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Journal of Clinical Neuromuscular Disease

Nicholas J Silvestri, Gil I Wolfe, David Lacomis, Mark B Bromberg
The Guillain-Barré syndrome (GBS) is one of the few neuropathies well known to the general public, in part because of its association with swine flu vaccinations in 1976. GBS has again reached the general public with its possible association with Zika virus. The virus, borne by infected Aedes aegypti mosquitos, is being linked to birth defects when pregnant women are bitten and infected. There are early reports also linking GBS to Zika infection, which could expose a wider range of infected people to the neuropathy...
September 2016: Journal of Clinical Neuromuscular Disease
Derrece Reid, Yuliya Zinger, Divisha Raheja
No abstract text is available yet for this article.
September 2016: Journal of Clinical Neuromuscular Disease
Samuel Arends, Paul Willem Wirtz
No abstract text is available yet for this article.
September 2016: Journal of Clinical Neuromuscular Disease
Chirag B Patel, Bhavesh Trikamji, Glenn Mathisen, Catherine Yim, Brian Zipser, Shrikant Mishra
No abstract text is available yet for this article.
September 2016: Journal of Clinical Neuromuscular Disease
Jafar Kafaie, Minsoo Kim, Erik Krause
OBJECTIVE: To identify clinical and quantitative relationship between vaccinations and small fiber neuropathy (SFN). SFN refers to damaged unmyelinated or thinly myelinated sensory and/or autonomic fibers. Diagnosis is primarily based on clinical presentation. Intraepidermal nerve fiber density can provide diagnostic confirmation with a sensitivity of 88% and a specificity of 91%. However, the possible association between vaccination and small fiber polyneuropathy is not well defined...
September 2016: Journal of Clinical Neuromuscular Disease
Roeland B van Leeuwen, Bart W Smits, Richard J Rodenburg, Baziel G van Engelen
In patients with a triad of sensory ataxic neuropathy, dysarthria, and ophthalmoparesis (SANDO), the presenting features are mainly ataxia or ptosis. SANDO patients often have impaired balance and gait, which is not surprising considering the combination of sensory ataxic neuropathy, and additional symptoms like cerebellar ataxia and limb girdle weakness. We describe a SANDO patient who noticed an increasingly impaired balance and gait, without any dizziness. Neurological investigation revealed an external ophthalmeplegia and a cerebellar ataxia; the head impulse test was not reliable because of eye movement disorders...
September 2016: Journal of Clinical Neuromuscular Disease
T Zambelis, S Kanelli, E Tzavellas, T Paparrigopoulos, N Karandreas
OBJECTIVES: To evaluate the occurrence of Ulnar neuropathy at the elbow (UNE) among alcohol dependent subjects. METHOD: The study sample comprised 99 consecutive alcohol dependent subjects treated for detoxification voluntarily in the specialized unit of the Athens University Psychiatric Clinic in an inpatient basis. RESULTS: UNE was found in 51 subjects (51.5%): in 40.4% on the right side, in 44.6% on the left and in 20.1% on both. Polyneuropathy was diagnosed in 41...
September 2016: Journal of Clinical Neuromuscular Disease
Hafiz A Elahi, Tulio E Bertorini, Masanori Igarashi, William H Mays, John N Whitaker
OBJECTIVES: We conducted a retrospective study analyzing the clinical features, laboratory findings, demographics, and long-term prognoses of patients with juvenile inflammatory myopathies to determine possible predictors indicating the use of aggressive immunotherapy and the response to and complications of treatment. METHODS: The medical records of 41 patients with juvenile inflammatory myopathies seen at University of Tennessee-affiliated hospitals in Memphis from 1969 to 2008 were evaluated...
September 2016: Journal of Clinical Neuromuscular Disease
Kristin L Jack, Wilma J Koopman, Denise Hulley, Michael W Nicolle
OBJECTIVES: Myasthenia gravis (MG) is an autoimmune disorder in which antibodies interfere with neuromuscular transmission. Azathioprine (AZA) is an immunosuppressant frequently used for treatment of various autoimmune conditions, including MG. The literature suggests that the rates of AZA-associated hepatotoxicity and myelosuppression in MG are highly variable. Published studies have not formally analyzed their pattern, severity, timing, and/or recovery. We assessed the prevalence, pattern and timing of AZA associated toxicity in a large group of MG patients...
September 2016: Journal of Clinical Neuromuscular Disease
Paola Ortiz-Salas, Alberto Velez-Van-Meerbeke, Camilo Alberto Galvis-Gomez, Jesús H Rodriguez Q
OBJECTIVES: To evaluate the efficacy and safety over a short time period of human intravenous immunoglobulin versus plasma exchange (PE) in the management of some autoimmune neurologic diseases. In addition, length of hospital stay and duration of ventilator support were compared. METHODS: Randomized controlled trials and analytical observational studies of more than 10 cases were reviewed. Cochrane Neuromuscular Disease Group trials, MEDLINE, EMBASE, HINARI, and Ovid, were searched as data source...
September 2016: Journal of Clinical Neuromuscular Disease
Nicholas J Silvestri, Gil I Wolfe, Mark Bromberg, David Lacomis
One of the first questions asked by patients and family members when a diagnosis of amyotrophic lateral sclerosis is made is "what about stem cells?" The term "stem cells" has attractiveness to it, with the assumption that stem cell treatment (stem nerve cells) can replace lost nerve cells. There are perhaps 2 types of stem cell trials, those that are vetted by the Food and Drug Administration and those that have no official oversight and whose results are infrequently published. The issue of the latter was discussed in the last edition of this column...
