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Multifocal Motor Neuropathy With Conduction Blocks

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Comparison of MRI and motor evoked potential with triple stimulation technique for the detection of brachial plexus abnormalities in multifocal motor neuropathy.

Giovanni Corazza, Thomas Le Corroller, Aude-Marie Grapperon, Emmanuelle Salort-Campana, Annie Verschueren, Shahram Attarian, Emilien Delmont,

BACKGROUND:Conduction blocks (CB) are the diagnostic hallmark of multifocal motor neuropathy (MMN). Conventional nerve conduction studies cannot detect CB above Erb's point. Our purpose was to compare the performance of the motor evoked potential with triple stimulation technique (MEP-TST) and MRI in the detection of abnormalities of the brachial plexus. ... Read more >>

Muscle Nerve (Muscle & nerve)
[2020, 61(3):325-329]

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Dose Adjustment of Subcutaneous IgG in Chronic Inflammatory Demyelinating Polyneuropathy.

Alanood Alsolaihim, Steven K. Baker,

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy that is characterized by a slowly progressive sensory and motor involvement lasting at least 2 months. We present a CIDP patient on subcutaneous Ig (SCIg). Upon fine-tuning his dose from 24 to 28 g/week, this showed a dramatic improvement in both ... Read more >>

Case Rep Neurol (Case reports in neurology)
[2020, 12(1):73-77]

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Conduction Block and Nerve Cross-Sectional Area in Multifocal Motor Neuropathy.

Yi Li, Jingwen Niu, Tanxin Liu, Qingyun Ding, Shuang Wu, Yuzhou Guan, Liying Cui, Mingsheng Liu,

Introduction: Motor nerve conduction block (CB) is the main electrophysiological feature of multifocal motor neuropathy (MMN). Increased cross-sectional area (CSA) can be detected by nerve ultrasound in MMN. In this study, we aim to analyze the correlation between CB and CSA in MMN. Methods: Twelve patients with MMN were recruited. ... Read more >>

Front Neurol (Frontiers in neurology)
[2019, 10:1055]

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Immune-Mediated Peripheral Neuropathy in Patients with Diffuse Panbronchiolitis.

You Ri Kang, Tai Seung Nam, Kyung Hwa Lee, Shina Kim, Hyeon Joong Park, Seung Jin Lee, Seung Han Lee, Byeong C Kim,

J Clin Neurol (Journal of clinical neurology (Seoul, Korea))
[2020, 16(1):166-168]

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Antiganglioside antibodies in neurological diseases.

Pitchaya Wanleenuwat, Piotr Iwanowski, Wojciech Kozubski,

Gangliosides are sialylated glycosphingolipids, highly abundant in our nervous system. Antibodies targeting gangliosides are usually developed as a consequence of molecular mimicry following infections. Antiganglioside antibodies are implicated in many neurological disorders such as acute and chronic polyradiculoneuropathies which includes different variants of Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and ... Read more >>

J. Neurol. Sci. (Journal of the neurological sciences)
[2020, 408:116576]

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Childhood-onset multifocal motor neuropathy with IgM antibodies to GM2 and GalNac-GD1a.

Hiroshi Maeda, Ryotaro Ishii, Susumu Kusunoki, Tomohiro Chiyonobu,

BACKGROUND:Multifocal motor neuropathy (MMN) is an acquired immune-mediated form of neuropathy characterized by upper and asymmetric limb weakness without sensory loss. The mean age of onset is 40 years (range, 20-70 years), and childhood-onset MMN is extremely rare. In the present report, we discuss a case of childhood-onset MMN in a patient ... Read more >>

Brain Dev. (Brain & development)
[2019, :]

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Multifocal motor neuropathy: controversies and priorities.

Wei Zhen Yeh, P James Dyck, Leonard H van den Berg, Matthew C Kiernan, Bruce V Taylor,

Despite 30 years of research there are still significant unknowns and controversies associated with multifocal motor neuropathy (MMN) including disease pathophysiology, diagnostic criteria and treatment. Foremost relates to the underlying pathophysiology, specifically whether MMN represents an axonal or demyelinating neuropathy and whether the underlying pathophysiology is focused at the node ... Read more >>

J. Neurol. Neurosurg. Psychiatry (Journal of neurology, neurosurgery, and psychiatry)
[2020, 91(2):140-148]

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Differential Diagnoses of Amyotrophic Lateral Sclerosis are More Variegated than Anticipated.

