Criteria For CIDP Research Articles

Motor chronic inflammatory demyelinating polyneuropathy in a child

Motor chronic inflammatory demyelinating polyneuropathy (M-CIDP) is a form of atypical CIDP. This article presents a clinical observation of M-CIDP in a 15-year-old boy, as well as a description of laboratory and instrumental diagnostic data. The boy had a chronic development (> 2 months) of flaccid tetraparesis, predominantly of the proximal muscles of the limbs, without sensory disorders. According to electroneuromyography, there were signs of demyelinating lesions of the proximal parts of the peripheral nerves. There was an increase in the thickness of the nerves of the upper limbs according to ultrasound. In the liquor protein-cell dissociation, as well as in the blood, IgG antibodies to the surface glycoprotein S of the SARS-CoV-2 coronavirus were found. The clinical and neurophysiological picture corresponded to the reliable criteria for CIDP. The therapy with intravenous immunoglobulins had a significant positive effect in the form of an increase in the strength of the limb muscles.

P 72 Antibody depletion in nodo-paranodopathy: Clinical and electrophysiological long-term follow-up

Nodo-paranodopathies are rare immune-mediated neuropathies with autoantibodies against proteins of the node of Ranvier. The antibodies cause conduction failure and a destruction of nodal architecture in vivo and in vitro. Affected patients show severe motor and sensory involvement and signs of demyelination in electroneurography, fulfilling EFNS/PNS criteria for CIDP. Response to standard treatment is poor, but good clinical response to rituximab has been reported. Little is known about long-term outcome after antibody depletion and electroneurographic follow-up. We conducted a cohort study of 7 patients with neuropathy with anti-paranodal antibodies treated at the University Hospital of Würzburg (n = 3 anti-contactin-1, n = 4 anti-Caspr-1) who showed an antibody decrease in the course of the disease either spontaneously (n = 2) or treatment-induced by B-cell depletion with Rituximab (n = 5). Antibody titers and Neurofilament Light-Chain (NfL) levels were assessed at first diagnosis/ recruitment and in follow-up serum samples by ELISA and Ella. Clinical data, scores and electrophysiological data were assessed retrospectively with a mean follow-up period of 48.6 (8-78) months and compared using paired t-test and Spearman correlation. At recruitment, mean disease duration was 43.6 (0.25-180) months. Patients showed sensorimotor tetraparesis with a mean Overall Disability Sum Score (ODSS) of 7.43 (5-10) and MRC sum score of 48.29 (40-57). The antibody titer ranged from 1:100 – 1:10,000 and did not correlate with inter-individual disease severity. Electroneurography revealed demyelinating features with highly elevated distal motor latency (dml), loss/ reduction of F-waves and reduced nerve conduction velocity, but also signs of severe, length-dependent compound motor action potential (CMAP) amplitude decrease and spontaneous activity in electromyography in 5/ 7 patients. After antibody depletion, we found a significant reduction in the Overall Disability Sum Score (ODSS, p < 0.007) and improvement in the MRC sum score. Electrophysiological demyelinating parameters improved significantly. The mean increase in tibial nerve dml correlated with the increase in MRC sum score (r = 0.922, p < 0.003). Median nerve CMAP amplitudes increased significantly (p < 0.009), with a significant negative correlation to ODSS (r = -0.7, p < 0.03). Tibial nerve CMAP on the other hand remained severely reduced. In patients with autoantibody-mediated nodo-paranodopathy, the depletion of antibodies leads to a clinical and electrophysiological improvement, even after a long disease duration. In our cohort, distal, length dependent axonal damages could not be fully reversed. Our data therefore suggest that early antibody depletion should be a main therapeutic goal in nodo-paranodopathy. Although the antibody titer cannot be used as an inter-individual indicator of disease severity, it may serve as a valuable intra-individual marker for treatment reevaluation and prognosis.

Nerve Ultrasound Distinguishes Non-Inflammatory Axonal Polyneuropathy From Inflammatory Polyneuropathy With Secondary Axonal Damage.

