Diagnosis Of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Research Articles

An atypical chronic inflammatory demyelinating polyradiculoneuropathy that radiologically mimicking neurofibromatosis: Case report

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an uncommon demyelinating disorder with a relapsing and remitting or continuously progressive course. Patients may have motor and sensory involvement, but generally motor involvement may be more prominent and more severe in lower extremities. CIDP is a treatable neuropathy that is challenging to diagnose and has a broad spectrum of presentations. When ranked by the descending frequency, postural tremor in the arms, peripheral nerve thickening, papilledema and facial or bulbar weakness, respiratory failure and autonomic dysfunction can be seen in patients with CIDP. Demyelinating neuropathy predominantly affects spinal roots, plexuses and proximal nerve trunks and thickened nerves can be palpable in about 10% of the patients. In patients with atypical presentation, the diagnosis of CIDP may be delayed and hypertrophic nerve roots have been reported in CIDP patients with delayed diagnosis. Magnetic Resonance Imaging (MRI) may be helpful in the diagnosis of CIDP by excluding the compressive or structural lesions that may lead to polyradiculopathy and MRI may also show hypertrophy of nerve roots and inflammatory processes in CIDP. We presented a patient with CIDP who had MRI findings of diffuse enlargement and mild enhancement of roots and extraforaminal segments of nerves in all segments.

Spinal cord involvement in chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and MRI study.

Concomitant central nervous system (CNS) involvement in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare. Although the spinal nerve roots may present MRI abnormalities in CIDP, hitherto, the spinal cord has been investigated in a single study. We retrospectively investigated clinically and with MRI a cohort of patients with definite CIDP diagnosis (EFNS/PNS criteria) for evidence of brain and spinal cord involvement, who were initially admitted in our department during the last 4 years. Among 12 patients with CIDP (men: 8, mean age: 59.3 years, mean disease duration: 3.8 years), nine patients had their MRI scan during a clinical relapse and three during remission. Brain MRI did not document typical multiple sclerosis lesions in any patient. We did not identify any MRI abnormalities in ten patients without clinical evidence of spinal cord involvement. Conversely, MRI disclosed extensive lesions of the thoracic cord in two patients with an overt spinal cord syndrome, whom we describe. This represents the biggest MRI study of CIDP patients who have been investigated for spinal cord involvement. Our data support earlier observations that a minority of CIDP patients may additionally develop CNS involvement of variable degree.

Somatosensory evoked potentials in chronic inflammatory demyelinating polyradiculoneuropathy.

Somatosensory evoked potentials (SEPs) offer complementary results to those of nerve conduction studies and contribute to the electrodiagnostic criteria of chronic inflammatory demyelinating polyradiculoneuropathy. We performed nerve conduction studies and SEPs in patients with symmetrical motor weakness, areflexia, and/or sensory disturbances lasting for at least 8 weeks. We determined two groups according to the electrodiagnostic criteria of the European Federation of Neurological Societies. Group 1 included patients who met the definite or probable electrodiagnostic criteria, and group 2 included patients who met the possible electrodiagnostic criteria. We also compared SEPs results with those of controls (group of healthy subjects). Sixteen patients (14 men; mean age, 59 ± 17.3 years) were included in the study. The latencies of potentials N9, N13, N7, and N22 and the intervals N9-N13 and N7-N22 were significantly increased in patients compared with controls. The N9/iP14 amplitude ratio was significantly lower in patients. There was no significant difference in the latencies of SEPs between the two groups of patients. We confirm the contribution of SEPs as complementary information to nerve conduction studies in chronic inflammatory demyelinating polyradiculoneuropathy diagnosis. In addition to the usual abnormalities, a decrease in the N9/iP14 amplitude ratio could potentially be used as an electrodiagnostic criterion.