Josef Finsterer,

Ann Indian Acad Neurol (Annals of Indian Academy of Neurology)
[2019, 22(4):513-514]

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Motor conduction block and conduction velocity in Lewis-Sumner syndrome and multifocal motor neuropathy.

Yi Li, Jingwen Niu, Tanxin Liu, Yuzhou Guan, Shuang Wu, Qingyun Ding, Liying Cui, Mingsheng Liu,

Motor conduction blocks (CBs) and decreased motor nerve conduction velocity (MCV) are both demyelination electrophysiological characteristics. Though CBs are both common in Lewis-Sumner syndrome (LSS) and multifocal motor neuropathy (MMN), they are two distinct disease groups, so their MCV and CBs electrophysiological characteristics may be different. In this paper, we ... Read more >>

J Clin Neurosci (Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia)
[2019, 67:10-13]

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Nerve ultrasound can identify treatment-responsive chronic neuropathies without electrodiagnostic features of demyelination.

H Stephan Goedee, Ingrid J T Herraets, Leo H Visser, Hessel Franssen, Jan-Thies H van Asseldonk, W Ludo van der Pol, Leonard H van den Berg,

INTRODUCTION:We present a case series of six treatment-naive patients with clinical phenotypes compatible with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy without electrodiagnostic features of demyelination but with abnormal peripheral ultrasound findings who responded to treatment. METHODS:All six patients underwent a complete set of ancillary investigations, including extensive nerve ... Read more >>

Muscle Nerve (Muscle & nerve)
[2019, 60(4):415-419]

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Sentinel Node Biopsy and Lumpectomy in a Patient with Machado-Joseph Disease.

N N Aldawoodi, A R Escher, A Padalia, D Padalia,

Spinocerebellar ataxia 3 (SCA3), also known as Machado-Joseph disease (MJD) is an autosomal dominant, progressive neurodegenerative disorder. Patients present with cerebellar ataxia, dystonia, rigidity, and neuropathy that worsen with time. On a molecular level, it occurs due to a CAG trinucleotide repeat expansion in the ATXN3 gene. Due to the ... Read more >>

Case Rep Anesthesiol (Case reports in anesthesiology)
[2019, 2019:2309598]

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Clinical and functional change in multifocal motor neuropathy treated with IVIg.

Peck Kee Chia, Stefanie Kar Yan Hung, Fu Liong Hiew,

We determined the clinical progression, disability and outcome of 11 Multifocal Motor Neuropathy (MMN) patients from Malaysia. Mean patient age was 46.8 (SD 13.3), with mean disease duration of 108.0 months (SD 80.2). All reported unilateral limb weakness at onset. At diagnosis, after mean 49.9 months (SD 73.5) delay, 7 (63.6%) had ... Read more >>

J Clin Neurosci (Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia)
[2019, 69:114-119]

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Comparison of Lewis-Sumner syndrome with chronic inflammatory demyelinating polyradiculoneuropathy patients in a tertiary care centre.

G Fargeot, T Maisonobe, D Psimaras, R Debs, T Lenglet, D Adams, C Vandendries, C Labeyrie, K Viala,

BACKGROUND AND PURPOSE:Whether the Lewis-Sumner syndrome (L-SS) is a distinct entity from other types of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP-ot) remains controversial. METHOD:The clinical/electrophysiological characteristics and long-term outcomes of 45 L-SS and 35 CIDP-ot patients were retrospectively compared. RESULTS:The CIDP-ot group was composed of 11 patients with a typical CIDP, ... Read more >>

Eur. J. Neurol. (European journal of neurology)
[2020, 27(3):522-528]

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Neurolymphomatosis of the lumbosacral plexus and its branches: case series and literature review.