IntroductionChronic inflammatory demyelinating polyneuropathy (CIDP) may have a similar clinical and electrophysiological presentation to non-inflammatory axonal polyneuropathies (NIAPs) when secondary axonal damage occurs. We aimed to investigate if nerve ultrasound can help to differentiate CIDP with additional secondary axonal damage from NIAP.MethodsIn a retrospective analysis, the cross-sectional area (CSA) of the peripheral nerves measured by ultrasound at six suitable nerve sites was compared in 95 patients with CIDP and 82 patients with NIAP. We developed the adjusted Bochum ultrasound score (aBUS) ranging from 0 to 6 resulting from the number of sites with enlarged CSA (median, ulnar, radial, and sural nerve).ResultsThe mean CSA of patients with CIDP was enlarged at all six nerve sites compared with the mean CSA of patients with NIAP. A total of 21 patients with CIDP did not meet 2010 electrophysiological diagnostic criteria (European Academy of Neurology/Peripheral Nerve Society Guideline, EFNS/PNS criteria) for CIDP at examination timepoint but only in further follow-up, while 25 patients with NIAP fulfilled electrophysiological EFNS/PNS criteria for CIDP as “possible” or “probable” CIDP. To increase diagnostic power, we included aBUS measured by ultrasound in patients classified as “possible” or “probable” resulting in an improved specificity of 94% and a sensitivity of 59%, compared to a specificity of the EFNS/PNS criteria alone of 60% and sensitivity of 78%.ConclusionUsing nerve ultrasound and the aBUS as a complementary method to distinguish CIDP from NIAP in case of secondary axonal damage can facilitate the diagnosis of CIDP.

CIDP mimics: a case series

BackgroundTo report our experience with a group of patients referred for refractory CIDP who fulfilled “definite” electrodiagnostic EFNS criteria for CIDP but were found to have an alternate diagnosis.MethodsPatients who were seen between 2017 and 2019 for refractory CIDP that fulfilled “definite” electrodiagnostic ENFS criteria for CIDP, but had an alternate diagnosis, were included. Patients who correctly had CIDP, anti MAG neuropathy, or MMN with conduction block, were excluded from the study. Demographics, clinical and electrophysiological characteristics, pertinent workup, final alternate diagnoses, and outcomes were collected.ResultsSeven patients were included: POEMS (n = 5), CANOMAD (n = 1), and neurolymphomatosis (n = 1). Most patients reported neuropathic pain and leg swelling (n = 6) or significant weight loss (n = 4). All patients had a monoclonal protein, and most patients who were tested had an elevated VEGF and CSF cyto-albuminologic dissociation. Electrophysiology showed pronounced intermediate more than distal demyelination, and axonal loss in the lower extremities. Response to steroids or IVIG varied, but some patients did respond to these treatments, especially early in the disease.ConclusionPain, systemic symptoms, suggestive electrophysiological findings, and/or a serum monoclonal protein should raise suspicion for CIDP mimics. Initial response to steroids or IVIG, over reliance on CSF, and electrophysiology findings can all be misleading.

CIDP associated with Sjögren's syndrome.

This study addresses the challenging characterisation and differentiation of CIDP versus CIDP in association with Sjögren's syndrome to facilitate the process in clinical routine. Patients with both CIDP and Sjögren's syndrome and CIDP without Sjögren's syndrome were compared concerning relevant differences in clinical, laboratory and electrophysiological findings. 154 patients who fulfilled the diagnostic EFNS/PNS criteria for CIDP were included in the analysis. 54 of these patients additionally fulfilled the ACR/EULAR classification criteria for Sjögren's syndrome. The frequency of female patients was higher in patients with CIDP and Sjögren's syndrome (52%) versus CIDP patients without Sjögren's syndrome (28%). Furthermore, the occurrence of cranial nerve impairment was significantly higher in patients with Sjögren's syndrome (39% versus 14%). There were no significant group differences in the evaluation of initial symptoms, severity of disability judged by INCAT disability scale score, presence or distribution of sensory deficits, limb weakness and the presence of ataxia, pain or dysautonomia, CSF laboratory or electrophysiological findings. In conclusion, our data indicate that cranial nerve impairment and female gender might represent red flags for an additional Sjögren's syndrome in patients with CIDP. The patterns of clinical disabilities and electrophysiological findings due to peripheral nerve damage are similar in both CIDP entities.

