Diagnosis Of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Research Articles

Impact of 2021 European Academy of Neurology/Peripheral Nerve Society diagnostic criteria on diagnosis and therapy of chronic inflammatory demyelinating polyradiculoneuropathy variants.

There are different criteria for the diagnosis of different variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines provide specific clinical criteria for each CIDP variant even if their therapeutical impact has not been investigated. We applied the clinical criteria for CIDP variants of the 2021 EAN/PNS guidelines to 369 patients included in the Italian CIDP database who fulfilled the 2021 EAN/PNS electrodiagnostic criteria for CIDP. According to the 2021 EAN/PNS clinical criteria, 245 patients achieved a clinical diagnosis of typical CIDP or CIDP variant (66%). We identified 106 patients with typical CIDP (29%), 62 distal CIDP (17%), 28 multifocal or focal CIDP (7%), four sensory CIDP (1%), 27 sensory-predominant CIDP (7%), 10 motor CIDP (3%), and eight motor-predominant CIDP (2%). Patients with multifocal, distal, and sensory CIDP had milder impairment and symptoms. Patients with multifocal CIDP had less frequently reduced conduction velocity and prolonged F-wave latency and had lower levels of cerebrospinal fluid protein. Patients with distal CIDP more frequently had reduced distal compound muscle action potentials. Patients with motor CIDP did not improve after steroid therapy, whereas those with motor-predominant CIDP did. None of the patients with sensory CIDP responded to steroids, whereas most of those with sensory-predominant CIDP did. The 2021 EAN/PNS criteria for CIDP allow a better characterization of CIDP variants, permitting their distinction from typical CIDP and more appropriate treatment for patients.

Cutting edge of diagnosis and treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) based on the EAN/PNS guideline 2021

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a most common chronic immune-mediated demyelinating neuropathy, and includes a number of clinical subtypes. The major phenotype is "typical CIDP", which is characterized by symmetric polyneuropathy and "proximal and distal" muscle weakness. During the historical changes in the concept of CIDP, multifocal motor neuropathy, anti-myelin-associated glycoprotein (MAG) neuropathy, and autoimmune nodopathy have been excluded. Currently CIDP is considered as a syndrome including typical CIDP and CIDP variant such as distal CIDP and multifocal CIDP. In 2021, the international guideline of diagnosis and treatment for CIDP, European Academy of Neurology (EAN)/Peripheral Nerve Society (PNS) Guideline, was published. This review article introduces the putline of the guideline with medical-social situation in Japan. The diagnosis of CIDP is based on (1) phenotype of typical CIDP or variant, (2) electrophysiologic evidence of peripheral nerve demyelination, and (3) exclusion criteria. The first-line treatments are corticosteroids or immunoglobulin therapy, and plasma exchange should be considered if the 2 treatments were not effective sufficiently. This guideline recommends intravenous or subcutaneous immunoglobulin as a maintenance therapy, and suggests other immune-suppressive agents. In the near future, new treatment with biologics, such as monoclonal antibodies against neonatal Fc receptors, complements, and CD19/20 will be approved.

Chronic inflammatory demyelinating polyradiculoneuropathy overdiagnosis in Russia

Introduction. Despite the improving diagnostic criteria for the chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), its verification is still an issue.
 Objective: to study the rate and the causes of CIDP misdiagnosis.
 Materials and methods. We prospectively and retrospectively analyzed the clinical and paraclinical data of 223 patients admitted to the Research Center of Neurology from 2018 to 2022 with a CIDP referral.
 Results. We revised the CIDP diagnosis in 150/223 patients (67%; median age 55.5 [43; 63] years; 75 males and 75 females; 3-year follow-up history [1.75; 5.25].) Once the definitive diagnosis was clarified, we divided the patients into the following groups: polyneuropathy of other etiology (n = 94; 63%), other neuromuscular disorders (n = 39; 27%), CNS disorders (n = 10; 7%), no structural NS disease (n = 7; 5%). Patients did not meet the 2021 EAN/PNS diagnostic criteria at the history-taking stage in 65% of cases, at the neurological examination stage in 39% of cases, and at the electroneuromyography stage in 92% of cases.
 Conclusions. The rate of CIDP misdiagnosis in Russia is 67%, and most often this refers to patients with polyneuropathy of other etiologies. The main cause for the CIDP misdiagnosis was inaccurate electroneuromyography. We should bear in mind that CIDP is a rare disorder with an extensive differential diagnosis, so it should be verified according to the current 2021 EAN/PNS diagnostic criteria.

