119 results match your criteria Multifocal Motor Neuropathy With Conduction Blocks


Multifocal motor neuropathy in Austria: a nationwide survey of clinical features and response to treatment.

J Neurol 2018 Dec 26;265(12):2834-2840. Epub 2018 Sep 26.

Department of Neurology, KH Wels-Grieskirchen, Wels, Austria.

Background And Objectives: Multifocal motor neuropathy (MMN) is a rare neuropathy and detailed descriptions of larger patient cohorts are scarce. The objective of this study was to evaluate epidemiological, clinical, and laboratory features of MMN patients and their response to treatment in Austria and to compare these data with those from the literature.

Methods: Anonymized demographic and clinical data about MMN patients until 31. Read More

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http://link.springer.com/10.1007/s00415-018-9071-9
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http://dx.doi.org/10.1007/s00415-018-9071-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6244652PMC
December 2018
7 Reads

Multifocal motor neuropathy presenting as a post-infectious complication of dengue: a CASE report.

BMC Infect Dis 2018 Aug 22;18(1):415. Epub 2018 Aug 22.

National Hospital of Sri Lanka, Colombo, Sri Lanka.

Background: Dengue infection is an endemic illness in the tropics and it is associated with a wide variety of post infectious complications. With the increasing prevalence of dengue infection in endemic regions, post-infectious neurological complications following dengue infection are now been reported more frequently. We report a patient who developed multifocal motor neuropathy (MMN) with conduction blocks following dengue infection during the immediate post-infectious period. Read More

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http://dx.doi.org/10.1186/s12879-018-3334-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6103970PMC
August 2018
4 Reads

A case of multifocal-motor-neuropathy-like disease with conduction blocks under infliximab with spontaneous progressive recovery.

Presse Med 2018 Mar 13;47(3):298-301. Epub 2018 Mar 13.

University Hospital of Pointe-à-Pitre, Rheumatology Unit, route de Chauvel, 97139 Abymes-Guadeloupe, France.

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http://dx.doi.org/10.1016/j.lpm.2017.10.021DOI Listing
March 2018
1 Read

Multifocal Motor Neuropathy Associated With Infliximab: A Case Report and a Literature Review.

Neurologist 2017 Jul;22(4):144-146

*Department of Neurology, Faculty of Medicine, Ondokuz Mayis University †Medicana Samsun Hospital, Rheumatology, Canik, Samsun, Turkey.

Multifocal motor neuropathy with conduction block (MMN-CB) is purely a motor neuropathy with progressive weakness that is characteristically caused by conduction blocks. Association with antiganglioside antibodies and a good response to immunomodulating therapies suggest an autoimmune etiology. In rare cases, MMN-CB has been reported as an adverse effect of infliximab, a tumor necrosis factor-α blocker. Read More

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http://dx.doi.org/10.1097/NRL.0000000000000132DOI Listing
July 2017
8 Reads

Normalization of sonographical multifocal nerve enlargements in a MADSAM patient following a good clinical response to intravenous immunoglobulin.

Neuromuscul Disord 2016 09 14;26(9):619-23. Epub 2016 Jun 14.

Department of Neurology, Tenri Hospital, Tenri, Japan.

Focal nerve enlargements at sites of conduction blocks can be visualized sonographically in patients with multifocal acquired demyelinating sensory and motor neuropathy (MADSAM). However, little is known about association between nerve morphological changes and treatment responses. Here we present a 73-year-old female MADSAM patient whose sonographical multifocal nerve enlargements normalized following a good treatment response. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09608966163009
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http://dx.doi.org/10.1016/j.nmd.2016.06.008DOI Listing
September 2016
6 Reads

[Multifocal motor neuropathy with conduction blocks].

Zh Nevrol Psikhiatr Im S S Korsakova 2016;116(6):70-72

Republican Clinical Hospital of Republic of Mari El, Yoshkar-Ola, Russia.

