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Chronic Inflammatory Demyelinating Polyneuropathy

CIDP

Chronic inflammatory demyelinating polyneuropathy (CIDP) or chronic relapsing polyneuropathy is an acquired form of neuropathy that is characterized by non-length dependent axonal neuropathy and proximal limb weakness (feature distinguishing it from distal axonal polyneuropathies). It is an immune disorder affecting the peripheral nervous system that causes inflammation of nerve roots and axons and disrupts the myelin covering of axons. The demyelination process is symmetrical in distribution and the course of disease varies in individuals. 

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Presentation

CIDP, in most cases, occurs in patients during the forth to sixth decade of life. Since auto-immunity to nerve components may arise in any age, younger age groups may also present with CIDP. The disease is slow in onset and progression, some consider an eight week or greater duration of symptoms for a case to be diagnosed as CIDP. 16% of cases present with acute or sub-acute onset and the majority of these are children. Some may recover spontaneously and in others, disease progresses in the following patterns:

  • Slowly progressive (linear fashion)
  • Step-wise progressive
  • Fluctuating [10] [11] [12] [5]

Symptoms may affect the motor, sensory, autonomic system, may arise in combinations (two or all three components of nervous systems involved in varying proportions). Motor system involvement may develop solitary (pure motor variant) or in combination with sensory symptoms, latter being most common. Some patients may present with sensory features alone (sensory variant), however, their electrophysiological features are the same as others and they also respond to the same treatment. 

Classical CIDP

In the classical form, CIDP presents with the following features:

  • Symmetrical limb weakness, involving both proximal and distal [13] extremities
  • Sensory loss (usually starting in the legs)

History and clinical features

The main symptom is weakness of muscles around the shoulders, hips or phalanges. The patient presents with a history of difficulty in walking, climbing and/or of falls. In case of weakness of the hand muscles, fine movements may be affected to varying degrees. Patients may complain of reduction in mental dexterity (for example problems with buttoning a shirt). However, this problem is of muscular origin. Deep reflexes are reduced or absent and inspection may reveal muscle atrophy. Proximal limb weakness is a core feature of CIDP, differentiating this disorder from other neuropathies, which often involve more distal portion of limbs. This shows that the demyelination is non-length dependent in case of CIDP. CIDP affects the proximal musculature as severely as the distal muscles of the limb. Sensory abnormalities include pain or tingling, burning or abnormal prickling sensations. Sometimes painful parasthesia may occur [14] [15] [16] in areas supplied by affected nerves. These symptoms may be accompanied by gait disturbances, a feature seen with sensory ataxia. Limb involvement is usually symmetrical and evolves slowly over weeks (about 2 months). CIDP may arise without an antecedent illness. Other features include dysphagia, diplopia, fatigue and clumsiness.

In the head and neck region, CIDP involves face and neck flexor muscles but typically spares extra-ocular muscles. Pure motor involvement is seen in 10% of cases. Some may present with autonomic involvement, and in these cases, main symptoms comprise bladder and bowel dysfunction, orthostatic dizziness or cardiac abnormalities. Relapses and recurrences are common, and in these cases, nerve impairment is step-wise progressive, each episode adding to the previous level. 

Chronic inflammatory demyelinating polyradiculoneuropathy begins with a slow onset. In 60% of the patients, the disease undergoes a progressive course and another 10% suffer from relapses, with complete recovery or progressive deterioration of nerve function with each relapse. The interval between the episodes may last weeks or months.

