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Patients usually present with a history of weakness, numbness, pain and difficulty in walking. Some patients may have sudden onset of back pain or neck pain radiating down the extremities. Usually diagnosed as radicular pain. These symptoms are usually progressive and may come and go.

On examination the patients may have weakness, loss of  deep tendon reflexes( rarely increased or normal).

There may be atrophy of muscles , fasiculations and loss of sensation.

Some patients present with a ALS like clinical picture.

Laboratory findings


The major laboratory tests for CIDP are electrophysiologic studies, CSF examination, and nerve biopsy.

  • EMG is useful to differentiate other causes of muscle weakness, particularly anterior horn cell diseases, other forms of peripheral neuropathies, disorders of neuromuscular transmission, and primary muscle diseases. In typical CIDP, the nerve conduction studies confirm demyelination as the predominant process. These findings include: (a) a non-uniform reduction in nerve conduction velocities in two or more motor nerves in a range considered to be indicative of a demyelinating disorder; (b) the presence of conduction block or abnormal temporal dispersion in at least one motor nerve; (c) prolonged distal latencies in at least two nerves; (d) absent F waves or prolonged minimum F wave latencies in at least two motor nerves.

     

  • CSF Examination: Cytoalbuminologic dissociation (elevated CSF protein >45 in the absence of a high cell count <10) is characteristic of CIDP. CSF pleocytosis is rare except in HIV-associated CIDP. An elevated IgG index and IgG synthesis rate is consistent with the immune-related nature of CIDP. An elevated Q-albumin shows an impairment of the blood-brain barrier, which in CIDP is at the level of the inflamed nerve root sheaths within the thecal sac.

Not all the patients will have lab abnormalities. In many the EMG/NCV findings will not show that they have CIDP yet they will still have the disease.

Is EMG/NCV Insensitive in the Diagnosis of CIDP?

The clinical profile of chronic inflammatory demyelinating polyneuropathy (CIDP) is variable. Nerve conduction studies are essential for the diagnosis of CIDP; however, current electrophysiologic criteria for CIDP may not be sensitive. These authors analyzed results of nerve conduction studies and nerve biopsies in 8 patients with CIDP who did not fulfill standard electrophysiologic criteria (of 44 patients with CIDP confirmed by nerve histology). Two standard electrophysiologic criteria were used: one proposed by an ad hoc committee of the AAN (Neurology 1991; 41:617) and another by the Inflammatory Neuropathy Cause and Treatment Group (Ann Neurol 2001; 50:195).

The main electrophysiologic abnormalities were those of simple axonopathy in 7 patients; the other patient had almost normal electrophysiologic results. In contrast, examination of nerve biopsies in all 8 patients revealed substantial loss of myelinated fibers; in addition, frequent histologic findings were naked axons, thinly myelinated fibers, and various degrees of onion bulbs. These histology findings were identical to those of the 36 other CIDP patients. The authors conclude that many patients with unrecognized CIDP are erroneously classified by electrodiagnosis as having chronic axonal neuropathy and that nerve biopsy should be considered to further investigate a chronic idiopathic neuropathy.

 

 
: Neurology. 2002 Dec 24;59(12 Suppl 6):S2-6.  
 
Diagnosis of CIDP.

Latov N.

Peripheral Neuropathy Center, Weill Medical College of Cornell University, New York, NY 10022, USA.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease that targets the myelin sheaths of peripheral nerves. In clinical practice the diagnosis is often difficult to make because of the clinical heterogeneity of the disease, its multifocality and predilection for proximal nerve segments, and the limitations of our electrophysiologic and pathologic techniques. Although there are rather stringent research criteria for selecting patients to clinical trials, there are no generally agreed-on clinical diagnostic criteria for CIDP, and application of the research criteria to routine clinical practice would miss the diagnosis in a majority of patients. Because of this uncertainty, the prevalence of CIDP is greatly underestimated, and patients are often left untreated despite progression of their disease. However, given what is known about the clinical presentation and pathophysiology of CIDP, patients with neuropathy of otherwise unknown etiology are more likely to have CIDP than idiopathic axonal neuropathy, and warrant a trial of therapy if they have nerve conduction velocities below the lower limits of normal, prolongation of F-waves beyond the normal range, or presence of conduction block or temporal dispersion. A favorable response to therapy, consisting of stabilization or improvement of the neuropathy, would confirm the diagnosis


