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Journal of the Peripheral Nervous System: JPNS

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https://www.readbyqxmd.com/read/29119642/protocol-of-a-dose-response-trial-of-iv-immunoglobulin-in-chronic-inflammatory-demyelinating-polyradiculoneuropathy-drip-study
#1
K Kuitwaard, W J R Fokkink, E Brusse, A F J E Vrancken, F Eftimov, N C Notermans, A J van der Kooi, I S J Merkies, B C Jacobs, P A van Doorn
High peak levels of serum IgG may not be needed for maintenance treatment of CIDP with IVIg. More frequent dosing of IVIg leads to more stable IgG levels and higher trough levels which may be related with improved clinical efficacy. More frequent lower dosing leads to lower peak levels and may induce less systemic side-effects. The DRIP study is a double-blind randomized controlled cross-over intervention study. CIDP patients ≥ 18 years old, proven IVIg dependent and receiving an individually established but stable maintenance dose and interval of IVIg (Kiovig) can be included...
November 9, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29119633/antibody-responses-to-galc-in-severe-and-complicated-childhood-guillain-barr%C3%A3-syndrome
#2
LETTER
Patrick M Meyer Sauteur, Ruth Huizinga, Anne P Tio-Gillen, Marie-Claire Y de Wit, Wendy W J Unger, Christoph Berger, Annemarie M C van Rossum, Bart C Jacobs
We recently presented a case series of seven children who developed severe and complicated Guillain-Barré syndrome (GBS) after infection with M. pneumoniae (Mp) (Meyer Sauteur et al., 2015). The disease was rapidly progressive and severe: one died, four had clinically defined central nervous system (CNS) involvement, and five required mechanical ventilation.
November 9, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29095548/epidemiology-of-guillain-barr%C3%A3-syndrome-in-finland-2004-2014
#3
Jussi Sipilä, Merja Soilu-Hänninen, Jori O Ruuskanen, Päivi Rautava, Ville Kytö
At total mean incidence of 0.84-1.1/100,000 the occurrence of Guillain-Barré syndrome (GBS) is reported to be low in Finland compared to other Caucasian populations. However, a recent study from Southwestern Finland reported an incidence of 1.82/100,000 which is comparable to other Caucasian populations. We analyzed discharge data covering the years 2004 through 2014 on all neurological admissions in all Finnish university and central hospitals with a primary diagnosis of GBS. 989 admissions due to GBS (917 individuals) were identified...
November 2, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29094786/intraneural-perineuriomas-diagnostic-value-of-magnetic-resonance-neurography
#4
L León Cejas, D Binaghi, M Socolovsky, A Dubrovsky, L Pirra, C Marchesoni, A Pardal, S Monges, G Peretti, A Taratuto, F Lubinieki, R Reisin
Intraneural perineurioma is an under-recognized hypertrophic peripheral nerve tumor. It affects young patients involving frequently the sciatic nerve and its branches and presents with a progressive, painless and predominantly motor deficit. Magnetic resonance neurography is a useful tool to localize the lesion, evaluate its extension, and discriminate between different etiologies. We reviewed the clinical records of 11 patients with pathologically confirm intraneural perineurioma. Eight patients were males with mean age 19 years...
November 2, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29092099/maintenance-iv-immunoglobulin-treatment-in-chronic-inflammatory-demyelinating-polyradiculoneuropathy
#5
REVIEW
K Kuitwaard, W J R Fokkink, E Brusse, A F J E Vrancken, F Eftimov, N C Notermans, A J van der Kooi, I S J Merkies, B C Jacobs, P A van Doorn
CIDP patients treated with intravenous immunoglobulin (IVIg) usually start with a standard dosage of 2 g/kg body weight. Only a minority of patients have a sustained improvement, and most require ongoing maintenance treatment. Preferred IVIg regimens however vary considerably between doctors and at present it is unknown which is optimal. Since there are also large differences in IVIg dosage and interval requirements between patients, optimal IVIg maintenance treatment of CIDP is even more complex. The lack of evidence based guidelines on how IVIg maintenance treatment should be administered may potentially lead to under- or overtreatment of this expensive therapy...
November 1, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29091318/early-electrophysiological-findings-in-acute-inflammatory-demyelinating-polyradiculoneuropathy-variant-of-guillain-barre-syndrome-in-the-pakistani-population-a-comparison-with-global-data
#6
Ahmed Wali, Sara Khan
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) are the most common variants of Guillian Barre syndrome documented in the Asian population. However the variability of early neurophysiologic findings in the Asian population compared to western data has not been documented. Eighty seven cases of AIDP were retrospectively reviewed for their demographic, clinical, electrophysiological and laboratory data. Mean age of subjects was 31 ± 8 years with males more commonly affected...
November 1, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29065233/-chronic-inflammatory-demyelinating-polyradiculoneuropathy-clinical-aspects-and-new-animal-models-of-auto-immunity-to-nodal-components
#7
Isabel Illa
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic and treatable disorder of the peripheral nerves with clinical and immunological heterogeneity.
