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Neurol Sci 2020 Jul 10. Epub 2020 Jul 10.

Humanitas Clinical and Research Institute, Milan, Italy.

In its typical presentation, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) occurs more often in old males as a progressive/recurrent motor and sensory nerve dysfunction with tendon areflexia. However, CIDP has also atypical clinical presentations, including pure sensory neuropathies, among which chronic immune sensory polyradiculopathy (CISP) accounts for only 0.5% of all CIDP, with no juvenile cases reported as yet. Read More

Rinsho Shinkeigaku 2020 Jul 7. Epub 2020 Jul 7.

Department of Neurology, Nihonkai General Hospital.

A 41-year-old man noticed numbness of the fingers and toes, and gradually developed limb weakness and sensory impairment. The patient was diagnosed with typical chronic inflammatory demyelinating polyradiculoneuropathy. Over the course of clinical diagnosis, the limb and trunk ataxia, and finger tremor became prominent, and the presence anti-neurofascin-155 antibody was examined and confirmed positive. Read More

J Peripher Nerv Syst 2020 Jul 5. Epub 2020 Jul 5.

Neurology Unit, Department of Neuroscience, University of Padova, Padova, Italy.

Background: Neurolymphomatosis, the infiltration of the peripheral nervous system from lymphoid cells, represents an uncommon manifestation of lymphomas. We describe the challenging diagnostic work-up in a patient with neurolymphomatosis.

Case Report: a 58-yr-old woman with previous breast diffuse large B-cell lymphoma (DLBCL) treated with chemo- and radiation-therapy, presented with dysesthesias, neuropathic pain at left abdomen and thigh, and weakness at left lower limb 9 years after disease remission. Read More

Child Neurol Open 2020 Jan-Dec;7:2329048X20934914. Epub 2020 Jun 19.

Children's Hospital of Eastern Ontario Research Institute, University of Ottawa, Ontario, Canada.

Exposure to n-hexane or toluene-containing solvents such as glue or gasoline can produce clinical symptoms and neurophysiological findings that can mimic chronic inflammatory demyelinating polyneuropathy. The authors present a case of a boy with severe sensorimotor polyneuropathy with demyelinating features. Cerebrospinal fluid testing and magnetic resonance imaging spine did not show findings typical of chronic inflammatory demyelinating polyneuropathy. Read More

J Neurol 2020 Jun 27. Epub 2020 Jun 27.

Academic Department of Neuroscience and Sheffield, NIHR Translational Neuroscience BRC, Sheffield Teaching Hospitals, NHS Foundation Trust, University of Sheffield, Sheffield, UK.

Objective: Determine toxicity and efficacy of autologous hematopoietic stem cell transplantation (HSCT) for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are dependent on intravenous immunoglobulins or plasmapheresis.

Methods: Unselected peripheral blood stem cells were re-infused on day 0 after conditioning with cyclophosphamide 200 mg/kg/intravenously (IV), rATG (thymoglobulin) 5.5 mg/kg/IV, and rituximab 1000 mg/IV. Read More

J Neuroimaging 2020 Jun 27. Epub 2020 Jun 27.

Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.

Background And Purpose: Both clinical autonomic dysfunction and involvement of autonomic nerves have been reported in a range of peripheral nerve disorders. We employed nerve ultrasound to assess the size of the vagus nerve in a serial study of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and Charcot-Marie-Tooth type 1B (CMT1B) as compared to healthy controls (HCs). We correlated these findings with involvement of the median and ulnar nerves. Read More

J Peripher Nerv Syst 2020 Jun 24. Epub 2020 Jun 24.

Department of Neurology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.

The diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is often a challenge. The clinical presentation is diverse, accurate biomarkers are lacking, and the best strategy to initiate and maintain treatment is unclear. The aim of this study was to determine how neurologists diagnose and treat CIDP. Read More

Brain Sci 2020 Jun 17;10(6). Epub 2020 Jun 17.

Dipartimento di Neuroscienze, Università Cattolica del Sacro Cuore, 00168 Rome, Italy.

Objective: Segmental demyelination is the pathological hallmark of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but other elementary lesions are frequently observed, configuring a series of different pathological pictures. In this article, we review the pathological findings of a large series of sural nerve biopsies from our cohort of CIDP patients.