MRI quantifies lumbosacral nerve root and sciatic nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy

PurposeChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a treatable, immune-mediated condition characterised by progressive or relapsing motor and sensory neurological deficits. The diagnosis is based on a combination of clinical, neurophysiological and supportive criteria, but can be challenging. In this study, we quantified the diameter and cross-sectional area of the lumbosacral nerve roots, and explored the imaging characteristics of the sciatic nerves, in patients with CIDP versus healthy controls using MRI. MethodsMRI of the lumbosacral plexus and both thighs was performed at 3 T. Orthogonal diameter and cross-sectional area of the lumbosacral nerve roots were measured, along with sciatic nerve cross-sectional area at the mid-thigh level. The MRI appearance of the sciatic nerves was also evaluated qualitatively. All measurements were performed by an observer blinded to the diagnosis. Results10 patients with CIDP and 10 healthy controls (age and sex-matched) were studied. Lumbosacral nerve root diameter and cross-sectional area were significantly increased in patients with CIDP compared to controls (mean diameter 6.0 ± 1.1 mm vs 4.8 ± 0.3 mm; p = 0.006), with a high sensitivity (89 %) and specificity (90 %) on ROC analysis. Sciatic nerve cross sectional area was also significantly increased in the CIDP group, and was accompanied by qualitative MRI changes. ConclusionsQuantitative MRI reveals significant hypertrophy of the lumbosacral nerve roots and sciatic nerves in patients with CIDP compared to controls. This study provides further evidence for the inclusion of lumbosacral nerve root and sciatic nerve hypertrophy on MRI as a supportive feature in the diagnostic criteria for CIDP.

CIDP Diagnostic Criteria and Response to Treatment

AbstractIntroduction: Diagnostic criteria for CIDP have been proven useful for clinical trials. However, use of these criteria in clinics has been limited by time constraints and unknown usefulness in predicting outcomes. Methods: A retrospective chart review of CIDP patients at the University of Kansas seen between 2008 and 2014 was performed. We determined the diagnostic criteria fulfilled by each patient and assessed treatment responses. A positive response was defined by improvement sensory or motor examination as determined by a neuromuscular physician.Results: There were 38 total patients included in the study. The response rate to IVIG in patients who fulfilled EFNS/PNS criteria was 20/22 (90.1%). Among patients who fulfilled AAN criteria, 8/9 (88.9%) responded positively to IVIG. Slightly lower response rates were seen in patients fulfilling INCAT criteria and Saperstein criteria at 10/15 (66.7%) and 12/17 (70.6%), respectively.Discussion: EFNS/PNS and AAN criteria can similarly predict IVIG treatment response.

Treatment of chronic inflammatory demyelinating polyradiculoneuropathy patients with antibodies against paranodal proteins

AbstractChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune‐mediated polyneuropathy characterized by relapsing or steadily progressive clinical course. A major feature of the syndrome is that it encompasses several pathogeneses, and it is actually assumed that both cellular and humoral immunity can be involved with superiority depending on the particular subtype. Although cellular immunity typified by macrophage‐derived demyelination can play an essential role in classical CIDP, other pathogeneses that involve humoral immunity, such as autoantibodies targeting node of Ranvier proteins (neurofascin155: NF155; contactin1: CNTN1; contactin‐associated protein1: Caspr1; and neurofascin186/140: NF186/140), can bring about functional demyelination and have been discovered in approximately 5–15% of CIDP patients. Interestingly, such antibodies commonly belong to the immunoglobulin G4 (IgG4) subclass, and thus lack complement‐binding sites and compete with other IgG subclasses for the antigens. Patients with these IgG4 autoantibodies share clinical presentations to a certain extent, and anti‐NF155 and anti‐CNTN1 antibodies are representative examples. Patients with these IgG4 subclass antibodies fulfill the conservative diagnostic criteria for CIDP: distal‐dominant weakness, tremor and sensory ataxia. These patients also show poor responses to conventional first‐line therapeutics, and are non‐responders to intravenous immunoglobulins in particular. Therefore, novel treatment strategies other than first‐line therapies are awaited, and to date, small retrospective studies and several case reports have suggested the efficacy of rituximab. Accordingly, the Efficacy and Safety of Rituximab in Refractory CIDP Patients with IgG4 Autoantibodies trial, a prospective trial investigating the efficacy and safety of rituximab, is currently in progress.

