918 results match your criteria Journal of the Peripheral Nervous System [Journal]


Serum neurofilament light chain in chronic inflammatory demyelinating polyneuropathy.

J Peripher Nerv Syst 2019 Apr 11. Epub 2019 Apr 11.

Amsterdam UMC, University of Amsterdam, Department of Neurology, Amsterdam Neuroscience, AZ, Amsterdam, The Netherlands.

Objective: Axonal damage in chronic inflammatory demyelinating polyneuropathy (CIDP) is the main predictor of poor outcome. We hypothesized that serum neurofilament light chain (sNfL) reflects disease activity by detecting ongoing neuro-axonal damage in CIDP.

Methods: Three prospective cohorts of CIDP patients were studied: 1) patients starting induction treatment (IT cohort, N:29) measured at baseline and six months after starting treatment; 2) patients on maintenance treatment starting IVIg withdrawal (MT cohort, N:24) measured at baseline and six months after IVIg withdrawal or at time of relapse and 3) patients in long-term remission without treatment (N:27). Read More

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http://dx.doi.org/10.1111/jns.12319DOI Listing

One-year follow-up study of neuropathic pain in chronic inflammatory demyelinating polyradiculoneuropathy.

J Peripher Nerv Syst 2019 Apr 11. Epub 2019 Apr 11.

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

Background: We sought to gather information about frequency and features of neuropathic pain (NeP) in CIDP patients and to investigate course of NeP during one-year follow-up.

Methods: Study included 105 patients diagnosed with CIDP. Patients with diabetes (N=26) were excluded. Read More

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http://dx.doi.org/10.1111/jns.12318DOI Listing

Beriberi neuropathy in oncological setting.

J Peripher Nerv Syst 2019 Mar;24(1):162-163

Neurosciences, University of Padova, Padova, Italy.

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http://dx.doi.org/10.1111/jns.12304DOI Listing

Novel GARS mutation presenting as autosomal dominant intermediate Charcot-Marie-Tooth disease: Intermediate or axonal?

J Peripher Nerv Syst 2019 Mar;24(1):161

Service of Clinical Neurophysiology, University Hospital "Marqués de Valdecilla (IDIVAL)", "Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)", Santander, Spain.

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http://dx.doi.org/10.1111/jns.12300DOI Listing

Hereditary sensory and autonomic neuropathy type IC accompanied by upper motor neuron abnormalities and type II juxtafoveal retinal telangiectasias.

J Peripher Nerv Syst 2019 Mar 13. Epub 2019 Mar 13.

Department of Neurology, Concord Repatriation General Hospital, Sydney, New South Wales, Australia.

Hereditary sensory and autonomic neuropathy type I (HSAN-1) is an autosomal dominant sensory neuropathy occurring secondary to mutations in the SPTLC1 and SPTLC2 genes. We present two generations of a single family with Ser384Phe mutation in the SPTLC2 gene located on chromosome 14q24 characterized by a typical HSAN-1c presentation, with additional findings upper motor neuron signs, early demyelinating features on nerve conduction studies, and type II juxtafoveal retinal telangiectasias also known as macular telangiectasias (MacTel II). Although HSAN1 is characterized as an axonal neuropathy, demyelinating features were identified in two subjects on serial nerve conduction studies comprising motor conduction block, temporal dispersion, and prolongation of F-waves. Read More

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http://dx.doi.org/10.1111/jns.12315DOI Listing

A novel family with axonal Charcot-Marie-Tooth disease caused by a mutation in the EGR2 gene.

J Peripher Nerv Syst 2019 Mar 6. Epub 2019 Mar 6.

Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples "Federico II", Naples, Italy.

EGR2 (Early Growth Response 2) is one of the most important transcription factors involved in myelination in the peripheral nervous system. EGR2 mutations typically cause different forms of demyelinating neuropathy, that is, Charcot-Marie-Tooth type 1D (CMT1D), Dejerine-Sottas Syndrome (DSS), and Congenital Hypomyelinating Neuropathy (CHN). However, the EGR2 gene has been recently associated with an axonal phenotype (CMT2) in a large CMT family. Read More

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http://dx.doi.org/10.1111/jns.12314DOI Listing
March 2019
2 Reads

POLG mutations presenting as Charcot-Marie-Tooth disease.

J Peripher Nerv Syst 2019 Mar 6. Epub 2019 Mar 6.

Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

We report on two patients, with different POLG mutations, in whom axonal neuropathy dominated the clinical picture. One patient presented with late onset sensory axonal neuropathy caused by a homozygous c.2243G>C (p. Read More

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http://dx.doi.org/10.1111/jns.12313DOI Listing

A population-based and cross-sectional study of the long-term prognosis in multifocal motor neuropathy.

J Peripher Nerv Syst 2019 Mar 3;24(1):64-71. Epub 2019 Mar 3.

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

A population-based, cross-sectional study of patients referred to the Danish hospital system between 1985 and 2006 was conducted to evaluate the long-term outcome in Danish patients treated for multifocal motor neuropathy (MMN). Thirty-four MMN patients were identified, three had died of unrelated diseases, 10 were excluded, one did not reply to study request and 20 were included. The median disease duration was 24 years (interquartile range: 18. Read More

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http://dx.doi.org/10.1111/jns.12311DOI Listing
March 2019
2 Reads

A novel pathogenic variant of NEFL responsible for deafness associated with peripheral neuropathy discovered through next-generation sequencing and review of the literature.

J Peripher Nerv Syst 2019 Mar 26;24(1):139-144. Epub 2019 Feb 26.

Univ. Limoges, MMNP, Limoges, France.

Neurofilaments are neuron-specific intermediate filaments essential for the radial growth of axons during development and the maintenance of axonal diameter. Pathogenic variants of Neurofilament Light (NEFL) are associated with CMT1F, CMT2E, and CMTDIG and have been observed in less than 1% of Charcot-Marie-Tooth (CMT) cases, resulting in the reporting of 35 variants in 173 CMT patients to date. However, only six variants have been reported in 17 patients with impaired hearing. Read More

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http://dx.doi.org/10.1111/jns.12310DOI Listing
March 2019
3 Reads

A cryptic splicing mutation in the INF2 gene causing Charcot-Marie-Tooth disease with minimal glomerular dysfunction.

J Peripher Nerv Syst 2019 Mar 8;24(1):120-124. Epub 2019 Feb 8.

Centre de Biologie et de Pathologie Est, Hospices Civils de Lyon, Bron, France.

Heterozygous mutations in the inverted formin-2 (INF2) gene provoke focal segmental glomerulosclerosis (FSGS) and intermediate Charcot-Marie-Tooth (CMT) disease with FSGS. Here, we report four patients from a three-generation family with a new cryptic splicing INF2 mutation causing autosomal dominant intermediate CMT with minimal glomerular dysfunction. Three males and one female with a mean age of 51 years (26-87) presented with a slowly progressive sensorimotor polyneuropathy, pes cavus, and kyphoscoliosis. Read More

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http://doi.wiley.com/10.1111/jns.12308
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http://dx.doi.org/10.1111/jns.12308DOI Listing
March 2019
14 Reads

ATL3 gene mutation in a Chinese family with hereditary sensory neuropathy type 1F.

J Peripher Nerv Syst 2019 Mar 21;24(1):150-155. Epub 2019 Feb 21.

Department of Neurology, The Fourth Affiliated Hospital, Zhejiang University School of Medicine, Yiwu, China.

Hereditary sensory neuropathy (HSN) comprises a group of progressive peripheral neuropathies predominantly affecting the sensory nerves. To date, two different ATL3 gene mutations have been reported to be responsible for HSN type 1F (HSN1F). Here, we report a family in which the members presented numbness of the lower limbs and recurrent foot ulceration. Read More

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http://doi.wiley.com/10.1111/jns.12309
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http://dx.doi.org/10.1111/jns.12309DOI Listing
March 2019
8 Reads
2.758 Impact Factor

Anti-allodynic and anti-inflammatory effects of 17α-hydroxyprogesterone caproate in oxaliplatin-induced peripheral neuropathy.

J Peripher Nerv Syst 2019 Mar 25;24(1):100-110. Epub 2019 Feb 25.

Laboratorio de Nocicepción y Dolor Neuropático, Instituto de Biología y Medicina Experimental - CONICET, Buenos Aires, Argentina.

Chemotherapy-induced peripheral neuropathy is a disabling condition induced by several frequently used chemotherapeutic drugs including the front-line agent oxaliplatin (OXA). Symptoms are predominantly sensory with the development of neuropathic pain. Alternative dosing protocols and treatment discontinuation are the only available therapeutic strategies. Read More

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http://dx.doi.org/10.1111/jns.12307DOI Listing

Patients' and physicians' interpretation of chemotherapy-induced peripheral neurotoxicity.

J Peripher Nerv Syst 2019 Mar 8;24(1):111-119. Epub 2019 Feb 8.

Center of Biostatistics for Clinical Epidemiology, School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.

