Search Our Scientific Publications & Authors

J Neurol 2021 Jun 12. Epub 2021 Jun 12.

Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, 12 Middle Wulumuqi Road, Shanghai, 200040, China.

Background: Current standard treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been proved effective, but it is poorly effective in refractory patients and unclear for anti-IgG4 antibody-associated CIDP. Rituximab is a B cell-depleting monoclonal antibody. It has been applied as one of the management strategies in CIDP, but its efficacy is unknown. Read More

Front Neurosci 2021 24;15:637336. Epub 2021 May 24.

Department of Neurology, The Second Xiangya Hospital, Central South University, Changsha, China.

To review the available evidence on sensitivity and specificity of anti-NF155 antibody detection in diagnosing a specific subset of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and to calculate the frequencies of different autoantibodies to paranodal proteins. Diagnosis of CIDP relies on clinical and neurophysiologic criteria and lacks useful diagnostic biomarkers. A subset of CIDP patients exhibit atypical clinical phenotypes and impaired response to conventional treatments. Read More

J Peripher Nerv Syst 2021 Jun 4. Epub 2021 Jun 4.

Yeditepe University Department of Pediatrics, İstanbul, Turkey.

Objective: To revise the 2010 consensus guideline on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Methods: Seventeen disease experts, a patient representative, and two Cochrane methodologists constructed 12 PICO questions regarding diagnosis and treatment to guide the literature search. Data were extracted and summarized in GRADE summary of findings (for treatment PICOs) or evidence tables (for diagnostic PICOs). Read More

Eur J Neurol 2021 Jun 1. Epub 2021 Jun 1.

Neurology Department, APHP, CHU de Bicêtre, Le Kremlin-Bicêtre, 94276, France.

Introduction: Charcot-Marie-Tooth (CMT) disease, an untreatable hereditary polyneuropathy, may mimic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a treatable neuropathy.

Objective: In this retrospective study, we analyzed the characteristics of CMT patients misdiagnosed as CIDP in 16 University Hospitals from 3 countries, compared these patients with a reference group of CIDP patients, and estimated the cost of misdiagnosis.

Results: Amongst 1104 CIDP cases, we identified 35 CMT patients misdiagnosed as CIDP (3. Read More

Front Immunol 2021 12;12:659038. Epub 2021 May 12.

Skull Base Research Center, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Protein inhibitors of activated STAT (PIAS) are involved in the regulation of the JAK/STAT signaling pathway and have interactions with NF-κB, p73 and p53. These proteins regulate immune responses; therefore dysregulation in their expression leads to several immune-mediated disorders. In the present study, we examined expression of - in peripheral blood of patients with acute/chronic inflammatory demyelinating polyradiculoneuropathy (AIDP/CIDP) compared with healthy subjects. Read More

Neurol Sci 2021 May 21. Epub 2021 May 21.

Dysimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Rome, Italy.

Introduction: Electrophysiological diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may be challenging. Thus, with the aim ofproviding some practical advice in electrophysiological approach to a patient with suspected CIDP, we analyzed electrophysiological data from 499 patients enrolled inthe Italian CIDP Database.

Methods: We calculated the rate of each demyelinating feature, the rate of demyelinating features per nerve, the diagnostic rate for upper andlower limb nerves, and, using a ROC curve analysis, the diagnostic accuracy of each couple of nerves and each demyelinating feature, for every CIDP subtype. Read More

J Clin Neuromuscul Dis 2021 Jun;22(4):200-208

Department of Neurology, University of Utah, Salt Lake City, UT.

Abstract: This edition of What is in the Literature focuses on chronic immune neuropathies as they represent treatable conditions. There are formal criteria to solidify the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but patients are encountered who have clinical and electrodiagnostic features of CIDP but do not fulfill diagnostic criteria. These patients are addressed in recent publications. Read More

Qual Life Res 2021 May 13. Epub 2021 May 13.

Neurology Clinic, University Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, 6, Dr. Subotic Sr. Street, 11 000, Belgrade, Serbia.

Neurol Sci 2021 May 4. Epub 2021 May 4.

