Chronic inflammatory demyelinating polyneuropathy (CIDP)

Chronic inflammatory demyelinating polyneuropathy (CIDP) is classified as a neurological disorder characterized by symmetrical progressing weakness and impaired sensory (ability to feel pain and other sensations) function in the legs and arms. As the condition progresses significant disability, reduction in mobility, and lower quality of life can occur. 

CIDP is a slowly developing autoimmune disease in which the body’s immune system attacks the myelin that insulates and protects your body’s nerves. This prolonged (progresses over more than 8 weeks)  reaction causes damage to the myelin sheath of the peripheral nerves. These nerves are located outside your brain and spinal cord. They relay information between your brain and the rest of your body. 

The location of the damage is important to remember because CIDP can sometimes be confused with another condition called multiple sclerosis which affects the central nervous system but includes the brain and spinal cord. CIDP is however closely related to Guillain-Barre syndrome in its pathology and symptoms. The main difference between the two is the length of time it takes for symptoms to progress. 

Whom does it affect?

CIDP can occur at any age but is more common in adults and men. It occurs in 8 out of 100,000 people with rates increasing in advanced age. The average age of diagnosis is 40–60 years. Symptoms include but are not limited to:

  • tingling or numbness 
  • weakness of the arms and legs, gait imbalance
  • loss of deep tendon reflexes (areflexia)
  • fatigue
  • painful paresthesias

Diagnosis of CIDP is challenging because there are shared symptoms with other chronic neuropathies. Misdiagnosis is common due to this. Diagnostic tools for this condition include:

  • Cerebrospinal fluid (CSF) evaluation which checks spinal fluid for infection or other immune components. CSF evaluation is the most sensitive test for this disease.
  • Nerve conduction studies which can help identify nerve damage.  
  • Nerve biopsy which could show signs of inflammation of the nerves.

After diagnosis, prognosis for patients varies widely among individuals. Some may have an acute case of CIDP followed by complete recovery, while others may have a partial recovery followed by many relapses. Early diagnosis helps to prevent permanent damage and loss of mobility. 

Treatment options for CIDP include:

  • Intravenous immunoglobulin (IVIg) therapy – Drugmaker Pfizer produces this product called Immune Globulin Intravenous [Human] – ifas (PANZYGA) which has been approved by the U.S. FDA in 2018. This medication is administered every three weeks over a period of 6 months. 
  • Corticosteroids such as prednisone – which are a type of anti-inflammatory drugs and are used for a period of three to six months. Patients are cautioned against long term use of steroids due to their side effects. 
  • Immunosuppressant drugs such as azathioprine, cyclophosphamide, ciclosporin, and methotrexate. 
  • Plasmapheresis (plasma exchange) given daily or every other day until symptoms resolve.  

*the above have been shown to be effective and are considered to be first line treatment options

  • Physiotherapy which may help to regain muscle strength, function, and mobility. It may also help to reduce the atrophy of muscles and tendons and distortions of the joints.

By Nadia Bhatti

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