The article describes a case of multifocal motor neuropathy with conduction blocks in a female patient, aged 27 years. The development of the disease, results of neurological, laboratory, instrumental examinations, including electroneuromyography, and their role for the diagnosis and differential diagnosis are presented. Read More

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http://dx.doi.org/10.17116/jnevro20161166170-72DOI Listing
September 2018
1 Read

Differential weakness of finger extensor muscles: A clinical pattern of multifocal motor neuropathy.

Muscle Nerve 2017 03 30;55(3):433-437. Epub 2016 Nov 30.

Department of Neurology, Hospital Universitari Son Espases, Carretera de Valldemossa, 79, 07120 Palma de Mallorca, Balearic Islands, Spain.

Introduction: Several studies have suggested that differential weakness in muscles supplied by the same motor nerve supports the diagnosis of multifocal motor neuropathy (MMN).

Methods: We describe the clinical, electrophysiological, neuroimaging, and laboratory findings of patients with a lower motor syndrome whose clinical presentation included differential finger extension weakness that we have seen in our neuromuscular clinic.

Results: We identified 3 patients with hand weakness and 1 patient with asymmetric weakness of the upper extremity. Read More

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http://dx.doi.org/10.1002/mus.25247DOI Listing
March 2017
4 Reads

[Clinical and electrophysiological characteristics and therapeutic analysis of 9 patients with Lewis-Sumner syndrome].

Zhonghua Yi Xue Za Zhi 2016 Mar;96(11):859-62

Department of Neurology, Peking University Third Hospital, Beijing 100191, China.

Objective: To investigate the clinical characteristics, electrophysiological findings and treatment response of Lewis-Sumner syndrome (LSS).

Methods: Data of nine patients with LSS, who were diagnosed and treated from May 2008 to August 2014 in Department of Neurology, Peking University Third Hospital, were analyzed retrospectively, including clinical features, electrophysiological studies, pathological characteristics, therapy and follow up.

Results: The nine cases included seven males and two females, with the average age being 29 years old (18-64 years old). Read More

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http://dx.doi.org/10.3760/cma.j.issn.0376-2491.2016.11.007DOI Listing
March 2016
9 Reads

Nerve ultrasound in the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis with predominant lower motor neuron disease (ALS/LMND).

J Neurol 2016 Jan;263(1):35-44

The objective of the study was to investigate nerve ultrasound (US) in comparison to nerve conduction studies (NCS) for differential diagnosis of amyotrophic lateral sclerosis with predominant lower motoneuron disease(ALS/LMND) and multifocal motor neuropathy(MMN). A single-center, prospective, examiner-blinded cross-sectional diagnostic study in two cohorts was carried out. Cohort I: convenience sample of subjects diagnosed with ALS/LMND or MMN (minimal diagnostic criteria:possible ALS (revised EL-Escorial criteria), possible MMN (European Federation of Neurosciences guidelines). Read More

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http://dx.doi.org/10.1007/s00415-015-7927-9DOI Listing
January 2016
14 Reads

The pathogenesis of multifocal motor neuropathy and an update on current management options.

Ther Adv Neurol Disord 2015 May;8(3):109-22

National Referral Center for rare Neuromuscular Diseases, Institut Hospitalo-Universitaire de Neurosciences, University Hospital Pitié-Salpêtrière and University Pierre et Marie Curie (Paris VI), Paris, France.

Multifocal motor neuropathy (MMN) is a rare and disabling disease. Several experimental studies and clinical data are strongly suggestive of an immune-mediated pathogenesis, although underlying mechanisms in MMN seem to be very specific, mainly because of the presence of IgM anti-GM1 serum antibodies and the dramatic response to intravenous immunoglobulins (IVIg). The origin of antiganglioside antibodies and the way in which they act at the molecular level remain unclear. Read More

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http://dx.doi.org/10.1177/1756285615575269DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4409549PMC
May 2015
5 Reads

Immunosuppressant and immunomodulatory treatments for multifocal motor neuropathy.

Cochrane Database Syst Rev 2015 Mar 4(3):CD003217. Epub 2015 Mar 4.

Department of Neurology, National Neuroscience Institute, 11 Jalan Tan, Tock Seng, Singapore, Singapore, 308433.

