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Neurology 2019 Feb 13. Epub 2019 Feb 13.

From the Department of Neurology (P.S.N.), National Neuroscience Institute, Singapore; Department of Neurology (P.S.N., P.J.D., R.S.L., M.V.P., P.J.B.D.), and Division of Biomedical Statistics and Informatics (P.T.), Mayo Clinic, Rochester, MN.

Objective: To determine the previously unknown incidence of lumbosacral-radiculoplexus neuropathy (LRPN) and its association with diabetes mellitus (DM).

Methods: LRPN defined by clinical and electrophysiologic criteria was identified among Olmsted County, Minnesota, residents during a 16-year period (2000-2015) using the unique facilities of the Rochester Epidemiology Project. DM was ascertained using American Diabetes Association criteria. Read More

J Neurol 2019 Feb 5. Epub 2019 Feb 5.

Department of Neurosciences, Odontostomatology and Reproductive Sciences, University of Naples " Federico II ", Via Pansini, 5, 81025, Naples, Italy.

Objective: The current clinical measures (ONLS, R-ODS, mRS, and MRC) may not be so sensitive in capturing minimal variations or measuring fatigue in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Our aim was to assess if 6-min walk test (6MWT) is able to increase the sensitivity in detecting response to therapy and to capture fatigue in CIDP patients.

Methods: We tested 6MWT in 42 CIDP patients. Read More

J Neuroimmunol 2019 Jan 29. Epub 2019 Jan 29.

Laboratory of Neuroimmunology, IRCCS Mondino Foundation, Pavia, Italy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with anti-neurofascin-155 antibodies is a subgroup of CIDP with tremor and poor response to intravenous immunoglobulins. A 23-year-old male presented with a 6-month history ataxic-stepping gait, stocking tactile hypoesthesia, areflexia, tremor at limbs and tongue. Neurophysiology and cerebrospinal fluid analysis supported the diagnosis of CIDP. Read More

Muscle Nerve 2019 Jan 30. Epub 2019 Jan 30.

Department of Neurology, University Hospitals Cleveland Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH, 44106, USA.

Introduction: Chronic immune sensorimotor polyradiculopathy (CISMP) is a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant affecting both sensory and motor nerve roots without evidence of peripheral nerve demyelination.

Method: We report a case series of nine patients with CISMP, identified from two tertiary centers. Clinical, electrodiagnostic, and neuroradiologic features, and treatment responses, were retrospectively reviewed. Read More

Neurol India 2019 Jan-Feb;67(Supplement):S94-S99

Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.

Introduction: Sjogren's syndrome (SS) is a systemic autoimmune disease that apart from involving the exocrine glands can affect any organ. Involvement of the peripheral nervous system results in a wide spectrum of neuropathic manifestations.

Objective: To evaluate the clinico-electrophysiological patterns as well as pathological characteristics of neuropathy in SS patients presenting to a neuromuscular clinic in a tertiary hospital from South India. Read More

J Child Neurol 2019 Jan 23:883073818825213. Epub 2019 Jan 23.

2 Institute of Child Neurology, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. During the acute phase, the disorder can be life-threatening by involving the respiratory muscles and the autonomic nervous system. Nevertheless, the prognosis is good, and most children achieve full recovery. Read More

Neuroepidemiology 2019 Jan 22;52(3-4):161-172. Epub 2019 Jan 22.

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

Background: Prevalence and incidence rates of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are required to determine the impact of CIDP on society. We aimed to estimate the prevalence and incidence of CIDP worldwide and to determine the effect of diagnostic criteria on prevalence and incidence.

Method: A systematic review was conducted for all published incidence and prevalence studies on CIDP until May 18, 2017. Read More

Mov Disord Clin Pract 2018 Sep-Oct;5(5):559-560. Epub 2018 Aug 24.

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

J Neurol Sci 2019 Feb 31;397:141-145. Epub 2018 Dec 31.

Department of Neurology and Neurophysiology, Amsterdam university medical centers, University of Amsterdam, Amsterdam, The Netherlands.