Patients And Methods: Patients with CIDP who underwent nerve biopsy were retrospectively selected from those referred to the Institute of Neurology of the "Università Cattolica del Sacro Cuore" in Rome, Italy, from 1982 to February 2020. Read More

Neurol Neurochir Pol 2020 Jun 18. Epub 2020 Jun 18.

University of Rzeszow, Collegium Medicum, Institute of Medical Sciences, Al. W. Kopisto 2A, 35-959 Rzeszów, Poland.

Clinical Rationale For Study: The sudden onset of autoimmune neurological diseases often threatens life. In such clinical situations, fast, effective and safe treatment is needed. Therapeutic plasma exchange (TPE) is an option in the treatment of autoimmune disorders. Read More

J Neuroimmunol 2020 Aug 8;345:577288. Epub 2020 Jun 8.

Neuromuscular and Neuroimmunology Service, Humanitas Clinical and Research Institute, Rozzano, Milan, Italy; Department of Medical Biotechnology and Translational Medicine, Milan University, Milan, Italy.

For the diagnosis of anti-MAG polyneuropathy the commercial ELISA manufacturer currently recommends a cut-off of 1000 Bühlmann Titer Units (BTU). We analyzed sera from 80 anti-MAG neuropathy patients and 383 controls (with other neuropathies or healthy controls) to assess the ELISA sensitivity and specificity at different thresholds. A better combination of sensitivity/specificity was found at a threshold >1500 BTU than at >1000 BTU. Read More

J Child Neurol 2020 May 29:883073820925302. Epub 2020 May 29.

Department of Child Neurology, State University of New York at Downstate, Brooklyn, NY, USA.

Objective: To analyze the available literature on papilledema in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), report the first detailed pediatric case, and explore the underlying pathophysiology.

Methods: First, we conducted a comprehensive literature review of all cases of papilledema in CIDP. Next, we reviewed each case, incorporating only those including cerebrospinal fluid analysis into the results. Read More

J Neurol 2020 May 19. Epub 2020 May 19.

Neuromuscular and Neuroimmunology Service, Humanitas Clinical and Research Center-IRCCS, Rozzano, Milan, Italy.

A few observational studies and randomized trials suggest that exercise and rehabilitation may improve activity limitation and quality of life (QoL) in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but the impact of other modifiable factors on the severity of the disease is not well understood. Using a structured questionnaire, we collected data on lifestyle and dietary habits of the patients included in the Italian CIDP database to investigate the possible influence of modifiable lifestyle factors on disability and QoL. Questionnaire data were available for 323 patients. Read More

Sci Rep 2020 May 13;10(1):7910. Epub 2020 May 13.

Unit of Neurology and Neuromuscular Diseases, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare and heterogeneous acquired sensory-motor polyneuropathy with autoimmune pathogenesis. Intravenous immunoglobulins (IVIG) are a well-established therapy for CIDP: it is well known that at least two-thirds of these patients need these infusions for several years. More recently, Subcutaneous Immunoglobulins (SCIg) have been proved to be effective: this finding has been confirmed either in isolated cases or in few randomized trials. Read More

J Peripher Nerv Syst 2020 04 28. Epub 2020 Apr 28.

Neuromuscular and Neuroimmunology Service, Humanitas Clinical and Research Institute, Rozzano, Milan, Italy.

Background and aims to report the clinical features and the relevance of diagnostic investigations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We retrospectively reviewed data from patients with a clinical diagnosis of CIDP included in a national database. Among the 500 included patients with a clinical diagnosis of CIDP, 437 patients (87%) fulfilled the EFNS/PNS criteria for CIDP (definite in 407, probable in 26, possible in four). Read More

Neurol Neuroimmunol Neuroinflamm 2020 Jul 16;7(4). Epub 2020 Apr 16.

From the Department of Internal Medicine (M.Z., D.A.H., M.W.F.H., A.E.W.), Division of Nephrology and Transplantation; Rotterdam Transplant Group (M.Z., D.A.H., M.W.F.H., A.E.W.); Department of Neurology (E.B., P.A.D., B.C.J.); and Immunology (E.B., P.A.D., B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands.

Can J Neurol Sci 2020 Apr 13:1-7. Epub 2020 Apr 13.

Department of Medicine, Divisions of Physical Medicine and Neurology, Peripheral Nerve Clinic, McMaster University, Hamilton, Ontario, Canada.

