Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy is a rare autoimmune condition that causes progressive muscle weakness and sensory changes. While the symptoms develop gradually over weeks and months, early diagnosis and treatment can help prevent permanent nerve damage and improve quality of life.

Table of contents

• What is chronic inflammatory demyelinating polyradiculoneuropathy?
• How common is CIDP?
• Signs and symptoms
• What causes CIDP?
• Relationship to Guillain-Barré syndrome
• Diagnosis and testing
• Treatment options
• Outlook and prognosis
• Living with CIDP

What is chronic inflammatory demyelinating polyradiculoneuropathy?

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare neurological condition that affects the peripheral nerves — the nerves outside of the brain and spinal cord. The name of the condition helps explain what happens in the body^[1]:

• Chronic means long-term. CIDP develops slowly over at least eight weeks. It can also improve and then return (relapse) over months or years.
• Inflammatory refers to excessive inflammation that damages the peripheral nerves due to problems with the immune system.
• Demyelinating describes the destruction of the myelin sheath, the protective covering wrapped around each nerve cell. This insulating layer allows electrical signals to travel quickly and efficiently along nerves.
• Polyradiculoneuropathy means the malfunction of many peripheral nerves and nerve roots throughout the body.

CIDP is an autoimmune disease, which means the immune system mistakenly attacks parts of the body it should protect^[1][2]. In CIDP, the immune system attacks the myelin surrounding peripheral nerves. When myelin is damaged, the nerves lose their ability to transmit messages from the brain to various parts of the body efficiently^[3].

The peripheral nervous system sends messages from the brain that help control essential functions such as moving muscles, maintaining a heartbeat, and digesting food. When these nerves are damaged, the body has trouble completing these essential tasks^[3].

How common is CIDP?

CIDP is a rare condition. Researchers estimate there are 0.8 to 8.9 new cases per 100,000 people in the United States each year^[1]. Up to 29,000 people in the United States are thought to have CIDP^[3]. The estimated worldwide prevalence is about 3 per 100,000^[10].

This wide range exists because CIDP can affect people in many different ways and is difficult to diagnose. The condition can affect anyone at any age, though people usually develop symptoms around 50 years old^[1][3]. Men are twice as likely as women to be affected by CIDP. While CIDP mainly impacts adults, about 10% of cases occur in children^[3].

Signs and symptoms

The most common symptom of CIDP is muscle weakness that gradually worsens over at least eight weeks^[1][2]. This weakness typically affects muscles equally on both sides of the body in the following areas^[1]:

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

A key feature that sets CIDP apart from most other nerve conditions is that it causes weakness in both the muscles close to the body (like shoulders and hips) and those farther away (like hands and feet)^[7].

Other common symptoms include^[1][3][6]:

• Tingling, numbness, or abnormal sensations in fingers and toes
• Loss of muscle mass in affected areas
• Difficulties with balance and coordination
• Loss or weakening of reflexes
• Difficulty walking
• Fatigue
• Burning pain in arms and legs
• Muscle cramping, especially in feet or hands

CIDP particularly affects the body’s ability to sense vibration and position more than pain and temperature^[2]. People with CIDP often experience “foot drop” — an inability to lift the front part of the foot — which makes walking challenging^[3].

In rare cases, people may experience difficulty swallowing or double vision^[1].

These symptoms may change in severity over time. They can develop slowly or rapidly and sometimes come and go. If you develop symptoms of CIDP, see your healthcare provider as soon as possible, as early diagnosis and treatment are key to recovery^[1].

Variants of CIDP

Typical CIDP is the most common form, with symmetric muscle weakness and sensory symptoms affecting both sides of the body. However, certain variants have different patterns^[1][7]:

• Multifocal motor neuropathy: This variant only causes muscle weakness. The weakness is asymmetric, affecting different parts of the body unevenly.
• Lewis-Sumner syndrome: This variant causes both weakness and sensory symptoms in specific nerve territories.
• Pure motor variants: These cause only muscle weakness without sensory symptoms.
• Pure sensory variants: These cause only sensory changes without weakness.
• Ataxic variants: These primarily affect balance and coordination.

What causes CIDP?

CIDP is caused when the immune system mistakenly attacks the myelin sheath surrounding peripheral nerves^[3][6]. This autoimmune attack causes inflammation that damages the protective covering of nerves.

In many cases, the specific trigger for CIDP cannot be identified^[6]. However, CIDP may occur alongside other conditions, including^[6]:

• Chronic hepatitis
• Diabetes
• HIV/AIDS
• Inflammatory bowel disease
• Overactive thyroid
• Cancer of the lymph system
• Immune system disorders due to cancer

Some cases of CIDP have been reported following vaccination, though scientific research has not established a clear and consistent causal link, and the risk appears to be extremely low^[5].

Relationship to Guillain-Barré syndrome

CIDP is closely related to Guillain-Barré syndrome (GBS). Healthcare providers consider CIDP to be the long-term form of GBS^[1][2]. Both are autoimmune conditions in which the immune system attacks peripheral nerves.

The main difference between the two conditions is timing. The most severe stage of GBS usually occurs around two to three weeks after symptoms start, and most people begin improving after this point. In contrast, CIDP symptoms worsen over at least eight weeks^[1]. Distinguishing between these two conditions is aided by assessing how quickly symptoms progress^[2].

Diagnosis and testing

Diagnosing CIDP can be challenging because it affects people in different ways and shares symptoms with other neurological conditions^[2]. Diagnosis is based on symptoms such as loss of sensation, abnormal sensations, loss of reflexes, and weakness^[15].

