AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

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• Introduction
• Clinical
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• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

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Background

The term chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been used to identify patients with a chronically progressive or relapsing symmetric sensorimotor disorder with cytoalbuminologic dissociation and interstitial and perivascular endoneurial infiltration by lymphocytes and macrophages. In many ways, CIDP can be considered the chronic equivalent of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), the most common form of Guillain-Barré syndrome (GBS).

A number of variants of CIDP have been described that have immune or inflammatory aspects and electrophysiologic and/or pathologic evidence of demyelination in common. No consensus exists on the best approach to the nomenclature of these disorders. CIDP is a major subset of chronic acquired demyelinating polyneuropathies (CADP). In this context, CIDP is considered when patients have a symmetric proximal and distal motor predominant disorder.

CIDP variants include patients with predominantly sensory symptoms, those with a distal symmetric disorder (DADS), those with multifocal sensorimotor neuropathy or sensorimotor mononeuropathy multiplex with prominent conduction block (also known as Lewis-Sumner neuropathy), and those with CIDP with associated CNS demyelination or with other systemic disorders.

The following disorders are considered distinct from CIDP because they have specific pathophysiologic features and respond to treatments differently than do patients with CIDP: Demyelinating neuropathies associated with immunoglobulin M (IgM) paraproteins, including those with anti–myelin-associated glycoprotein (MAG) antibodies; polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome; and multifocal motor neuropathy.

Pathophysiology

CIDP is presumed to occur because of immunologic antibody-mediated reaction along with interstitial and perivascular infiltration of the endoneurium with inflammatory T cells and macrophages. The consequence is a segmental demyelination of peripheral nerves.

Human leukocyte antigens Dw3, DRw3, A1, and B8 occur more frequently in patients with CIDP than in the healthy population.

Cytoalbuminologic dissociation is a characteristic finding in cerebrospinal fluid (CSF) pointing to nerve root involvement. Occasionally, CSF studies reveal mild lymphocytic pleocytosis and elevation of gamma globulin level, but this is observed most frequently in HIV-positive patients.

Frequency

International

CIDP is uncommon. The prevalence of CIDP is difficult to ascertain but estimates have ranged from 0.8-1.9 cases per 100,000 population.

Mortality/Morbidity

CIDP most commonly has an insidious onset and either chronic progressive or relapsing course. Occasionally, complete remissions occur. Quadriplegia, respiratory failure, and death have been described but are rare.

Race

No racial predilection has been identified.

Sex

Both sexes are affected. Of CADP variants, multifocal motor neuropathy has a male predominance of at least 2:1 based on a survey of the largest case series.

Age

CIDP may occur at any age, but it is more common in the fifth and sixth decades. Relapsing course is associated with younger age of patients (third and fourth decades). CIDP has been described in childhood.

CLINICAL

Section 3 of 11  

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History

CIDP most frequently starts insidiously and evolves slowly, either in a slowly progressive (more than 60% of patients) or relapsing manner (approximately one third of patients), with partial or complete recovery between recurrences.

• Periods of worsening and improvement usually last weeks or months. Patients with a younger age of onset are said to have a higher frequency of relapsing course.
• Because of the insidious onset of CIDP, documenting precipitating illnesses or events is very difficult. However, preceding infection has been reported infrequently. Both respiratory and gastrointestinal infections have been cited, but no causative organism has been identified.
• Initial symptoms include weakness of the limbs, both proximal and distal, with proximal muscles affected at least as severely as distal.
• Sensory symptoms are common, such as tingling and numbness of hands and feet, but usually motor symptoms predominate.
• Only a small proportion of patients (approximately 16%) have a relatively acute or subacute onset of symptoms, with subsequent steadily progressive or fluctuating course.
• Children usually have a more precipitous onset of symptoms.
• Most experts consider the necessary duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made.
• Autonomic system dysfunction can occur; in such a case, the patient would complain of orthostatic dizziness, problems with bowel and bladder functions, and cardiac problems.

Physical

Pertinent findings are limited to the nervous system, except for cases of CADP associated with other diseases, as already mentioned. Depending on the associated systemic disorder, abnormalities on physical examination may be found in multiple organ systems. Patients should be examined in detail for signs of autoimmune, inflammatory, and neoplastic conditions.