Spectrum of electrophysiology findings in metabolic disorders

Metabolic neuropathy includes peripheral nerve disorders with systemic diseases of metabolic origin. These include diabetes mellitus, uremia, hypothyroidism, hepatic failure, hypoglycemia, polycythemia, amyloidosis, porphyria, disorders of lipid / glycolipid metabolism, nutritional / vitamin deficiencies, mitochondrial disorders. To study prevalence & patterns of Peripheral Neuropathy in metabolic disorders like chronic kidney disease on maintenance Hemodiaysis, Diabetes mellitus and Hypothyroidism. 100 patient with single metabolic factor as a cause of neuropathy were included, 50 were Chronic Kidney Disease(CKD), 35 Diabetic 15 Hypothyroidic. Clinical profile, Electrophysiology finding were analyzed. Uremic group (50 patients) CRF polyneuropathy in 39 (13 females / 26 males). 21 clinical symptomatic polyneuropathy 10 patients objective neuropathy without symptoms of polyneuropathy and 8 patients had only electrophysiological evidence. Hyperkalemia in 24 out of 50 patients. Positive correlation between serum potassium and polyneuropathy (P value 0.007). 36 axonal type 3 have primary axonal changes with secondary demyelination. 35 (19 males / 16 females). 34 type 2, 1 type 1 diabetes mellitus. All patients are having neuropathy. All are symptomatic. 30 are axonal neuropathy, and 2 are primary axonal neuropathy with secondary demyelination, 3 had of which 1 met criteria for CIDP. Females 10 / Males - 5.Electrophysiologically CTS in 5 Cases. Electrophysiological abnormalities in 5. Common parameters affected were median motor and sensory latencies, CVs, prolonged F wave latencies suggestive of CTs, followed by sural amplitudes and CVs. Polyneuropathy in CKD, diabetes mellitus, Hypothyroidism still considerable despite awareness and advancements in management.

Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome.

Objective: Sjögren's syndrome is a heterogeneous inflammatory disorder frequently involving peripheral nerves with a wide spectrum of sensory modalities and distribution patterns. The objective of this cross-sectional study was to determine characteristics of Sjögren's syndrome as a cause for severe neuropathy with limb weakness.Methods: One hundred and eighty four patients with polyneuropathy associated with limb weakness underwent routine diagnostics including investigations for Sjögren's syndrome. Forty-four patients with Sjögren's syndrome (ACR-EULAR classification criteria) and severe neuropathy were identified.Results: Sjögren's syndrome was found at a median age of 63 years and the gender distribution showed a balanced female-male ratio of 1:1. Anti-SSA(Ro) antibodies were detected in 48% while seronegative patients were diagnosed with Sjögren's syndrome based on sialadenitis on minor salivary gland biopsy with a focus score ≥1. The majority of patients (93%) were diagnosed with Sjögren's syndrome after neurological symptoms appeared. Limbs were symmetrically involved in 84% of patients (57% tetraparesis, 27% paraparesis). Sensory function was not affected in 11% of patients indicating that Sjögren's syndrome associated neuropathy can present as a pure motor syndrome. Electrophysiological measurements did not reveal pathognomonic findings (23% demyelinating pattern, 36% axonal pattern, 41% both demyelinating and axonal damage signs). More than half of our patients fulfilled the European Federation of Neurological Societies (EFNS) diagnostic criteria for CIDP indicating that distinction between Neuro-Sjögren and other causes of neuropathy such as CIDP is challenging.Interpretation: Our findings show that severe neuropathy with limb weakness is often associated with Sjögren's syndrome. This is of great importance in identifying and understanding the causes of immune mediated polyneuropathy.