The diagnostic process typically includes^[6][15]:

Physical examination: A healthcare provider will perform a neurological exam focusing on muscle strength, reflexes, and sensory function.

Nerve conduction studies: These tests measure how fast electrical signals move through nerves. In CIDP, these studies usually show evidence of demyelination — damage to the myelin sheath.

Electromyography (EMG): This test checks the electrical activity in muscles and can help identify nerve damage.

Spinal tap (lumbar puncture): This test examines the fluid surrounding the brain and spinal cord. In CIDP, the protein level is usually elevated while the cell count remains normal.

Blood and urine tests: These help rule out other disorders that may cause similar symptoms and look for unusual proteins.

Nerve biopsy: In some cases, a small piece of nerve tissue may be removed for examination under a microscope.

Prompt identification and treatment are important to prevent mortality and prolonged disability^[2]. Early diagnosis and treatment are key to preventing irreversible nerve damage^[7].

Treatment options

CIDP is treatable, and several treatment options are available^[1][9][10]. The goal of treatment is to reverse the immune system’s attack on the nerves and prevent the disease from getting worse^[6].

First-line treatments

Current evidence-based first-line therapies include^[10]:

Intravenous immunoglobulin (IVIg): This treatment involves infusing antibodies from healthy donors into the bloodstream to reduce the effect of antibodies causing the disease^[6]. IVIg is typically given by a medical professional every 3 weeks, either at a medical facility or at home^[15].

Subcutaneous immunoglobulin (SCIg): This is another form of immunoglobulin therapy that is injected just below the skin. SCIg is typically self-administered using an infusion pump once weekly^[15].

Corticosteroids: These medications help reduce inflammation and relieve symptoms^[6][10].

Plasmapheresis (plasma exchange): This procedure removes antibodies from the blood that are attacking the nerves^[6][10].

Additional treatment options

For people who do not respond well to first-line therapies, other medications that suppress the immune system may be used. These include cyclophosphamide, rituximab, and mycophenolate mofetil, though evidence for their effectiveness is limited^[10].

A new therapeutic option called efgartigimod has recently been shown to significantly lower relapse rates compared to placebo after withdrawal from previous treatment^[10].

In rare cases of severe, treatment-resistant disease, hematopoietic autologous stem cell transplants may be effective, with high remission rates^[10].

Supportive care

Physical and occupational therapy can play an important role in managing CIDP. Regular exercise can help improve nerve and muscle strength, though it’s important to choose the right level of exercise and take rest periods to avoid fatigue or injury^[13].

Outlook and prognosis

The outlook for CIDP varies from person to person^[6]. The condition may continue long-term, or people may have repeated episodes of symptoms. Complete recovery is possible, though permanent loss of nerve function can occur.

CIDP is both chronic and progressive, meaning it’s a long-term condition that can worsen over time. However, CIDP can go into remission, so early diagnosis and treatment are important for moving forward with a management plan^[3].

For some people, symptoms may gradually and steadily increase over time. For others, CIDP may follow a pattern of remission — when symptoms reduce or disappear for weeks or months — followed by relapse, when symptoms reappear^[13]. During a relapse, old symptoms may come back or new symptoms may appear, lasting for several months.

In a study of nearly 500 CIDP patients, 20% missed school or work, and 47% stopped working altogether due to their condition^[3].

Possible complications include permanent weakness or paralysis in affected areas, permanent loss of sensation, repeated or unnoticed injury, and side effects from medications used to treat the condition^[6].

Living with CIDP

Living with CIDP can be challenging, as it impacts many aspects of daily life. However, with proper management and support, people with CIDP can maintain quality of life.

Impact on daily activities

The symptoms of CIDP can affect a person’s ability to perform everyday tasks^[3], including:

• Daily grooming, such as washing and brushing hair
• Getting dressed, including buttoning clothes or using zippers
• Eating, including holding utensils
• Carrying or lifting items like groceries
• Turning keys or opening bottles

Physical and mental health

Looking after both physical and mental health is important when living with CIDP. Being diagnosed with a long-term, physically disabling condition can be difficult to come to terms with. There may be times of fear about the future or frustration with symptoms^[13].

It’s important to ask for help when needed. Close family and friends can provide support, and healthcare providers can refer patients to mental health services^[13].

Lifestyle considerations

A healthy lifestyle can help prevent other illnesses and infections that may trigger relapses and can generally improve feelings of wellbeing^[13].

Diet: A nutritious and balanced diet ensures adequate intake of vitamins and minerals. There is no evidence of special dietary requirements for CIDP, though managing weight is sensible to avoid putting excess strain on muscles^[13][17]. Foods with anti-inflammatory properties, such as fruits, vegetables, nuts, seeds, and foods rich in omega-3 fatty acids, may help reduce inflammation^[17].

Exercise: Regular physical exercise can help improve nerve and muscle strength. Working with a doctor or physiotherapist to build an appropriate exercise plan can help avoid fatigue or injury^[13].

Social life and relationships

CIDP can impact social activities and relationships. Acknowledging physical limitations and setting boundaries is important. For example, if symptoms make certain activities difficult, it’s acceptable to join friends for parts of social gatherings that are manageable^[12].

Because CIDP is rare and symptoms can vary, explaining the condition to friends and family may help them better understand the challenges faced. Some symptoms, like burning pain or tingling, are invisible, so describing these experiences can improve understanding and support^[12].

It’s important to keep people updated about changing abilities and symptoms, as they might not know how someone is currently feeling^[12].

chronic relapsing polyneuropathy, chronic inflammatory demyelinating polyradiculoneuropathy