Department of Neurology, Military Hospital, Tunis, Tunisia.

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare affection of the peripheral nervous system. Its diagnostic criteria have evolved since 1975. The aim of our work is to study the epidemiological, clinical, and paraclinical aspects of CIDP. Read More

Clin Neurol Neurosurg 2021 Apr 20;205:106654. Epub 2021 Apr 20.

Neurology Clinic, University Clinical Centre of Serbia, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia.

Objectives: At a time of global health crisis, fear, anxiety, and stress levels increase. The effects of protracted social isolation, and media related misinformation's about the coronavirus disease 2019 (COVID-19) resulting in increased fear/stress related to the insufficiently known illness. The aim was to assess the influence of the COVID-19 health crisis on patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Read More

Cureus 2021 Mar 27;13(3):e14146. Epub 2021 Mar 27.

Neurology, Yale School of Medicine, New Haven, USA.

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a relapsing-remitting or progressive inflammatory neuropathy, which can present in a multitude of phenotypes. It can be a challenging condition to diagnose and requires thorough clinical evaluation and electrodiagnostic testing. With the outbreak of coronavirus disease in 2019 (COVID-19), large portions of the medical field converted to telemedicine to facilitate patient visits. Read More

BMJ Case Rep 2021 Apr 22;14(4). Epub 2021 Apr 22.

Internal Medicine, All India Institute Of Medical Science, Rishikesh, Uttarakhand, India

Chronic inflammatory demyelinating polyneuropathy (CIDP) is probably the best-recognised progressive immune-mediated peripheral neuropathy. It presents with symmetrical, motor predominant peripheral neuropathy that produces both distal and proximal weakness. Here we report a case of a 38-year-old man who presented with chronic additive large and small joint inflammatory polyarthritis, associated with morning stiffness, anasarca associated with frothy urine and progressive episodic, relapsing and remitting, sensorimotor lower motor neuron type quadriparesis without any bladder and bowel involvement. Read More

Brain 2021 May;144(4):1183-1196

Neuromuscular Diseases Unit, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Spain.

Previous studies have described the clinical, serological and pathological features of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and antibodies directed against the paranodal proteins neurofascin-155, contactin-1 (CNTN1), contactin-associated protein-1 (Caspr1), or nodal forms of neurofascin. Such antibodies are useful for diagnosis and potentially treatment selection. However, antibodies targeting Caspr1 only or the Caspr1/CNTN1 complex have been reported in few patients with CIDP. Read More

Surg Neurol Int 2021 24;12:114. Epub 2021 Mar 24.

Department of Neurosurgery, Salford Royal NHS Foundation Trust, Greater Manchester, United Kingdom.

Background: Spinal cord compression secondary to nerve root hypertrophy is often attributed to hereditary neuropathies. However, to avoid misdiagnosis, rare immune-mediated neuropathy such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) should not be overlooked. This report presents a case of multilevel nerve root hypertrophy leading to significant cord compression from CIDP. Read More

Clin Geriatr Med 2021 May 23;37(2):327-345. Epub 2021 Mar 23.

Department of Neurology, Virginia Commonwealth University, 1101 East Marshall Street, PO Box 980599, Richmond, VA 23298, USA. Electronic address:

This article discusses the chronic immune-mediated polyneuropathies, a broad category of acquired polyneuropathies that encompasses chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), the most common immune-mediated neuropathy, the CIDP variants, and the vasculitic neuropathies. Polyneuropathies associated with rheumatological diseases and systemic inflammatory diseases, such as sarcoidosis, will also be briefly covered. These patients' history, examination, serum studies, and electrodiagnostic studies, as well as histopathological findings in the case of vasculitis, confirm the diagnosis and differentiate them from the more common length-dependent polyneuropathies. Read More

J Neurol Neurosurg Psychiatry 2021 Apr 13. Epub 2021 Apr 13.

