment may include:

• Taking medications to control inflammation and manage sarcoidosis symptoms
• Using assistive devices, like glasses, hearing aids, a walker or a wheelchair
• Participating in physical therapy

Neurosarcoidosis medications

The following are common medications to treat symptoms of neurosarcoidosis:

• Corticosteroids as a first-line treatment to reduce inflammation
• Immunosuppressants to reduce inflammation in the long term
• Hormone replacements if symptoms affect your pituitary gland
• Varying medications to manage symptoms like fatigue, nerve pain, spasticity, bowel and bladder issues and sexual dysfunction

Talk to your provider about the side effects of each medication before you start taking it. This helps you know what to look out for. Let them know if you experience any side effects during treatment or if your symptoms change.

When should I see a healthcare provider?

If you notice symptoms of neurosarcoidosis, contact a healthcare provider. Common symptoms include:

• Changes in sensation
• Loss of vision
• Movement problems
• Ongoing headaches
• Weakness or drooping on one side of your face

These symptoms can happen with other conditions, too, not just sarcoidosis. So, it’s best to get them checked by a provider.

What’s the outlook for neurosarcoidosis?

The prognosis varies from person to person. Your healthcare provider can give you an idea of what your outlook is based on your situation.

Many people improve with treatment. Others continue to have long-term symptoms, depending on which parts of the nervous system it affects and how quickly they receive a diagnosis and begin treatment.

Some side effects can happen when you take certain medications for a long time. For example, corticosteroids may cause many long-term side effects, and immunosuppressants can increase your risk of infections.

Your provider will monitor how the condition affects you over time. Even if your symptoms improve, regular check-ups can help catch and manage symptoms if they return.

What’s the life expectancy of a person with neurosarcoidosis?

Most people diagnosed with this condition have a normal life expectancy. This can vary from person to person. It depends on the severity and where the disease is located. Your provider will give you the most accurate information about what to expect.