Chronic inflammatory demyelinating polyradiculoneuropathy

In this review, we focus on less recognised signs and symptoms in typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and on the clinical presentation, course of disease and response to treatment of the atypical CIDP phenotypes. Frequently occurring and often disabling symptoms in CIDP such as fatigue, pain and tremor have recently been emphasised, whereas autonomic dysfunction, if present, is usually mild. Sensory CIDP is probably the most frequent atypical CIDP phenotype and diagnosis can be difficult in the absence of clear demyelinating features on nerve conduction studies. The most important study comparing intravenous immunoglobulin treatment with intravenous methylprednisolone showed a lower rate of discontinuation due to inefficacy, adverse events or intolerance with intravenous immunoglobulin treatment. However, improvement after corticosteroids seems to be more long-lasting than after intravenous immunoglobulin suggesting superior long-term immunosuppressive and immunomodulating effect of corticosteroids in CIDP. Symptoms other than the classical motor and sensory symptoms can lead to significant disability in CIDP patients. Based on limited evidence from largely small retrospective case series, we conclude that atypical CIDP phenotypes often have a different course of disease and sometimes response to treatment when compared with typical CIDP. Prospective multicentre cohort studies using standardised clinical description, electrophysiological parameters and outcome measures are needed to study the natural disease course of these phenotypes including response to different treatments.

MRI of the cervical nerve roots in the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy: a single-institution, retrospective case–control study

ObjectiveTo systematically evaluate the usefulness of assessing the cervical nerve roots by MRI for the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).DesignSingle-institution, retrospective case–control study.SettingA regional referral hospital.ParticipantsWe retrospectively enrolled...

Chronic Inflammatory Demyelinating Polyradiculoneuropathy: From Bench to Bedside

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common treatable chronic autoimmune neuropathy. Multiple diagnostic criteria have been established, with the primary goal of identifying neurophysiologic hallmarks of acquired demyelination. Treatment modalities have expanded to include numerous immunomodulatory therapies, although the best evidence continues to be for corticosteroids, plasma exchange, and intravenous immunoglobulin (IVIg). This review describes the pathology, epidemiology, pathogenesis, diagnosis, and treatment of CIDP.

Amyloid neuropathy mimicking chronic inflammatory demyelinating polyneuropathy

Amyloid neuropathy is a rare peripheral neuropathy that classically presents as a progressive sensory neuropathy with prominent autonomic involvement. We describe 5 patients with amyloid neuropathy (familial amyloid polyneuropathy or acquired amyloidosis) who were initially mistaken to have chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) based on history, clinical examination, electrodiagnostic studies, and cerebrospinal fluid (CSF) analysis. The diagnosis of CIDP had been retained on clinical and electrophysiological grounds for all patients, but we observed no improvement after immunomodulatory treatment. Nerve biopsy confirmed amyloid deposits in nerves, and molecular genetic analysis showed a mutation of the transthyretin (V30M) gene for 3 patients; the 2 other patients had acquired amyloidosis. This report emphasizes the need to look for an alternative diagnosis in CIDP patients who do not respond to treatment and to look carefully for symptoms or signs of autonomic involvement in such patients.

Segmental evaluation of the peripheral nerve using tibial nerve SEPs for the diagnosis of CIDP

SEPs can evaluate the proximal portion of the peripheral nerve, including the nerve root, which is preferentially involved in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We retrospectively investigated the utility of tibial nerve SEPs for the diagnosis of CIDP. Subjects were 12 clinically-defined CIDP patients and 17 patients with diabetic polyneuropathy (DPN). We segmentally evaluated the entire peripheral nerve using the N8, P15, and N21 components. We defined findings suggestive of proximal-dominant involvement (proximal-dominant findings), and investigated their frequency in both groups. Ten out of 12 CIDP patients showed at least one of the proximal-dominant findings, whereas none of the DPN patients did, when appropriate parameters were selected. The sensitivity was higher than conventional electrodiagnostic criteria such as the European Federation of Neurological Societies/Peripheral Nerve Societies criteria (definite in 8/12). Both a proximal-dominant involvement in CIDP patients and a distal-dominant involvement in DPN patients were evident by the Z-score plots. Tibial nerve SEPs were highly sensitive and specific, at least in comparison with DPN, measures for the diagnosis of CIDP. Segmental evaluation of the peripheral nerve using tibial nerve SEPs is expected as a promising tool to document proximal-dominant involvement in CIDP.