Intravenous immunoglobulins (IVIg) are an efficacious treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN). IVIg is considered in patients who have a high suspicion of an inflammatory neuropathy, but do not meet diagnostic criteria. The objective of this retrospective study was to assess which diagnostic results led to the decision to administer IVIg and to determine the rate of improvement. Read More

Neurochem Int 2018 Dec 22. Epub 2018 Dec 22.

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.

Demyelination diseases involving the central and peripheral nervous systems are etiologically heterogeneous with both cell-mediated and humoral immunities playing pathogenic roles. Recently, autoantibodies against nodal and paranodal proteins, such as neurofascin186 (NF186), neurofascin155 (NF155), contactin-1 (CNTN1), contactin-associated protein 1 (CASPR1) and gliomedin, have been discovered in not only chronic demyelinating conditions, such as multiple sclerosis (MS) and chronic inflammatory demyelinating polyradiculoneuropathy, but also in acute demyelinating conditions, such as Guillain-Barré syndrome. Only a minority of these patients harbor anti-nodal/paranodal protein antibodies; however, these autoantibodies, especially IgG4 subclass autoantibodies to paranodal proteins, are associated with unique features and these conditions are collectively termed nodopathy or paranodopathy. Read More

eNeurologicalSci 2018 Dec 22;13:38-39. Epub 2018 Nov 22.

Department of Neurology, University of Florida, Gainesville, FL 32610, United States.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired disorder of peripheral nerves and nerve roots. Its cause is unknown, but recently antibodies to nodal and paranodal proteins have been discovered in a small subset of CIDP patients. These contactin and neurofascin-related immune-mediated neuropathies are thought to be variants of CIDP and often respond suboptimally to standard therapy. Read More

Medicine (Baltimore) 2018 Dec;97(49):e13538

Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei.

Rationale: Autonomic symptoms are not uncommon in chronic inflammatory demyelinating polyneuropathy (CIDP), but they are mostly mild and transient and are overshadowed by somatic manifestations. Here, we report a very unusual case of CIDP with severe autonomic symptom, intestinal obstruction, as initial and persistent symptom which responded well to high-dose glucocorticoid and intravenous immunoglobulin treatment.

Patient Concerns: We described a patient with CIDP with precedent and long-lasting incomplete intestinal obstruction. Read More

Medicine (Baltimore) 2018 Dec;97(49):e13304

Department of Nephrology, Huashan Hospital, Fudan University, Shanghai, China.

Rationale: Inflammatory demyelinating neuropathies such as Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and focal segmental glomerulosclerosis (FSGS) are autoimmune disorders that may have a common pathogenesis. Here, we describe 2 unique cases of FSGS, 1 with GBS and the other with CIPD. We believe that reviewing these multisystemic diseases will help in better understanding of FSGS pathogenesis. Read More

Muscle Nerve 2018 Nov 29. Epub 2018 Nov 29.

Institute for Neurosciences of Montpellier, INSERM U1051, Montpellier University, Hopital Gui de Chauliac, Montpellier, France.

Neurobiol Dis 2019 Apr 22;124:240-247. Epub 2018 Nov 22.

Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health, Klinik und Hochschulambulanz für Neurologie, Berlin, Germany; Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health, Cluster of Excellence NeuroCure, Berlin, Germany; Berlin Institute of Health, 10178 Berlin, Germany; Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health, Center for Stroke Resarch Berlin, Berlin, Germany; German Center for Neurodegenerative Diseases (DZNE), Berlin, Germany; DZHK (German Center for Cardiovascular Research), partner site Berlin, Berlin, Germany.

Background: It was previously reported that high salt dietary conditions can drive autoimmunity and worsen severity and symptoms of autoimmune diseases. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a common autoimmune condition of the peripheral nervous system which leads to progressive paralysis and sensory deficits due to a demyelination and secondary axonal loss of peripheral nerves. We used a previously described model with a knockout of CD86 in non-obese diabetic mice (CD86 NOD), which results in the spontaneous development of an autoimmune peripheral neuropathy similar to CIDP and investigated the influence of a high salt diet on functional impairment, electrophysiological parameters, demyelination and neuroinflammation in these mice. Read More

J Neurol Neurosurg Psychiatry 2019 Feb 1;90(2):138-140. Epub 2018 Nov 1.