To test if and how chemotherapy-induced peripheral neurotoxicity (CIPN) is perceived differently by patients and physicians, making assessment and interpretation challenging. We performed a secondary analysis of the CI-PeriNomS study which included 281 patients with stable CIPN. We tested: (a) the association between patients' perception of activity limitation in performing eight common tasks and neurological impairment and (b) how the responses to questions related to these daily activities are interpreted by the treating oncologist. Read More

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http://dx.doi.org/10.1111/jns.12306DOI Listing
March 2019
4 Reads

Efficacy and safety of IVIG in CIDP: Combined data of the PRIMA and PATH studies.

J Peripher Nerv Syst 2019 Mar 15;24(1):48-55. Epub 2019 Feb 15.

CSL Behring, Marburg, Germany, and King of Prussia, Pennsylvania.

Intravenous immunoglobulin (IVIG) is a potential therapy for chronic inflammatory demyelinating polyneuropathy (CIDP). To investigate the efficacy and safety of the IVIG IgPro10 (Privigen) for treatment of CIDP, results from Privigen Impact on Mobility and Autonomy (PRIMA), a prospective, open-label, single-arm study of IVIG in immunoglobulin (Ig)-naïve or IVIG pre-treated subjects (NCT01184846, n = 28) and Polyneuropathy And Treatment with Hizentra (PATH), a double-blind, randomized study including an open-label, single-arm IVIG phase in IVIG pre-treated subjects (NCT01545076, IVIG restabilization phase n = 207) were analyzed separately and together (n = 235). Efficacy assessments included change in adjusted inflammatory neuropathy cause and treatment (INCAT) score, grip strength and Medical Research Council (MRC) sum score. Read More

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http://dx.doi.org/10.1111/jns.12302DOI Listing
March 2019
14 Reads

Restabilization treatment after intravenous immunoglobulin withdrawal in chronic inflammatory demyelinating polyneuropathy: Results from the pre-randomization phase of the Polyneuropathy And Treatment with Hizentra study.

J Peripher Nerv Syst 2019 Mar 1;24(1):72-79. Epub 2019 Mar 1.

Department of Neurology, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, the Netherlands.

In patients with chronic inflammatory demyelinating polyneuropathy (CIDP), intravenous immunoglobulin (IVIG) is recommended to be periodically reduced to assess the need for ongoing therapy. However, little is known about the effectiveness of restabilization with IVIG in patients who worsen after IVIG withdrawal. In the Polyneuropathy And Treatment with Hizentra (PATH) study, the pre-randomization period included sudden stopping of IVIG followed by 12 weeks of observation. Read More

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http://dx.doi.org/10.1111/jns.12303DOI Listing
March 2019
13 Reads

Mutational screening of the SH3TC2 gene in Greek patients with suspected demyelinating recessive Charcot-Marie-Tooth disease reveals a varied and unusual phenotypic spectrum.

J Peripher Nerv Syst 2019 Mar 6;24(1):125-130. Epub 2019 Feb 6.

Neurogenetics Unit, 1st Department of Neurology, Eginition Hospital, Medical School National and Kapodistrian University of Athens, Athens, Greece.

Charcot-Marie-Tooth disease type 4 C (CMT4C) is an autosomal recessive form of demyelinating peripheral neuropathy caused by mutations in SH3TC2, characterized by early onset, spine deformities, and cranial nerve involvement. We screened SH3TC2 in 50 unrelated Greek patients with suspected demyelinating Charcot-Marie-Tooth disease and pedigree compatible with recessive inheritance. All patients had been previously screened for PMP22, GJB1, and MPZ mutations. Read More

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http://dx.doi.org/10.1111/jns.12305DOI Listing
March 2019
3 Reads

Peripheral Neuropathy Research Registry: A prospective cohort.

J Peripher Nerv Syst 2019 Mar 29;24(1):39-47. Epub 2019 Jan 29.

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD.

The Peripheral Neuropathy Research Registry (PNRR) is a prospective cohort of peripheral neuropathy (PN) patients focused on idiopathic axonal peripheral neuropathy. Patients with diabetic, human immunodeficiency virus-, and chemotherapy-induced peripheral neuropathies are enrolled as comparison groups. The PNRR is a multi-center collaboration initiated and funded by the Foundation for Peripheral Neuropathy (FPN) with the objective to recruit a well characterized cohort of patients with different phenotypes and symptoms in each diagnostic category, and to advance research through development of biomarkers and identification of previously unknown causes of PN. Read More

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http://doi.wiley.com/10.1111/jns.12301
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http://dx.doi.org/10.1111/jns.12301DOI Listing
March 2019
13 Reads

International chronic inflammatory demyelinating polyneuropathy outcome study (ICOS): Protocol of a prospective observational cohort study on clinical and biological predictors of disease course and outcome.