• Cranial nerves
• • Cranial nerves may be involved, particularly CN VII, with paralysis of both upper and lower facial muscles. Diplopia can occur with the involvement of CN III, IV, or VI. Rarely, bulbar muscles (eg, palate, tongue) can be affected.
  • Papilledema with pseudotumor cerebri syndrome (eg, headaches, transient visual obscurations, pulsatile tinnitus, visual field defects) are observed rarely in patients with CIDP and are due to a very high CSF protein level (usually >1000 mg/mL).
• Gait frequently is abnormal.
• Type of gait depends on location of weakness and degree of proprioceptive loss. With significant weakness in the lower extremities, patients may walk with steppage (ie, high elevation of both feet to compensate for weakness of foot dorsiflexors) or a slapping gait (ie, due to deficit of proprioception in the feet).
• Children are said to have more profound gait abnormalities than adults.
• Motor system
• • Usually relatively symmetric weakness of both proximal and distal muscles is present in upper and lower extremities.
  • Muscle tone can be normal or decreased. Hypotonia, atrophy, and fasciculations may be present.
• Deep tendon reflexes: Reflexes characteristically are diminished or absent even in regions with only mild weakness.
• Sensory system
• • Large-diameter, heavily myelinated fibers are affected most severely, leading to proprioceptive and vibratory deficits.
  • Loss or decrease of pain (ie, pinprick) and temperature sensations is less common.
  • Stocking-glove distribution of sensory deficits is typical.
  • Neuropathic pain in affected extremities may occur.
• Coordination: Patients may have sensory ataxia with positive Romberg sign due to damage to the large nerve fibers that convey proprioception.
• Pathological reflexes: Pathological reflexes (eg, Babinski, Chaddock, Oppenheim) usually are not observed in patients with CIDP.

Causes

CIDP is most frequently an idiopathic illness, but it has been known to occur with several conditions. In those cases, the associated condition is included in the main diagnosis (eg, CIDP with systemic lupus erythematosus, CIDP with HIV infection) to separate those cases from the idiopathic variety. Most reported conditions associated with CIDP are listed below.

• CIDP associated with other disorders cannot be distinguished clinically from isolated CIDP except where mentioned in this section. The disease mechanisms for all these disorders are not known. They appear to be immunologic; in some instances, antibody-mediated mechanisms have been shown to play a role.
• Vasculitis does not seem to be involved; physiologic and pathologic differences can distinguish the multifocal variants of CADP from vasculitic mononeuritis multiplex. Vasculitic neuropathies cause wallerian degeneration with minimal signs, if any, of segmental demyelination on biopsy and electromyographic studies (EMG). The multifocal variants of CADP have prominent conduction block and slowing—hallmarks of segmental demyelination.
• HIV infection: In these patients, mild lymphocytic pleocytosis and increased gamma globulin level in the CSF are seen frequently. CIDP has been observed with early disease and later on in the course of AIDS.
• Hodgkin lymphoma: Neuropathy associated with Hodgkin lymphoma is not caused by direct infiltration of the peripheral nerves but is a consequence of the autoimmune cascade that occurs with this disease; the mechanism is not completely clear.
• Paraproteinemias and/or plasma cell dyscrasias
• • CADP is seen with monoclonal gammopathies (eg, monoclonal gammopathy of unknown significance [MGUS]), most frequently gammopathy of IgM. Evidence exists to suggest that CADP with IgM MGUS has specific clinical and electrophysiologic characteristics. Patients usually have predominance of distal sensory symptoms that are greater than motor symptoms. Conduction slowing on nerve conduction testing is accentuated in distal nerve segments. Fifty percent of patients with IgM-associated neuropathies have antibodies directed against MAG, a protein found in noncompact myelin of peripheral nerves. Whether any clinical difference exists between patients with IgM gammopathy without anti-MAG antibodies and those with anti-MAG antibodies is not clear. In both cases, the response to immunosuppressive and/or immunomodulatory treatment is poor. However, encouraging reports describe a response to rituximab, a monoclonal antibody directed against B cells.
  • Some paraproteinemias occur as isolated phenomena and some are by-products of malignant cells, as in the cases of Waldenström macroglobulinemia and myeloma. In myeloma-associated neuropathy, the abnormal paraprotein usually consists mostly of lambda light chain component. Combination of osteosclerotic myeloma, organomegaly, endocrinopathy, M protein, sensorimotor neuropathy, and pigmentary skin changes is referred to as POEMS syndrome. In POEMS syndrome, the M protein is typically immunoglobulin G (IgG).
  • The association of CIDP with IgG or immunoglobulin A (IgA) gammopathy is less clear. IgG paraproteins can occur in 5% of the population and it is unclear that the incidence of IgG paraproteins is excessive in patients with demyelinating neuropathy. Patients with CIDP and IgG or IgA paraproteins have identical clinical and electrophysiologic features to patients with CIDP and no paraprotein. Response to treatment is also the same.
• Multiple sclerosis: Reports describe CNS white matter changes in patients with CIDP. Whether a true association exists between CIDP and multiple sclerosis remains unclear.
• Systemic lupus erythematosus
• Chronic active hepatitis (B or C): CIDP associated with hepatitis should be differentiated from cryoglobulinemic vasculitis. The latter causes either symmetric distal sensorimotor polyneuropathy or mononeuropathy multiplex but on pathologic examination shows wallerian degeneration and not the segmental demyelination seen in CIDP.
• Inflammatory bowel disease: CIDP has been described in association with Crohn disease and other inflammatory bowel conditions, although no direct correlation between the 2 afflictions is known. The mechanism of development of CIDP is presumed to be an autoimmune abnormality that is also causing the primary problem in inflammatory bowel disease, although the details are not known.
• Diabetes mellitus: Whether an increased incidence of CIDP occurs in patients with diabetes mellitus remains unclear. The most recent literature has not corroborated earlier reports of an association.
• Pregnancy: Pregnancy is known to exacerbate CIDP. Worsening usually occurs in the third trimester or in the postpartum period.