Serum neurofilament light chain does not detect self-reported treatment-related fluctuations in chronic inflammatory demyelinating polyneuropathy.

Serum neurofilament light chain (sNfL) is a marker for axonal degeneration. Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) often report a fluctuation of symptoms throughout one treatment cycle with intravenous immunoglobulins (IVIG). The aim of this study was to determine whether sNfL is suitable to quantify patient-reported symptom fluctuations. Twenty-nine patients with the diagnosis of CIDP or a CIDP-variant under treatment with IVIG were recruited in this study and underwent examination before IVIG infusion, in the middle of the treatment interval, and before their next IVIG infusion. Patients were surveyed regarding symptom fluctuations at the last visit and divided into two groups: those with and without fluctuations of symptoms. At the first visit, sociodemographic and disease-specific data were collected. Clinical scores were assessed at every examination. sNfL values were compared between both groups at the different time points after conversion into Z-scores-adjusted for age and body mass index. Patients with CIDP show elevated sNfL Z-scores (median at baseline: 2.14, IQR: 1.0). There was no significant change in sNfL Z-scores or questionnaire scores within the treatment cycle in either group. There was no significant difference in sNfL levels between the patients with and without symptom fluctuations. CIDP patients show elevated sNfL levels. However, sNfL is not suitable to reflect patient-reported fluctuations of symptoms. This indicates that symptom fluctuations during treatment with IVIG in patients with CIDP are not caused by a neuroaxonal injury. Furthermore, repeated sNfL measurements within one treatment cycle with IVIG seem to have no benefit for symptom monitoring.

The diagnostic value of quantitative assessment of MR neurography in chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis.

The purpose of this study was to evaluate the diagnostic value of quantitative magnetic resonance neurography (MRN) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We also compared various MRN parameters and determined the best performing one. Through literature searches in PubMed, Embase, Cochrane, Ovid MEDLINE and ClinicalTtrials.gov until March 1, 2023, we selected studies with the diagnostic performance of MRN in CIDP patients. The pooled estimated sensitivity and specificity of quantitative MRN parameters were determined by a bivariate random-effects model. Subgroup analysis was performed to evaluate the proper quantitative parameters and nerve sites. A total of 14 quantitative MRN studies with 23 results gave a pooled sensitivity of 0.73 (95% CI 0.66-0.79) and a pooled specificity of 0.89 (95% CI 0.84-0.92). The area under the curve (AUC) was 0.89 (95%CI 0.86-0.92). Subgroup analysis of quantitative parameters showed the fractional anisotropy (FA) with the highest sensitivity of 0.85 (95% CI 0.77-0.90) and cross-sectional area (CSA) with the highest specificity of 0.95 (95% CI 0.85-0.99). The pooled correlation coefficient for interobserver agreements was 0.90 (95%CI 0.82-0.95). Quantitative MRN has considerable diagnostic value in CIDP patients with accuracy and reliability. FA and CSA can be promising parameters in the future diagnosis of CIDP patients. This is the first meta-analysis of quantitative MRN in the diagnosis of CIDP.We have selected reliable parameters with cut-off value and provided new insights for subsequent diagnosis of CIDP.