J Peripher Nerv Syst 2019 Mar 22;24(1):34-38. Epub 2019 Jan 22.

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

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous immune-mediated disorder with extensive variation in clinical presentation, electrophysiological phenotype, treatment response and long-term outcome. This heterogeneity may reflect the existence of distinct subtypes of CIDP with a different pathogenesis that require personalized treatment. The International CIDP Outcome Study (ICOS) is a prospective, observational, multicenter cohort study that aims to describe this variation and to define clinical and biological determinants and predictors of these subtypes, disease activity, treatment response and outcome. Read More

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http://doi.wiley.com/10.1111/jns.12296
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http://dx.doi.org/10.1111/jns.12296DOI Listing
March 2019
9 Reads

Genetic epidemiology, demographic, and clinical characteristics of Charcot-Marie-tooth disease in the island of Gran Canaria (Spain).

J Peripher Nerv Syst 2019 Mar 16;24(1):131-138. Epub 2019 Jan 16.

Research Unit, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria.

Charcot-Marie-Tooth (CMT) disease is the most common hereditary neuromuscular disorder. This study involves the entire known CMT patient registry in Gran Canaria, represented by 256 patients belonging to 79 unrelated families, who were clinically and genetically characterized, along with physical and neurophysiological evaluation on 181 and 165 patients, respectively. Complete genotyping showed an estimated prevalence of CMT disease of 30. Read More

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http://doi.wiley.com/10.1111/jns.12299
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http://dx.doi.org/10.1111/jns.12299DOI Listing
March 2019
6 Reads

Acute flaccid paralysis: Do not forget beriberi neuropathy.

J Peripher Nerv Syst 2019 Mar 16;24(1):145-149. Epub 2019 Jan 16.

Department of Neurology, National Neuroscience Institute, Singapore, Singapore.

We aimed to elucidate characteristics of beriberi neuropathy (BB) in a general hospital (GH) setting. Nerve conduction studies (NCS), cross-referenced with clinical records of patients admitted to a GH (May 2011-July 2017), were reviewed for diagnosis of BB. Thirteen patients (age range 23-64 years; five women) were diagnosed with BB. Read More

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http://dx.doi.org/10.1111/jns.12297DOI Listing
March 2019
6 Reads

Small-fiber neuropathy: Expanding the clinical pain universe.

J Peripher Nerv Syst 2019 Mar 8;24(1):19-33. Epub 2019 Jan 8.

Department of Neurology, School of Mental Health and Neuroscience, Maastricht University Medical Centre+, Maastricht, The Netherlands.

Small-fiber neuropathy (SFN) is a disorder of thinly myelinated Aδ and unmyelinated C fibers. SFN is clinically dominated by neuropathic pain and autonomic complaints, leading to a significant reduction in quality of life. According to international criteria, the diagnosis is established by the assessment of intraepidermal nerve fiber density and/or quantitative sensory testing. Read More

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http://doi.wiley.com/10.1111/jns.12298
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http://dx.doi.org/10.1111/jns.12298DOI Listing
March 2019
16 Reads

Peripheral nervous system involvement in lymphomas.

J Peripher Nerv Syst 2019 Mar 8;24(1):5-18. Epub 2019 Jan 8.

Hematology and Clinical Immunology Unit, Department of Medicine, University of Padova, Padova, Italy.

The peripheral nervous system may be involved at any stage in the course of lymphoproliferative diseases. The different underlying mechanisms include neurotoxicity secondary to chemotherapy, direct nerve infiltration (neurolymphomatosis), infections, immune-mediated, paraneoplastic or metabolic processes and nutritional deficiencies. Accordingly, the clinical features are heterogeneous and depend on the localization of the damage (ganglia, roots, plexi, and peripheral nerves) and on the involved structures (myelin, axon, and cell body). Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/jns.12295
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http://dx.doi.org/10.1111/jns.12295DOI Listing
March 2019
12 Reads
2.758 Impact Factor

Peripheral axonopathy in sciatic nerve of adult Wistar rats following exposure to vanadium.

J Peripher Nerv Syst 2019 Mar 16;24(1):94-99. Epub 2018 Dec 16.

Neuroscience Unit, Department of Veterinary Anatomy, Faculty of Veterinary Medicine, University of Ibadan, Ibadan, Nigeria.

