CIDP (Chronic Inflammatory Demyelinating Polyneuropathy)

CIDP (Chronic Inflammatory Demyelinating Polyneuropathy)

Overview

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.

Symptoms

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.

Causes

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.

Diagnosis

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.

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.

Treatment

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

Type of Doctor Department : A neurologist or neuromuscular specialist