Chronic inflammatory demyelinating polyradiculoneuropathy in a patient with systemic lupus erythematosus without systemic activity.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an uncommon subtype of peripheral neuropathy, especially in systemic lupus erythematosus (SLE). We report a case of SLE presenting with CIDP successfully treated. The patient presented with bilateral, progressive, ascending, sensory, and motor neuropathy. Electrodiagnostic tests reported active motor and sensitive demyelinating polyneuropathy, and the diagnosis of CIDP was confirmed according to the European Federation of Neurological Societies/Peripheral Nerve Society criteria. Initial management with intravenous immunoglobulin and high-dose steroids was administered, then 6-month intravenous cyclophosphamide was initiated with improvement according to clinical scales. In conclusion, CIDP in SLE is rare, reported in just 0.2%. Immunosuppressive therapy should be considered whether initial improvement is not evidenced, as seen in our case requiring cyclophosphamide; interestingly, systemic activity was in remission as the peripheral nervous system is not part of neurological compromise, and we suggest evaluating this unusual presentation into rheumatological practice.

Presence of deep tendon reflexes may not exclude a diagnosis of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) (P11-8.015)

<h3>Objective:</h3> To identify if there are chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients who do not meet clinical criteria but satisfy electrodiagnostic criteria. Our hypothesis is that these patients do not differ from patients who meet criteria for CIDP and possible CIDP. <h3>Background:</h3> The diagnosis of CIDP remains challenging due to lack of a biomarker or a single diagnostic test. The European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) 2021 revision of the diagnostic criteria for CIDP advocate a stepwise approach to the diagnosis of CIDP which requires clinical criteria including absent or reduced deep tendon reflexes before the application of electrodiagnostic criteria. <h3>Design/Methods:</h3> We applied the 2021 EAN/PNS criteria to our cohort of 39 patients receiving intravenous immunoglobulin (IVIG) for CIDP. We compared clinical and electrodiagnostic criteria between those who fulfilled electrodiagnostic criteria but not clinical criteria and patients with CIDP or possible CIDP <h3>Results:</h3> Of our cohort 39-patients, 21 (53.8%) were classified as CIDP, 6 (15.3%) as possible CIDP and 12 patients didn’t meet criteria. Of the 10 patients who didn’t meet clinical criteria, 6/10 (60%) met electrodiagnostic criteria. These patients did not qualify as CIDP or Possible CIDP due to the presence of reflexes. There were no significant differences in the clinical phenotypes and demographics between these patients and CIDP or possible CIDP patients in our cohort. There was also no significant difference in the electrodiagnostic parameters between these patients and those with CIDP or possible CIDP. However, the patients with preserved reflexes tended to have more frequent conduction velocity slowing (p = 0.08) than patients with CIDP or possible CIDP. <h3>Conclusions:</h3> The presence of reflexes should not exclude the diagnosis of CIDP if the patient meets electrodiagnostic criteria. This could be indicative of a subset of patients with CIDP who have more involvement of the intermediate segments of nerve rather than proximal segments. <b>Disclosure:</b> Dr. Strathdee has nothing to disclose. The institution of Dr. Rodrigues has received research support from Australian Brain Foundation. The institution of Dr. Kapoor has received research support from Bethlehem Griffiths Research Foundation . Dr. Zhang has nothing to disclose. Dr. Faragher has stock in Argo P/L. Dr. Faragher has stock in Australian Foundation P/L. Dr. Faragher has stock in Argo Infrastructure P/L. Bruce Day has nothing to disclose. The institution of Dr. Hutton has received research support from Lundbeck. The institution of Dr. Hutton has received research support from Daewoong Pharmaceuticals . The institution of Dr. Hutton has received research support from Xalud. The institution of Dr. Hutton has received research support from Ipsen. The institution of Dr. Hutton has received research support from Abbvie. The institution of Dr. Hutton has received research support from Cerecin. Dr. Hutton has a non-compensated relationship as a Board Member with Brain Foundation (Vic) that is relevant to AAN interests or activities. Dr. Hutton has a non-compensated relationship as a President-elect with Australian and New Zealand Headache Society that is relevant to AAN interests or activities. Dr. Stark has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Lundbeck. Dr. Stark has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abbvie. Dr. Stark has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Teva. Dr. Stark has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eli Lilly. Dr. Stark has stock in CSL. Dr. Stark has stock in Multiple ASX listed (non-medical) companies. Dr. Stark has received personal compensation in the range of $10,000-$49,999 for serving as a TCMC Committee chair with AMA Victoria.

Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuropathy after Ad26.COV2.S COVID-19 Vaccination (P13-8.006)

<h3>Objective:</h3> NA <h3>Background:</h3> Acute onset demyelinating neuropathy has been reported after coronavirus disease 2019 (COVID-19) vaccination. Only a limited number of cases continued progression and became chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), mostly after ChAdOx1 nCoV-19 vaccination. Here, we report the first case of acute-onset CIDP after Ad26.COV2.S (Johnson &amp; Johnson) vaccination. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 67-year-old man presented with one-week history of progressive left facial droop, unsteady gait, slurred speech, double vision, and patchy numbness; he reported Ad26.COV2.S vaccination three weeks before presentation. On initial examination, he had left facial palsy, partial left abducens nerve palsy, generalized areflexia, severe lower extremities ataxia, and diminished vibratory sensation in toes. Cerebrospinal fluid analysis showed albuminocytologic dissociation. He received intravenous immunoglobulin (IVIG) for five days for presumptive diagnosis of Guillain-Barré syndrome (GBS). Electrodiagnostic studies demonstrated impersistent F-waves, decreased recruitment in lower extremities, and bilaterally absent blink reflexes. He developed right facial weakness on day three and asymmetric appendicular muscle weakness on day five of treatment. Seven weeks after symptom onset, his lower extremities weakness worsened, and received a second cycle of IVIG. The lower extremity weakness did not improve but remained stable until seventeen weeks after symptom onset when he returned with progressive upper extremities weakness and dyspnea. Electrodiagnostic studies demonstrated demyelinating features consistent with CIDP. Neuromuscular ultrasound demonstrated multifocal median nerve enlargements at non-entrapment sites. At this point, given the clinical course, electrodiagnostic and ultrasonographical findings, CIDP diagnosis was established. <h3>Conclusions:</h3> This patient’s acute-onset CIDP and other reported GBS cases after adenoviral vector COVID-19 vaccinations shared similar and atypical presenting symptoms, which were not seen in GBS after mRNA COVID-19 vaccinations. While a causal relationship cannot be established to his COVID-19 vaccination, this case adds to the evolving literature of COVID-19 vaccination-associated neurological events. <b>Disclosure:</b> Dr. Bendi has nothing to disclose. Dr. Chen has nothing to disclose. Dr. Danielson has nothing to disclose.

Unclassified clinical presentations of chronic inflammatory demyelinating polyradiculoneuropathy

BackgroundTo assess the ability of the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) clinical criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to include within their classification the whole spectrum...

Comparison of the diagnostic accuracy of the 2021 EAN/PNS and 2010 EFNS/PNS diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy

ObjectivesTo compare the sensitivity and specificity of the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with those of the 2010 European...

Usefulness and prognostic value of diagnostic tests in patients with possible chronic inflammatory demyelinating polyradiculoneuropathy.

Recent guidelines define chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and possible CIDP. The aims of our study were to evaluate the value of diagnostic tests to support the diagnosis of CIDP in patients with possible CIDP and to identify prognostic factors of therapeutic success. We conducted an observational retrospective two-center study between 2014 and 2019. We selected patients with a clinical presentation suggesting CIDP, but whose electrodiagnostic (EDX) test results did not meet the EFNS/PNS 2021 criteria. We analyzed epidemiologic and clinical features, axonal loss on EDX, cerebrospinal fluid (CSF), somatosensory evoked potentials (SSEPs), plexus magnetic resonance imaging (MRI), nerve biopsy, and therapeutic response. We selected 75 patients, among whom 30 (40%) responded to treatment. The positivity rates of CSF analysis, MRI and SSEPs were not influenced by the clinical presentation or by the delay between symptom onset and medical assessment. A high protein level in CSF, female gender, and a relapsing-remitting course predicted the therapeutic response. It is important to properly diagnose suspected CIDP not meeting EFNS/PNS 2021 EDX criteria by using supportive criteria. Specific epidemiological factors and a raised CSF protein level predict a response to treatment. Further prospective studies are needed to improve diagnosis and the prognostic value of diagnostic tests in CIDP.