Depletion of myelin and neurobehavioural deficits are indications that vanadium crosses the blood-brain barrier and such neurotoxic effects of vanadium on the brain of Wistar rats have been elucidated. The effect however on the peripheral nerves, is yet to be reported. Thus, this work was designed to evaluate the axonal and myelin integrity of sciatic nerves in Wistar rats following exposure to vanadium. Read More

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http://dx.doi.org/10.1111/jns.12294DOI Listing
March 2019
16 Reads

Morphometric analysis of peripheral myelinated nerve fibers through deep learning.

J Peripher Nerv Syst 2019 Mar 11;24(1):87-93. Epub 2018 Dec 11.

Department of Neurology, Wayne State University School of Medicine, Detroit, Michigan.

Irrespective of initial causes of neurological diseases, these disorders usually exhibit two key pathological changes-axonal loss or demyelination or a mixture of the two. Therefore, vigorous quantification of myelin and axons is essential in studying these diseases. However, the process of quantification has been labor intensive and time-consuming because of the requisite manual segmentation of myelin and axons from microscopic nerve images. Read More

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http://doi.wiley.com/10.1111/jns.12293
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http://dx.doi.org/10.1111/jns.12293DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6420354PMC
March 2019
18 Reads
2.758 Impact Factor

IqYmune® is an effective maintenance treatment for multifocal motor neuropathy: A randomised, double-blind, multi-center cross-over non-inferiority study vs Kiovig®-The LIME Study.

J Peripher Nerv Syst 2019 Mar 11;24(1):56-63. Epub 2018 Dec 11.

Maastricht University Medical Center, Maastricht, The Netherlands.

Intravenous immunoglobulin (IVIg) is the gold-standard for maintenance treatment of multifocal motor neuropathy (MMN). This phase III, randomised, double-blind, multi-centre, active-control, crossover study, aimed to evaluate the non-inferiority of IqYmune® relative to Kiovig®, primarily based on efficacy criteria. Twenty-two adult MMN patients, treated with any brand of IVIg (except Kiovig® or IqYmune®) at a stable maintenance dose within the range of 1 to 2 g/kg every 4 to 8 weeks, were randomised to receive either Kiovig® followed by IqYmune®, or IqYmune® followed by Kiovig®. Read More

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http://dx.doi.org/10.1111/jns.12291DOI Listing
March 2019
10 Reads

Increased incidence of axonal Guillain-Barré syndrome in La Spezia area of Italy: A 13-year follow-up study.

J Peripher Nerv Syst 2019 Mar 11;24(1):80-86. Epub 2018 Dec 11.

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

Guillain-Barré syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy with a worldwide incidence of 0.81-1.89 per 100 000 person-years. Read More

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http://dx.doi.org/10.1111/jns.12292DOI Listing
March 2019
3 Reads

Novel GARS mutation presenting as autosomal dominant intermediate Charcot-Marie-Tooth disease.

J Peripher Nerv Syst 2019 Mar 23;24(1):156-160. Epub 2018 Nov 23.

Department of Neurology, Graduate School of Medical Sciences, University of Yamanashi, Yamanashi, Japan.

We report the first family with a glycyl-tRNA synthetase (GARS) mutation with autosomal dominant intermediate Charcot-Marie-Tooth disease (DI-CMT). The proband and the proband's father presented with gait disturbance and hand weakness. Both patients displayed moderately decreased conduction velocities (MNCV) (ranging from 29. Read More

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http://doi.wiley.com/10.1111/jns.12289
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http://dx.doi.org/10.1111/jns.12289DOI Listing
March 2019
20 Reads

Nociceptin/orphanin FQ opioid peptide-receptor expression in pachyonychia congenita.

J Peripher Nerv Syst 2018 Dec 16;23(4):241-248. Epub 2018 Oct 16.

Neurology, The Johns Hopkins School of Medicine, Baltimore, Maryland.

Nociceptin/orphanin FQ opioid peptide (NOP)-receptor (NOP-R) is a member of the opioid receptor family. NOP-R activation has demonstrated analgesic effects in preclinical pain models without the addiction risks associated with other opiate targets. Pachyonychia congenita (PC) is a palmoplantar keratoderma characterized by neuropathic pain in affected skin. Read More

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https://www.researchgate.net/topic/Opioid-Peptides
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http://doi.wiley.com/10.1111/jns.12288
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http://dx.doi.org/10.1111/jns.12288DOI Listing
December 2018
18 Reads

Rituximab in chronic inflammatory demyelinating polyradiculoneuropathy with associated diseases.

