Chronic Inflammatory Demyelinating Polyneuropathy
Also known as: CIDP, Chronic relapsing polyneuropathy
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder in which the immune system attacks the myelin sheath that insulates peripheral nerves, slowing or blocking the signals between the brain and the body. It causes progressive weakness and numbness, typically on both sides of the body and in both the arms and legs, developing over at least eight weeks. CIDP is considered the chronic counterpart of Guillain-Barré syndrome and may follow a steadily progressive or relapsing course. Untreated, it can lead to significant disability, but it is one of the more treatable neuropathies: immune therapies such as intravenous immunoglobulin, corticosteroids, and plasma exchange help most people regain strength.
At a Glance
- Common symptoms
- Progressive, symmetrical weakness in the legs and arms, affecting both close-to-body and distal muscles, Tingling, numbness, or loss of sensation in the hands and feet, Difficulty walking, climbing stairs, or rising from a chair
- Main treatments
- Intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin as first-line therapy, Corticosteroids, either oral or in pulsed intravenous doses, Plasma exchange to remove harmful antibodies, especially for severe or rapidly worsening disease
- Category
- Neurological · Rare; the most common treatable chronic autoimmune neuropathy, more frequent in older men
- See a doctor if
- Weakness or numbness in the arms and legs that is progressing over weeks
Symptoms
Causes
- An autoimmune attack on peripheral nerve myelin; the exact trigger is unknown
- Antibody- and cell-mediated inflammation of the nerve roots and nerves
- In a small subgroup, specific antibodies against proteins at the nodes of Ranvier
- Possible association with preceding infections in some cases
Risk Factors
- Being male and older adult, though CIDP can occur at any age
- Diabetes, which is more common in people with CIDP
- Other autoimmune conditions
- Certain blood disorders such as monoclonal gammopathy
- HIV infection in rare cases
Diagnosis
- Neurological examination showing symmetrical weakness, sensory loss, and reduced reflexes progressing over two months or more
- Nerve conduction studies and electromyography showing demyelination
- Lumbar puncture, which typically shows raised protein with normal cell counts
- Blood tests to exclude diabetes, vitamin deficiencies, paraproteins, and other causes of neuropathy
- MRI of the nerve roots or nerve ultrasound, and occasionally nerve biopsy, in uncertain cases
Treatment
- Intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin as first-line therapy
- Corticosteroids, either oral or in pulsed intravenous doses
- Plasma exchange to remove harmful antibodies, especially for severe or rapidly worsening disease
- Immunosuppressant medications or newer targeted therapies for people who do not respond to standard treatment
- Physiotherapy and occupational therapy to maintain strength, balance, and independence
- Supportive: regular, moderate exercise within tolerance to prevent deconditioning
- Supportive: fall prevention at home, orthotics or walking aids when needed, and attention to foot care given reduced sensation
Prevention
- There is no known way to prevent CIDP
- Seek prompt assessment of progressive weakness or numbness, since early treatment limits nerve damage
- Adhere to maintenance treatment schedules to prevent relapses
- Keep diabetes and other health conditions well controlled
- Stay physically active to preserve muscle strength
When to See a Doctor
- Weakness or numbness in the arms and legs that is progressing over weeks
- Increasing difficulty walking, climbing stairs, or using your hands
- Falls or loss of balance
- Rapidly worsening weakness or trouble breathing or swallowing (seek urgent care)
- Return of symptoms after a period of improvement, which may signal relapse
Frequently Asked Questions
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