Weakness
  • Sometimes leading to need to limp and feeling weak. 6 3.0 Upper-body 2015 Upper body weakness, intermittent pain, control loss. 6.1 2.0 hand pain & weak General hand & wrist weakness and pain. 6.2 1.0 arms & shoulders General arm & shoulder weakness and[cidplog.com]
  • She remained stable for 6 years until developing proximal and distal weakness. Nerve conduction studies showed multiple conduction blocks. She developed quadriparesis despite first-line treatment for CIDP.[ncbi.nlm.nih.gov]
  • At age 44 years, paresthesias and dysesthesias of both lower legs and mild right lower leg weakness additionally developed.[ncbi.nlm.nih.gov]
  • The patient's neuropathy and muscle weakness improved on treatment with intravenous immunoglobulin (IVIG) and high-dose steroids.[ncbi.nlm.nih.gov]
  • CASE: A 32-year-old male patient was admitted with the complaints of abdominal pain and diarrhea for three years and an ascending weakness of all four extremities since two years.[ncbi.nlm.nih.gov]
Pain
  • By 2015 lower legs & feet in continuous pain 4 1.0 Right Foot Pain Intermittent pain on top of right foot, internal pain, ankle weakness: restricting ability to walk. 5 1.0 Left Leg Use & Pain Excessive pain and cramp tendency in left leg, especially[cidplog.com]
  • Entering 2017 I find pain management a dominating need: movement, exercise, stretching as well as keeping "overheated" seem helpful in controlling my CIDP nerve & muscle pain.[cidplog.com]
  • Low-dose vaporized cannabis significantly improves neuropathic pain. Journal of Pain , 14(2), 136-48. Retrieved from .[medicalmarijuanainc.com]
  • Abstract A 50-year-old villager with chronic inflammatory demyelinating polyradiculoneuropathy developed pain abdomen, diarrhea, and vomiting after 8 weeks of prednisolone (40 mg/day) therapy.[ncbi.nlm.nih.gov]
  • 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 be intermittent.[en.wikipedia.org]
Fatigue
  • We present a patient with CIDP in whom fatigue remained the only symptom, hereby stressing the importance of adding this disease to the differential diagnosis of fatigue.[ncbi.nlm.nih.gov]
  • Fatigue Loss of reflexes Tingling Numbness Pain Weakness Paralysis The degree of symptom severity varies with each individual. It may take months or a year for severe symptoms to develop.[peripheralneuropathycenter.uchicago.edu]
  • It often presents with symptoms that include tingling or numbness (beginning in the toes and fingers), weakness of the arms and legs, loss of deep tendon reflexes (areflexia), fatigue, and abnormal sensations.[ninds.nih.gov]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: January 30, 2019[patientslikeme.com]
  • Symptoms include: Tingling or numbness (beginning in the toes and fingers) Weakness of the arms and legs Loss of deep tendon reflexes (areflexia) Fatigue Abnormal sensations CIDP is closely related to Guillain-Barre syndrome and it is considered the chronic[smartdraw.com]
Falling
  • CASE PRESENTATION: A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year.[ncbi.nlm.nih.gov]
  • […] canal vestibular function in 21 CIDP patients (15M/6F) by the video head impulse test and postural stability with a battery comprising the modified Clinical Test of Sensory Integration and Balance, the Berg Balance Scale, the Dynamic Gait Index, the Fall[ncbi.nlm.nih.gov]
  • My gait got clumsy and I was falling frequently. My feet felt like they were on fire, the pain was excruciating. Over time, I lost over 50 pounds. Down to 165 and I thought I was dying.[globalgenes.org]
  • There are other less well established variants most of which would fall into the category of CIDP. Living with CIDP Post-treatment life depends on whether the disease was caught early enough to benefit from treatment options.[gbs-cidp.org]
  • Thomason (DD-760) USS Joseph Strauss (DDG-161) USS Mahan (DLG-11) USS Mansfield (DD-728) USS Maury (AGS-16) USS Montrose (APA-212) USS Newell (DER-322) USS Niagara Falls (AFS-3) USS Okanogan (APA-220) USS Orleck (DD-886) USS Perkins (DD-877) USS Picking[hillandponton.com]
Difficulty Walking
  • The most aggressive treatment is only given if you have difficulty walking or if symptoms don't allow you to care for yourself or work.[ufhealth.org]
  • The most aggressive treatment is only given if you have difficulty walking, breathing, or if symptoms don't allow you to care for yourself or work.[medlineplus.gov]
  • Patients commonly experience foot drop and difficulties walking.[medicalmarijuanainc.com]
  • Diagnosis of CIDP is based on the symptoms of the patient: Symptoms such as loss of sensation (numbness), abnormal sensation (tingling and pain), loss of reflexes, and weakness (difficulty walking, foot drop) Tests such as nerve conduction and EMG (usually[gbs-cidp.org]