Recommendations on diagnostic strategies for chronic inflammatory demyelinating polyradiculoneuropathy

Background:Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune mediated treatable peripheral neuropathy, the diagnosis of which is straightforward in more than half of cases. Numerous sets of electrophysiological criteria have...

Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated With Multiple Sclerosis

To describe temporal profile of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients with definite, relapsing multiple sclerosis (MS). Peripheral demyelinating neuropathy has been rarely reported in association with central nervous system demyelinating disorder indistinguishable from MS. In addition to usual diagnostic studies for CIDP and MS in all 5 patients, we studied proximal segments of nerves using deep tendon reflex latency measurements of biceps reflex, patellar reflex, and ankle reflex. All patients with MS subsequently (4-22 years) developed definite CIDP. Two of these patients developed multiple cranial nerve and spinal root enhancement on subsequent imaging without new intraparenchymal enhancement after a diagnosis of CIDP. The deep tendon reflex latencies were prolonged at more than 2 sites in all patients. Cerebral spinal fluid protein increased (70 +/- 19 to 144.8 +/- 17.4 mg/dL, P = 0.0001) at time of diagnosis of CIDP. Clinical improvement was observed in all patients after intravenous immunoglobulin therapy. When patients with MS develop CIDP, manifestations of central and peripheral disease involvement seem to respond to intravenous immunoglobulin. These cases suggest that there may be common antigenic targets in central and peripheral nervous system in this subset of patients.

Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial

Short-term studies suggest that intravenous immunoglobulin might reduce disability caused by chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) but long-term effects have not been shown. We aimed to establish whether 10% caprylate-chromatography purified immune globulin intravenous (IGIV-C) has short-term and long-term benefit in patients with CIDP. 117 patients with CIDP who met specific neurophysiological inflammatory neuropathy cause and treatment (INCAT) criteria participated in a randomised, double-blind, placebo-controlled, response-conditional crossover trial. IGIV-C (Gamunex) or placebo was given every 3 weeks for up to 24 weeks in an initial treatment period, and patients who did not show an improvement in INCAT disability score of 1 point or more received the alternate treatment in a crossover period. The primary outcome was the percentage of patients who had maintained an improvement from baseline in adjusted INCAT disability score of 1 point or more through to week 24. Patients who showed an improvement and completed 24 weeks of treatment were eligible to be randomly re-assigned in a blinded 24-week extension phase. Analysis was by intention to treat. This trial is registered with ClinicalTrials.gov, number NCT00220740. During the first period, 32 of 59 (54%) patients treated with IGIV-C and 12 of 58 (21%) patients who received placebo had an improvement in adjusted INCAT disability score that was maintained through to week 24 (treatment difference 33.5%, 95% CI 15.4-51.7; p=0.0002). Improvements from baseline to endpoint were also recorded for grip strength in the dominant hand (treatment difference 10.9 kPa, 4.6-17.2; p=0.0008) and the non-dominant hand (8.6 kPa, 2.6-14.6; p=0.005). Results were similar during the crossover period. During the extension phase, participants who continued to receive IGIV-C had a longer time to relapse than did patients treated with placebo (p=0.011). The incidence of serious adverse events per infusion was 0.8% (9/1096) with IGIV-C versus 1.9% (11/575) with placebo. The most common adverse events with IGIV-C were headache, pyrexia, and hypertension. This study, the largest reported trial of any CIDP treatment, shows the short-term and long-term efficacy and safety of IGIV-C and supports use of IGIV-C as a therapy for CIDP.

Spinal Nerve Root Hypertrophy on MRI: Clinical Significance in the Diagnosis of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

