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Journal of the Peripheral Nervous System: JPNS

Noor E Taams, Nicolette C Notermans, Willem-Jan R Fokkink, Anne P Tio-Gillen, Ruth Huizinga, Marco W J Schreurs, Bart C Jacobs
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), MFS (N=10), MF-GBS (N=28), monoclonal gammopathy of unknown significance neuropathy (MGUS,N=101), chronic inflammatory demyelinating polyneuropathy (CIDP,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)...
August 12, 2018: Journal of the Peripheral Nervous System: JPNS
Thirugnanam Umapathi, Yiu-Wing Kam, O Ohnmar, Brandon C J Ng, Yixiang Ng, M Premikha, Yee-Sin Leo, Lisa F P Ng
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. Four hundred and fifty five 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. Eleven GBS cases, consecutively enrolled into our prospective GBS database from onset to 4 weeks after outbreak, and 6 controls, comprising 3 GBS patients enrolled before outbreak and 3 non-GBS patients, were examined for evidence of recent ZIKV infection...
August 2, 2018: Journal of the Peripheral Nervous System: JPNS
Dinkar Kulshreshtha, Kiran Preet Malhotra, Hardeep Singh xMalhotra, Anup Kumar Thacker, Ravindra Kumar Garg, Ajai Kumar Singh, Pradeep Kumar Maurya
BACKGROUND: Pure neuritic leprosy (PNL) accounts for 5-10% 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. METHODS AND MATERIAL: Patients from two tertiary care referral centres were enrolled in this incident case study...
August 1, 2018: Journal of the Peripheral Nervous System: JPNS
Stefanie Kar Yan Hung, Fu Liong Hiew, Shanthi Viswanathan, Santhi Puvanarajah
OBJECTIVES: 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. PATIENTS AND METHODS: We evaluated the outcome of conventional and unconventional therapies in 31 CIDP patients. RESULTS: Overall response rate with conventional first-line immunotherapies was 77% (20/26), comparable between IVIG and corticosteroids (80% vs 70%)...
July 20, 2018: Journal of the Peripheral Nervous System: JPNS
Bogdan Bjelica, Ivana Basta, Ivo Bozovic, Aleksandra Kacar, Ana Nikolic, Aleksandra Dominovic-Kovacevic, Zoran Vukojevic, Vesna Martic, Aleksandar Stojanov, Gordana Djordjevic, Milutin Petrovic, Miroslav Stojanovic, Stojan Peric
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. Following measures were used: questionnaire on employment status, Medical Research Council Sum Score, INCAT disability score, Beck Depression Inventory, and Krupp's Fatigue Severity Scale. At the moment of testing, 2% of patients were students, 15% were employed, 9% were unemployed due to CIDP, 9% were unemployed but not due to CIDP, 28% were retired early due to disability caused by CIDP, and finally 37% were in old-age pension...
July 12, 2018: Journal of the Peripheral Nervous System: JPNS
Margherita Marchi, Vincenzo Provitera, Maria Nolano, Marcello Romano, Simona Maccora, Ilaria D'Amato, Erika Salvi, Monique Gerrits, Lucio Santoro, Giuseppe Lauria
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...
July 5, 2018: Journal of the Peripheral Nervous System: JPNS
Sara Mariotto, Alessia Farinazzo, Roberta Magliozzi, Daniela Alberti, Salvatore Monaco, Sergio Ferrari
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...
July 4, 2018: Journal of the Peripheral Nervous System: JPNS
Preet G S Makker, José M Matamala, Susanna B Park, Justin G Lees, Matthew C Kiernan, David Burke, Gila Moalem-Taylor, James Howells
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...
June 19, 2018: Journal of the Peripheral Nervous System: JPNS
Jin He, Lingling Guo, Guorong Xu, Liuqing Xu, Shan Lin, Wanjin Chen, Ning Wang
Demyelinating Charcot-Marie-Tooth disease 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. To investigate the profile of genes mutated and clinical features in demyelinating CMT of Chinese descent, we collected a cohort of 44 demyelinating CMT patients and screened them using multiplex ligation-dependent probe amplification (MLPA) and targeted next-generation sequencing technology...
June 12, 2018: Journal of the Peripheral Nervous System: JPNS
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No abstract text is available yet for this article.
June 2018: Journal of the Peripheral Nervous System: JPNS
Veronica I Shubayev, Douglas W Zochodne
No abstract text is available yet for this article.
June 2018: Journal of the Peripheral Nervous System: JPNS
Jetter Robertson, Jeffrey Raizer, James S Hodges, William Gradishar, Jeffrey A Allen
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. A multivariate analysis identified predictors with the strongest association with a change in rTNS...