Dyspnea
  • After 2 months, severe extremity pain and dyspnea developed again, and steroid pulse therapy was initiated. CONCLUSION: Besides GBS, acute-onset CIDP can occur with hantavirus and HBV coinfection.[ncbi.nlm.nih.gov]
  • […] getting better with IVIG, CIDP pain, GBS/CIDP Foundation, sensory, autonomic, motor, strength, ALS, neuron, neurological, neurologist, CIDP muscle weakness, assessing IVIG success, CIDP Canada, CIDP specialist, CIDP doctor, breathing, shortness of breath, Dyspnea[cidplog.com]
Dysphagia
  • Other features include dysphagia, diplopia, fatigue and clumsiness. In the head and neck region, CIDP involves face and neck flexor muscles but typically spares extra-ocular muscles. Pure motor involvement is seen in 10% of cases.[symptoma.com]
  • Her symptoms progressed to dysphagia requiring feeds via naso-gastric tube, and eventual gastromy tube. % cell population Pre-rituximab Post-rituximab Normal Range CD3 85 77 56-85% Activated T cells 41 19 0-15% CD19 2 9-29% CD4 39 20 32-64% CD8 43 53[autoimmunediseases.imedpub.com]
Vascular Disease
  • Except in the presence of IgA deficiency, renal failure, and vascular disease or cardiac insufficiency complications, IVIg is generally well tolerated and easy to administer; therefore, IVIg rather than PE should be the initial therapy.[doi.org]
Skin Lesion
  • This led to remission of the skin lesions in two patients and to marked improvement in the other two patients. IVIg had to be restarted in two patients because of a relapse of CIDP which led to worsening of the skin lesions.[ncbi.nlm.nih.gov]
Muscle Weakness
  • BACKGROUND AND PURPOSE: Muscle atrophy is generally mild in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) compared with the severity and duration of the muscle weakness.[ncbi.nlm.nih.gov]
  • Abstract An 11-year-old boy presented with progressive proximal muscle weakness and areflexia. He also had scoliosis with right convexity in the thoracic spine. Nerve conduction studies showed demyelination with conduction blocks.[ncbi.nlm.nih.gov]
  • The article presents a boy with progressive generalized muscle weakness and areflexia since the age of two, developed after viral infection. Electromyoneurography showed severe neurogenic lesion, with myopathic pattern in proximal muscles.[ncbi.nlm.nih.gov]
  • The patient's neuropathy and muscle weakness improved on treatment with intravenous immunoglobulin (IVIG) and high-dose steroids.[ncbi.nlm.nih.gov]
  • Although the patient noticed little muscle weakness, an electrophysiological study demonstrated slow conduction velocities and prolonged distal latencies, with definite conduction blocks in the median, ulnar, and tibial nerves.[ncbi.nlm.nih.gov]
Numbness of the Feet
  • CIDP often causes limb weakness but may just cause problems with balance from numbness in the feet and legs. This is one of the few types of polyneuropathy where the underlying cause be treated.[phoenixneurology.com]
Diplopia
  • Other features include dysphagia, diplopia, fatigue and clumsiness. In the head and neck region, CIDP involves face and neck flexor muscles but typically spares extra-ocular muscles. Pure motor involvement is seen in 10% of cases.[symptoma.com]
  • One had a relapsing course after initial presentation with weakness and diplopia that spontaneously improved without diagnosis. Subsequently, this patient had more typical relapses and was confirmed to have CIDP.[annalsofian.org]
  • Initial diplopia is usually followed by internal ophthalmoplegia and bilateral facial weakness. Approximately one quarter of patients with MFS have some weakness and sensory changes of the extremities.[clinicaladvisor.com]
  • Diplopia can occur with the involvement of CN III, IV, or VI. Rarely, bulbar muscles (eg, palate, tongue) can be affected.[emedicine.medscape.com]
  • Signs of cranial nerve (CN) involvement (eg, facial muscle paralysis or diplopia) 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[emedicine.medscape.com]
Polyradiculoneuropathy
  • , Chronic Inflammatory Demyelinating/blood Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/complications Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/drug therapy Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/immunology[ncbi.nlm.nih.gov]
  • Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst.[doi.org]
  • Abstract A 50-year-old villager with chronic inflammatory demyelinating polyradiculoneuropathy developed pain abdomen, diarrhea, and vomiting after 8 weeks of prednisolone (40 mg/day) therapy.[ncbi.nlm.nih.gov]
  • Elizabeth's Medical Center, 736 Cambridge Street, Boston, MA 02135, USA. kengorson@comcast.net Abstract Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune disorder of the peripheral nervous system.[ncbi.nlm.nih.gov]