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA

Objective: To describe an expanded teased nerve fibre classification in disease association.

Methods: We reviewed four newly proposed teased nerve fibre types (): Type J, rope-like fibres; K, fibril-like clumps of osmium positivity; L, cellular debris along and within nerve fibres; M, circular axonal inclusions surrounded by thin myelin. Different clinical pathological entities were studied for these fibre types including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP: N=20); amyloid polyneuropathy (N=20); intraneural B-cell lymphoma (N=20) or adult-onset polyglucosan body disease (APBD: N=6) in comparison with 112 disease controls. Read More

Medicine (Baltimore) 2018 Oct;97(41):e12469

Department of Neurology, The Second Affiliated Hospital of Nanchang University.

Rationale: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disorder characterized by a symmetrical, sensorimotor involvement and slowly progressive onset peripheral neuropathy. Peripheral neuropathies have been reported in some central demyelination patients. However, the central nervous system focus in the CIDP patient can mimic neuromyelitis optica have not been recognized by most of us. Read More

Nat Rev Dis Primers 2018 Oct 11;4(1):31. Epub 2018 Oct 11.

Department of Neurology, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany.

Since the discovery of an acute monophasic paralysis, later coined Guillain-Barré syndrome, almost 100 years ago, and the discovery of chronic, steroid-responsive polyneuropathy 50 years ago, the spectrum of immune-mediated polyneuropathies has broadened, with various subtypes continuing to be identified, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN). In general, these disorders are speculated to be caused by autoimmunity to proteins located at the node of Ranvier or components of myelin of peripheral nerves, although disease-associated autoantibodies have not been identified for all disorders. Owing to the numerous subtypes of the immune-mediated neuropathies, making the right diagnosis in daily clinical practice is complicated. Read More

J Neurol 2018 Dec 10;265(12):2927-2933. Epub 2018 Oct 10.

Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), IRCCS San Raffaele Scientific Institute, Milan, Italy.

Although anti-myelin-associated glycoprotein (MAG) antibody neuropathy is reported as a slowly progressive disease, it can lead to significant disability and impairment of health-related quality of life (HR-QoL) and social participation. The aim of this cross-sectional study was to evaluate the functioning and HR-QoL determinants in 67 patients with anti-MAG neuropathy in terms of the International Classification of Functioning, Disability, and Health (ICF). Evaluations included: Medical Research Council (MRC) sum score, Sensory Modality Sum score (SMS), Berg balance scale (BBS), Fatigue Severity Scale (FSS), Visual Analogue Scale (VAS) for pain, 9-Hole Peg Test (9-HPT), 6-min Walk Distance (6MWD), Impact on Participation and Autonomy (IPA) and the physical component score (PCS) and mental component score (MCS) of the short-form-36 health status scale (SF-36) HR-QoL measure. Read More

J Neurol Neurosurg Psychiatry 2019 Feb 8;90(2):125-132. Epub 2018 Oct 8.

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

Objectives: A few variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been described, but their frequency and evolution to typical CIDP remain unclear. To determine the frequency and characteristics of the CIDP variants, their possible evolution to typical CIDP, and treatment response.

Methods: We applied a set of diagnostic criteria to 460 patients included in a database of Italian patients with CIDP. Read More

Zhonghua Nei Ke Za Zhi 2018 Oct;57(10):749-752

Department of Neurology, Peking University Third Hospital, Beijing 100191, China.

To explore clinical characteristics, electrophysiological findings and treatment response in diabetic patients with chronic inflammatory demyelinating polyradiculoneuropathy (DM-CIDP). Thirty-six CIDP patients were identified, 10 were DM-CIDP and 26 were non-DM-CIDP. Clinical medical records, electrophysiological data and treatment were retrospectively reviewed. Read More

J Neurol 2018 Dec 6;265(12):2906-2915. Epub 2018 Oct 6.

Universitätsklinik Ulm, RKU, Oberer Eselsberg 45, 89081, Ulm, Germany.