Background: The response of chronic inflammatory demyelinating polyneuropathy (CIDP) to intravenous immunoglobulins (IVIgs) treatment is well established. However, it remains unclear whether patients not responding to two IVIg treatments or those whose condition stabilizes (ICE trial) may benefit from additional doses. We aim to identify the time period required to reach maximal strength gains from IVIg treatment. Read More

J Neurol 2020 Apr 7. Epub 2020 Apr 7.

Department of Neurology, The Second Xiangya Hospital, Central South University, Renmin Road 139#, Changsha, 410011, Hunan, China.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is considered to be an immune-mediated heterogeneous disease involving cellular and humoral immunity. In recent years, autoantibodies against nodal/paranodal protein neurofascin155 (NF155), neurofascin186 (NF186), contactin-1 (CNTN1), and contactin-associated protein 1 (CASPR1) have been identified in a small subset of patients with CIDP, which disrupt axo-glial interactions at nodes/paranodes. Although CIDP electrodiagnosis was made in patients with anti-nodal/paranodal component autoantibodies, macrophage-induced demyelination, the characteristic of typical CIDP, was not observed. Read More

JMIR Res Protoc 2020 Apr 1;9(4):e17117. Epub 2020 Apr 1.

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated peripheral neuropathy that is currently classified into several clinical subtypes, which are presumed to have different pathogenic mechanisms. Recently, studies identified a subgroup of patients with CIDP who were positive for IgG4 autoantibodies against paranodal proteins, such as neurofascin-155 and contactin-1, who respond poorly to first-line therapies for typical CIDP, including intravenous immunoglobulin therapy.

Objective: This study aims to evaluate the efficacy and safety of intravenous rituximab according to IgG4 autoantibody status in patients with refractory CIDP. Read More

Muscle Nerve 2020 05 4;61(5):549-551. Epub 2020 Apr 4.

Department of Neurology, Cedars-Sinai Medical Center, Los Angeles, California.

Case Rep Neurol 2020 Jan-Apr;12(1):73-77. Epub 2020 Feb 14.

Neuromuscular Disease Clinic, Divisions of Physical Medicine and Neurology, Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy that is characterized by a slowly progressive sensory and motor involvement lasting at least 2 months. We present a CIDP patient on subcutaneous Ig (SCIg). Upon fine-tuning his dose from 24 to 28 g/week, this showed a dramatic improvement in both hand grip (13-25%) and dorsiflexion (73-278%). Read More

Neurol Sci 2020 Mar 24. Epub 2020 Mar 24.

Department of Neurology, Wroclaw Medical University, Wroclaw, Poland.

Introduction: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system, sometimes including the central nervous system. The aim of the study was the assessment of the prevalence of central sensory impairment and its reliance on peripheral nerve damage in patients with CIDP.

Material And Methods: Multimodal (visual-VEP, brainstem auditory-BAEP, somatosensory-SEP) evoked potentials (EPs) were studied in 24 patients diagnosed with CIDP. Read More

J Clin Neurosci 2020 May 19;75:228-231. Epub 2020 Mar 19.

Department of Neurology, Universidade Federal de São Paulo, SP, Brazil.

Charcot Marie Tooth (CMT) due to myelin protein zero (MPZ) mutations, may cause a wide variation of phenotypes, depending on the localization of the mutation within the gene. Among the most common phenotypes are: an infantile onset disease with extremely slow nerve conduction velocities (CMT1B) and an adult onset phenotype with nerve velocities in the axonal range (CMT2I). We reported a patient with CMT1B (MPZ p. Read More

J Neurol Sci 2020 Jun 19;413:116777. Epub 2020 Mar 19.

Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, Genoa, Italy; IRCCS Ospedale Policlinico San Martino, Genoa, Italy.

In chronic polyneuropathies associated with hematologic malignancy (HM) the optimal treatment management is primarily focused on the HM, but the parallel response of the neuropathy is still unclear. Rituximab is a recognized therapeutic choice in anti-MAG antibody polyneuropathy, that might be useful also in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with HM. The efficacy of immunochemotherapy, which is the standard approach to malignant lymphoproliferative diseases, has been poorly investigated in polyneuropathies. Read More

Eur Cytokine Netw 2019 Dec;30(4):130-134

Department of Neurosciences, Division of Neurology, Geneva University Hospitals, Geneva, Switzerland, Department of Diagnostic Division of Laboratory Medicine, Geneva University Hospitals, Geneva, Switzerland, Department of Pathology and Immunology, Faculty of Medicine, University of Geneva, Geneva, Switzerland.