  • Electrodiagnostic studies were consistent with a chronic demyelinating polyradiculoneuropathy with conduction blocks. After he developed systemic symptoms, tissue biopsies revealed granulomatous disease. Symptoms improved with steroid therapy.[ncbi.nlm.nih.gov]
Tingling
  • Fatigue Loss of reflexes Tingling Numbness Pain Weakness Paralysis The degree of symptom severity varies with each individual. It may take months or a year for severe symptoms to develop.[peripheralneuropathycenter.uchicago.edu]
  • It often presents with symptoms that include tingling or numbness (beginning in the toes and fingers), weakness of the arms and legs, loss of deep tendon reflexes (areflexia), fatigue, and abnormal sensations.[ninds.nih.gov]
  • The symptoms are of a slowly progressive numbness and tingling that usually starts in the feet, but later spreads to the legs and hands.[bcm.edu]
  • This type of neuropathy is usually characterized by a stabbing or burning sensation or itching or tingling.[phoenixneurology.com]
  • Symptoms include: Tingling or numbness (beginning in the toes and fingers) Weakness of the arms and legs Loss of deep tendon reflexes (areflexia) Fatigue Abnormal sensations CIDP is closely related to Guillain-Barre syndrome and it is considered the chronic[smartdraw.com]
Ataxia
  • Gov't MeSH terms Adrenal Cortex Hormones/therapeutic use Adult Aged Aged, 80 and over Ataxia/blood Ataxia/complications Ataxia/drug therapy Ataxia/immunology* Autoantibodies/blood Autoantibodies/immunology* Biomarkers, Pharmacological/blood* Case-Control[ncbi.nlm.nih.gov]
  • Twenty-eight patients (74%) presented with sensory ataxia, 16 (42%) showed tremor, 5 (13%) presented with cerebellar ataxia associated with nystagmus, 3 (8%) had demyelinating lesions in the CNS, and 20 of 25 (80%) had poor response to IV immunoglobulin[ncbi.nlm.nih.gov]
  • On admission, the patient exhibited bifacial, extremity weakness, ataxia, impaired sensation, and areflexia. Electrophysiology and nerve biopsy suggested demyelination. Spinal fluid revealed increased protein content.[ncbi.nlm.nih.gov]
  • […] the video head impulse test and postural stability with a battery comprising the modified Clinical Test of Sensory Integration and Balance, the Berg Balance Scale, the Dynamic Gait Index, the Fall Efficiency Scale, and the International Cooperative Ataxia[ncbi.nlm.nih.gov]
  • This syndrome, a dominantly transmitted hereditary ataxia with tremor, was first described in 1926 by Gustave Roussy and Gabrielle Lévy.[movementdisorders.org]
Paresthesia
  • Abstract The authors present the case of a 65-year-old woman who was admitted for paraparesis and paresthesias in the inferior limbs.[ncbi.nlm.nih.gov]
  • RESULTS: She had distal numbness and paresthesias, minimal distal weakness and impaired vibratory sensation. She had anti-MAG antibodies, negative Western blot, and lacked a monoclonal gammopathy. There were prolonged distal motor latencies.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: A 17-year-old male had experienced three separate attacks of motor weakness and paresthesia of all four extremities over a period of 7 months.[ncbi.nlm.nih.gov]
  • At age 44 years, paresthesias and dysesthesias of both lower legs and mild right lower leg weakness additionally developed.[ncbi.nlm.nih.gov]
  • DDx: Thickened, enlarged nerve roots: Lymphoma, sarcoidosis, neurofibromatosis type 1 Brain lesions: MS and other infectious and inflammatory disorders Rx: Immunotherapy A 35-year-old woman with bilateral foot drop and recurrent upper extremity paresthesias[ajnr.org]
Limb Weakness
  • He was admitted with bilateral lower limb weakness which was ascending in nature. He eventually required ventilation. It was initially thought to be isoniazid-neuropathy.[ncbi.nlm.nih.gov]
  • We described a young girl with CIDP underlying type 2 diabetes mellitus, presenting with progressive numbness and limb weakness, who was initially misdiagnosed to have DPN. Finally immunosuppressive therapy got good response.[ncbi.nlm.nih.gov]
  • Although at 25 years of age she had already been diagnosed with limb weakness associated with chronic inflammatory demyelinating polyneuropathy (CIDP), she had never experienced double vision until her latest condition.[ncbi.nlm.nih.gov]
  • BACKGROUND: Ventilatory involvement is rarely reported in chronic inflammatory demyelinating polyneuropathy (CIDP), but small prospective studies showed frequent involvement of phrenic nerves, which is usually overshadowed by severe limb weakness.[ncbi.nlm.nih.gov]
  • The disease presents with progressive development of proximal and distal limb weakness; muscles of hands and feet may be involved, affecting fine movements.[symptoma.com]