Living with CIDP

Coping with uncertainty

CIDP may follow a pattern of relapses and remissions or a more gradual increase in symptoms. During a relapse new symptoms occur or old symptoms which had previously subsided may recur. Relapses can last for several months and may be relatively slight or quite severe. A remission occurs when the symptoms experienced during the relapse disappear either partially or completely over a period of time which may last weeks, months or even years.

CIDP does not always have these patterns of being 'better' or 'worse'; sometimes symptoms can gradually increase over a period of many years and it may be difficult to identify `better' or `worse' times.

It is impossible to predict with certainty how CIDP is going to affect an individual in the future. The pattern of relapses and remissions varies greatly from person to person. A period of relapse can be very disturbing but many people make a good recovery. Coping with this uncertainty is one of the most difficult aspects of 'living with CIDP'. You should try and accept this variability without getting too worried about it.

You and your family and friends

A diagnosis such as CIDP of a chronic condition with an uncertain prognosis, may well throw a strain on family and other relationships. You may find it difficult to accept help when you need it, or your family and friends may feel that they cannot give help or become overprotective toward you. It is difficult to carry on family life as if nothing has happened. Everyone concerned may have to take on new roles. If you and your family and friends are able to speak openly and honestly with each other you will probably find that you are able to help each other through difficult times with the result that the bonds are strengthened.

Instinctively children are aware that something is wrong and that you are worried. It is important that their questions are answered as and when they occur. Older children can become surprisingly mature and a source of strength. Trying to keep your problems to yourself will not spare them any anxiety.

You and your doctor

It is important to build a good relationship with your doctors, both GP and specialist. Because of the rarity of the illness, many doctors will not have encountered it before. The symptoms are difficult to describe and may not be taken seriously at first. Each case of CIDP is different, and relapses, if they occur, may bring new symptoms and problems. Because of the variability in severity and progression of the disease, the doctor will not be able to give you a definite prognosis.

Although there is not one single overall treatment for CIDP, there is much that your doctor can do to help. Each person responds in different ways to different treatments. A period of experimentation with different treatment regimes is likely to be necessary in order to discover the regime which is most appropriate for you.

Attitude to life

It is important to be as positive as possible about everything. Our emotional state plays a large part in our health and although the norms of life may have to change for a while, the majority of patients with CIDP can expect a good quality of life.

Modification of ones lifestyle may be necessary but it is better to emphasise strengths, undertaking what can be achieved rather than failing to achieve the impossible. It is a natural reaction to become frustrated but the acceptance and understanding of the problem is more than half the battle. Addressing the problems of CIDP can be seen as bringing a new challenge.

Being positive can take a lot of effort, determination and even courage and can be helped by a similar attitude in those that support and help you.

What you can do to help yourself

You should follow as healthy a lifestyle as possible. This will help to prevent other illnesses and infections which have been shown to trigger relapses.

A nutritionally balanced diet will ensure you are getting all the vitamins and minerals you require. There is no evidence of any special dietary requirements for CIDP sufferers. It is sensible to keep your weight down, since more weight is more difficult for weak legs to carry. (see our diet page for more info).Diet

Regular exercise is important for overall health and should be taken according to individual limits and capabilities. Over exertion causes fatigue. However a little regular exercise will help to minimize muscle wastage and give you a good feeling of well-being. Any form of exercise that you enjoy and can comfortably follow will prove beneficial. Ask your physiotherapist to show you.

Adequate rest periods are essential to avoid fatigue. Stress and tension may irritate the symptoms of CIDP and therefore relaxation will allow you to unwind and `recharge'.

Some patients find it useful to record their progress in a diary so that they can discuss changes of treatment in the light of their recent progress. Others find that this can increase their anxiety about the disease and is counter productive.

 

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