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PubMed1994

[A pathogenic study of chronic inflammatory demyelinating polyradiculoneuropathy in a patient with hepatitis B infection].

We immunologically examined the pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy in a patient with HB hepatitis. A 41-year-old male clerk has been suffered from muscle weakness, tingling and numbness in the distal portion of all limbs. All symptoms were compatible with the typical patterns of chronic inflammatory demyelinating polyradiculoneuropathy. We examined the patient's serum and biopsied sural nerve, using histochemical and immunological techniques. We detected the band that reacted with anti-HBs antibody in the sural nerve in western blotting. The result indicated that HBs antigen was expressed on the peripheral nerves in the patient. There were no anti-peripheral nerve antibodies neither in the sural nerve or serum. There was no increase of immune complex in the serum. No deposition of immunoglobulins and complements were detected in the sural nerve. Immunoadsorption therapy had no effect on this patient, but administration of prednisolone improved his symptoms drastically. These findings suggest that a cytotoxic T cell may had played a more important role than humoral factors in this patient's nerve injury. Though the pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy remains unclear, our findings seem to be very interesting in that they go some way toward clarifying the pathogenesis of this disease.

Adult↗
PubMed1994

The spectrum of acquired demyelinating polyradiculoneuropathy.

Guillain-Barré Syndrome (GBS) is best viewed as a clinical syndrome which can have at least two clinical substrates: the commonest is an acquired demyelinating polyradiculoneuropathy and the other is an acute motor axonal neuropathy. An acute acquired neuropathy can also sometimes cause the Miller Fisher syndrome of ophthalmoplegia, areflexia and ataxia, and even more rarely a pure sensory neuropathy. The demyelinating form of GBS also forms one end of a spectrum which has Chronic Idiopathic Demyelinating Polyradiculoneuropathy (CIDP) at its other pole. CIDP usually produces a mixed sensory and motor, albeit predominantly motor deficit, but some patients have pure multifocal motor neuropathy (MMN) and other even less common patients have pure sensory CIDP. According to the definitions of international committees, the symptoms of GBS reach their nadir within 4 weeks and those of CIDP in not less than 8 weeks. The spectrum is completed by patients with a monophasic illness reaching its nadir in 4 to 8 weeks (subacute idiopathic demyelinating polyradiculoneuropathy (SIDP). In addition there are patients with discrete attacks of acute demyelinating neuropathy which have been called recurrent GBS. The differential diagnosis of acquired demyelinating neuropathy differs according to the position of the individual case on this spectrum. The pathogenesis of each case may depend on the nature of the autoantigen, the balance between T cell and antibody-mediated autoimmune mechanisms, and the tempo of the inflammatory process. These differences may explain the empirical observations that steroids are helpful in CIDP but not GBS. Removal of antibodies by plasma exchange or flooding the immune system with normal immunoglobulin is beneficial in GBS and some cases of CIDP.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMed2001

A case of acute inflammatory demyelinating polyradiculoneuropathy in early pregnancy.

Acute or chronic inflammatory demyelinating polyradiculoneuropathy is a rare disorder that presents with weakness, hyporeflexia, and sensory loss. Treatment consists of immunosuppression and/or plasma exchange. A woman in the first trimester of pregnancy presents with flaccid quadriplegia and numbness. Electromyography demonstrated a demyelinating polyneuropathy with active denervation. The diagnosis of acute inflammatory demyelinating polyradiculoneuropathy was made. She was treated with corticosteroids and plasmapheresis. Despite slow improvement, she decided to terminate the pregnancy at 18 weeks' gestation. At 1 year postpartum she is still in remission. Inflammatory demyelinating polyradiculoneuropathy should be considered in the differential diagnosis of every pregnant woman with new onset peripheral neuropathy. It is suggested that relapse occurs three times more common during pregnancy. Plasmapheresis is the recommended treatment.

Adult↗
PubMed2004

Fulminant acute inflammatory demyelinating polyradiculoneuropathy: case report and literature review.

BACKGROUND: The inflammatory polyradiculoneuropathies (Guillain-Barré Syndrome) represent a diverse spectrum of diseases, with variable pathophysiological mechanisms (demyelinating versus axonal), clinical manifestations (motor, sensory, and autonomic), presentation patterns, and degrees of severity. Fulminant cases of total body paralysis with loss of all cranial nerve reflexes have been described, whereby patients appear to have lost all central nervous system (CNS) function. METHODS: The case of a patient with a fulminant polyradiculoneuropathy in whom consideration was given to discontinuation of care and organ donation is presented. A positive oculocardiac response verified ongoing brainstem function and led to the diagnosis of an inflammatory polyradiculoneuropathy. The patient was successfully treated with plasmapheresis and intravenous immunoglobulins. CONCLUSION: The oculocardiac response may be a useful addition to the routine examination in patients who are suspected of being cerebrally dead or of having absent brainstem function, especially in cases where a central lesion or underlying disease is not evident to explain the condition. Additional study and validation of the oculocardiac response in these situations is proposed.