Workup

Important diagnostic tests that can be employed for the diagnosis of CIDP include:

  • Cerebrospinal fluid (CSF) examination
  • Nerve conduction study (NCS)
  • Nerve biopsy
  • Magnetic resonance imaging (MRI)

CSF examination

CSF examination is one of the most important and most sensitive studies for the diagnosis of CIDP. Elevated proteins are found in CSF in around 94% of patients with normal white cell count [17] [18].
An elevated protein and a reduced white blood cell count, called albumino-cytologic dissociation and demyelination findings on electrodiagnostic testing are found in both Gullian Barre syndrome and CIDP. A WBC greater than 10/mm3 in CSF should raise the suspicion of human immuno-deficiency virus (HIV) infection, leukemia and lymphoma, as well as other autoimmune diseases like sarcoidosis and Lyme disease. A parasitic disease should be kept in mind if the CSF examination reveals an elevated white cell count.

Lumbar puncture shows an elevated CSF pressure. Oligoclonal bands are identified in 65% of the cases [19]. Similarly lymphoproliferative disorders (myelomas and lymphomas) cause an elevation of the paraprotein level in immunofixation, or an elevation of serum lambda and kappa free light chains (light chains of immunoglobulins are raised in response to myelomas as osteosclerotic myelomas).

Nerve conduction study

A reduced conduction velocity is suggestive of CIDP, on nerve conduction study and electromyography (EMG).

Nerve biopsy

This is rarely required. In cases where biopsy is taken, nerve specimen shows segmental demyelination and other characteristic features of CIDP.

Magnetic resonance imaging

This is also an important diagnostic test. On contrast enhancement, enlargement on the T2 sequence of the spinal cord nerve roots is seen. It may also show signs of inflammation.