To investigate the clinical usefulness of measuring diameters of spinal nerve roots on magnetic resonance imaging (MRI) in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with regard to the diagnosis and estimation of neurofunctional impairment. Fourteen patients with CIDP (mean age, 38.9+/-19.2 years) and 10 controls were enrolled in this study. Diameters of cervical and lumbosacral spinal nerve roots were determined on the short tau inversion recovery image of MRI. Correlations between these diameters and clinical indices, including the conduction velocity of median and tibial nerves, were examined. Mean diameters of cervical and lumbosacral spinal nerve roots in CIDP patients were 6.0 to 6.8 mm and 7.3 to 10.4 mm, respectively. CIDP patients showed higher values of the diameter in C5 (p<0.05), C6 (p<0.05), C7 (p<0.005) and C8 (p<0.01) than controls. C7 and C8 showed significantly negative correlations between diameters of spinal nerve roots and the F-wave conduction velocity (FWCV) (p<0.05). In the lumbosacral region, L3, L4 and S1 showed significantly negative correlations between diameters of spinal nerve roots and FWCV (p<0.005, p<0.0005 and p<0.005, respectively). The latency-time difference between F- and M-waves increased with diameters of spinal nerve roots, and there were significantly positive correlations between them in L3 (p<0.05) and L4 (p<0.005). Hypertrophy of spinal nerve roots shown on MRI may be useful as a clue to the diagnosis of CIDP and also as a clinical marker suggesting impairment of peripheral nerve conduction, particularly FWCV.

Fatigue as the main presenting symptom of chronic inflammatory demyelinating polyradiculoneuropathy: a study of 11 cases

Fatigue has been shown to be more frequent than previously thought in immune-mediated polyneuropathies. However, fatigue has not been reported as the main cause of referral in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients. Between January 2001 and December 2003, we investigated 11 patients referred for fatigue, for which we established a final diagnosis of CIDP. All patients had at least two clinical examinations including assessment of the fatigue severity scale (FSS) and one electrophysiological and laboratory work up. Additionally, 10 of the 11 patients had a nerve biopsy. There were 11 male patients. Mean age at onset was 53 +/- 11 years. Main cause of referral was fatigue in all patients. Additional symptoms included cramps (one case), distal paresthesias (six cases), limb pain (seven cases) and vasomotor disturbances (one case). Cerebrospinal fluid (CSF) analysis displayed a moderate increase in protein content in four patients. Electrophysiological analysis showed abnormalities in all patients. Among 11 patients, one fulfilled the American Academy of Neurology electrodiagnostic criteria for CIDP and three fulfilled the inflammatory neuropathy cause and treatment group or the Nicolas et al. criteria. In the eight remaining patients, a nerve biopsy confirmed the diagnosis of CIDP. Ten patients were treated, among which seven showed a significant improvement based on the FSS scale. This study shows that fatigue is a possible cause of referral for patients with CIDP and, like previous reports, emphasizes the lack of sensitivity of widely accepted electrophysiological criteria of CIDP. Long-term follow up of these patients is warranted to determine the prognosis of these minimal forms of CIDP and establish the best therapeutic strategy in such cases.

New frontiers in therapy for chronic inflammatory demyelinating polyradiculoneuropathy

In November 2001, neurologists specializing in the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were convened for a roundtable meeting entitled “Current Opinions on the Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy.” The purpose of this meeting was to discuss issues in the diagnosis and management of CIDP, with a focus on the potential use of immunomodulatory agents (IMAs) for the treatment of CIDP. In September 2002, a second roundtable meeting was held. The presentations at these meetings form the basis of the articles contained in this supplement. In addition, results from a phase II study in patients with treatment-resistant CIDP are presented. The therapeutic options currently available for patients with CIDP are limited. Therefore, …

Improvement of Neurologic Function in Chronic Inflammatory Demyelinating Polyradiculoneuropathy Following Intravenous -Globulin Infusion

To the Editor. —Recently, Dyck et al1reported that plasma exchange produces improvement in patients with severe chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) when treatment is begun early in the course of the disease. The cause of the CIDP is unknown, but an abnormality of immunity has been presumed.2 Immunoglobulin has been shown to suppress the patient's own antibodies or act through a blockade of the reticuloendothelial system, and immunoglobulin infusions have been suggested to be beneficial in patients with immune disorders.3,4This suggested a rationale for immunoglobulin infusion in a patient with CIDP and hypogammaglobulinemia. The marked clinical improvement following such therapy prompted this report. Report of a Case. —A 41-year-old woman presented at our institution with worsening neuropathic pain and decreasing motor function over the previous month. A diagnosis of CIDP had been established by biopsy eight years previously. She had been managed on maintenance prednisone