Electromyography↗
PubMedDate not supplied

[Immunochemical findings in the cerebrospinal fluid in patients with inflammatory demyelinating polyradiculoneuropathy].

The aim of the study was to check the hypothesis of involvement of the structure of the central nervous system on the basis of the analysis of the integrity of blood-brain barrier and the degree of IgG synthesis in the intrathecal space of patients with inflammatory demyelinization polyradiculoneuropathy. The study involved 27 patients with acute and 14 patients with chronic inflammatory demyelinization polyradiculoneuropathy. The analysis of liquor was performed in different stages of the disease. The results have shown that in the acute phase, in the phase of the maximal functional deficit there are signs of damaged integrity of the blood-brain barrier but without signs of increased intrathecal IgG synthesis. It has been concluded that there are no reliable signs of increased immunologic activity in the intrathecal space of patients with inflammatory demyelinization polyradiculoneuropathy.

Adult↗
PubMed2005

Multifocal motor sensory demyelinating neuropathy: inflammatory demyelinating polyradiculoneuropathy.

The authors present two cases that provide the first autopsy findings in multifocal acquired demyelinating sensory and motor neuropathy (MADSAMN). Both cases documented multifocal but asymmetric demyelinating neuropathy with rare axonal degeneration. One case clearly documented an inflammatory polyradiculoplexoneuropathy, confirming the inflammatory nature of this neuropathy. This study showed that MADSAMN is an inflammatory demyelinating polyradiculoneuropathy that shares histologic features observed in chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy (MMN), suggesting a similar immunopathogenesis for these entities.

Anti-Inflammatory Agents↗
PubMed1989

Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria.

Over a 10-year period, we followed up 60 patients (35 men and 25 women) with chronic inflammatory demyelinating polyradiculoneuropathy. Diagnosis was based on previously outlined criteria. Patients were treated in a uniform manner and the overwhelming majority, 56 (94.9%) of 59 treated patients, initially responded to immunosuppressive therapy. The time for initial improvement was 1.9 +/- 3.6 months while the time to reach a clinical plateau was 6.6 +/- 5.4 months. The course was monophasic in 32 patients (53.3%) and relapsing in 28 (46.6%). Despite the initial responsiveness, only 24 (40%) of 60 patients are in partial or complete remission, receiving no medication. Two patients died. We were unable to identify specific clinical or laboratory features at the time of diagnosis that predicted outcome. Our data analysis, along with previous reports, suggests that chronic inflammatory demyelinating polyradiculoneuropathy may be more heterogeneous than previously emphasized. In this light, we have proposed diagnostic criteria that allow for the heterogeneity but at the same time provide for a more consistent approach to better establish the natural history of this condition.

Biopsy↗
PubMed1977

Polyradiculoneuropathy accompanying procainamide-induced lupus erythematosus: evidence for drug-induced enhanced sensitization to peripheral nerve myelin.

Factors involved in the development of an insidious polyradiculoneuropathy in association with a procainamide-induced, lupuslike syndrome were explored. A 73-year-old man with this clinical syndrome had sural nerve changes consisting of loss of large myelinated fibers with evidence of remyelination and Schwann cell proliferation. The patient's lymphocytes showed marked incorporation of tritiated thymidine when cultured with either procainamide or extracts of human peripheral nerve myelin, and there was an enhanced response with the combination. We also found that procainamide-treated rats showed acceleration of lymphocyte sensitization to peripheral nerve myelin as judged by the early development of inhibition of macrophage migration and positive skin tests to extracts of peripheral nerve myelin. These studies suggest that procainamide can enhance lymphocyte sensitization to peripheral nerve myelin and may have predisposed this individual to development of a polyradiculoneuropathy.

Aged↗
PubMed2004

Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy.