White Matter Lesions
  • Magnetic resonance imaging showed presence of an intracranial white matter lesion and enhancing, thickened cranial and spinal nerves. This disorder has been described very infrequently in the radiology literature.[ncbi.nlm.nih.gov]

Treatment

CIDP is a treatable, acquired neuropathy and an early intervention is recommended in order to hamper the possible nerve loss and axonal damage. Treatment follows these principles:

  • Reduction of symptoms
  • Maintaining long term remission
  • Improving the functional status of the patient

Conventional therapy includes corticosteroids (CS), immuno-suppressive drugs, intravenous immunoglobulins (IVIG) and plasma exchange (PE) therapy or plasmapheresis. Standard therapy (CS, IVIG and PE) [20] [1] [2] has shown improvement in 50-80% of the patients.

Corticosteroids

Corticosteroids may be used alone, or in case of insufficient response, in conjunction with other therapies e.g. immunosuppressive drugs. In many cases, patients respond to corticosteroids like prednisone, but some experience adverse effects of long term use of corticosteroids, hence combined therapies are commonly employed. 

Immuno-suppressive drugs

Azathioprine, cyclosporine, mycophenolate , methotrexate and cyclophosphamide are used in CIDP patients. These agents inhibit auto-immune response in patients with CIDP, but they also have an untoward effect of depressing the patient's immunity against infectious agents. Long term trials have also shown them to be less effective. Hence patients on immuno-suppressive drugs alone, or those receiving combined treatment of corticosteroids and immuno-suppressive drugs, require prophylaxes and prevention against various opportunistic organisms.

Plasma exchange/ plasmapheresis

The purpose of this procedure is to remove offending or harmful material that is present in the plasma. Many autoimmune disease treatments include this procedure in which plasma and its constituents are removed.

The patient's blood is removed and the corpuscular part is separated, leaving behind plasma, that is exchanged with normal plasma, then the blood cells are transfused back. Since normal plasma does not contain agglutinogen, an immune reaction does not occur. However, this therapy is effective only for a few weeks and the patient may require intermittent plasma exchange, in symptomatic or active phases of the disease.

Intravenous immunoglobulins

IVIG are also effective for a short term and improve the immune status of the patient. Initial treatment with IVIG may require very high doses, especially in those with severe signs and symptoms at the onset of the disease. 

Physiotherapy

Physiotherapy is a rehabilitation approach that improves muscle strength and mobility, and hence preserves and improves the residual function of muscle and movement at joints. It prevents distortion of joints. It also minimizes shrinkage in muscles or tendons, and impedes atrophy.

Prognosis

CIDP is a treatable form of acquired neuropathy. Loss of nerve axons can be prevented by early intervention. The disease can occur either in bouts with prompt recovery or in multiple bouts with partial recovery between the relapses. Prognosis varies from patient to patient and depends on the following factors:

  • Age of patient: An earlier age of onset is associated with a long term prognosis.
  • Mode of onset: Patients with acute or sub-acute onset show a better prognosis.
  • Time to initiate the treatment.
  • Response to treatment: Patients who respond better to corticosteroids are more likely to have a better prognosis and experience less complications as compared to non-responders. An earlier treatment usually correlates with better results. However, some patients treated with corticosteroids become dependent, Others may only perceive a short term effect. Young patients respond better to treatment than elderly.
  • Area of weakness: Proximal involvement is associated with better prognosis than distal weakness [7] [8].
  • Strength of auto-immune response affects the prognosis. Negative prognostic factors are a progressive course of disease and axonal degeneration [9].

Relapses may occur, but about 70% of the patients recover from these. However, some may be left with numbness or partial weakness in the limbs. About 90% of the patients respond well to immuno-suppressive therapy. Gait instability and loss of limb function are significant causes of morbidity in patients with CIDP. Complete remission is likely in patients presenting with acute or sub-acute onset, symmetrical limb involvement, conduction abnormalities predominantly in distal extremities and good response to corticosteroids. These factors can also predict long term outcome in patients.