June 2016: Journal of Clinical Neuromuscular Disease
Homa Sadeghian, Hamid Arasteh, Rouzbeh Motiei-Langroudi
No abstract text is available yet for this article.
June 2016: Journal of Clinical Neuromuscular Disease
Artemios K Artemiadis, Christos Peppas, Sotiris Giannopoulos, Vasiliki Zouvelou, Nikos Triantafyllou
Amyotrophic lateral sclerosis (ALS) constitutes the main type of motor neuron disease. Familial ALS is characterized by the presence of positive family history and accounts for 10% of ALS cases. Although familial ALS is the main culprit for early-onset disease, there are rare cases of early- or young-onset ALS with negative family history or sporadic ALS. We describe a 23-year-old man with clinical and electrophysiological evidence of probable sporadic ALS according to the revised EI Escorial criteria. Interestingly, brain neuroimaging revealed bilaterally increased T2 signals across corona radiata, posterior limb of the internal capsule, and descending motor tracts in the brainstem and hypointensity rim of the motor cortex on T2-weighted images...
June 2016: Journal of Clinical Neuromuscular Disease
Jenny Lin, Sumit Verma
OBJECTIVE: To study the electrodiagnostic abnormalities in tick paralysis. METHODS: A 7-year-old girl with acute onset proximal muscle weakness and an engorged dog tick attached to her scalp was evaluated. RESULTS: The routine motor nerve conduction study showed normal compound muscle action potential amplitude. The stimulated jitter analysis of the orbicularis oculi muscle showed normal jitter with no blocking. The quantitative electromyography of the proximal muscles showed decreased mean duration of the motor unit potentials...
June 2016: Journal of Clinical Neuromuscular Disease
Norman Latov, Mary L Vo, Russell L Chin, Bridget T Carey, Jennifer A Langsdorf, Naomi T Feuer
Abnormal concentrations of nutritional factors were found in 24.1% of 187 patients with neuropathy who were newly seen at our academic neuropathy referral center over a 1-year period. All patients presented with sensory axonal or small fiber neuropathy. In 7.3%, they were present in association with at least one other identifiable cause for neuropathy. Elevated levels of pyridoxal phosphate or mercury occurred more frequently than deficiencies in vitamins B1, B12, or B6. The nutritional abnormalities are amenable to correction by dietary intervention...
June 2016: Journal of Clinical Neuromuscular Disease
Brin Freund, Manoj Maddali, Thomas E Lloyd
INTRODUCTION: Morvan syndrome is a rare autoimmune/paraneoplastic disorder involving antibodies to the voltage-gated potassium channel complex. It is defined by subacute encephalopathy, neuromuscular hyperexcitability, dysautonomia, and sleep disturbance. It may present a diagnostic dilemma when trying to differentiate from amyotrophic lateral sclerosis with frontotemporal dementia. METHODS: A 76-year-old man with a history of untreated prostate adenocarcinoma was evaluated for subacute cognitive decline, diffuse muscle cramps, and hyponatremia...
June 2016: Journal of Clinical Neuromuscular Disease
Kensuke Ikeda, Madoka Mori-Yoshimura, Toshiyuki Yamamoto, Masahiro Sonoo, Shigeaki Suzuki, Yoshiyuki Kondo, Harumasa Nakamura, Kana Mitsuhashi, Meiko Hashimoto Maeda, Jun Shimizu, Yukiko K Hayashi, Ichizo Nishino, Yasushi Oya, Miho Murata
OBJECTIVES: To report cases of chronic autoimmune necrotizing myopathy with anti-signal recognition particle antibodies (anti-SRP myopathy) initially misdiagnosed as muscular dystrophy, in particular, facioscapulohumeral muscular dystrophy (FSHD). METHODS: Medical records of patients with anti-SRP myopathy in our institution were retrospectively reviewed. RESULTS: All 6 patients were initially diagnosed with muscular dystrophy because of the long-term clinical course and lack of inflammation on biopsy; 5 were diagnosed with FSHD based on a winged scapula...
June 2016: Journal of Clinical Neuromuscular Disease
Mohamed Kazamel, Eric J Sorenson, Margherita Milone
OBJECTIVE: To compare the clinical and electrophysiological findings in hereditary inclusion body myopathy (hIBM) and sporadic inclusion body myositis (sIBM) patients. METHODS: We retrospectively identified 8 genetically proven hIBM patients and 1 DNAJB6 myopathy with pathological features of hIBM, and compared their clinical, electromyographic, and serological data with a group of 51 pathologically proven sIBM patients. RESULTS: hIBM patients had a younger mean age of onset (36 vs...
June 2016: Journal of Clinical Neuromuscular Disease
Rocio Garcia-Santibanez, Stephen N Scelsa
OBJECTIVES: Patients with carpal tunnel syndrome (CTS) and paracervical pain (PCP) are often incorrectly diagnosed with cervical radiculopathy. The objective of the study is to determine how frequently such patients have electrophysiologic evidence of radiculopathy. METHODS: We reviewed charts of patients with clinical features of CTS and at least 1 median nerve conduction parameter showing slowing across the wrist. Patients were divided into those with and without PCP...
June 2016: Journal of Clinical Neuromuscular Disease
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