This pilot study was designed to compare the levels of interleukin-8 (IL-8), a pro-inflammatory chemokine, in the cerebrospinal fluid (CSF) of patients with Guillain-Barre syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), non-inflammatory polyneuropathy (PNP), and other non-inflammatory neurological diseases (functional syndrome or migraine). The results show elevated CSF IL-8 levels in GBS compared to the other groups (p < 0.05). Read More

Clin Neurophysiol 2020 Apr 6;131(4):921-927. Epub 2020 Feb 6.

Referral Centre for Neuromuscular Diseases and ALS, La Timone hospital, Marseille, France; Aix-Marseille University, Timone Neuroscience Institute, UMR CNRS 7289, 13005 Marseille, France. Electronic address:

Objective: Chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) with antibodies against neurofascin 155 (Nfasc155) or contactin-1 (CNTN1) have distinctive clinical features. Knowledge on their electrophysiological characteristics is still scarce. In this study, we are investigating whether these patients have specific electrophysiological characteristics. Read More

eNeurologicalSci 2020 Mar 30;18:100224. Epub 2020 Jan 30.

Department of Neurology, Seoul National University College of Medicine, Neuroscience Research Institute, Seoul National University Medical Research Council, Seoul National, Republic of Korea, University-Seoul Metropolitan Government Boramae Medical Center, Seoul, South Korea.

Treatment-related fluctuation (TRF), only defined in Guillain-Barre syndrome (GBS), refer to the deterioration of symptoms following treatment-induced improvement, and implies disease activity lasting beyond the effect of immunotherapy. Here, we first propose the concept of TRF in subacute inflammatory demyelinating polyneuropathy (SIDP) with description of a corresponding case. A 27-year-old female presented with acute flaccid paralysis, and experienced two sequential episodes of TRF, the latter occurring around 8 weeks from disease onset. Read More

Muscle Nerve 2020 05 17;61(5):600-607. Epub 2020 Feb 17.

Department of Neurology, University Hospital Aachen, Aachen, Germany.

Background: Muscle MRI is of increasing importance for neuromuscular patients to detect changes in muscle volume, fat-infiltration, and edema. We developed a method for semi-automated segmentation of muscle MRI datasets.

Methods: An active contour-evolution algorithm implemented within the ITK-SNAP software was used to segment T1-weighted MRI, and to quantify muscle volumes of neuromuscular patients (n = 65). Read More

PLoS One 2020 4;15(2):e0228679. Epub 2020 Feb 4.

University Hospital Frankfurt, Department of Neurology, Frankfurt am Main, Germany.

Background: Chronic autoimmune demyelinating polyneuropathies (CADP) result in impaired sensorimotor function. However, anecdotal clinical observations suggest the development of cognitive deficits during the course of disease.

Methods: We tested 16 patients with CADP (11 patients with chronic inflammatory demyelinating polyneuropathy, 4 patients with multifocal motor neuropathy and 1 patient with multifocal acquired demyelinating sensory and motor neuropathy) and 40 healthy controls (HC) with a neuropsychological test battery. Read More

Int J Low Extrem Wounds 2020 Jan 29:1534734619896462. Epub 2020 Jan 29.

Pisa University Hospital, Pisa, Italy.

Guillain-Barré syndrome (GBS) represents an acute inflammatory immune-mediated demyelinating polyradiculoneuropathy with an incidence of 0.6 per 100 000 people. In this article, we report the case of a 19-year-old girl affected by GBS since the age of 2 who presented at our clinic complaining for a chronic plantar hindfoot-infected ulceration. Read More

Zh Nevrol Psikhiatr Im S S Korsakova 2019 ;119(12):94-99

Yaroslavl State Medical University, Yaroslavl, Russia.