Etiology

There is a strong evidence in favor of an auto-immune etiology, however the precipitating cause for auto-immunity is not known. This is due to the insidious pattern of the onset of the disease. Both respiratory and gastrointestinal illnesses have been cited to be causative in CIDP, but there is a lack of evidence suggestive of a specific infectious agent.

Epidemiology

CIDP can affect any age group, though it is commonly seen between the fourth and sixth decade of life. The prevalence of this rare disease is around 5 to 7 cases per 100,000 population. CIDP is more common in males than females and its average age of onset is 50 years. The disease represents 20% of initially undiagnosed cases of neuropathy and 10% of cases seeking out neuromuscular clinics. The relapse rate is found to be higher in young patients, in the third and fourth decade of life. Chronic inflammatory polyradiculoneuropathy, a variant of CIDP, can occur at any age, but is more common in the 40 to 60 years age group.

Sex distribution
Age distribution

Pathophysiology

CIDP is an auto-immune disease which occurs in response to cross-reacting antigens; molecules in the body that resemble some foreign protein. As compared to a normal immune response that does not attack the body's natural proteins, auto-immunity occurs when a response against harmful foreign agents creates self-reactive auto-antibodies or lymphocytes. Although incompletely understood, pathogenesis of CIDP is thought to involve both humoral and cell mediated immune responses against the myelin sheath [1] [2]. The antigen is unknown, however, like various other demyelinating diseases, CIDP presents microscopically with segmental demyelination of nerve fibers. The disease affects peripheral nerves, where T-cells and macrophages are seen to invade the epineurium and endoneurium. Another view is that these macrophages infiltrate the interstitial and perivascular spaces of the endoneurium of nerves. Macrophages sometimes invade the basement membrane of cells causing the stripping of myelin lamellae at various places along the length of the nerve fiber [3] [4], hence giving rise to segmental demyelination. Nerve biopsy specimens reveal an ongoing process of inflammation and demyelination, followed by remyelination [5] [6].

Classical CIDP:

The classical form of the disease is characterized by proximal limb weakness, sensory disturbances and loss of reflexes with a progressive or relapsing course. Electrophysiological features in this form of disease characterize:

  • Demyelination
  • Reduced conduction velocities
  • Temporal dispersion (hallmark of demyelinating diseases)
  • Prolonged F-wave latency
  • Prolonged distal motor latency
  • Conduction block

Nerve biopsy shows areas of inflammation, demyelination and re-myelination in a skipping pattern. Laboratory tests reveal an albumino-cytological dissociation in CSF.

Chronic inflammatory demyelinating polyradiculoneuropathy:

This variant involves an immune response consisting of auto-antibodies, inflammatory T-cells and macrophages which infiltrate the interstitial and perivascular spaces of the endoneurium.

Prevention

The disease is auto-immune in nature and arises in response to an unknown antigen. A prior disease may play an inciting role, however, there is a lack of scientific data in recognition of the antigen. Prevention at primary level is insignificant. Rehabilitation and disability limitation is possible and can be adequately achieved through consultation of a neurologist who evaluates the treatment of the disease and/or inciting pathologies. The patient may have to be referred to other consultants including hematologist, rheumatologist or oncologist (in case of paraneoplastic syndrome) for abolishing or treating the cause. A physical therapist and rehabilitation specialist may apply various therapies to mitigate and restore an optimum function in the affected limbs. Orthotic devices and exercises are required in patients for physiotherapy and vocational rehabilitation. 

