Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

What is Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)?

CIDP or Chronic Inflammatory Demyelinating Polyradiculoneuropathy is a condition that affects the nervous system as a result of the immune system attacking it. It results in slowly progressive weakness and loss of feeling in the legs and arms. The symptoms are of a slowly progressive numbness and tingling that usually starts in the feet, but later spreads to the legs and hands. The patients complain of weakness usually starting in the legs, but soon involving the arms. With further involvement of the sensory system, other modalities of sensations, such as balance, are affected and the patients complain of inability to walk or maintain balance in the dark. There is usually no bowel or bladder involvement. On rare occasions, cranial nerves are involved and their symptoms range from difficulty in swallowing to double vision and numbness involving the face. Cognitive skills are not affected by CIDP.

What causes Chronic Inflammatory Demyelinating Polyradiculoneuropathy?

It can start after food poisoning, enteritis, vaccination, chemical exposure, injury, surgery & severe exercise. Stress is important factor in the immune dysfunction.

How is Chronic Inflammatory Demyelinating Polyradiculoneuropathy diagnosed?

The diagnosis of CIDP is suspected with a history of progressive sensory motor neuropathy. Physical examination consistent with distal sensory loss in the upper and lower extremities, in conjunction with motor weakness that can be more proximal than distal supports the clinical diagnosis.

Patients may present with pain, numbness or weakness. One of the early signs is that a patient has to use their hands to go upstairs or rise from the squatting position. Some have vasomotor symptoms like difficulty to maintain their blood pressure, burning sensations which may be misdiagnosed as Reflex Sympathetic Dystrophy. Even the Complex regional pain syndrome is more likely to be CIDP. Many patients are placed on chronic Narcotics which suppress the immune functions and end up making the disease even worse.

Common causes of neuropathy such as alcoholism, or medication induced neuropathies and vitamin deficiency need to be ruled out.  Diabetic neuropathy is more likely to be CIDP. The spinal tap may show a rise in the protein level of the spinal fluid. Electromyography with nerve conduction studies supports the diagnosis. Usually the picture is of slowing in the conduction velocities of the peripheral nerves. Sometimes when only the small fibers of the nerves are involved the EMG/NCV can be normal.

Patients maybe misdiagnosed as ALS. Some have CIDP with upper motor neuron involvement. If demyelination involves the cervical spinal region and there is peripheral nerve involvement in the legs. Physician examination will show increased reflex’s along with atrophy and fasciculation’s in muscles, thus leading to a diagnosis of ALS.  Dr King Engle at USC has helped these patients with IVIg.

Current standards to diagnose CIDP do not recommend a nerve biopsy: As the skip lesions of CIDP may or may not be seen in  Sural nerve biopsy. After biopsy the patient may have a permanent sensory deficit. Which may be worse then the original disease. We have seen patients who are worse off due to biopsy.

(Finding of inflammation on the nerve biopsy, although rare, definitely confirms the diagnosis. However, the absence of inflammation does not entirely rule it out. Findings of predominant demyelination on the nerve biopsy can be used to confirm the clinical presentation and suggest a diagnosis of CIDP.)

What is the treatment?

Treatment for CIDP is aimed at suppressing the immune system. First line treatment is usually with intravenous immunoglobulin. If this is not available, plasmapheresis (plasma exchange) or oral medications which suppress the immune system such as steroids may be used.

Physiotherapy may improve muscle strength, function and mobility, and minimize the development of contractures.

What is the prognosis?

The course of CIDP varies widely among individuals. Some may have a bout of CIDP followed by spontaneous recovery, while others may have many bouts with partial recovery in between relapses. The disease is a treatable cause of acquired neuropathy and initiation of early treatment to prevent loss of nerve cells is recommended. However, some individuals are left with some residual numbness or weakness.

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