The authors report the clinical case of a 7-year-old child with gradual development in left leg weakness considering by the neurologist as the unknown lesion of the sciatic nerve. According to the results of clinical and instrumental examinations, the diagnosis was established as a focal form of chronic inflammatory demyelinating polyneuropathy (CIDP). The dysimmune cause of the disease was confirmed by MRI of the lumbosacral plexus with contrast enhancement of neural structures and response to therapy with high-dose intravenous immunoglobulin in combination with corticosteroids. Read More

J Peripher Nerv Syst 2020 Mar 4;25(1):4-8. Epub 2020 Feb 4.

Department of Neurology, Johns Hopkins University School of Medicine Meyer 6-181a, North, Wolfe Street, Baltimore, US.

Mult Scler Relat Disord 2020 May 14;40:101943. Epub 2020 Jan 14.

Department of Internal Medicine, University of Zambia School of Medicine, Lusaka, Zambia; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address:

Overlap syndromes, where otherwise distinct autoimmune processes of the central and peripheral nervous systems are present in the same patient, are uncommon and have not been previously reported in sub-Saharan Africa. We present a case of a 32-year-old man who was found to have both clinically isolated syndrome and chronic inflammatory demyelinating polyneuropathy, highlighting the importance of continued efforts to establish the prevalence of demyelinating disease in the region given the limited treatment options currently available for autoimmune disease. Read More

Turk J Pediatr 2019 ;61(2):275-278

Division of Child Neurology, Department of Pediatrics, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey.

Edem P, Karakaya M, Wirth B, Okur TD, Yiş U. Giant axonal neuropathy: A differential diagnosis of consideration. Turk J Pediatr 2019; 61: 275-278. Read More

Discov Med 2019 08;28(152):107-111

Indiana University - Purdue University Indianapolis, Indianapolis, IN 46202, USA.

Importance: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune neurological disorder that is characterized by symmetrical progressive worsening or relapsing weakness and numbness of the limbs. There are no reliable diagnostic tests or definitive diagnostic criteria, and the diagnosis remains one of excluding other cases of polyneuropathy. Typical treatment for CIDP includes corticosteroids, intravenous immunoglobulin (IVIG), and plasma exchange. Read More

Eur J Radiol 2020 Mar 3;124:108816. Epub 2020 Jan 3.

Department of Radiology, Huashan Hospital, Fudan University, 12 Middle Wulumuqi Rd, Shanghai, 200040, China. Electronic address:

Purpose: MR neurography(MRN) is an advanced imaging technique to visualize peripheral nerves. Our aim was to determine the value of morphological features of lumbosacral nerve roots on MRN in diagnosing chronic inflammatory demyelinating polyradiculoneuropathy(CIDP) and analyze their correlations with electrophysiological parameters.

Methods: MRN of lumbosacral plexus was performed in 21 CIDP patients and 21 healthy volunteers. Read More

J Neuropathol Exp Neurol 2020 03;79(3):247-255

Department of Neurology, Nerve-Muscle Unit, CHU Bordeaux (Pellegrin University Hospital), Bordeaux, France.

Whatever the cause of myelin damage of the peripheral nervous system, the initial attack on myelin by a dysimmune process may begin either at the internodal area or in the paranodal and nodal regions. The term "nodo-paranodopathy" was first applied to some "axonal Guillain-Barré syndrome" subtypes, then extended to cases classified as chronic inflammatory demyelinating polyradiculoneuropathy bearing IgG4 antibodies against paranodal axoglial proteins. In these cases, paranodal dissection develops in the absence of macrophage-induced demyelination. Read More

Muscle Nerve 2020 05 21;61(5):575-579. Epub 2020 Jan 21.

Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas.

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder in which early effective treatment is important to minimize disability from axonal degeneration. It has been suggested that some patients with CIDP may benefit from rituximab therapy, but there is no definitive evidence for this.

Methods: Baseline and post-rituximab-therapy neuromuscular Medical Research Council (MRC) sum scores, Inflammatory Neuropathy Cause and Treatment (INCAT) disability score, and functional status were assessed in 11 patients with refactory CIDP. Read More

J Peripher Nerv Syst 2020 Mar 14;25(1):19-26. Epub 2020 Jan 14.

Department of Neurosciences, University of Padova, Padova, Italy.

Hereditary neuropathies may be misdiagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). A correct diagnosis is crucial for avoiding unnecessary therapies and access genetic counseling. We report on nine patients (seven men, mean age 49. Read More

J Neurol 2020 Jan 6. Epub 2020 Jan 6.