Electrodiagnosis plays an important role in the early detection and characterization of inflammatory demyelinating polyradiculoneuropathies, because timely treatment reduces morbidity and disability. The challenge consists of defining electrodiagnostic criteria that are highly specific for primary demyelination but sufficiently sensitive to be useful in clinical practice. We compared 10 published sets of criteria in 53 patients with demyelinating Guillain-Barré syndrome (GBS) and 28 with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Specificity of criteria sets was tested in 40 patients with amyotrophic lateral sclerosis (ALS) and 32 with diabetic polyneuropathy (DPN). Sensitivity ranged from 24 to 83% (mean, 54.3%) in GBS and 39 to 89% (mean, 64.9%) in CIDP. With regard to ALS, specificity was 100% for nine sets but was 97% in one. In contrast, 3-66% of DPN patients fulfilled criteria in eight of ten sets. We propose a set of criteria with 72% and 75% sensitivity in our GBS and CIDP patient series, respectively, and 100% specificity with regard to ALS and DPN. Our data illustrate that most, but not all, patients can be electrodiagnostically ascertained.

Adolescent↗
PubMed2005

Chronic inflammatory demyelinating polyradiculoneuropathy with autonomic involvement.

We report a case of chronic acquired neuropathy predominantly affecting sensory and autonomic nerves. Investigations showed a demyelinating polyradiculoneuropathy with axonal degeneration and depletion of postganglionic noradrenergic fibers in the rectal mucosa. Intravenous immunoglobulin and corticosteroid administration were effective in alleviating symptoms and improving electrophysiological abnormalities. This neuropathy may be a novel variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), in which autoimmunoreactivity is directed not only against myelin but also against axon- or ganglion-composing protein. Autonomic nerve involvement does not exclude a diagnosis of CIDP.

Autonomic Nervous System Diseases↗
PubMed1985

Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy.

Eleven consecutive patients with progressive chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) underwent plasma exchange. Eight patients were previously unresponsive to prednisone, two were started on prednisone with plasma exchange, and one did not receive corticosteroids. Electrodiagnostic studies revealed evidence of an acquired demyelinating polyradiculoneuropathy with varying degrees of axonal degeneration. Neurologic impairment was monitored using conventional functional status index. Five patients demonstrated substantial clinical improvement, beginning 2 days to 3 weeks after initiating plasma exchange. Two additional patients improved following a second course of plasma exchange, and four patients demonstrated minimal or no change. Comparison of responding and nonresponding patients showed no differences related to the presence or absence of antecedent illness, duration of disease, duration of maximum weakness, or severity of impairment prior to plasma exchange. Responders had significantly prolonged F-response and motor distal latencies compared to nonresponders. Results in this unselected, consecutive patient trial suggest a temporal relationship between plasma exchange and clinical improvement in some patients with progressive CIDP.

Adolescent↗
PubMed1994

Cervical magnetic stimulation in children and adolescents: normal values and evaluation of the proximal lesion of the peripheral motor nerve in cases with polyradiculoneuropathy.

Cervical magnetic stimulation was used to establish the normal values of the peripheral motor nerve conduction of the upper extremity muscle in normal children and adolescents. Seven patients with peripheral neuropathy were also examined to evaluate a lesion in the proximal site of the peripheral motor nerve. In normal subjects, onset latencies and negative wave durations tended to increase with age. The developmental profile of the latency, corrected for arm length, revealed a significant decline until the age of about 5 years. In 4 cases with polyradiculoneuropathy, motor evoked potentials following cervical magnetic stimulation showed increased latencies, prolonged durations and polyphasic shapes. The time differences between latencies by magnetic stimulation and peripheral motor conduction times by F technique were significantly prolonged. Motor evoked potentials obtained in 3 cases of axonal degeneration, on the other hand, showed slightly increased latencies and normal durations. The time differences between latencies and peripheral motor conduction times were within the normal range. Thus, we consider that cervical magnetic stimulation is a useful method for study of peripheral motor conduction in children and adolescents, and in particular to evaluate the proximal lesion of the nerve in patients with polyradiculoneuropathy.

Adolescent↗
PubMed2005

Cyclosporine in chronic inflammatory demyelinating polyradiculoneuropathy.

This study demonstrates the efficacy of cyclosporine included in a regimen for the treatment of steroid-resistant chronic inflammatory demyelinating polyradiculoneuropathy in two children. Clinical response was characterized by either decreased frequency of recurrent weakness or normalized motor function. Nerve conduction studies and monitoring of cyclosporine levels were included in the serial follow-up evaluations, and their results were used in formulating a treatment plan. One of the two children, who had been monitored for 56 months since the onset of the disease, was able to maintain normal muscle strength without recurrent weakness for 39 months, with 5 mg/kg daily of cyclosporine. The other child, who had been taking prednisolone 0.3 mg/kg daily and cyclosporine 5 mg/kg daily, regained ambulation without support while demonstrating a reduction of recurrent weakness. None had adverse effects caused by cyclosporine therapy. We conclude that cyclosporine is an effective drug in the treatment of children with steroid-resistant chronic inflammatory demyelinating polyradiculoneuropathy.