Objective: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, autoimmune-inflammatory disease of the peripheral nervous system. Recently, various immunoglobulin G4 (IgG4) type auto-antibodies have been described in patients with CIDP which can effectively be removed by immunoadsorption (IA). Therefore, we prospectively evaluated the therapeutic effect of IA in 17 patients with progressive CIDP not responding to other treatment. Read More

BMJ Case Rep 2018 Oct 2;2018. Epub 2018 Oct 2.

Department of Pediatrics, UNC Health Care System, Chapel Hill, North Carolina, USA.

J Neurol Neurosurg Psychiatry 2019 Feb 2;90(2):133-137. Epub 2018 Oct 2.

Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan

Objective: To propose the optimal diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome using appropriate statistical methods and disease controls.

Methods: This retrospective cohort study included 104 consecutive patients with suspected POEMS syndrome, among whom a gold standard group of 60 patients with definitive POEMS syndrome diagnosis were followed for at least 12 months to strictly exclude other disorders mimicking POEMS syndrome and to confirm response to POEMS syndrome-specific treatment. Thirty patients with chronic inflammatory demyelinating polyradiculoneuropathy (demyelinating polyradiculoneuropathy controls) and 30 with multiple myeloma or immunoglobulin light chain amyloidosis (monoclonal plasma cell proliferation controls) were also included. Read More

Magn Reson Imaging 2019 Jan 25;55:112-117. Epub 2018 Sep 25.

Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, Japan.

Purpose: MR neurography is known to be useful to evaluate nerve pathology. The purpose of this study was to evaluate the usefulness of simultaneous apparent T2 mapping and neurography with nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation enhancement imaging (SHINKEI) to distinguish patients with chronic inflammatory demyelinating polyneuropathy (CIDP) from healthy subjects.

Materials And Methods: This retrospective study included 13 patients with CIDP and five healthy subjects from 2015 to 2017. Read More

Fortschr Neurol Psychiatr 2018 09 24;86(9):566-574. Epub 2018 Sep 24.

Friedrich-Baur-Institut, Neurologische Klinik und Poliklinik, Klinikum der LMU München.

This paper is a practical survey of immune-mediated, inflammatory and hereditary neuropathies along with recommendations for diagnostic procedures. The large group of immune-mediated, inflammatory neuropathies includes the Guillain-Barré syndrome and chronic-inflammatory demyelinating polyradiculoneuropathy and their subtypes, vasculitic, paraneoplastic and paraproteinemic neuropathies as well as neuropathies resulting from connective tissue disorders. Besides clinical features such as time-dependent progression and distribution of sensorimotor deficits, characteristic electroneurographic findings and antibody profiles are considered. Read More

Brain Behav 2018 Oct 21;8(10):e01115. Epub 2018 Sep 21.

Department of Neurology, the First Affiliated Hospital of China Medical University, Shenyang, China.

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most commonly observed phenotype among chronic acquired demyelinating polyneuropathies and is clinically variable. The aim of this meta-analysis was to evaluate the diagnostic value and characteristics of CIDP targeting neurofascin 155 (NF155).

Methods: A systematic literature search was performed on March 2018, and two reviewers independently extracted data and assessed the risk of bias on MEDLINE, EMBASE, the Web of Science, and the Cochrane Library to identify relevant articles. Read More

Neurodegener Dis Manag 2018 10 18;8(5):285-288. Epub 2018 Sep 18.

Aston Brain Centre, School of Life & Health Sciences, Aston University, Birmingham, UK.

J Peripher Nerv Syst 2018 Dec 7;23(4):235-240. Epub 2018 Oct 7.

Department of Clinical Neurophysiology, APHP, Pitié-Salpêtrière Hospital, Paris, France.

We aimed to analyse the response to rituximab in a cohort of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients with associated disorders. We conducted a clinical and electrophysiological retrospective monocentric study in 28 CIDP patients. Response to rituximab was defined as (a) a five-point increase in the Medical Research Council sum score or a one-point decrease in the Overall Neuropathy Limitations Scale score, compared to the score at the first rituximab infusion, or (b) the discontinuation of, or reduced need for, the last treatments before rituximab initiation. Read More

Br J Radiol 2018 Dec 17;91(1092):20180501. Epub 2018 Sep 17.