Amyloidosis Center Foundation, IRCCS Policlinico San Matteo, San Matteo, Italy.

Amyloid transthyretin (ATTR) amyloidosis with polyneuropathy (PN) is a progressive, debilitating, systemic disease wherein transthyretin protein misfolds to form amyloid, which is deposited in the endoneurium. ATTR amyloidosis with PN is the most serious hereditary polyneuropathy of adult onset. It arises from a hereditary mutation in the TTR gene and may involve the heart as well as other organs. Read More

Muscle Nerve 2020 04 22;61(4):521-526. Epub 2020 Jan 22.

Department of Neurology, Otto-von-Guericke University, Magdeburg, Germany.

Background: We present one patient with an initial diagnosis of Guillain-Barré syndrome (GBS) and one with Charcot-Marie-Tooth disease (CMT) type 1A.

Methods: Both patients underwent ankle tibial nerve fusion-imaging of high-resolution ultrasound (HRUS) with 7T MR neurography (MRN).

Results: In GBS, the nerve was enlarged, T2-hyperintense, and showed increased vascularization 21 months after symptom onset. Read More

Clin Neurophysiol Pract 2019 9;4:190-193. Epub 2019 Sep 9.

Department of Neurology, Henry Ford Health System, Detroit, MI, USA.

Objectives: To evaluate sensory electrophysiology, terminal latency index (TLI), and treatment response in idiopathic and diabetic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Methods: We performed a retrospective review of 147 patients with CIDP who underwent electrodiagnostic evaluation (January 2000-December 2015). Eighty-nine patients fulfilled electrophysiological criteria described by the Ad hoc Subcommittee of the American Academy of Neurology and Albers et al. Read More

J Neuroimmunol 2020 02 16;339:577139. Epub 2019 Dec 16.

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan. Electronic address:

To clarify the immunogenetic background of patients with immunoglobulin G (IgG)4 anti-neurofascin 155 (NF155) antibody-positive chronic inflammatory demyelinating polyneuropathy (CIDP), we genotyped the extended human leukocyte antigen (HLA) haplotypes in 22 Japanese patients with this disorder and compared them with those of healthy Japanese controls. All IgG4 anti-NF155 antibody-positive CIDP patients exclusively carried either HLA-DRB1*15:01-DRB5*01:01-DQA1*01:02-DQB1*06:02 or -(A*24:02)-B*52:01-C*12:02-DRB1*15:02-DRB5*01:02-DQA1*01:03-DQB1*06:01, resulting in significantly increased HLA-DRB1*15, -DRB1*15:01, -DQB1*06:01/06:02, -DQB1*06:02, and -DRB1*15:01-DQB1*06:02 frequencies compared with healthy Japanese controls. These findings indicate the involvement of specific HLA class II molecules in the pathomechanisms of IgG4 anti-NF155 antibody-positive CIDP. Read More

Neurol India 2019 Nov-Dec;67(6):1532-1535

Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

Distal acquired demyelinating symmetric neuropathy (DADS) is a variant of chronic inflammatory demyelinating polyneuropathy (CIDP) characterized by symmetrical, distal, sensory or sensorimotor involvement. DADS with M-protein (DADS-M) is less responsive to immunotherapy compared to those without M-protein (DADS-I). We report a case of DADS-I with severe clinical presentation viz. Read More

Neurology 2020 02 18;94(6):e635-e638. Epub 2019 Dec 18.

From the National Hospital of Neurology and Neurosurgery (J.S., C.E., S.S.-G., M.P.L., A.C.); MRC Centre for Neuromuscular Diseases, Department of Neuromuscular Diseases (M.K., A.R., H.M., M.M.R.), UCL Institute of Neurology; Department of Cardiology (R.B.), University College London Hospital; and Department of Neuroimmunology (M.P.L.), Institute of Neurology, London, UK.

Our objective was to evaluate whether IV immunoglobulin (IVIg) increases the risk of thromboembolic events in neurology outpatients with inflammatory neuropathies, as there is conflicting evidence supporting this hypothesis, mainly from non-neurologic cohorts. We investigated this question over 30 months in our cohort of patients with inflammatory neuropathies receiving regular IVIg and found a greater incidence of arterial and venous thromboembolic events than population-based rates determined by hospital admissions data. Vascular risk factors were more common in the event group but there were no IVIg administration factors that contributed to the risk. Read More

Neurology 2020 01 11;94(1):e62-e74. Epub 2019 Dec 11.