DIFFERENTIALS

Section 4 of 11  

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Other Problems to be Considered

Variants of CADP

Multifocal motor neuropathy
Chronic ataxic polyneuropathy
Polyneuropathy associated with monoclonal gammopathies
Multifocal sensorimotor demyelinating neuropathy with persistent conduction block (ie, Lewis-Sumner syndrome)
CIDP associated with diabetes mellitus
Chronic inflammatory sensory polyneuropathy
Hereditary Motor and Sensory Neuropathies

WORKUP

Section 5 of 11  

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Lab Studies

• Test CSF on all patients in whom CIDP is suspected. Protein level is increased significantly in 80% of patients (usually between 50 and 200 mg/dL, but can be higher); 10% of patients also have mild lymphocytic pleocytosis ( <50 cells) and increased gamma globulin (usually associated with HIV infection).
• CBC count, sedimentation rate, antinuclear antibody, biochemistry profile, and serum and urine immunoelectrophoresis are necessary to exclude important associated systemic disorders.
• In certain instances, genetic testing may be helpful. Examples include patients with positive family history, very insidious symmetric course of the disease, or some atypical features, including lack of treatment effect. In such cases, genetic testing for Charcot-Marie-Tooth disease might be indicated. Hereditary neuropathy with predisposition to pressure palsies can be suspected and tested for in some patients. Charcot-Marie-Tooth disease type 1X and adult-onset Charcot-Marie-Tooth disease type 1B have been confused with CIDP.

Imaging Studies

• Imaging studies can provide supportive evidence of CIDP.
• MRI of the spine with gadolinium enhancement may show enhancement of nerve roots.

Other Tests

• EMG is a critical test to determine whether the disorder is truly a peripheral neuropathy and whether the neuropathy is demyelinating. Findings of a demyelinating neuropathy are as follows:
• • Multifocal conduction block or temporal dispersion of compound muscle action potential (see Image 1)
  • Prolonged distal latencies and dispersion of the distal compound motor action potential
  • Variable conduction slowing to less than 70% of normal
  • Absent or prolonged F wave latencies (see Image 2)
• As the disease progresses, patients tend to develop secondary axonal degeneration.
• Reports exist of a predominantly axonal neuropathy with clinical course and response to treatment similar to those of CIDP. Most cases of axonal neuropathy are not immune or inflammatory. However, some patients with an aggressive axonal neuropathy have been treated effectively with immunosuppressive and/or immunomodulatory therapy, raising the question of whether a chronic axonal inflammatory neuropathy, akin to the acute axonal variants of GBS, may be present. The relationship of these chronic axonal variants to CIDP is unclear.

Procedures

• Peripheral (sural) nerve biopsy is considered as supportive evidence of CIDP.
• • Consider biopsy for those patients in whom the diagnosis is not completely clear, when other causes of neuropathy (eg, hereditary, vasculitic) cannot be excluded, or when profound axonal involvement is observed on EMG.
  • Some experts recommend biopsy for most patients prior to initiating immunosuppressive therapy, but more recent guidelines no longer recommend biopsy.

Histologic Findings

Tissue collected on biopsy of the sural nerve may demonstrate evidence of interstitial and perivascular infiltration of the endoneurium with inflammatory T cells and macrophages with local edema. Evidence exists of segmental demyelination and remyelination with occasional onion bulb formation, particularly in relapsing cases (see Image 3). Some evidence of axonal damage also is observed, with loss of myelinated nerve fibers. The inflammatory infiltrate with neutrophil infiltration is observed in only a minority of patients.

