Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a neurological disorder characterized by progressive weakness and impaired sensory function in the arms and legs.
What is Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?
CIDP is a neurological disorder characterized by progressive weakness and impaired sensory function,
including loss of reflexes.
CIDP is considered an autoimmune disorder, in which the immune system begins to attack the peripheral nerves,
although the exact mechanisms by which this happens are still not clearly defined.
CIDP is the most common treatable chronic neuropathy worldwide. The number of new cases of CIDP is
between 1 and 2 per 100,000 people per year, but since the disease can be present in a person for years prior to diagnosis,
the prevalence (accumulation of cases over time) may be as high as 9 per 100,000.
CIDP most commonly occurs between ages 40 and 60, though it can also affect children and the elderly. It is more common in men than in women.
CIDP is one of a spectrum of acquired demyelinating neuropathies that differ in their time course and clinical features. CIDP has a similar presentation to that of Guillain-Barre Syndrome (GBS), except that GBS is acute and usually peaks within 4 weeks, whereas CIDP is chronic (8 weeks or more), and may be progressive or relapsing.
What are the symptoms of CIDP?
The classic clinical symptoms of CIDP include:
progressive, symmetric weakness of the arms and legs, especially in proximal muscles
loss of sensation (numbness); abnormal sensations (tingling, buzzing)
progressive or relapsing loss of reflexes (areflexia)
unsteady gait (usually resulting from sensory loss and weakness)
Diagnostic tests typically find:
electrophysiological features of demyelination (e.g., reduced conduction velocities)
high protein levels in spinal fluid analysis
inflammation and demyelination observed in nerve biopsy samples
The symptoms of CIDP are caused by damage to the myelin sheath of peripheral nerves, but
the particular nerves affected can vary from one patient to another.
The course of CIDP varies widely. Some may have a monophasic course of CIDP followed by recovery,
while others may have progressive or relapsing-remitting courses.
There are several phenotypes within CIDP, suggesting that it may be a spectrum of related
conditions more than a discrete disease.
improve functional status (reduce disability and handicap)
maintain long-term remission, if possible
Standard treatments for CIDP include:
Intravenous immunoglobulin (IVIG) therapy at high doses
IVIG is the only treatment that has official approval in the US, Canada, and Europe for treating CIDP.
Corticosteroids, which are similar to naturally occurring anti-inflammatory hormones made by the body
Corticosteroids often improve strength, are conveniently taken by mouth, and are inexpensive, but side effects can limit long-term use.
Plasma exchange (PE) or plasmapheresis (PLEX), which may help remove harmful substances in the plasma
Each of these treatments has been demonstrated to be superior to placebo in randomized controlled trials,
and between 50 and 80% of patients experience improvement, at least initially. Unfortunately, these agents lack a durable response in many patients, so chronic treatment is necessary. Side effects, expense, time,
and logistical hassles of these treatment options can become more problematic with long-term treatment.
Without treatment, about 30% of CIDP patients will progress to wheelchair dependence. Early recognition and proper treatment can prevent loss of nerve function and avoid a significant amount of disability.
Post-treatment life depends on whether the disease was caught early enough to benefit from treatment options.
The gradual onset of CIDP can delay diagnosis by several months or even years, resulting in significant nerve damage that may limit and delay the response to therapy.
The chronic nature of CIDP requires long-term care of patients. Accommodations in the home may be needed to facilitate daily living activities.
Physical and occupational therapy may help maintain range of motion, prevent joint distortions and muscle shrinkage, and assist in gait retraining. Moderate exercise can help reduce fatigue and maintain muscle strength.