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

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http://doi.wiley.com/10.1111/jns.12287
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http://dx.doi.org/10.1111/jns.12287DOI Listing
December 2018
6 Reads

Second IVIg course in Guillain-Barré syndrome patients with poor prognosis (SID-GBS trial): Protocol for a double-blind randomized, placebo-controlled clinical trial.

J Peripher Nerv Syst 2018 Dec 24;23(4):210-215. Epub 2018 Sep 24.

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

One course of intravenous immunoglobulins (IVIg) of 2 g/kg is standard treatment in Guillain-Barré syndrome (GBS) patients unable to walk independently. Despite treatment some patients recover poorly, in part related to rapid consumption of IVIg, indicating that they may benefit from a second course of IVIg. The aim of the study is to determine whether a second course of IVIg, administered 1 week after start of the first course in patients with GBS and predicted poor outcome improves functional outcome on the GBS disability scale after 4 weeks. Read More

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http://dx.doi.org/10.1111/jns.12286DOI Listing
December 2018
6 Reads

Clinical relevance of serum antibodies to GD1b in immune-mediated neuropathies.

J Peripher Nerv Syst 2018 Dec 12;23(4):227-234. Epub 2018 Sep 12.

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

Antibodies to the ganglioside GD1b have been reported in various forms of immune-mediated neuropathy, but their clinical relevance for diagnosis and prognosis is unknown. We investigated the prevalence of anti-GD1b antibodies in acute and chronic immune-mediated neuropathies, and the clinical presentation and outcome in Guillain-Barré syndrome (GBS) and Miller Fisher-GBS overlap syndrome (MF-GBS). Anti-GD1b, anti-GM1 and anti-GQ1b antibodies were tested in serum of patients with GBS (N = 165), Miller Fisher syndrome (N = 10), MF-GBS (N = 28), monoclonal gammopathy of unknown significance neuropathy (MGUS; N = 101), chronic inflammatory demyelinating polyneuropathy (N = 29), paraneoplastic syndrome with anti-Hu-associated neuropathy (PNS; N = 11), other auto-immune diseases (AID; N = 60), and healthy controls (HC; N = 60). Read More

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http://dx.doi.org/10.1111/jns.12285DOI Listing
December 2018
11 Reads

The 2016 Singapore Zika virus outbreak did not cause a surge in Guillain-Barré syndrome.

J Peripher Nerv Syst 2018 Sep 21;23(3):197-201. Epub 2018 Aug 21.

Singapore Immunology Network, Agency for Science, Technology and Research (A*STAR), Singapore.

Although individuals with Zika virus (ZIKV) antibodies were reported in Malaya in mid-1950s, entomological and human surveillance in Singapore did not identify autochthonous transmission until the outbreak of August-November, 2016. A total of 455 cases from 15 separate clusters were identified. We asked if this ZIKV outbreak increased the incidence of Guillain-Barré syndrome (GBS) and aimed to characterize these cases. Read More

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http://doi.wiley.com/10.1111/jns.12284
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http://dx.doi.org/10.1111/jns.12284DOI Listing
September 2018
30 Reads

Mandating nerve biopsy: A step towards personalizing therapy in pure neuritic leprosy.

J Peripher Nerv Syst 2018 Sep 27;23(3):190-196. Epub 2018 Aug 27.

Department of Neurology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India.

Pure neuritic leprosy (PNL) accounts for 5% to 10% of leprosy patients who usually present with asymmetrical neuropathy in the absence of lepra bacilli on slit-skin smears. However, nerve biopsies in PNL lack appropriate categorization in current immunologic terms. We aimed to classify nerve biopsies according to the immune spectrum of leprosy and assess the role of histologic classification of nerve biopsies in treating PNL. Read More

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http://dx.doi.org/10.1111/jns.12283DOI Listing
September 2018
4 Reads

Conventional and unconventional therapies in typical and atypical chronic inflammatory demyelinating polyneuropathy with different clinical course of progression.

J Peripher Nerv Syst 2018 Sep 14;23(3):183-189. Epub 2018 Aug 14.

Department of Neurology, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia.

Intravenous immunoglobulin (IVIG), corticosteroids and therapeutic plasma exchange (TPE) are evidence-based conventional treatments for chronic inflammatory demyelinating polyneuropathy (CIDP). In many centres, unconventional treatments are frequently used as alternatives. We evaluated the outcome of conventional and unconventional therapies in 31 CIDP patients. Read More

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http://dx.doi.org/10.1111/jns.12282DOI Listing
September 2018
9 Reads

Employment status of patients with chronic inflammatory demyelinating polyradiculoneuropathy.