Clinical and diagnostic features of anti-neurofascin-155 antibody-positive neuropathy in Han Chinese.

ObjectiveTo investigate the clinical features of Han Chinese patients with anti‐neurofascin‐155 (NF155) antibody‐positive neuropathy.MethodsWe screened 194 patients with peripheral neuropathy for NF155 antibodies using a cell‐based assay (CBA) and teased‐fiber immunofluorescence assay. We summarized the clinical findings of seropositive patients.ResultsThe sera from 17 patients reacted to human embryonic kidney 293 cells transfected with NF155. Eleven of these patients had the immunoglobulin G (IgG) 4 isotype, a younger onset age, tremor, higher levels of cerebrospinal fluid protein, a larger diameter of the lumbosacral nerve root on magnetic resonance imaging, and the distal demyelinating symmetric phenotype. Most patients responded to steroids and rituximab. For the remaining six seropositive patients in CBA, the predominant antibody isotype was IgG3, IgG1, or undetectable, and only one patient with IgG3 showed a positive result in the teased‐fiber immunofluorescence assay. These patients did not share the typical features displayed by patients with the IgG4 isotype.InterpretationIn the Han Chinese population, a significant proportion of patients who fulfilled the criteria for chronic inflammatory demyelinating polyradiculoneuropathy diagnosis had anti‐NF155 IgG4 antibody‐positive neuropathy and displayed specific phenotypes. Ambiguous staining patterns may appear, and the potential for false positivity should be considered. For patients who presented with specific phenotypes, identifying antibodies and subtypes involved a significant laboratory workup.

A diagnostic score for anti-myelin-associated-glycoprotein neuropathy or chronic inflammatory demyelinating polyradiculoneuropathy in patients with anti-myelin-associated-glycoprotein antibody.

A diagnostic score was developed to discriminate anti-myelin-associated-glycoprotein (MAG) neuropathy from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and applied it to patients with atypical anti-MAG neuropathy. The clinical and electrophysiological features of patients with a diagnosis of typical anti-MAG neuropathy were compared to those of patients with a diagnosis of CIDP. The association of each feature with the diagnosis was assessed in the two groups. Features showing a significant association with the diagnosis were included in a multivariable logistic regression model and adjusted odds ratios were estimated for each feature. A score ranging from 1 to 3 was applied to each feature based on the magnitude of the estimated odds ratios. The score was then applied to patients with a clinical diagnosis of CIDP who also had high anti-MAG antibody titers (CIDP-MAG). Thirty-one anti-MAG neuropathy patients, 45 typical CIDP patients and 16 CIDP-MAG patients were included. Scores in anti-MAG antibody patients ranged from 1 to 5 and in CIDP patients from -7 to -1. Using the score, 4/16 CIDP-MAG patients were diagnosed to have anti-MAG neuropathy and 12/16 patients to have CIDP. Response to intravenous immunoglobulin in the CIDP-MAG patients classified as CIDP was similar to that of definite CIDP patients and higher than that of anti-MAG neuropathy patients. Our score allowed an accurate discrimination to be made, amongst patients with anti-MAG antibodies, of those affected by CIDP and the patients with anti-MAG neuropathy. This score may help proper treatment to be chosen for patients with anti-MAG antibodies with a CIDP-like presentation.