Pierre R Bourque, Marcos Loreto Sampaio, Jodi Warman-Chardon, Sam Samaan, Carlos Torres,

BACKGROUND:Neurolymphomatosis (NL) is a direct process of invasion of peripheral nerves by lymphoma. It occurs in roughly 5% of patients with lymphoma and represents a particularly difficult diagnostic dilemma when it is the presenting focal manifestation of occult lymphoma. CASE PRESENTATION:We present 3 examples of invasion of the lumbosacral plexus ... Read more >>

BMC Cancer (BMC cancer)
[2019, 19(1):1149]

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Amyotrophic Lateral Sclerosis-like Syndrome after Chikungunya.

Felipe César Andrade, Vinicius Vergetti, Gabriella Cozza, Maria Clara Falcao, Gustavo Azevedo,

Amyotrophic lateral sclerosis (ALS)-like syndrome refers to a group of conditions whose outcome is similar to that of amyotrophic lateral sclerosis, but with different characteristics in the initial phase and response to therapy. The involvement of an earlier age group, the subacute course, and the stabilization or improvement of the ... Read more >>

Cureus (Cureus)
[2019, 11(10):e5876]

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Evidence-based recommendations for examination and diagnostic strategies of polyneuropathy electrodiagnosis.

H Tankisi, K Pugdahl, S Beniczky, H Andersen, A Fuglsang-Frederiksen,

The purpose of this report is to recommend evidence-based strategies for polyneuropathy (PNP) electrodiagnosis based on a large cohort of patients examined prospectively. Nerve conduction studies (NCS) of bilateral tibial, peroneal and sural nerves, the latter with both near-nerve-technique (NNT) and surface recordings, were done in 313 patients with clinically ... Read more >>

Clin Neurophysiol Pract (Clinical neurophysiology practice)
[2019, 4:214-222]

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Ultrasound in Multifocal Motor Neuropathy: Clinical and Electrophysiological Correlations.

Ari Breiner, Hamid Ebadi, Vera Bril, Carolina Barnett, Hans D Katzberg,

OBJECTIVES:Multifocal motor neuropathy (MMN) is a treatable autoimmune polyneuropathy, which may prove challenging diagnostically in the setting of absent conduction blocks or advanced axonal loss. Relatively few studies have examined the role of ultrasound (US) in MMN. METHODS:Retrospective, cross-sectional study of patients with MMN who underwent peripheral nerve US. Charts ... Read more >>

J Clin Neuromuscul Dis (Journal of clinical neuromuscular disease)
[2019, 20(4):165-172]

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Brainstem dysfunction in critically ill patients.

Sarah Benghanem, Aurélien Mazeraud, Eric Azabou, Vibol Chhor, Cassia Righy Shinotsuka, Jan Claassen, Benjamin Rohaut, Tarek Sharshar,

The brainstem conveys sensory and motor inputs between the spinal cord and the brain, and contains nuclei of the cranial nerves. It controls the sleep-wake cycle and vital functions via the ascending reticular activating system and the autonomic nuclei, respectively. Brainstem dysfunction may lead to sensory and motor deficits, cranial ... Read more >>

Crit Care (Critical Care)
[2020, 24(1):5]

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Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype.

Andrea Cortese, Raffaella Lombardi, Chiara Briani, Ilaria Callegari, Luana Benedetti, Fiore Manganelli, Marco Luigetti, Sergio Ferrari, Angelo M Clerici, Girolama Alessandra Marfia, Andrea Rigamonti, Marinella Carpo, Raffaella Fazio, Massimo Corbo, Anna Mazzeo, Fabio Giannini, Giuseppe Cosentino, Elisabetta Zardini, Riccardo Currò, Matteo Gastaldi, Elisa Vegezzi, Enrico Alfonsi, Angela Berardinelli, Ludivine Kouton, Constance Manso, Claudia Giannotta, Pietro Doneddu, Patrizia Dacci, Laura Piccolo, Marta Ruiz, Alessandro Salvalaggio, Chiara De Michelis, Emanuele Spina, Antonietta Topa, Giulia Bisogni, Angela Romano, Sara Mariotto, Giorgia Mataluni, Federica Cerri, Claudia Stancanelli, Mario Sabatelli, Angelo Schenone, Enrico Marchioni, Giuseppe Lauria, Eduardo Nobile-Orazio, Jérôme Devaux, Diego Franciotta,