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

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http://dx.doi.org/10.1111/jns.12281DOI Listing
September 2018
11 Reads

A novel SCN9A splicing mutation in a compound heterozygous girl with congenital insensitivity to pain, hyposmia and hypogeusia.

J Peripher Nerv Syst 2018 Sep 23;23(3):202-206. Epub 2018 Jul 23.

Neuroalgology Unit, IRCCS Foundation "Carlo Besta" Neurological Institute, Milan, Italy.

Congenital insensitivity to pain (CIP) is a rare autosomal recessive disorder presenting with a spectrum of clinical features caused by mutations in different genes. A 10-year-old girl with CIP, hyposmia and hypogeusia, and her unaffected twin and parents underwent next generation sequencing of SCN9A exons and flanking splice sites. Transcript analysis from whole blood successfully assayed the effect of the mutation on the mRNA splicing by polymerase chain reaction amplification on cDNA and Sanger sequencing. Read More

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http://dx.doi.org/10.1111/jns.12280DOI Listing
September 2018
6 Reads

Serum and cerebrospinal neurofilament light chain levels in patients with acquired peripheral neuropathies.

J Peripher Nerv Syst 2018 Sep 24;23(3):174-177. Epub 2018 Jul 24.

Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy.

Neurofilament light chain (NFL) levels reflect axonal damage in different inflammatory and neurodegenerative central nervous system conditions, in correlation with disease severity. Our aim was to determine the possible diagnostic and prognostic value of serum and cerebrospinal fluid (CSF) NFL levels in subjects with different forms of acquired peripheral neuropathies (PN). Paired serum and CSF samples of 25 patients with acquired PN were analysed for NFL using an ultrasensitive technique (Quanterix, Simoa, Lexington, MA, USA) and compared with a group of 25 age-matched healthy subjects. Read More

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http://dx.doi.org/10.1111/jns.12279DOI Listing
September 2018
11 Reads

A unified model of the excitability of mouse sensory and motor axons.

J Peripher Nerv Syst 2018 Sep 15;23(3):159-173. Epub 2018 Jul 15.

Brain and Mind Centre, The University of Sydney, Sydney, Australia.

Non-invasive nerve excitability techniques have provided valuable insight into the understanding of neurological disorders. The widespread use of mice in translational research on peripheral nerve disorders and by pharmaceutical companies during drug development requires valid and reliable models that can be compared to humans. This study established a novel experimental protocol that enables comparative assessment of the excitability properties of motor and sensory axons at the same site in mouse caudal nerve, compared the mouse data to data for motor and sensory axons in human median nerve at the wrist, and constructed a mathematical model of the excitability of mouse axons. Read More

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http://dx.doi.org/10.1111/jns.12278DOI Listing
September 2018

Clinical and genetic diversities of Charcot-Marie-Tooth disease with MFN2 mutations in a large case study.

Authors:

J Peripher Nerv Syst 2018 Jun 11;23(2):149-150. Epub 2018 Apr 11.

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http://dx.doi.org/10.1111/jns.12263DOI Listing
June 2018
1 Read

Clinical and genetic investigation in Chinese patients with demyelinating Charcot-Marie-Tooth disease.

J Peripher Nerv Syst 2018 Dec 24;23(4):216-226. Epub 2018 Sep 24.

Department of Neurology and Institute of Neurology, First Affiliated Hospital, Fujian Medical University, Fuzhou, China.

Demyelinating Charcot-Marie-Tooth disease (CMT) is the most common subtype of CMT. It is caused mainly by 17p11.2 heterozygous duplication, but also by mutations in more than 20 genes which affect development and function of Schwann cells. Read More

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http://dx.doi.org/10.1111/jns.12277DOI Listing
December 2018
5 Reads

Robert R. Myers: In Memoriam (April 15, 1946-April 19, 2018).

J Peripher Nerv Syst 2018 Jun 25;23(2):76-77. Epub 2018 May 25.

Neuroscience and Mental Health Institute, University of Alberta, Alberta, Canada.

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http://dx.doi.org/10.1111/jns.12272DOI Listing

Risk factors for the development of paclitaxel-induced neuropathy in breast cancer patients.

J Peripher Nerv Syst 2018 Jun 11;23(2):129-133. Epub 2018 May 11.

Department of Neurology, University of Minnesota, Minneapolis, Minnesota.