June 2018: Journal of the Peripheral Nervous System: JPNS
Valeria Prada, Laura Mori, Susanna Accogli, Matteo Rivarola, Sara Schizzi, Mernhaz Hamedani, Angelo Schenone
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. We concentrated to the upper limbs (UL). We recruited 120 subjects, 60 CMT patients and 60 normal controls and evaluated the strength of the tripod pinch and of the hand-grip with a dynamometer, the opposition ability with the thumb opposition test (TOT) and applied an innovative instrumental testing of hand function using the sensor engineered glove test (SEGT), which previously demonstrated its sensitiveness to measure severity of hands dysfunction in CMT patients...
June 2018: Journal of the Peripheral Nervous System: JPNS
M S Panduranga, Deepti Vibha, Kameshwar Prasad, Achal Kumar Srivastava, Garima Shukla
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 (25.5%) amongst 212 patients with chronic polyneuropathy. Idiopathic axonal polyneuropathy was present in 14.2% patients...
June 2018: Journal of the Peripheral Nervous System: JPNS
Satoshi Kuwabara, Sonoko Misawa, Masahiro Mori, Yuta Iwai, Kazuhide Ochi, Hidekazu Suzuki, Hiroyuki Nodera, Akira Tamaoka, Masahiro Iijima, Tatsushi Toda, Hiroo Yoshikawa, Takashi Kanda, Ko Sakamoto, Susumu Kusunoki, Gen Sobue, Ryuji Kaji
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. This study was an open-label phase 3 clinical trial, enrolling 13 MMN patients. After an induction IVIg therapy (0.4 g/kg/d for 5 consecutive days), maintenance dose (1.0 g/kg) was given every 3 weeks for 52 weeks. The major outcome measures were the Medical Research Council (MRC) sum score and hand-grip strength at week 52...
June 2018: Journal of the Peripheral Nervous System: JPNS
David R Cornblath, Hans-Peter Hartung, Hans D Katzberg, Ingemar S J Merkies, Pieter A van Doorn
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, 1.0, and 2.0 g/kg) of IVIg 10% (panzyga) administered every 3 weeks for 24 weeks. The primary efficacy endpoint is the rate of treatment response, defined as a decrease in adjusted inflammatory neuropathy cause and treatment disability score of ≥1 point, in the IVIg 1...
June 2018: Journal of the Peripheral Nervous System: JPNS
Lena Sagi-Dain, Lilach Shemer, Nathanel Zelnik, Yusri Zoabi, Sadeh Orit, Vardit Adir, Aharon Schif, Amir Peleg
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. We describe a 13-year-old girl presenting with progressive bilateral leg weakness and gait instability. Extensive laboratory studies and spinal magnetic resonance imaging scan were normal. Nerve conduction studies revealed severe lower limb peripheral neuropathy with prominent demyelinative component...
June 2018: Journal of the Peripheral Nervous System: JPNS
Metin Mercan, Vildan Yayla, Serdar Altinay, Serhat Seyhan
Tangier disease (TD) (OMIM#205400) is a rare cause of inherited metabolic neuropathies characterized by marked deficiency of high-density lipoproteins and accumulation of cholesterol esters in various tissue resulting from reverse cholesterol transport deficiency. We report a case of a patient with TD with multifocal demyelinating neuropathy with conduction block who presents with winging scapula, tongue, and asymmetric extremity weakness. We also present a review of all studies published from 1960 to 2017 regarding peripheral neuropathy in TD...
June 2018: Journal of the Peripheral Nervous System: JPNS
Jeffrey A Allen, Melvin Berger, Luis Querol, Krista Kuitwaard, Robert D Hadden
Despite the well-recognized importance of immunoglobulin therapy individualization during the treatment of chronic inflammatory demyelinating polyneuropathy (CIDP), the pathway to best achieve optimization is unknown. There are many pharmacokinetic and immunobiologic variables that can potentially influence the appropriateness of any individual therapy. Although identification of specific autoantibodies and their targets has only been accomplished in a minority of patients with CIDP, already the diagnostic and treatment implications of specific autoantibody detection are being realized...
June 2018: Journal of the Peripheral Nervous System: JPNS
Raquel Guimarães-Costa, Yoland Schoindre, Arnaud Metlaine, Jean-Pascal Lefaucheur, Jean-Philippe Camdessanché, Thierry Maisonobe, Jean-Marc Léger
A 59-year-old woman presented with progressive paresthesias of all of her limbs for 4 years, associated with neuropathic pain, tingling in the tongue and allodynia, consistent with small fiber neuropathy (SFN). Several systemic symptoms and signs were found on clinical examination and laboratory work-up. Neurological investigations including neurophysiologic test and skin biopsy supported the diagnosis of SFN. Chronic exposure to N-hexane was then disclosed and suspected to be the cause of the disease. Following the discontinuation of chronic N-hexane exposure, the patient had a progressive improvement of all signs and symptoms, reinforcing the correlation between exposure to N-hexane, and development of SFN...
June 2018: Journal of the Peripheral Nervous System: JPNS
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