Summary

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological autoimmune disorder that affects the myelin sheath (the layer of fatty tissue formed by Schwann cell, over axons that lie outside the CNS). This disease represents 20% of initially undiagnosed cases of neuropathy and 10% of cases seeking out neuromuscular clinics. Myelin is present in excess in fast conduction fibers and contributes to high speed with which action potential travels in these and other peripheral nerve fibers. A disruption of the myelin sheath in CIDP affects the speed of conduction. The disease progressively leads to a loss of axon fibers due to the damage of the protection afforded by myelin. This disease is considered to be a chronic counterpart of Gullian Barre syndrome, an acute auto-immune demyelinating disorder. Chronic inflammatory demyelinating polyradiculopathy is a variant of CIDP.

The period for the diagnosis of CIDP depends upon the severity of the immune response. As a general criterion, a period of signs and symptoms greater than 8 weeks is considered diagnostic. CIDP presents in a symmetrical fashion and affects males more commonly than females. This abnormality presents with muscle weakness, fatigue, ataxia/gait abnormalities, numbness, tingling sensations or pain, loss of deep reflexes or paralysis. The age of onset varies and spontaneous resolution can occur in few patients while others require steroidal or other medical therapy and rehabilitation interventions. 

Patient Information

Chronic inflammatory demyelinating polyneuropathy is a chronic, acquired and treatable neurological disease. At the onset of the disease, symptoms develop slowly, over a period of two months. This disease is comparable to Gullian Barre syndrome, except that in latter, onset is sudden and paralysis ensues in ascending fashion. CIDP occurs due to an auto-immune reaction against the myelin sheath. The myelin sheath protects the nerves and maintains the high speed of conduction in the nerve fibers. Both humoral and cell mediated responses are initiated and nerve fibers undergo patchy, segmental demyelination. The severity of inflammation varies in individuals. No specific inciting cause has yet been identified. 

The disease presents with progressive development of proximal and distal limb weakness; muscles of hands and feet may be involved, affecting fine movements. The patient presents with complaints like difficulty in getting up, climbing stairs or buttoning a shirt. Apart from motor function, sensory disturbances also occur and include a prickling or burning sensation, pain, numbness and ataxia (an imbalanced gait). The disease progresses in slowly ascending or relapsing fashion. Recovery is possible. However, recurring episodes may lead to progressive and permanent loss of nerve function. Diagnosis of CIDP is made by CSF examination, nerve biopsy, MRI and nerve conduction study. 

Treatment includes medical treatment and rehabilitation. Corticosteroids and immuno-suppressive agents are recommended to control immune response. Response to treatment differs in patients. Physical, occupational and psychological rehabilitation is necessary to regain an optimum level of functioning in patients.

References

Article

  1. Köller H, Kieseier BC, Jander S, Hartung HP. Chronic inflammatory demyelinating polyneuropathy. N Engl J Med. 2005 Mar 31;352(13):1343-56.
  2.  Dalakas MC; Medscape. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol. 2011 Aug 16;7(9):507-17. doi: 10.1038/nrneurol.2011.121.
  3. Hafer-Macko CE, Sheikh KA, Li CY, Ho TW, Cornblath DR, McKhann GM, Asbury AK, Griffin JW. Immune attack on the Schwann cell surface in acute inflammatory demyelinating polyneuropathy. Ann Neurol. 1996 May;39(5):625-35.
  4. Matsumuro K, Izumo S, Umehara F, Osame M. Chronic inflammatory demyelinating polyneuropathy: histological and immunopathological studies on biopsied sural nerves. J Neurol Sci. 1994 Dec 20;127(2):170-8.
  5. Dyck P, Prineas J, Pollard J. (1993) Chronic inflammatory demyelinating polyneuropathy. In: Dyck P, Thomas P, Griffin J, Low P, Podulos J, editors. (eds), Peripheral Neuropathy. Philadelphia, PA: Saunders.
  6. Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--First Revision. J Peripher Nerv Syst. 2010 Mar;15(1):1-9. doi:10.1111/j.1529-8027.2010.00245.x. Erratum in: J Peripher Nerv Syst. 2010
    Dec;15(4):373.
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Last updated: 2018-06-21 16:47