Peripheral neuropathy is a common side effect of many chemotherapeutic agents including paclitaxel. We prospectively evaluated demographic and laboratory data in a cohort of 61 woman with breast cancer prior to paclitaxel exposure to explore factors that predispose to neuropathy development. Neuropathy was graded based on the total neuropathy score reduced version (rTNS) at baseline and at 4 months after initiation of chemotherapy. Read More

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http://dx.doi.org/10.1111/jns.12271DOI Listing
June 2018
4 Reads

Testing overwork weakness in Charcot-Marie-tooth disease: Is it true or false?

J Peripher Nerv Syst 2018 Jun 7;23(2):124-128. Epub 2018 May 7.

Department of Neurology, Rehabilitation, Ophthalmology, Genetics and Child/Maternal Sciences (DINOGMI), University of Genoa, Genoa, Italy.

The occurrence of the overwork weakness (OW) in Charcot-Marie-tooth (CMT) disease has been debated for a long time. Especially at the hands level, it is still unclear as to whether OW occurs. Contrasting results may relate to the different muscle groups evaluated and the instruments used. Read More

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http://dx.doi.org/10.1111/jns.12270DOI Listing
June 2018
8 Reads

Clinical spectrum and quality of life in patients with chronic polyneuropathy: A cross-sectional study.

J Peripher Nerv Syst 2018 Jun 7;23(2):120-123. Epub 2018 May 7.

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.

Chronic polyneuropathy is a disabling condition of the peripheral nerves, characterized by symmetrical sensory motor symptoms and signs. There is paucity of studies on the etiological spectrum of polyneuropathy and its impact on quality of life (QoL). The present cross-sectional study in a referral based tertiary care center in North India found diabetic neuropathy as the commonest cause (25. Read More

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http://dx.doi.org/10.1111/jns.12269DOI Listing
June 2018
4 Reads

Intravenous immunoglobulin for maintenance treatment of multifocal motor neuropathy: A multi-center, open-label, 52-week phase 3 trial.

J Peripher Nerv Syst 2018 Jun 24;23(2):115-119. Epub 2018 Apr 24.

Department of Neurology, Tokushima University School of Medicine, Tokushima, Japan.

Intravenous immunoglobulin (IVIg) therapy is currently the only established treatment in patients with multifocal motor neuropathy (MMN), and many patients have an IVIg-dependent fluctuation. We aimed to investigate the efficacy and safety of every 3 week IVIg (1.0 g/kg) for 52 weeks. Read More

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http://dx.doi.org/10.1111/jns.12268DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6033107PMC
June 2018
2 Reads

A randomised, multi-centre phase III study of 3 different doses of intravenous immunoglobulin 10% in patients with chronic inflammatory demyelinating polyradiculoneuropathy (ProCID trial): Study design and protocol.

J Peripher Nerv Syst 2018 Jun 26;23(2):108-114. Epub 2018 Apr 26.

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

Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) show varying degrees of response to intravenous immunoglobulin (IVIg) therapy. This randomised phase III study in patients with CIDP (ProCID trial) will compare the efficacy and safety of 3 different doses (0.5, 1. Read More

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http://doi.wiley.com/10.1111/jns.12267
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http://dx.doi.org/10.1111/jns.12267DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6033152PMC
June 2018
10 Reads

Differences between acute-onset chronic inflammatory demyelinating polyneuropathy and acute inflammatory demyelinating polyneuropathy in adult patients.

J Peripher Nerv Syst 2018 Sep 25;23(3):154-158. Epub 2018 Jun 25.

Center for Research on Neuroimmunological Diseases (CIEN), Raúl Carrea Institute for Neurological Research (FLENI), Buenos Aires, Argentina.

Acute inflammatory demyelinating polyneuropathy (AIDP) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A-CIDP diagnosed at our institution between January 2006 and July 2017 were retrospectively reviewed. Read More

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http://dx.doi.org/10.1111/jns.12266DOI Listing
September 2018
5 Reads

Whole-exome sequencing reveals a novel missense mutation in the MARS gene related to a rare Charcot-Marie-Tooth neuropathy type 2U.

J Peripher Nerv Syst 2018 Jun 9;23(2):138-142. Epub 2018 May 9.

The Human Genetics institute, Carmel Medical Center, Haifa, Israel.

Charcot-Marie-Tooth (CMT) is a heterogeneous group of progressive disorders, characterized by chronic motor and sensory polyneuropathy. This hereditary disorder is related to numerous genes and varying inheritance patterns. Thus, many patients do not reach a final genetic diagnosis. Read More

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http://dx.doi.org/10.1111/jns.12264DOI Listing
June 2018
6 Reads