CIDP (Chronic Inflammatory Demyelinating Polyneuropathy)

What is CIDP?

CIDP (chronic inflammatory demyelinating polyneuropathy) is a rare neurological condition that causes worsening (progressive) muscle weakness, numbness and other symptoms.

Breaking down the name of the condition helps to understand it:

• Chronic: “Chronic” means “long-term.” CIDP slowly develops over at least eight weeks. It can also improve and then come back (relapse) over the course of months or years.
• Inflammatory: Researchers think CIDP happens due to issues with your immune system, specifically a type of autoimmune attack. This causes excessive inflammation that damages your peripheral nerves— the nerves outside of your brain and spinal cord.
• Demyelinating: The inflammation specifically affects the myelin sheath of your nerves. This is the protective sleeve (sheath) that’s wrapped around each nerve cell (neuron). Demyelination refers to the destruction of the myelin sheath.
• Polyneuropathy: This is the malfunction of many peripheral nerves throughout your body. “Poly-” means “many.” Neuropathy is the umbrella term for any kind of damage to your peripheral nerves. Neuropathy can cause many issues, including muscle weakness and abnormal sensations (paresthesia) like numbness.

What’s the difference between Guillain-Barré syndrome and CIDP?

CIDP is closely related to Guillain-Barré syndrome (GBS). Healthcare providers consider CIDP to be the long-term form of GBS. GBS is a rare autoimmune condition in which your immune system attacks your peripheral nerves.

The most severe stage of GBS is usually around two to three weeks after symptoms start. But most people then start improving after this point. CIDP lasts longer — the symptoms worsen over at least eight weeks.

How common is CIDP?

Researchers estimate that there are 0.8 to 8.9 new cases of CIDP per 100,000 people in the United States each year. This range is wide because CIDP can affect people in many different ways and is difficult to diagnose because of this.

CIDP can affect anyone at any age, but it more commonly affects people assigned male at birth (AFAB).

What are the symptoms of CIDP?

The symptoms of CIDP can vary based on the variant (type). But the most common symptom is muscle weakness that gets worse over at least eight weeks. It typically affects the muscles in the following areas typically equally on both sides of your body:

• Hips and thighs.
• Shoulders and upper arms.
• Hands
• Feet.

Other symptoms of CIDP may include:

• Loss of muscle mass (atrophy) in affected muscles.
• Tingling, prickliness or numbness in your fingers and toes (paresthesia).
• Difficulties with balance and coordination (clumsiness).
• Loss of mobility
• Loss or weakening of deep tendon (muscle stretch) reflexes.
• Neuropathic pain.

In rare cases, you may also experience:

• Difficulty swallowing (dysphagia) and weakness above your neck.
• Double vision.

These symptoms may change in severity over time. They may come on slowly or rapidly and sometimes come and go over time. If you develop symptoms of CIDP, see your healthcare provider as soon as possible. Early diagnosis and treatment are key to recovery.

Variants of CIDP

Certain variants (types) of CIDP have different symptoms. Typical CIDP is the most common form. It has symmetric (affecting both sides of your body) muscle weakness and abnormal sensations (sensory symptoms).

Some atypical variants include:

• Multifocal motor neuropathy: This variant only causes muscle weakness. The weakness is asymmetric, which means it affects different parts of your body.
• Lewis-Sumner syndrome: This variant has asymmetric muscle weakness and sensory symptoms.
• Pure sensory CIDP: This variant involves numbness, pain, balance issues and an abnormal gait (walking pattern). It doesn’t have muscle weakness as a symptom.
• Pure motor CIDP: This variant involves symmetric muscle weakness and loss of reflexes but no sensory symptoms.

Researchers are currently studying other variants of CIDP.

What causes CIDP?

Researchers believe CIDP happens because of issues with your immune system. For unknown reasons, your immune system sees myelin as dangerous and attacks it (autoimmune reaction).

The myelin sheath is the protective layer around your nerve cells. It wraps around the nerve axon — the long, wire-like part of a nerve cell. Myelin allows electrical impulses to efficiently travel along your nerves. When myelin is damaged or removed, it slows down or loses these electrical impulses. And the “messages” may never reach their intended destination. This causes the symptoms of CIDP.

How is CIDP diagnosed?