Correction: Absence of pathogenic mutations in CD59 in chronic inflammatory demyelinating polyradiculoneuropathy

[This corrects the article DOI: 10.1371/journal.pone.0212647.].

Clinical and laboratory features of anti-MAG neuropathy without monoclonal gammopathy

Antibodies against myelin-associated glycoprotein (MAG) almost invariably appear in the context of an IgM monoclonal gammopathy associated neuropathy. Very few cases of anti-MAG neuropathy lacking IgM-monoclonal gammopathy have been reported. We investigated the presence of anti-MAG antibodies in 69 patients fulfilling diagnostic criteria for CIDP. Anti-MAG antibodies were tested by ELISA and confirmed by immunohistochemistry. We identified four (5.8%) anti-MAG positive patients without detectable IgM-monoclonal gammopathy. In two of them, IgM-monoclonal gammopathy was detected at 3 and 4-year follow-up coinciding with an increase in anti-MAG antibodies titers. In conclusion, anti-MAG antibody testing should be considered in chronic demyelinating neuropathies, even if IgM-monoclonal gammopathy is not detectable.

Absence of pathogenic mutations in CD59 in chronic inflammatory demyelinating polyradiculoneuropathy.

ObjectiveMutations in CD59 cause CIDP-like polyneuropathy in children with inherited chronic hemolysis. We hypothesized that mutations in CD59 might be found in a subset of sporadic CIDP patients.Methods35 patients from two centers, fulfilling the EFNS/PNS diagnostic criteria for CIDP were included. CD59 coding region was amplified by PCR and Sanger sequenced.ResultsOne rare variant was detected in a patient which resulted in a synonymous change and predicted to be neutral. Pathogenic variants were absent in our cohort.InterpretationOur pilot study suggests that mutations in CD59 are absent in adult-onset sporadic CIDP.

Corticosteroids in chronic inflammatory demyelinating polyneuropathy

BackgroundChronic inflammatory demyelinating polyneuropathy (CIDP) can be treated with corticosteroids or intravenous immunoglobulins. Various corticosteroid regimens are currently used in CIDP, but it is unknown whether they are equally efficacious. In this retrospective study, we compared efficacy and safety of three corticosteroid regimens in CIDP patients.MethodsWe included treatment naïve patients that fulfilled the EFNS/PNS criteria for CIDP. Patients were treated with corticosteroids according to the local protocol of three CIDP expertise centres. Corticosteroid regimens consisted of daily oral prednisolone, pulsed oral dexamethasone, or pulsed intravenous methylprednisolone. Outcomes were number of responders to treatment, remission rate of treatment responders, overall probability of 5-year remission, and the occurrence of adverse events.ResultsA total of 125 patients were included. Sixty-seven (54%) patients received daily prednisone or prednisolone, 37 (30%) pulsed dexamethasone, and 21 (17%) pulsed intravenous methylprednisolone. Overall, 60% (95% CI 51–69%) responded to corticosteroids, with no significant difference between the three treatment regimens (p = 0.56). From the 75 responders, 61% (95% CI 50–73%) remained in remission, during a median follow-up of 55 months (range 1–197 months). The probability of responders reaching 5-year remission was 55% (95% Cl 44–70%), with no difference between the three groups. Adverse events leading to a change in treatment occurred in ten patients (8%). Two patients had a serious adverse event.ConclusionCorticosteroids lead to improvement in 60% of patients and to remission in 61% of treatment responders. There were no differences between treatment modalities in terms of efficacy and safety.