October 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29065229/prediction-of-disease-progression-in-miller-fisher-and-overlap-syndromes
#8
Christine Verboon, Heleen van Berghem, Pieter A van Doorn, Liselotte Ruts, Bart C Jacobs
BACKGROUND: Patients with Miller Fisher syndrome (MFS) may have a relatively mild clinical course or progress to Guillain-Barré syndrome with limb weakness (MFS-GBS overlap syndrome). Other variants in this spectrum are GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE). AIM: To compare the clinical course of MFS and overlap syndromes and to identify predictors of disease progression. METHODS: In a prospective study of 170 patients with GBS and variant forms, 37 (22%) had a MFS, MFS-GBS overlap syndrome, ophthalmoplegic GBS or BBE...
October 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28837237/pseudodominant-inheritance-pattern-in-a-family-with-cmt2-caused-by-gdap1-mutations
#9
Barbara W van Paassen, Marieke Bronk, Camiel Verhamme, Fred van Ruissen, Frank Baas, Karin Y van Spaendonck-Zwarts, Marianne de Visser
We report a family in which an autosomal dominantly inherited Charcot-Marie-Tooth (CMT) disease type 2 was suspected. The affected family members (proband, sister, father, and paternal aunt) showed intrafamilial clinical variability. The proband needed walking aids since adolescence because of generalized muscle weakness. The sister showed the same symptoms although to a lesser extent. The father and paternal aunt had foot deformity and atrophy of lower legs. A homozygous GDAP1 mutation was found in the proband and in the sister...
August 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28834584/a-de-novo-dominant-mutation-in-kif1a-associated-with-axonal-neuropathy-spasticity-and-autism-spectrum-disorder
#10
Pedro J Tomaselli, Alexander M Rossor, Alejandro Horga, Matilde Laura, Julian C Blake, Henry Houlden, Mary M Reilly
Mutations in the kinesin family member 1A (KIF1A) gene have been associated with a wide range of phenotypes including recessive mutations causing hereditary sensory neuropathy and hereditary spastic paraplegia and de novo dominant mutations causing a more complex neurological disorder affecting both the central and peripheral nervous system. We identified by exome sequencing a de novo dominant missense variant, (c.38G>A, p.R13H), within an ATP binding site of the kinesin motor domain in a patient manifesting a complex phenotype characterized by autism spectrum disorder (ASD), spastic paraplegia and axonal neuropathy...
August 23, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28543957/a-novel-mutation-in-the-fgd4-gene-causing-charcot-marie-tooth-disease
#11
Panagiotis Zis, Mary M Reilly, Dasappaiah G Rao, Pedro Tomaselli, Alex M Rossor, Marios Hadjivassiliou
No abstract text is available yet for this article.
September 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28485482/deletion-of-sarm1-gene-is-neuroprotective-in-two-models-of-peripheral-neuropathy
#12
Elliot Turkiew, Debbie Falconer, Nicole Reed, Ahmet Höke
Distal axon degeneration seen in many peripheral neuropathies is likely to share common molecular mechanisms with Wallerian degeneration. Although several studies in mouse models of peripheral neuropathy showed prevention of axon degeneration in the slow Wallerian degeneration (Wlds) mouse, the role of a recently identified player in Wallerian degeneration, Sarm1, has not been explored extensively. In this study, we show that mice lacking the Sarm1 gene are resistant to distal axonal degeneration in a model of chemotherapy induced peripheral neuropathy caused by paclitaxel and a model of high fat diet induced putative metabolic neuropathy...
September 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28833828/markers-for-guillain-barr%C3%A3-syndrome-with-poor-prognosis-a-multi-center-study
#13
Yuko Yamagishi, Hidekazu Suzuki, Masahiro Sonoo, Satoshi Kuwabara, Takanori Yokota, Kyoichi Nomura, Atsuro Chiba, Ryuji Kaji, Takashi Kanda, Kenichi Kaida, Shu-Ichi Ikeda, Tatsuro Mutoh, Ryo Yamasaki, Hiroshi Takashima, Makoto Matsui, Kazutoshi Nishiyama, Gen Sobue, Susumu Kusunoki
Guillain-Barré syndrome (GBS) is an acute monophasic neuropathy. Prognostic tools include the modified Erasmus GBS outcome score (mEGOS), Erasmus GBS respiratory insufficiency score (EGRIS), and increase in IgG levels (ΔIgG) 2 weeks after intravenous immunoglobulin (IVIg) treatment. Given that proportions of GBS subtypes differ between Western countries and Japan, the usefulness of these tools in Japan or other countries remains unknown. We enrolled 177 Japanese patients with GBS from 15 university hospitals and retrospectively obtained mEGOS and EGRIS for all and ΔIgG status for 79 of them...
August 18, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28786512/the-role-of-nutrition-as-risk-factor-for-polyneuropathy-a-case-control-study
#14
Nora A Visser, Nicolette C Notermans, Jeanne H M de Vries, Leonard H van den Berg, Alexander F J E Vrancken
OBJECTIVE: The aim of this case-control study is to investigate the role of nutrition as risk factor for polyneuropathy. METHODS: Three hundred eighteen patients with chronic idiopathic axonal polyneuropathy and 636 matched controls completed a validated food frequency questionnaire that covered nutrient intake and alcohol consumption. As risk estimates we calculated adjusted odds ratios for the intake of energy and nutrients. RESULTS: Energy and nutrient intake did not differ between patients and controls, regardless of moderate alcohol consumption...