Department of Neurosciences (DNS), University of Padova, Padova, Italy

Background: Cerebrospinal fluid (CSF) albumincytologic dissociation represents a supportive diagnostic criterion of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).Few studies have investigated possible systemic or intrathecal humoral immune response activation in CIDP.Aim of our study was to investigate whether the search of oligoclonal IgG bands (OCBs) might provide additional data helpful in CIDP diagnostic work-up. Read More

Qual Life Res 2021 Apr 8. Epub 2021 Apr 8.

Neurology Clinic, University Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, 6, Dr. Subotic Sr. Street, 11 000, Belgrade, Serbia.

Purpose: Even treated chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) continues to pose a significant burden in patients' everyday functioning and may continuously affect their quality of life (QoL). The aims of our prospective study were to analyze health-related QoL in CIDP patients during a 1-year follow-up period in real-life settings and to compare QoL changes with changes in disability and with patient impression of change.

Methods: The study comprised 59 patients diagnosed with CIDP. Read More

Front Neurol 2021 22;12:645263. Epub 2021 Mar 22.

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

Intravenous immunoglobulin (IVIG) is effective as standard first line therapy for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but some patients remain dependent on its long-term use. Recently, we have reported that autologous non-myeloablative hematopoietic stem cell transplantation (HSCT) is an effective second line therapy for CIDP. To compare the cost of chronic IVIG vs. Read More

Ophthalmic Plast Reconstr Surg 2021 Mar 31. Epub 2021 Mar 31.

Orbital Plastics and Lacrimal Unit, Royal Victorian Eye and Ear Hospital, East Melbourne, Victoria, Australia Centre for Eye Research, University of Melbourne, Melbourne, Australia Department of Surgery, Royal Melbourne Hospital, University of Melbourne, Parkville, Victoria, Australia.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, which commonly causes peripheral neuropathy. It has rarely been associated with cranial nerve hypertrophy and neuro-ophthalmic manifestations. Proptosis secondary to cranial nerve hypertrophy has been reported in association with CIDP. Read More

Front Neurol 2021 15;12:653734. Epub 2021 Mar 15.

Ellen & Martin Prosserman Centre for Neuromuscular Diseases, Toronto General Hospital, University Health Network, University of Toronto, Toronto, ON, Canada.

The variants of chronic inflammatory demyelinating polyneuropathy (CIDP) differ not just in their clinical, pathological and electrophysiological characteristics, but often in their indifferent response to conventional immunosuppressive agents which are effective in typical CIDP. High quality evidence is lacking as far as the management of these atypical variants is concerned. In this review, we summarize the treatment approaches to each of these CIDP variants based on existing data. Read More

Neurol Sci 2021 Jun 29;42(6):2223-2233. Epub 2021 Mar 29.

Department of Neurology, Renji Hospital Shanghai Jiaotong University School of Medicine, 160 Pujian Road, Shanghai, 200127, China.

There are bunch of autoantibodies, particularly autoantibodies against proteins located at the node of Ranvier, have been discovered and transformed the clinical management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Neurofascin (NF) plays an important role in both the nodal and paranodal regions of the node of Ranvier. In this review, we focus on the two characteristic forms of neurofascin: NF186 and NF155, comparing the similarities and differences between them, reviewing the current knowledge on genetic backgrounds, pathogenesis, clinical manifestations, and management of patients with anti-neurofascin positive CIDP. Read More

J Clin Neurosci 2021 Apr 20;86:276-283. Epub 2021 Feb 20.

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

We aimed to establish the cross-sectional area (CSA) reference values for peripheral nerves of lower extremities in a healthy Chinese population, and to determine their diagnostic values for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and Charcot-Marie-Tooth disease type1A (CMT1A). One hundred eleven healthy subjects, 15-70 years of age, as well as 104 CIDP patients and 26 CMT1A patients were recruited. CSA at predetermined sites of the tibial, fibular, sciatic and sural nerves was measured. Read More

Rev Med Interne 2021 Mar 24. Epub 2021 Mar 24.