From the Departments of Radiology and Nuclear Medicine (J.O., S.D.R., M.P.E., M.W.A.C., M.M., A.J.N.), Neurology (F.E., I.N.v.S., M.d.V., C.V.), and Biomedical Engineering and Physics (G.J.S., M.W.A.C.), Amsterdam UMC, University of Amsterdam; Departments of Radiology (J.J.S.) and Neurology (P.A.v.D.), Erasmus Medical Center, Rotterdam; and Departments of Radiology (M.F.) and Neurology (H.S.G.), University Medical Center Utrecht, the Netherlands.

Objective: To assess and compare the diagnostic performance of qualitative and (semi-)quantitative MRI and ultrasound for distinguishing chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) from segmental spinal muscular atrophy (sSMA).

Methods: Patients with CIDP (n = 13), MMN (n = 10), or sSMA (n = 12) and healthy volunteers (n = 30) were included. MRI of the brachial plexus, using short tau inversion recovery (STIR), nerve-specific T2-weighted (magnetic resonance neurography [MRN]), and diffusion tensor imaging (DTI) sequences, was evaluated. Read More

Bull Cancer 2020 Jan 7;107(1S):S104-S113. Epub 2019 Dec 7.

AP-HP, hôpital St-Louis, centre de référence des maladies auto-immunes systémiques rares d'Île-de-France, unité de médecine interne : maladies auto-immunes et pathologie vasculaire (UF 04), 1, avenue Claude Vellefaux, 75010 Paris, France; Institut de recherche Saint-Louis, université Paris Denis Diderot, Sorbonne Paris Cité, EA 3518, Paris, France; Department of Internal Medicine, McGill University, Montreal, Canada. Electronic address:

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a chronic autoimmune disease involving the peripheral nervous system, characterized by focal and segmental demyelination accounting for neurological deficit. CIDP diagnosis is based on several criteria and requires the presence of specific clinical symptoms and of demyelinating criteria on the electroneuromyogram (ENMG) or of additional supportive criteria (spinal fluid examination with dissociation between albumin level and cellular abnormalities, nervous abnormalities on MRI or other minor abnormalities on ENMG, demyelinating features on nerve biopsy or patient improvement under so-called first-line therapy with immunodulator treatment). After failure of two successive first line immunomodulating drug therapies (corticosteroids, immunomodulating immunoglobulins, or plasma exchange), several options can be considered as second line therapies. Read More

Presse Med 2019 11;48(11 Pt 2):317-318

Université Paris Descartes, Hôpital Cochin, Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases: Vasculitis and Scleroderma, Paris, France. Electronic address:

BMJ Case Rep 2019 Dec 2;12(12). Epub 2019 Dec 2.

Department of Neurology, University Hospital of Wales, Cardiff, UK.

This case of chronic inflammatory demyelinating polyneuropathy (CIDP) shows that a patient's condition can evolve from the point of admission, gradually manifesting its underlying cause. Our patient's initial presentation of backpain and lower limb weakness prompted investigations which ruled out compressive myelopathy and neuropathy. As upper limb weakness developed later, along with a more proximal and symmetrical pattern of lower limb weakness, the clinical picture suggested polyneuropathy. Read More

Muscle Nerve 2020 03 1;61(3):316-324. Epub 2020 Jan 1.

Department of Neurology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.

Introduction: The effect of long-lasting immune-modulating therapy was studied in patients with chronic inflammatory demyelinating polyneuropathy (CIDP).

Methods: A population-based, cross-sectional study of treated patients referred to the Danish health-care system between 1985 and 2006.

Results: The 51 participating patients had a median disease duration of 16 (interquartile range, 14-21) years. Read More

Eur J Neurol 2020 Apr 22;27(4):692-701. Epub 2019 Dec 22.

INSERM U1051, Institut des Neurosciences de Montpellier (INM), Université de Montpellier, Montpellier, France.

Background And Purpose: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogeneous autoimmune disorder critically lacking diagnostic biomarkers. Autoantibodies to nodal and paranodal components have recently been described in a small subset of patients. Here, the diagnostic value of immune reactivity toward the myelin compartment was investigated. Read More