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Journal of the Peripheral Nervous System: JPNS

Jetter Robertson, Jeffrey Raizer, James S Hodges, William Gradishar, Jeffrey A Allen
No abstract text is available yet for this article.
April 25, 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 long time. Especially at the hands level, it is still unclear as to whether OW occurs. Contrasting results may relate to the different muscles 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...
April 24, 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. 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. Leprosy presenting as confluent mononeuritis multiplex constituted 11...
April 23, 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 only one 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/day 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...
April 10, 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 three 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...
March 30, 2018: Journal of the Peripheral Nervous System: JPNS
Lucas Alessandro, José M Pastor Rueda, Miguel Wilken, Luis A Querol Gutiérrez, Mariano Marrodán, Julián N Acosta, Alberto Rivero, Fabio Barroso, Mauricio F Farez
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...
March 30, 2018: Journal of the Peripheral Nervous System: JPNS
Lena Sagi-Dain, Lilach Shemer, Nathanel Zelnik, Yusri Zoabi, Orit Sadeh, Vardit Adir, Aharon Schif, Amir Peleg
BACKGROUND: 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. PATIENT: We describe a 13-years old girl presenting with progressive bilateral leg weakness and gait instability. Extensive laboratory studies and spinal Magnetic resonance imaging scan were normal...
March 26, 2018: Journal of the Peripheral Nervous System: JPNS
Metin Mercan, Vildan Yayla, Serdar Altinay, Serhat Seyhan
Tangier disease (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 Tangier disease 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 Tangier disease...
March 26, 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...
March 23, 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...
March 15, 2018: Journal of the Peripheral Nervous System: JPNS
Melissa R Mandarakas, Kristy J Rose, Oranee Sanmaneechai, Manoj P Menezes, Kathryn M Refshauge, Joshua Burns
A functional outcome measure for infants (aged 0-3 years) with Charcot-Marie-Tooth (CMT) disease is needed for upcoming disease-modifying trials. A systematic review of outcome measures for infants with neuromuscular disorders was completed to determine if validated measures were available for the CMT infant population. We assessed 20,375 papers and identified seven functional outcome measures for infants with neuromuscular disorders. Six were developed and validated for spinal muscular atrophy (SMA). There were no CMT-specific outcome measures identified; however, one (motor function measure) assessed a range of neuromuscular disorders including 13 infants and children with CMT...
March 9, 2018: Journal of the Peripheral Nervous System: JPNS
Carolina Lavigne-Moreira, Vanessa D Marques, Marcus V M Gonçalves, Mauricio F de Oliveira, Pedro J Tomaselli, José C Nunez, Osvaldo J M do Nascimento, Amilton A Barreira, Wilson Marques
To present the genetic heterogeneity of a sample of the Brazilian population with transthyretin (TTR) mutations. This cohort study was descriptive and retrospective, and enrolled patients with peripheral neuropathy of unknown cause that were found to have a mutation in the TTR gene during the process of etiological investigation, between July 1997 to January 2016. Over the study period, 129 point mutations were identified in 448 tested patients, of whom 128 were of Brazilian origin. The TTR Val30Met mutation was identified in 116 patients (90...
March 9, 2018: Journal of the Peripheral Nervous System: JPNS
Pedro J Tomaselli, Mahima Kapoor, Andrea Cortese, James M Polke, Alexander M Rossor, Mary M Reilly
No abstract text is available yet for this article.
March 9, 2018: Journal of the Peripheral Nervous System: JPNS
(no author information available yet)
No abstract text is available yet for this article.
April 2018: Journal of the Peripheral Nervous System: JPNS
(no author information available yet)
No abstract text is available yet for this article.
March 2018: Journal of the Peripheral Nervous System: JPNS
Diego Franciotta, Matteo Gastaldi, Elisabetta Zardini, Eduardo Nobile-Orazio
No abstract text is available yet for this article.
March 2018: Journal of the Peripheral Nervous System: JPNS
Hajime Tanabe, Yujiro Higuchi, Jun-Hui Yuan, Akihiro Hashiguchi, Akiko Yoshimura, Satoshi Ishihara, Satoshi Nozuma, Yuji Okamoto, Eiji Matsuura, Hiroyuki Ishiura, Jun Mitsui, Ryotaro Takashima, Norito Kokubun, Kengo Maeda, Yuri Asano, Yoko Sunami, Yu Kono, Yasunori Ishigaki, Shosaburo Yanamoto, Jiro Fukae, Hiroshi Kida, Mitsuya Morita, Shoji Tsuji, Hiroshi Takashima
Mutations in small heat shock protein beta-1 (HspB1) have been linked to Charcot-Marie-Tooth (CMT) disease type 2F and distal hereditary motor neuropathy type 2B. Only four cases with HSPB1 mutations have been reported to date in Japan. In this study between April 2007 and October 2014, we conducted gene panel sequencing in a case series of 1,030 patients with inherited peripheral neuropathies (IPNs) using DNA microarray, targeted resequencing, and whole-exome sequencing. We identified HSPB1 variants in 1.3% (13 of 1,030) of the patients with IPNs, who exhibited a male predominance...
March 2018: Journal of the Peripheral Nervous System: JPNS
Aleksandra Kacar, Bogdan Bjelica, Ivo Bozovic, Stojan Peric, Ana Nikolic, Mina Cobeljic, Milutin Petrovic, Aleksandar Stojanov, Gordana Djordjevic, Zoran Vukojevic, Aleksandra Dominovic-Kovacevic, Miroslav Stojanovic, Zorica Stevic, Vidosava Rakocevic-Stojanovic, Dragana Lavrnic, Ivana Basta
To date, generic questionnaires have been used to investigate quality of life (QoL) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients. Although these measures are very useful, they are not usually precise enough to measure all specific characteristics of the disease. Our aim was to investigate QoL using the neuromuscular disease-specific questionnaire (individualized neuromuscular quality of life, INQoL) in a large cohort of patients with CIDP. Our study comprised 106 patients diagnosed with CIDP...
March 2018: Journal of the Peripheral Nervous System: JPNS
Guohua Zhao, Jie Song, Mi Yang, Xiuhua Song, Xiaomin Liu
Charcot-Marie-Tooth (CMT) disease is the most common inherited peripheral neuropathy characterized by progressive distal muscle weakness and atrophy with decreased or absent tendon reflexes. Mutations in LRSAM1 have been identified to cause CMT disease type 2P. We report a novel LRSAM1 mutation c.2021-2024del (p.E674VfsX11) in a Chinese autosomal dominant CMT disease type 2 family. The phenotype was characterized by late onset and mild sensory impairment. Electrophysiological findings showed normal or mildly to moderately reduced motor and sensory nerve conduction velocities in lower and upper limb nerves...
March 2018: Journal of the Peripheral Nervous System: JPNS
Binbin Sun, Shuangyi Fan, Kai Yao, Yifan Li, Xusheng Huang
Mercury and its compounds possess strong neurotoxicity and patients with mercury poisoning often report pain and numbness in the distal extremities that conform to the "stocking-glove" pattern. However, no study has investigated whether damage to small nerve fibers is associated with mercury poisoning. The aims of the present study were to evaluate the effects of different doses of mercury chloride (HgCl2 ) on intraepidermal nerve fibers density (IENFD) and Langerhans cells (LCs) in the plantar skin of rats and to assess the possible relationship between changes in IENFD and sensory testing...
March 2018: Journal of the Peripheral Nervous System: JPNS
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