Juvenile-Onset Relapsing Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Case Report

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, immune-mediated condition. It is a symmetric, motor-predominant neuropathy that results in both proximal and distal limb muscle weakness and is characterized by electrodiagnostic or pathologic features of demyelination. CIDP is a treatable disease that is known to be rare in the pediatric population. This case report describes a 12-year-old female who presented with gait disturbance and progressive upper and lower limb weakness. The electrophysiologic findings were compatible with demyelinating polyneuropathy. Combined with the clinical features, the diagnosis of CIDP was made and treatment was administered. The first-line immunomodulatory treatment seemed to be effective, as shown by improvements in electrophysiologic and clinical parameters, but the relapsing-remitting clinical course required additional immunomodulatory treatment. Herein, we describe the patient’s clinical and electrophysiologic course according to the treatment. Keywords: Child; Polyradiculoneuropathy, chronic inflammatory demyelinating; Electrodiagnosis

CIDP and hemopathies, an underestimated association

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated and treatable disease that may be associated with various systemic conditions. Our objective is to describe the clinical, electrophysiological and pathological data of a series of patients with both CIDP and hemopathy. In this retrospective study, we analyzed 21 patients with CIDP and various hemopathies (malignant or not), consecutively observed for almost five years. In this particular context (with a risk of neurological complications of the hemopathy), a nerve biopsy was taken from each patient (after written consent). All the patients fulfilled the EAN/PNS electrodiagnostic criteria (2021) of CIDP: 16 with ‘CIDP’ and 2 with ‘possible CIDP’ (no data for 3 patients). For each patient, pathological analysis of nerve biopsy was compatible with the diagnosis of CIDP, and there was no evidence for hematological complication of the peripheral nervous system. In cases of peripheral neuropathy and malignant hemopathy, the possibility that the peripheral neuropathy is CIDP should not be overlooked because CIDP is clearly accessible to appropriate therapies, with high potential for a positive clinical response. If the diagnosis of CIDP is usually suspected clinically and electrophysiologically, it should be confirmed by pathological study (nerve biopsy) in certain cases. The management of such patients benefits from the collaboration of neurologists, hematologists and oncologists.

Atypical chronic inflammatory demyelinating polyradiculoneuropathy: recent advances on classification, diagnosis, and pathogenesis.

In recent years, there has been an intense debate in literature regarding the definition of the individual variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), their possible pathogenetic mechanisms, and impact in the diagnosis of CIDP. The 2021 European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines revised the definition of the individual CIDP variants and implemented their diagnostic criteria. Diagnosis of atypical CIDP is challenging and misdiagnosis is frequent, leading to diagnostic delay and consequent greater accumulation of disability and treatment dependency. Recent studies suggest that patients with typical CIDP have an antibody-mediated mechanism of neuropathy whereas in those with Lewis--Sumner syndrome (LSS) neuropathy is preferentially mediated by macrophages and T cells. Although the validity of the 2021 EFNS/PNS diagnostic criteria for atypical CIDP is unknown, they will hopefully lead to greater uniformity in the selection of patients to be enrolled in future studies and to a greater diagnostic accuracy. New data are emerging on the possible pathological mechanisms of individual variants and this could result in the discovery of specific diagnostic biomarkers and new therapies.

Chronic inflammatory demyelinating polyneuropathy with hypoglossal nerve involvement and inverted Beevor\u2019s sign: case report

BackgroundCranial nerve involvement is not commonly encountered in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); this is especially true for involvement of the hypoglossal nerve. Neither Beevor's sign nor its inverted form has previously been described in CIDP.Case presentationA 28-year-old man presented with distal-predominant limb weakness and numbness at the age of 18. A diagnosis of CIDP was made, which was confirmed by electrodiagnostic evidence of demyelination. He responded well to intravenous immunoglobulin and glucocorticoid treatment and achieved remission for 5 years. However, the same symptoms relapsed at the age of 28 and lasted for 10 months. On examination, in addition to limb sensory impairment and muscle weakness, mild bilateral facial paresis, tongue atrophy and fasciculations, and inverted Beevor's sign were also observed. A brief literature review of cranial nerve involvements in CIDP and Beevor's sign or its inverted form were also performed.ConclusionsCranial nerves may be affected in patients with CIDP. Facial palsy is most frequently present, while hypoglossal nerve involvement is rare. Inverted Beevor's sign can appear in CIDP patients.