OBJECTIVE:To assess the prevalence and isotypes of anti-nodal/paranodal antibodies to nodal/paranodal proteins in a large chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) cohort, compare clinical features in seronegative vs seropositive patients, and gather evidence of their isotype-specific pathogenic role. METHODS:Antibodies to neurofascin-155 (Nfasc155), neurofascin-140/186 (Nfasc140/186), contactin-1 (CNTN1), and contactin-associated protein 1 (Caspr1) ... Read more >>

Neurol Neuroimmunol Neuroinflamm (Neurology(R) neuroimmunology & neuroinflammation)
[2020, 7(1):]

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Conduction block in immune-mediated neuropathy: paranodopathy versus axonopathy.

N Garg, S B Park, J Howells, S Vucic, S Vucic, C Yiannikas, E K Mathey, T Nguyen, Y Noto, M H Barnett, A V Krishnan, J Spies, H Bostock, J D Pollard, M C Kiernan,

BACKGROUND AND PURPOSE:Conduction block is a pathognomonic feature of immune-mediated neuropathies. The aim of this study was to advance understanding of pathophysiology and conduction block in chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). METHODS:A multimodal approach was used, incorporating clinical phenotyping, neurophysiology, immunohistochemistry and structural assessments. RESULTS:Of ... Read more >>

Eur. J. Neurol. (European journal of neurology)
[2019, 26(8):1121-1129]

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Peripheral nerve disease secondary to systemic conditions in children.

Jo M Wilmshurst, Robert A Ouvrier, Monique M Ryan,

This review is an overview of systemic conditions that can be associated with peripheral nervous system dysfunction. Children may present with neuropathic symptoms for which, unless considered, a causative systemic condition may not be recognized. Similarly, some systemic conditions may be complicated by comorbid peripheral neuropathies, surveillance for which is ... Read more >>

Ther Adv Neurol Disord (Therapeutic advances in neurological disorders)
[2019, 12:1756286419866367]

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Clinical evaluation of a 31-year-old woman with crural monomelic amyotrophy.

Austin R Thompson, Brooke Beckett, Erik R Ensrud,

This report describes the clinical presentation of a female patient diagnosed with crural MMA. Careful clinical correlation is necessary to distinguish crural MMA from other motor neuron diseases. When crural MMA is diagnosed, treatment options aim to alleviate symptoms. ... Read more >>

Clin Case Rep (Clinical case reports)
[2020, 8(1):68-70]

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Flexion-Induced Cervical Cord Compression: Hirayama Disease

Sena Tolu, Fikret Aysal, Tuğrul Örmeci, İbrahim Ethem Kirez, Nurbanu Hindioğlu,

Balkan Med J (Balkan medical journal)
[2019, 36(3):190-191]

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New family with HSPB8-associated autosomal dominant rimmed vacuolar myopathy.

Sejad Al-Tahan, Lan Weiss, Howard Yu, Sha Tang, Mario Saporta, Anna Vihola, Tahseen Mozaffar, Bjarne Udd, Virginia Kimonis,

Objective:We clinically and molecularly characterize a new family with autosomal dominant rimmed vacuolar myopathy (RVM) caused by mutations in the HSPB8 gene. Methods:We performed whole-exome and whole-genome sequencing in the family. Western blot and immunocytochemistry were used to analyze 3 patient fibroblasts, and findings were compared with their age- and ... Read more >>

Neurol Genet (Neurology. Genetics)
[2019, 5(4):e349]

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Guillain-Barré Syndrome and Posterior Reversible Encephalopathy Syndrome following Spinal Surgery.

Yui Sanpei, Akira Hanazono, Sachiko Kamada, Masashiro Sugawara,

Guillain-Barré syndrome (GBS) typically occurs after gastroenteritis and respiratory tract infection, but surgery has also been considered one of the triggers. Posterior reversible encephalopathy syndrome (PRES) is a rare complication of GBS. A normotensive female in her 70s presented ascending paralysis and frontal-parieto-occipital subcortical lesions with intermittent hypertension after spinal ... Read more >>

Case Rep Neurol (Case reports in neurology)
[2019, 11(3):284-289]

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