Combined central and peripheral demyelination: Clinical features, diagnostic findings, and treatment

Combined central and peripheral demyelination (CCPD) is rare, and current knowledge is based on case reports and small case series. The aim of our study was to describe the clinical features, diagnostic results, treatment and outcomes in a large cohort of patients with CCPD. Thirty-one patients entered this retrospective, observational, two-center study. In 20 patients (65%) CCPD presented, after an infection, as myeloradiculoneuropathy, encephalopathy, cranial neuropathy, length-dependent peripheral neuropathy, or pseudo-Guillain-Barré syndrome. Demyelinating features of peripheral nerve damage fulfilling European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria for CIDP were found in 23 patients (74%), and spatial dissemination of demyelinating lesions on brain MRI fulfilling the 2010 McDonald criteria for multiple sclerosis (MS) in 11 (46%). Two thirds of the patients had a relapsing or progressive disease course, usually related to the appearance of new spinal cord lesions or worsening of the peripheral neuropathy, and showed unsatisfactory responses to high-dose corticosteroids and intravenous immunoglobulins. The clinical presentation of CCPD was severe in 22 patients (71%), who were left significantly disabled. Our data suggest that CCPD has heterogeneous features and shows frequent post-infectious onset, primary peripheral nervous system or central nervous system involvement, a monophasic or chronic disease course, inadequate response to treatments, and a generally poor outcome. We therefore conclude that the current diagnostic criteria for MS and CIDP may not fully encompass the spectrum of possible manifestations of CCPD, whose pathogenesis remains largely unknown.

Electromyoneurography in the diagnosis of critical-illness patients

Critical illness polyneuropathy and myopathy (CIP and CIM) are characterized by muscle weakness and respiratory failure in patients who are treated intensive care units. CIP is sensorimotor axonal form of the disease that is usually associated with sepsis, septic syndromes and multiple-organ failure. Clinically, CIM has similar characteristics to the CIP, but the degree of muscle atrophy is greater. We analyzed 38 patients with diagnosis od acute inflammatory demyelinating polyneuropathy (GBS) from January 2013 till July 2014 treated at the neurological department of the Emergency Center of Clinical Center of Serbia. There were 20 men (mean age was 61.1 years) and 18 female patients (mean age was 54.82 years). Electrophysiological diagnosis of GBS was confirmed in 17 patients (47.22%). Two patients (5.5%) had criteria for CIDP; four patients (8.33%) for AMAN and/or AMSAN. Two patients had no motor and sensory responses (CMAP and SNAP) (5.5%) – CIP. Other patients had brachial plexopathy, polyneuropathy or Miller-Fisher syndrome. None of the patients was found to have CIM. Electromyoneurography is electrophysiological method for diagnosis CIP and/or CIM, but also to estimate the prediction of the development of these entities in more severe forms of GBS.

Different electrophysiological profiles and treatment response in ‘typical’ and ‘atypical’ chronic inflammatory demyelinating polyneuropathy

BackgroundChronic inflammatory demyelinating polyneuropathy (CIDP) is currently classified into ‘typical’ CIDP and ‘atypical’ subtypes such as multifocal acquired demyelinating sensory and motor neuropathy (MADSAM).ObjectivesTo assess the frequency of CIDP subtypes,...

Winging scapula due to demyelinating polyneuropathy

A 47-year-old male was admitted to our hospital with numbness and clumsiness in both hands. These symptoms developed 4 months ago and exacerbated progressively. A neurological examination revealed mild distal symmetrical sensory loss. He had no apparent muscle weakness in his shoulder muscles, but the medial section of the scapula was winging, especially after 120 of elevation and when he was asked to push the wall with both hands. The patient did not complain of pain. Nerve conduction studies (Table 1) demonstrated conduction blocks, slowed nerve conduction velocities, and prolonged distal latencies and F latencies in more than 2 peripheral nerves, meeting the electrophsyiological diagnostic criteria for CIDP [1]. By needle electromyography, we observed spontaneous denervation potentials in the serratus anterior muscle. There were no abnormalities in the other neck and shoulder muscles. His spinal magnetic resonance images were within normal limits. Examination of cerebrospinal fluid (CSF) revealed nothing except elevated protein levels (80 mg/dL). The patient was negative for anti-GM1, and no elevation of monoclonal antibodies was noted by blood or urine electrophoresis. There was nothing in his medical or family history that helped us exclude underlying hereditary polyneuropathy. Due to symmetrical diffuse demyelinating involvement in the nerve conduction studies, duration of the complaints, and elevated CSF protein levels, the patient was diagnosed with CIDP after excluding paraneoplastic conditions, diabetes mellitus, vasculitis, and rheumatological and hematological diseases. The patient was treated daily with 1 mgr/kg cts. His clinical symptoms, including the winging scapula, improved by the end of Week 8. In addition, the results of the nerve conduction studies improved slightly, and the conduction block abated (Table 2). Further, there were no abnormalities by repeated needle electromyography.