Unité de médecine interne, maladies auto-immunes et pathologie vasculaire UF04, Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France MATHEC Hôpital Saint-Louis, UF04, Filière 'FAI2R', 1, avenue Claude-Vellefaux, 75475 Paris, France; Université de Paris, EA 3518, Paris, France; Département de Médecine, Université McGill, Montreal, QC, Canada.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a dysimmune neuropathy with sensory and/or motor symptoms due to destruction of the myelin sheat secondary to an auto-immune attack. A quarter to a third of patients do not respond to immunomodulatory first line recommended therapies. No second line treatment has shown its effectiveness with a sufficient level of evidence. Read More

Diabet Med 2021 Mar 27:e14563. Epub 2021 Mar 27.

Unit of Pediatric Diabetology, Department of Mother and Child Healths, Sapienza University of Rome, Rome, Italy.

BMJ Case Rep 2021 Mar 25;14(3). Epub 2021 Mar 25.

King Fahad Hospital Jeddah, Jeddah, Makkah, Saudi Arabia.

This is a case report of a 42-year-old female patient with chronic inactive hepatitis B virus (HBV) infection who presented with relapsing chronic inflammatory demyelinating polyneuropathy (CIDP). Her initial attack was of acute onset (ie, acute CIDP) resembling Guillain-Barré syndrome that responded well to intravenous immunoglobulin (IVIG) therapy. The second episode was chronic and refractory to IVIG. Read More

J Neuroinflammation 2021 Mar 22;18(1):78. Epub 2021 Mar 22.

Departments of Clinical Neurosciences and Cell Biology and Anatomy, Hotchkiss Brain Institute, University of Calgary, Calgary, AB, T2N 4N1, Canada.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consists of various autoimmune subtypes in which the peripheral nervous system (PNS) is attacked. CIDP can follow a relapsing-remitting or progressive course where the resultant demyelination caused by immune cells (e.g. Read More

Ther Adv Neurol Disord 2021 5;14:1756286421999631. Epub 2021 Mar 5.

Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany.

Background: Up to 20% of patients with chronic immune-mediated sensorimotor neuropathies (CIN) do not respond adequately to first-line therapies. However, studies on further treatment are scarce.

Methods: We analyzed retrospectively 200 CIN patients regarding disease characteristics and response to therapy with cyclophosphamide (CYP), rituximab (RTX), and bortezomib (BTZ). Read More

Intern Med 2021 Mar 22. Epub 2021 Mar 22.

Department of Neurology, Kansai Electric Power Hospital, Japan.

Multifocal enlargements with the alteration of a normal fascicular pattern are considered to be sonographic peripheral nerve features in multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), a subtype of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We herein present the case of an 18-year-old patient with MADSAM in whom intensive sonological assessments revealed multifocal nerve enlargement within clinically affected cranial nerves. Our case demonstrated that, if systematically investigated with ultrasound, morphological changes similar to those in the peripheral nerves may be detected in a large proportion of clinically affected cranial nerves in MADSAM, boosting the future applications of cranial nerve ultrasound in CIDP. Read More

Muscle Nerve 2021 Jun 8;63(6):881-884. Epub 2021 Apr 8.

Department of Neurology, Division of Neuromuscular Medicine, University of Michigan School of Medicine, Ann Arbor, Michigan, USA.

Introduction: It is unknown how often patients with electrodiagnostic evidence of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a potentially treatable condition, present with a distal symmetric polyneuropathy (DSP) phenotype.

Methods: We reviewed the records of patients who presented to our electrodiagnostic laboratory between January 1, 2011, to December 31, 2019, and fulfilled electrodiagnostic criteria for CIDP to identify those who presented with a sensory predominant DSP phenotype.

Results: One hundred sixty-two patients had a chronic acquired demyelinating neuropathy, of whom 138 met criteria for typical or atypical CIDP. Read More

J Int Med Res 2021 Mar;49(3):300060521998896

Department of Physical Medicine and Rehabilitation, College of Medicine, Yeungnam University, Daegu, Republic of Korea.

Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), a subtype of chronic inflammatory demyelinating polyneuropathy, is a non-compressive peripheral nerve disorder. Symptoms of MADSAM include asymmetrical weakness and sensory deficits in the distribution of individual peripheral nerves, which are frequently noted in the distal portion of peripheral nerves. MADSAM can be easily misdiagnosed as any of the various compressive peripheral neuropathies. Read More