2. Learning Objectives
By the end of this presentation, learners will be able to:
• Define the term Chronic inflammatory demyelinating polyneuropathy
• Enlist its causes and risk factors
• Enumerate the clinical manifestations
• Elaborate its management
3. Chronic inflammatory demyelinating
• It is an acquired immune-mediated
inflammatory disorder of the peripheral
• The disorder is sometimes called chronic
relapsing polyneuropathy (CRP) or chronic
inflammatory demyelinating poly
radiculoneuropathy (because it involves the
• Chronic inflammatory demyelinating polyneuropathy (or
polyradiculoneuropathy) is considered an autoimmune disorder
destroying myelin, the protective covering of the nerves.
• CIDP is closely related to Guillain-Barre syndrome (GBS).
• With GBS, once treated, most people recover fairly quickly.
• CIDP, on the other hand, tends to be a longer-term problem. In rare
cases, people who don’t recover from GBS may develop CIDP.
5. Signs and Symptoms
• CIDP typically starts insidiously and evolves slowly, in either a slowly
progressive or a relapsing manner, with partial or complete recovery
between recurrences; periods of worsening and improvement usually
last weeks or months.
• Most experts consider the necessary duration of symptoms to be
greater than 8 weeks for the diagnosis of CIDP to be made.
• Symptoms reported include the following:
• Preceding infection (infrequent)
• Initial limb weakness, both proximal and distal
• Sensory symptoms (eg, tingling and numbness of hands and feet)
6. Signs and Symptoms
• Motor symptoms (usually predominant) muscle
fasciculations, "vibration" feelings, loss of balance,
general muscle cramping and nerve pain
• In children, usually a more quick onset of symptoms
• Symptoms of autonomic system dysfunction (eg,
• Pertinent physical findings are limited to the nervous
system, except when the condition is associated with
7. Signs and Symptoms
• Such findings may include the following.
• Signs of cranial nerve (CN) involvement (eg, facial muscle paralysis or
• Gait abnormalities
• Motor deficits (eg, symmetric weakness of both proximal and distal
muscles in upper and lower extremities)
• Diminished or absent deep tendon reflexes
• Sensory deficits (typically in stocking-glove distribution)
• Impaired coordination
8. Typical diagnostic tests include:
• Electrodiagnostics – electromyography (EMG) and nerve conduction study.
• Serum test to exclude other autoimmune diseases.
• Lumbar puncture .
• Sural nerve biopsy; biopsy is considered for those patients in whom the
diagnosis is not completely clear, when other causes of neuropathy (e.g.,
hereditary, vasculitic) cannot be excluded, or when
profound axonal involvement is observed on EMG.
• Ultrasound of the peripheral nerves may show swelling of the affected
• MRI can also be used in the diagnosic workup
• Early treatment is key. It can help prevent nerve damage. That can
help stop symptoms from becoming severe.
Treatment may include:
• Corticosteroids. These medications bring down inflammation and
slow the immune system.
• Intravenous immunoglobulin (IVIG). Your doctor may give you
injections of concentrated antibodies from healthy people to slow
your body’s immune response.
• Plasma exchange (PE). This treatment involves receiving a part of
blood called plasma through an IV to slow down your immune
• Immunotherapy. These drugs interrupt your immune system to help
stop it from attacking the myelin.
• Stem cell transplant. In rare cases, your doctor may inject healthy
stem cells (either yours or donated by someone else) to "reset" your
• Your doctor may also recommend physical therapy.
• Moderate exercise may give you more energy.
• You may recover entirely from CIDP. Some people do, but they may
have symptoms from nerve damage, such as numbness and
weakness, for the rest of their lives.