A Novel Marker for Neuroinflammation in Diabetic Neuropathy (P03.195)

Objective: To report a new marker for neuroinflammation in diabetic distal symmetric sensorimotor polyneuropathy. Background A conduction slowing seldom fulfilling the AAN criteria for CIDP has been observed in diabetic distal symmetric sensorimotor polyneuropathy (DSP) in several studies. Design/Methods: Urine secretory phopholipase activity (sPLA2) was measured in 34 DSP patients with at least one nerve conduction velocity (CV) slowing in the AAN demyelinating range (DSPs), 52 diabetic patients without CV slowing (DSPns), 5 diabetic patients without neuropathy (DWN) and 5 CIDP. Using multiple linear regression analysis, we related conduction velocity, (CV) ,distal latency (DL) and F latency to distal CMAP amplitude of median, ulnar, peroneal and tibial nerve in 76 patients with CIDP. The developed equations that assessed the range of slowing in CIDP were used to study conduction slowing in the above DSP patients. Results: sPLA2 activity was significantly higher is the DSPs in nmol/min/ml (1411.2±281.09) and CIDP (1349.1 ±328.45) compared to controls (581.56±73.95) p Conclusions: Urine sPLA2 activity is significantly higher in DSPn compared to DSPns. When combined to CV slowing, it could be used as a marker of inflammatory demyelination in diabetic neuropathy. Disclosure: Dr. Maybodi has nothing to disclose. Dr. Ende has nothing to disclose. Dr. Chen has nothing to disclose. Dr. Souayah has received personal compensation for activities with Walgreens as a consultant. Dr. Souayah has received research support from Talecris.

Une polyneuropathie avec atteinte des nerfs crâniens associée à une IgM monoclonale à activité anti-MAG/SGPG/SGPLG/sulfatides

A typically distal and symmetrical, slowly progressive sensorimotor demyelinating neuropathy is caused by monoclonal IgM against myelin-associated glycoprotein (MAG) and SGPG, SGLPG glycolipids in the context of a benign IgM paraproteinemia. We studied a patient with a neuropathy that fulfilled the diagnostic criteria for CIDP in whom IgM kappa anti-MAG/SGPG/SGLPG were detected. The patient was a 57-year-old man who had developed a slowly progressive distal sensorimotor neuropathy, involving the lower then upper limbs, with cranial nerves palsies (oro-pharyngo-laryngo territory). ENMG showed a demyelinating neuropathy with a disproportionate slowing of conduction in distal segments of motor and axonal features in the lower limbs. The first routine laboratory analysis revealed negative or normal findings. Several serum protein electrophoreses were normal. The third cerebrospinal fluid examination demonstrated a moderate and late rise in CSF protein level with no cells. Monoclonal IgM-kappa against MAG/SGPG/SGLPG, was detected; anti-MAG antibody titre in the serum was 20 059 BTU (N<1000). A small IgM-kappa paraprotein was identified by immunofixation. Electron microscopy failed to show nerve fibers with widening of outer lamellae of the myelin. There is no clinical improvement after different treatments, immunoglobulins IV, cortisteroids, plasma exchange, rituximab. It is not known whether this neuropathy is an atypical form of PNMAG or an CIDP associated with anti-MAG. When ENMG show a disproportionate slowing of conduction in distal segments of motor nerves, one should screen the serum with immunofixation to identify small monoclonal components. If IgM-MGUS is present, search should be undertaken for anti-MAG/SGPG/SGLPG antibodies. Diagnosis enables optimal treatment using, in severe cases, expensive current strategies with immunoglobulins IV, plasma exchange, and corticosteroids, or, in the event of no response, rituximab before resorting to more toxic drugs like cyclophosphamide.