2 Clinical Radiology, Graduate School of Medical Sciences, Kyushu University , Fukuoka , Japan.

Objective:: To evaluate the usefulness of simultaneous T mapping and neurography with nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation enhancement imaging (SHINKEI) in the lumbar plexus to distinguish patients with chronic inflammatory demyelinating polyneuropathy (CIDP) from healthy controls.

Methods:: Our institutional review boards approved this retrospective study, and written informed consent was waived. 10 patients with CIDP from 2015 to 2017 were studied along with 5 healthy controls on a 3 T scanner. Read More

J Clin Neurosci 2018 Nov 23;57:198-201. Epub 2018 Aug 23.

Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, 50603 Kuala Lumpur, Malaysia.

Diabetic patients with poor glycaemic control can demonstrate demyelinating distal sensorimotor polyneuropathy (D-DSP) on electrophysiology. Distinguishing D-DSP from chronic inflammatory demyelinating polyneuropathy (CIDP) can be challenging. In this study, we investigated the role of nerve ultrasound in differentiating the two neuropathies. Read More

Curr Opin Neurol 2018 Oct;31(5):551-558

Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.

Purpose Of Review: To provide an overview of polyneuropathy organomegaly endocrinopathy M-protein and skin changes (POEMS) syndrome, detailing new insights into pathogenesis, prognostic factors, treatments, and outcome scores.

Recent Findings: With the development of large multicentre national cohorts of patients, POEMS syndrome is evolving into a well characterized multisystem hematoneurological syndrome. Without early diagnosis significant disability results from the neuropathy. Read More

BMC Neurol 2018 Aug 14;18(1):113. Epub 2018 Aug 14.

Department of Neurology, Essen University Hospital, Hufelandstrasse 55, 45147, Essen, Germany.

Background: We present a patient fulfilling the electrophysiological criteria for definite chronic inflammatory demyelinating polyneuropathy (CIDP), antibody-positive myasthenia gravis (MG), and membranous glomerulonephritis (MGN) confirmed by biopsy. To our knowledge, this is the first case of the concomitant appearance of these three autoimmune diseases in a single patient.

Case Representation: A 42-year-old Caucasian male presented with rapidly progressive gait disturbance, distal weakness of the lower extremities, ascending hypoesthesia, impaired fine motor skills, and beginning cranial nerve palsy showing dysarthrophonia, facial paralysis, and eye movement abnormalities and was diagnosed as rapid onset (atypical) CIDP. Read More

Curr Opin Neurol 2018 Oct;31(5):534-540

Department of Neurology, University of Würzburg, Würzburg, Germany.

Purpose Of Review: To give an overview of recent data on the use of nerve and skin biopsy as a diagnostic tool in neuropathies.

Recent Findings: Axonal damage in a biopsy from a patient with chronic inflammatory demyelinating polyradiculoneuropathy may point to the presence of autoantibodies to paranodal proteins. In nonsystemic vasculitis of the peripheral nervous system, nerve biopsy is still the only means to make a definite diagnosis. Read More

Neuromuscul Disord 2018 Sep 12;28(9):757-765. Epub 2018 Jun 12.

The Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, 30 Guilford St, London, and MRC Centre for Neuromuscular Diseases & Neuroscience Unit, Great Ormond Street Hospital, London, UK.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a treatable chronic disorder of the peripheral nervous system. We retrospectively studied 30 children with a suspected diagnosis of CIDP. The diagnosis of CIDP was compared against the childhood CIDP revised diagnostic criteria 2000. Read More

Autoimmun Rev 2018 Sep 10;17(9):873-881. Epub 2018 Jul 10.

AP-HP, Pitié-Salpetrière Hospital, Department of Internal Medicine and Clinical Immunology, Paris, France.