CIDP can be difficult to diagnose, especially since different variants have different symptoms. To diagnose CIDP, your healthcare provider will:

• Assess your symptoms and learn about your medical history.
• Do physical and neurological exams.
• Request certain tests to help confirm the diagnosis or rule out other possible causes.

What tests will be done to diagnose CIDP?

Test that can help confirm CIDP and/or rule out other possible causes of your symptoms include:

• Electromyography (EMG) and nerve conduction tests: These tests evaluate the health and function of your skeletal muscles and the nerves that control them. They specifically look for demyelination changes (like slowing and blocks) in nerves to distinguish CIDP from the much more common types of polyneuropathy.
• Spinal tap (lumbar puncture): For this procedure, your healthcare provider inserts a needle into your lower back to get a sample of cerebrospinal fluid (CSF). They send the sample to a lab where a pathologist examines the substances in it. In about 85% to 90% of CIPD cases, there’s a normal amount of white blood cells and an elevated CSF protein level. Other abnormalities in CSF may point to other conditions.
• MRI (magnetic resonance imaging) of your lumbar (lower back) spine: If nerve roots in your lumbar spine pick up contrast dye as part of an MRI, it can support the diagnosis of CIDP.
• Blood tests: Your provider may recommend certain blood tests to rule out other conditions that can cause neuropathy, like diabetes, vitamin deficiencies, thyroid disease, Lyme disease, HIV or AIDS, lymphoma, and more. They may also check for certain antibodies that have been linked to CIDP.
• Nerve biopsy: In rare cases, your provider may recommend a nerve biopsy to look for evidence of demyelination.

How is CIDP treated?

There are three go-to (first-line) treatments for CIDP, including:

• Corticosteroids: Prescription corticosteroids like prednisone can help improve inflammation. Many people see improvement in their symptoms with corticosteroids alone. However, corticosteroids can cause serious side effects, which limits how long you can take the medication. Your provider may also prescribe other medications, like those that suppress your immune system (immunosuppressants), alongside corticosteroids.
• Plasma exchange (plasmapheresis): In this treatment, a machine separates the plasma from your blood, treats it, and then returns the plasma and blood to your body. Plasma exchange filters out the antibodies in your plasma that are attacking your nerves. Plasma exchange is typically only effective for a few weeks. You may need continued intermittent (on-and-off) treatments over the course of months or years.
• Intravenous immunoglobulin therapy (IVIG): This treatment involves intravenous (IV) injections of immunoglobulins, which are proteins that your immune system naturally makes to attack invading organisms. The immunoglobulins come from a collection of thousands of healthy donors. IVIG can lessen your immune system’s attack on your nerves. You may receive very high doses of IVIG initially and may need continued intermittent treatments over the course of months or years.

Researchers are currently studying other forms of treatment for CIDP. You may be able to participate in a clinical trial for CIDP. Talk with your provider to see if this is an option.

Can I prevent CIDP?

As researchers don’t know what triggers CIDP, there’s no known way to prevent developing it.

What is the prognosis for people with CIDP?

The long-term prognosis (outlook) depends on many factors, including:

• Your age when CIDP began.
• How the condition develops and the variant type.
• How quickly you receive a diagnosis and treatment.
• How your body initially responds to the treatment.

About 90% of the people with CIDP improve with treatment with a 50% relapse rate. Overall long-term prognosis is good for people with CIDP diagnosed at a younger age. If you don’t receive any treatment for CIDP, you may develop permanent nerve damage that results in disability.

Your healthcare provider will be able to give you a better understanding of what to expect given your unique case.

What is the life expectancy of a person with CIDP?

CIDP isn’t a fatal condition, and people with it typically have the same life expectancy as someone without the condition.

When should I see my healthcare provider?

If you’ve received a CIPD diagnosis, you’ll need to see your healthcare provider regularly to monitor your symptoms and receive treatment. CIPD symptoms can come and go over months or years, requiring ongoing treatment.

When should I go to the ER?

In rare cases, CIPD can affect the muscles that you need to breathe. If you have trouble breathing, call 911 (or your local emergency services number) or go to the nearest emergency room.

A note from Cleveland Clinic

Feeling like you’ve lost control of your body can be scary. But the good news is that the symptoms of CIDP (chronic inflammatory demyelinating polyneuropathy) are treatable, especially if you start treatment right away. Know that your healthcare team will be with you every step of the way to navigate this condition.