August 8, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28660751/clinical-and-genetic-diversities-of-charcot-marie-tooth-disease-with-mfn2-mutations-in-a-large-case-study
#15
Masahiro Ando, Akihiro Hashiguchi, Yuji Okamoto, Akiko Yoshimura, Yu Hiramatsu, Junhui Yuan, Yujiro Higuchi, Jun Mitsui, Hiroyuki Ishiura, Ayako Umemura, Koichi Maruyama, Takeshi Matsushige, Shinichi Morishita, Masanori Nakagawa, Shoji Tsuji, Hiroshi Takashima
Charcot-Marie-Tooth disease (CMT) constitutes a heterogeneous group affecting motor and sensory neurons in the peripheral nervous system. MFN2 mutations are the most common cause of axonal CMT. We describe the clinical and mutational spectra of CMT patients harboring MFN2 mutations in Japan. We analyzed 1,334 unrelated patients with clinically suspected CMT referred by neurological and neuropediatric departments throughout Japan. We conducted mutation screening using a DNA microarray, targeted resequencing, and whole-exome sequencing...
June 29, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28646538/a-novel-attr-l32v-mutation-causes-familial-amyloid-polyneuropathy-in-a-bolivian-family
#16
Pedro L Martínez-Ulloa, Manuela Vallejo, Iñigo Corral, Nuria García-Barragán, Alberto Alcazar, Emma Martínez-Alonso, Javier Martínez-Poles, Hector Pian, Adriano Jiménez-Escrig
We report a new transthyretin (ATTR) gene c.272C>G mutation and variant protein, p.Leu32Val, in a kindred of Bolivian origin with a rapid progressive peripheral neuropathy and cardiomyopathy. Three individuals from a kindred with peripheral nerve and cardiac amyloidosis were examined. Analysis of the TTR gene was performed by Sanger direct sequencing. Neuropathologic examination was obtained on the index patient with mass spectrometry study of the ATTR deposition. Direct DNA sequence analysis of exons 2, 3, and 4 of the TTR gene demonstrated a c...
June 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28600844/pain-in-chemotherapy-induced-peripheral-neurotoxicity
#17
REVIEW
Paola Marmiroli, Arianna Scuteri, David R Cornblath, Guido Cavaletti
Chemotherapy-induced peripheral neurotoxicity (CIPN) is a potentially dose-limiting side effect of the treatment of several cancers. CIPN is predominantly or exclusively sensory, and it is frequently associated with unpleasant symptoms, overall referred to as "pain." However, given the markedly different clinical presentation and course of CIPN depending on the antineoplastic drug used, the broad term "pain" in the specific context of CIPN needs to be reconsidered and refined. In fact, a precise identification of the features of CIPN has relevant implication in the design of rational-based clinical trials and in the selection of possible active drugs...
June 10, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28573768/nodes-of-ranvier-in-skin-biopsies-of-patients-with-diabetes-mellitus
#18
Kathrin Doppler, Franziska Frank, Ann-Cathrin Koschker, Karlheinz Reiners, Claudia Sommer
Paranodal demyelination has been discussed as a potential mechanism of nerve fiber damage in diabetic neuropathy (DNP). Studies on human tissue are limited, as nerve biopsies are invasive and only rarely performed in patients with confirmed DNP. Skin biopsy has recently been suggested as a tool to analyze paranodal and nodal changes of myelinated fibers. We analyzed the myelinated fibers of skin biopsies of 35 patients with DNP, 17 patients with diabetes mellitus (DM) without neuropathy, and 30 normal controls...
June 2, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28594116/minimum-clinically-important-difference-analysis-confirms-the-efficacy-of-igpro10-in-cidp-the-prima-trial
#19
LETTER
Ingemar S J Merkies, John-Philip Lawo, Jonathan M Edelman, Jan L De Bleecker, Claudia Sommer, Wim Robberecht, Mika Saarela, Jerzy Kamienowski, Zbigniew Stelmasiak, Orell Mielke, Björn Tackenberg, Jean-Marc Léger
No abstract text is available yet for this article.
June 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28447405/high-mortality-from-guillain-barr%C3%A3-syndrome-in-bangladesh
#20
Tanveen Ishaque, Mohammad B Islam, Gulshan Ara, Hubert P Endtz, Quazi D Mohammad, Bart C Jacobs, Zhahirul Islam
Although Guillain-Barré syndrome (GBS) has higher incidence and poor outcome in Bangladesh, mortality from GBS in Bangladesh has never been explored before. We sought to explore the frequency, timing, and risk factors for deaths from GBS in Bangladesh. We conducted a prospective study on 407 GBS patients who were admitted to Dhaka Medical College Hospital, Dhaka, Bangladesh from 2010 to 2013. We compared deceased and alive patients to identify risk factors. Cox regression model was used to adjust for confounders...
June 2017: Journal of the Peripheral Nervous System: JPNS
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