We reviewed the efficacy of SCIg administration in terms of muscle strength maintenance and patient satisfaction comparing with IVIg in the treatment of auto-immune neuromuscular diseases. A systematic review was conducted, and identified studies from databases (PUBMED, EMBASE, EBSCO, Web of Science and Google Scholar) which were analyzed. The methodological quality of the selected publications was evaluated using the Newcastle-Ottawa Scale. Read More

Lancet Neurol 2018 Aug 9;17(8):689-698. Epub 2018 Jul 9.

Department of Neurology, Universitätsklinikum Düsseldorf and Center for Neurology and Neuropsychiatry LVR Klinikum, Heinrich-Heine-University, Düsseldorf, Germany.

Background: Fingolimod is approved for the treatment of relapsing-remitting multiple sclerosis and was effective in experimental autoimmune neuritis in rats, a possible model for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We aimed to evaluate the efficacy of fingolimod in delaying disability progression in patients with CIDP who withdrew from currently effective treatments (intravenous immunoglobulin [IVIg] or corticosteroids).

Methods: This double-blind, multicentre, randomised, placebo-controlled, parallel-group, event-driven study was done at 48 neurology centres in Australia, Canada, Israel, Japan, the USA, and nine countries in Europe. Read More

J Peripher Nerv Syst 2018 Sep 29;23(3):178-182. Epub 2018 Jul 29.

Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia.

It has been previously shown that patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are unemployed or retired have worse quality of life. The aim of this study was to assess predictors of early retirement in CIDP. One hundred five patients with CIDP were included. Read More

Clin Neurol Neurosurg 2018 Sep 3;172:90-92. Epub 2018 Jul 3.

Department of Neurology, Fujita Health University School of Medicine, Toyoake, Aichi, Japan. Electronic address:

We herein report the first combined central and peripheral demyelination (CCPD) case originally diagnosed as multifocal acquired demyelinating sensory and motor neuropathy followed by CNS impairments with a long lag time (160 days after first attack). The anti-neutral glycolipids antibodies, especially anti-lactosylceramide antibodies but not anti-NF155 antibodies typical for CCPD were detected in the acute phase of sera and cerebrospinal fluid (CSF) and disappeared in the recovery phase. This case strongly illustrated the significance of examining anti-neutral glycolipids antibodies in future anti-NF 155 antibodies-negative CCPD cases. Read More

J Neurol 2018 Sep 2;265(9):2052-2059. Epub 2018 Jul 2.

Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.

Background: Chronic 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. Read More

Neurologist 2018 Jul;23(4):131-134

Departments of Neurology.

Brucellosis, an endemic zoonosis in Portugal, is a multisystem disease, presenting with neurological manifestations in up to 25% of cases. Neurobrucellosis diagnostic criteria include evidence of central nervous system invasion, either by documenting increased blood-brain barrier permeability that normalizes after treatment or by Brucella isolation. We report 2 patients with systemic brucellosis presenting with neurological symptoms: A 28-year-old female with progressive hemiparesis associated with severe refractory thoracic and lumbar pain, whose spinal magnetic resonance imaging identified longitudinally extensive myelitis. Read More

J Neurol 2018 Aug 20;265(8):1916-1921. Epub 2018 Jun 20.

Department of Orthopaedics, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.

Objective: To assess the value of triple stimulation technique (TST) for diagnose of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Methods: Seven clinically suspected CIDP patients who did not fulfill EFNS/PNS electrodiagnostic criteria for demyelinating neuropathy were enrolled in our study. Routine nerve conduction studies, lumbar puncture, spinal cord magnetic resonance imaging and TST were detected. Read More

Muscle Nerve 2018 Sep;58(3):407-412

Department of Neurology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, South Korea.

Introduction: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients possess multiple risk factors for osteoporosis, but few studies have evaluated bone mineral density (BMD) in CIDP patients.

Methods: We retrospectively compared the BMD of CIDP patients with that of normal controls, and evaluated the clinical factors associated with osteoporosis.

Results: Total BMD was lower in CIDP patients than in normal controls (P = 0. Read More

J Autoimmun 2018 Aug 5;92:114-125. Epub 2018 Jun 5.