The neurophysiological lesson from the Italian CIDP database

IntroductionElectrophysiological diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may be challenging. Thus, with the aim ofproviding some practical advice in electrophysiological approach to a patient with suspected CIDP, we analyzed electrophysiological data from 499 patients enrolled inthe Italian CIDP Database.MethodsWe calculated the rate of each demyelinating feature, the rate of demyelinating features per nerve, the diagnostic rate for upper andlower limb nerves, and, using a ROC curve analysis, the diagnostic accuracy of each couple of nerves and each demyelinating feature, for every CIDP subtype.Moreover, we compared the electrophysiological data of definite and probable CIDP patients with those of possible and not-fulfilling CIDP patients, and by a logisticregression analysis, we estimated the odds ratio (OR) to make an electrophysiological diagnosis of definite or probable CIDP.ResultsThe ulnar nerve had the highestrate of demyelinating features and, when tested bilaterally, had the highest diagnostic accuracy except for DADS in which peroneal nerves were the most informative.In possible and not-fulfilling CIDP patients, a lower number of nerves and proximal temporal dispersion (TD) measurements had been performed compared to definiteand probable CIDP patients. Importantly, OR for each tested motor nerve and each TD measurement was 1.59 and 1.33, respectively.ConclusionOur findingsdemonstrated that the diagnosis of CIDP may be missed due to inadequate or incomplete electrophysiological examination or interpretation. At the same time, thesedata taken together could be useful to draw a thoughtful electrophysiological approach to patients suspected of CIDP.

Misdiagnosis and diagnostic pitfalls of chronic inflammatory demyelinating polyradiculoneuropathy.

Background and purposeThe aim of this study was to determine the frequency of over‐ and underdiagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and to identify related diagnostic pitfalls.MethodsWe conducted a retrospective study in Dutch patients referred to the Erasmus University Medical Centre Rotterdam between 2011 and 2017 with either a diagnosis of CIDP or another diagnosis that was revised to CIDP. We used the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) 2010 diagnostic criteria for CIDP to classify patients into three groups: overdiagnosis, underdiagnosis, or confirmed diagnosis of CIDP. Clinical and laboratory features and treatment history were compared between groups.ResultsA referral diagnosis of CIDP was revised in 32% of patients (31/96; overdiagnosis). Of 81 patients diagnosed with CIDP, 16 (20%) were referred with another diagnosis (underdiagnosis). In the overdiagnosed patients, 20% of muscle weakness was asymmetric, 48% lacked proximal muscle weakness, 29% only had distal muscle weakness, 65% did not fulfil the electrodiagnostic criteria for CIDP, 74% had an elevated cerebrospinal fluid (CSF) protein level, and 97% had another type of neuropathy. In the underdiagnosed patients, all had proximal muscle weakness, 50% had a clinically atypical CIDP, all fulfilled the electrodiagnostic criteria for CIDP, and 25% had an increased CSF protein level.ConclusionOver‐ and underdiagnosis of CIDP is common. Diagnostic pitfalls include lack of attention to proximal muscle weakness as a diagnostic hallmark of CIDP, insufficient recognition of clinical atypical phenotypes, overreliance on CSF protein levels, misinterpretation of nerve conduction studies and poor adherence to electrodiagnostic criteria, and failure to exclude other causes of polyneuropathy.

Carrefour des spécialités : autogreffe de cellules souches hématopoïétiques périphériques pour la prise en charge thérapeutique des polyradiculonévrites inflammatoires démyélinisantes chroniques

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a dysimmune neuropathy with sensory and/or motor symptoms due to destruction of the myelin sheat secondary to an auto-immune attack. A quarter to a third of patients do not respond to immunomodulatory first line recommended therapies. No second line treatment has shown its effectiveness with a sufficient level of evidence. Autologous hematopoietic stem cell transplantation (AHSCT) is a promising therapy for autoimmune disease, especially for CIDP in recent works. We present in this article an update on the diagnosis of CIDP, its conventional treatments as well as the results of AHSCT in this indication, which was the subject of French recommendations under the aegis of the SFGMTC and neuromuscular disease french faculty (FILNEMUS) as a third line therapy after failure of two first-line and one second-line treatments.