TREATMENT

Section 6 of 11  

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Medical Care

Untreated, CIDP is characterized by accumulating disability that requires physical and occupational therapy, orthotic devices, and long-term treatment. Close follow-up care with a physician knowledgeable in the field is necessary to adjust treatment.

Surgical Care

Surgical and orthopedic consultation may be required for sural nerve biopsy or in severe disease with joint deformities that require corrective surgery.

Consultations

• Consult a neurologist for evaluation, diagnosis, and treatment of CIDP and possible associated diseases.
• Consult a physical medicine and rehabilitation specialist for physical and occupational therapy and evaluation for orthotic devices.
• Other consultations (eg, internist, hematologist, oncologist, rheumatologist) may be necessary if associated diseases are present. Some treatments may be best accomplished with the assistance of a hematologist or rheumatologist.

Diet

No specific diet has been proven beneficial in CIDP.

Activity

Activity generally depends on the level of disability. Encourage physical therapy and an active lifestyle.

MEDICATION

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If associated conditions are identified (HIV infection, lupus, paraproteinemia, lymphoma), treat them accordingly. The mainstay of treatment is immunosuppressive or immunomodulatory intervention.

Drug Category: Immunomodulatory/immunosuppressive agents

These agents include intravenous immunoglobulin (IVIg), plasmapheresis, prednisone, azathioprine, methotrexate, mycophenolate, cyclosporine, and cyclophosphamide. Their use is based on the proposed pathogenesis of CIDP as an immune-mediated condition.

Drug Category: Antiepileptic medications

In patients with CIDP, a variety of medications is used for treatment of neuropathic pain. Antiepileptic medications are quite effective. The 2 most frequently used medications, gabapentin and carbamazepine, are described.

Drug Category: Tricyclic antidepressants

These medications are used frequently for the treatment of neuropathic pain. The most traditionally used medication, amitriptyline, is discussed.

FOLLOW-UP

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Further Inpatient Care

• Most care is delivered on an outpatient basis, although patients may have to be admitted for a short stay for the initiation of plasmapheresis or IVIg treatment, because of complications of CIDP or treatment, or for inpatient physical therapy.

Further Outpatient Care

• Outpatient care consists of visits to specialists such as neurologists and physiatrists and of treatment visits for IVIg infusions or to the plasmapheresis unit.

In/Out Patient Meds

• Most medications are administered on an outpatient basis. IVIg can be administered as a home infusion or during an outpatient visit. An exception is plasmapheresis, which requires visits to a specialized pheresis center.

Complications

• If the disease becomes severe, swallowing and breathing functions can be affected. Aspiration pneumonia, atelectasis, and respiratory failure can occur.
• If autonomic function is involved, GI motility and bladder function can be abnormal. Orthostatic hypotension and cardiac conduction defects can occur.
• As already discussed, complications of treatment also must be considered.

Prognosis

• Some have suggested that patients with the relapsing disease have a better prognosis than patients with the chronic progressive course. Approximately 70% of patients are said to make relatively good recovery from their relapses, and close to 90% of patients respond to initial immunosuppressive therapy. Some patients do not respond to the usual treatments and accumulate significant disability. Some patients have only a short treatment effect and become treatment dependent.

Patient Education

• Refer to physical and occupational therapists and to a physiatrist for optimal outpatient therapy, orthotic devices, and adaptation at home.

MISCELLANEOUS

Section 9 of 11  

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Medical/Legal Pitfalls

• CIDP and its variants are uncommon disorders and can be difficult to diagnose and treat. Frequently, patients with this disorder require referral to a medical center specializing in neuropathic disorders.

Special Concerns

• Associated conditions need to be excluded and treated appropriately.

MULTIMEDIA

Section 10 of 11  

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Media file 1:  Electromyography of a patient with chronic inflammatory demyelinating polyradiculoneuropathy illustrating conduction block, temporal dispersion of compound muscle action potential, prolonged distal latencies, and slowed conduction.
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Media file 2:  Prolonged F wave latencies (normal is <31).
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Media file 3:  Electron micrograph of the peripheral nerve of a patient with chronic inflammatory demyelinating polyradiculoneuropathy. Note "onion bulb" formation in the myelin sheath of the nerve fibers due to continuous demyelination and remyelination. Courtesy of A. Sima, MD, Department of Pathology, Wayne State University.
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Media type:  Image

REFERENCES

Section 11 of 11

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Article Last Updated: Jan 8, 2007