Fédération de Médecine Translationnelle de Strasbourg (FMTS), France; CNRS UMR7242 Biotechnology and Cell Signaling, University of Strasbourg, France /Laboratory of Excellence Medalis, France; University of Strasbourg Institute for Advanced Study (USIAS), Strasbourg, France. Electronic address:

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease of the peripheral nerves evolving with diffuse sensory and motor symptoms. Although it is claimed that in neurodegenerative pathologies, a common feature is the failure of proteolytic systems to adequately eliminate aggregated or misfolded proteins, it has not been addressed whether autophagy, a central "clearance" system delivering damaged intracellular components to lysosomes, is affected in CIDP. The focus of the present investigation was therefore to determine if some defects exist in autophagy processes in this setting and if they can be corrected or minimized using an appropriate treatment targeting this survival pathway. Read More

Mayo Clin Proc 2018 Jun;93(6):777-793

Department of Neurology, Mayo Clinic, Rochester, MN.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is probably the best recognized progressive immune-mediated peripheral neuropathy. It is characterized by a symmetrical, motor-predominant peripheral neuropathy that produces both distal and proximal weakness. Large-fiber abnormalities (weakness and ataxia) predominate, whereas small-fiber abnormalities (autonomic and pain) are less common. Read More

Zh Nevrol Psikhiatr Im S S Korsakova 2018;118(4):113-120

Sechenov First Moscow State Medical University, Moscow, Russia.

Diabetic polyneuropathy is the most often complication of diabetes mellitus. However, patients with diabetes may have other neuropathies, which need to be recognized and treated. Chronic nnflammatory demyelinating polyneuropathy is the most common non-diabetic polyneuropathy in patients with diabetes. Read More

Clin Exp Rheumatol 2019 Jan-Feb;37(1):146-155. Epub 2018 May 24.

Department of Medical Science, Section of Rheumatology, University of Ferrara and Azienda Ospedaliero-Universitaria S.Anna, Cona, Ferrara, Italy.

In the past years the peripheral nervous system (PNS) involvement in systemic lupus erythematosus (SLE) has received little attention despite its potential significant impact. The true prevalence of PNS in SLE reported in studies is variable and strongly influenced by American College of Rheumatology (ACR) case definition that includes seven PNS manifestations (acute inflammatory demyelinating polyradiculoneuropathy, autonomic disorder, mononeuropathy, myasthenia gravis, cranial neuropathy, plexopathy and polyneuropathy). Other peripheral manifestations, such as chronic inflammatory demyelinating polyradiculoneuropathy and small fibre neuropathy, not included in the ACR nomenclature, have not been well characterised in SLE. Read More

Eur J Paediatr Neurol 2018 Sep 13;22(5):870-877. Epub 2018 Apr 13.

Center for Pediatrics, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany; Center for Pediatrics, Department of Neuropediatrics and Muscle Disorders, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany.

CD59 is involved in lymphocyte signal transduction and regulates complement-mediated cell lysis by inhibiting the membrane attack complex. In the cases reported so far, congenital isolated CD59 deficiency was associated with recurrent episodes of hemolytic anemia, peripheral neuropathy, and strokes. Here, we report on a patient from a consanguineous Turkish family, who had a first episode of hemolytic anemia at one month of age and presented at 14 months with acute Guillain-Barré syndrome (GBS). Read More

Immunotherapy 2018 Aug 16;10(11):919-933. Epub 2018 May 16.

CSL Behring, GmbH. Marburg, Germany.

Subcutaneous IgG (SCIG) administration may be preferred over the intravenous route (IVIG) in chronic inflammatory demyelinating polyneuropathy (CIDP) because it minimizes 'end of cycle' treatment-related fluctuations, reduces systemic adverse effects, improves convenience/quality of life and potentially lowers overall costs. Early reports of the use of highly concentrated SCIG preparations suggested they were effective and well-tolerated in chronic inflammatory demyelinating polyneuropathy. This was confirmed in the Polyneuropathy and Treatment with Hizentra study of 172 subjects randomized to receive maintenance therapy with placebo or one of two doses of IgPro20 (20% IgG stabilized with L-Proline) for 6 months. Read More

J Fam Health 2017 May;27(3):26-28