Child, Preschool↗
PubMed1999

Multifocal polyradiculoneuropathy and carcinoma of the thymus.

We studied a patient with polyradiculoneuropathy with anaplastic carcinoma of the thymus. Motor manifestations dominated. Postmortem examinations indicated that the primary changes were in the spinal nerve roots, peripheral nerves and, possibly, the spinal anterior horn cells. The posterior funiculi and posterior root ganglia were also affected, implying multifocal and multiphasic degeneration. This unusual polyradiculoneuropathy is a form of carcinomatous neuropathy.

Aged↗
PubMed1984

Beneficial effects of plasma exchange in acute inflammatory polyradiculoneuropathy.

The results of a controlled trial in which 38 patients with severe acute inflammatory polyradiculoneuropathy took part indicate that plasma exchange favourably influenced the course of the disease. Significant benefits were seen in time until onset of improvement, course of muscular weakness, improvement in disability grades over the first 2 months, and working capacity after 1 month. Cost-benefit analysis showed that the exchange treatment resulted in net financial savings. The results suggest that plasma exchange may have a role in the treatment of severe acute inflammatory polyradiculoneuropathy.

Acute Disease↗
PubMed2005

Recurrent polyradiculoneuropathy and PMP22 defects.

BACKGROUND: Although immunologic factors play an important role in the pathogenesis of the inflammatory neuropathies, the mechanisms of recurrent episodes of Guillain-Barré syndrome (GBS) and chronic relapsing polyneuropathies (CRP) are not known. Hereditary neuropathy with liability to pressure palsy (HNPP) is an inherited disease caused by a deletion or point mutation in the peripheral myelin protein 22 (PMP22) gene, which may manifest as a recurrent polyradiculoneuropathy. This study tried to elucidate the relationship between PMP22 and recurrent GBS and CRP. METHODS: Between 1993 and 2003, we saw 114 patients with polyradiculoneuropathies or their variants. Only 4 patients had recurrent episodes: 2 had recurrent GBS and 2 had CRP. We analyzed the PMP22 gene to determine its genetic role in these 4 patients. Genomic DNA was extracted from peripheral lymphocytes of all 4 patients using a previously described procedure, and molecular detection of PMP22 deletion was performed. RESULTS: The results showed no duplication, deletion or point mutation in the PMP22 gene. CONCLUSION: PMP22 gene deletion did not play a role in our patients with recurrent GBS and CRP.

Adult↗
PubMed2003

The treatment of chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy is an inflammatory disorder of nerve that usually presents with slowly progressive weakness and sensory loss and areflexia. It is among the most treatable of the peripheral nerve disorders, and several modalities have been shown to be effective in prospective, randomized controlled trials. Although most patients show a gratifying early response to treatment, in many cases the patients relapse. The cumulative effects of the neuropathic impairments, along with side effects from long-term immunosuppressive treatment, combine to produce significant long-term morbidity and loss of function. This review will cover the epidemiology, clinical features, laboratory findings, and pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy; the current status of the treatment of this disorder will be reviewed, highlighting those therapies shown to be effective in randomized controlled trials.

Anti-Inflammatory Agents↗
PubMed1986

Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy.

Plasma exchange has been reported to be efficacious in chronic inflammatory demyelinating polyradiculoneuropathy. We performed a prospective double-blind trial in which patients with static or worsening disease were randomly assigned to plasma exchange (n = 15) or to sham exchange (n = 14) for three weeks. After three weeks, we observed statistically significant differences in combined measurements of nerve conduction (total, motor, proximal, velocity, and amplitude) favoring patients who had received plasma exchange. Improvement to a greater degree than for any patient receiving sham exchange was detected in the neurologic-disability score in five patients (P = 0.025) and in subset scores for weakness and reflex in four patients (P less than 0.057). We conclude that for some patients with chronic inflammatory demyelinating polyradiculoneuropathy, plasma exchange has an ameliorating effect on neurologic dysfunction and nerve conduction, but in others no improvement is observed. Because plasma was replaced with normal serum albumin, a humoral factor or factors may have a role in the neurologic deficit of this disorder.

Chronic Disease↗