Chronic inflammatory demyelinating polyneuropathy

A neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms

Chronic inflammatory demyelinating polyneuropathy
CIDP Histopathology Teased fibre.jpg
Histopathology showing a teased nerve fiber from a patient with CIDP.
Synonyms	CIDP
Specialty	Neurology
Symptoms	Progressive muscle weakness, numbness, tingling, fatigue, loss of reflexes, difficulty walking
Complications	Permanent nerve damage, muscle atrophy, mobility impairment
Usual onset	Gradual, often over weeks or months
Duration	Chronic; may be relapsing-remitting or progressive
Types	Typical CIDP, multifocal CIDP (Lewis-Sumner syndrome), pure motor or pure sensory CIDP
Causes	Autoimmune attack on the myelin sheath of peripheral nerves
Risk factors	Associated with other autoimmune diseases; may occur post-infection or post-vaccination
Diagnosis	Nerve conduction studies, electromyography, lumbar puncture, nerve biopsy
Differential diagnosis	Guillain–Barré syndrome, multiple sclerosis, diabetic neuropathy, Charcot–Marie–Tooth disease
Prevention	None known
Treatment	Corticosteroids, intravenous immunoglobulin (IVIG), plasma exchange, immunosuppressive therapy
Medication	Prednisone, IVIG, azathioprine, rituximab
Prognosis	Varies; many improve with treatment, some may have long-term disability
Frequency	Estimated 1–8 per 100,000 people
Deaths	Rare; usually due to complications rather than the disease itself

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms. It is considered an autoimmune disorder, where the body's immune system mistakenly attacks the myelin sheath, the protective covering of the nerves. CIDP is related to Guillain-Barré syndrome, but it progresses more slowly and lasts longer.

Pathophysiology

CIDP is caused by inflammation of the peripheral nerves and nerve roots, leading to the destruction of the myelin sheath. This demyelination disrupts the normal conduction of electrical impulses along the nerves, resulting in muscle weakness, sensory disturbances, and loss of reflexes. The exact cause of the autoimmune response in CIDP is not fully understood, but it is believed to involve both cellular and humoral immune mechanisms.

Symptoms

The symptoms of CIDP can vary widely among individuals, but they typically include:

• Progressive muscle weakness, particularly in the arms and legs
• Tingling or numbness in the extremities
• Loss of deep tendon reflexes
• Fatigue
• Difficulty walking
• Sensory ataxia

The progression of symptoms can be gradual, occurring over several months, and may fluctuate in severity.

Diagnosis

Diagnosing CIDP involves a combination of clinical evaluation, laboratory tests, and electrophysiological studies. Key diagnostic criteria include:

• Clinical presentation of progressive weakness and sensory dysfunction
• Electromyography (EMG) and nerve conduction studies showing demyelination
• Elevated protein levels in the cerebrospinal fluid (CSF) without a significant increase in white blood cells

A nerve biopsy may be performed in some cases to confirm the diagnosis by demonstrating demyelination and inflammation.

Treatment

The primary goal of treatment for CIDP is to reduce inflammation and suppress the immune response. Common treatment options include:

• Corticosteroids such as prednisone
• Intravenous immunoglobulin (IVIG)
• Plasmapheresis (plasma exchange)
• Immunosuppressive drugs such as azathioprine or rituximab

Physical therapy and occupational therapy are also important components of managing CIDP, helping to maintain muscle strength and improve functional abilities.

Prognosis

The prognosis for individuals with CIDP varies. Some patients experience significant improvement with treatment, while others may have a more chronic course with relapses and remissions. Early diagnosis and treatment are crucial for improving outcomes and preventing long-term disability.

Related pages

• Guillain-Barré syndrome
• Peripheral neuropathy
• Autoimmune disease

External links

• CIDP at NINDS
• CIDP at GBS/CIDP Foundation International