Background: Multifocal motor neuropathy (MMN) is characterised by progressive, predominantly distal, asymmetrical limb weakness and usually multiple partial motor nerve conduction blocks. Intravenous immunoglobulin (IVIg) is beneficial but the role of immunosuppressive agents is uncertain. This is an update of a review first published in 2002 and previously updated in 2003, 2005, 2008 and 2011. Read More

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http://dx.doi.org/10.1002/14651858.CD003217.pub5DOI Listing
March 2015
11 Reads

Nerve ultrasound for differentiation between amyotrophic lateral sclerosis and multifocal motor neuropathy.

J Neurol 2015 28;262(4):870-80. Epub 2015 Jan 28.

Department of Neurology, Neuromuscular Center, Basel University Hospital, University Basel, Petersgraben 4, 4000, Basel, Switzerland,

Ultrasound is useful for non-invasive visualization of focal nerve pathologies probably resulting from demyelination, remyelination, edema or inflammation. In patients with progressive muscle weakness, differentiation between multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS) is essential regarding therapy and prognosis. Therefore, the objective of this study was to investigate whether nerve ultrasound can differentiate between ALS and MMN. Read More

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http://dx.doi.org/10.1007/s00415-015-7648-0DOI Listing
January 2016
9 Reads

Nerve ultrasound in a case of multifocal motor neuropathy without conduction block.

Muscle Nerve 2015 Aug 30;52(2):294-9. Epub 2015 Jun 30.

Department of Neurology, St. Josef Hospital, Ruhr-University of Bochum, Gudrunstr. 56, 44791, Bochum, Germany.

Introduction: Multifocal nerve enlargements and ultrastructural changes either corresponding or not to sites of existing conduction blocks have been described in demyelinating polyneuropathies using multiple imaging techniques.

Methods: Using the emerging technique of peripheral nerve ultrasonography we investigated the peripheral nerves of a patient with multifocal motor neuropathy (MMN) without conduction block.

Results: In this case of MMN without conduction blocks we found multifocal nerve enlargements in the ultrasonography and electrodiagnostic signs of acute and chronic denervation associated with positive anti-GM1 IgM antibodies. Read More

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http://dx.doi.org/10.1002/mus.24583DOI Listing
August 2015
4 Reads

Multifocal-motor-neuropathy-like disease associated with Infliximab treatment in a patient with Crohn's disease.

J Neurol Sci 2015 Feb 9;349(1-2):246-8. Epub 2015 Jan 9.

Department of Neurophysiology, University Hospital of León, León, Spain.

Multifocal motor neuropathy is an immune-mediated disorder characterized by motor-conduction block in nerve-conduction studies. It is recognized that anti-TNF-α therapies are associated with immune-mediated conditions as adverse events. We report a case of multifocal-motor-neuropathy-like disease associated with the use of Infliximab in a patient with Crohn's disease. Read More

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http://dx.doi.org/10.1016/j.jns.2015.01.003DOI Listing
February 2015
2 Reads

Diagnosis and treatment of chronic acquired demyelinating polyneuropathies.

Authors:
Norman Latov

Nat Rev Neurol 2014 Aug 1;10(8):435-46. Epub 2014 Jul 1.

Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 1305 York Avenue, Suite 217, New York, NY 10021, USA.

Chronic neuropathies are operationally classified as primarily demyelinating or axonal, on the basis of electrodiagnostic or pathological criteria. Demyelinating neuropathies are further classified as hereditary or acquired-this distinction is important, because the acquired neuropathies are immune-mediated and, thus, amenable to treatment. The acquired chronic demyelinating neuropathies include chronic inflammatory demyelinating polyneuropathy (CIDP), neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein (MAG; anti-MAG neuropathy), multifocal motor neuropathy (MMN), and POEMS syndrome. Read More

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http://www.nature.com/articles/nrneurol.2014.117
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http://dx.doi.org/10.1038/nrneurol.2014.117DOI Listing
August 2014
6 Reads

Multifocal motor neuropathy with high titers of anti-MAG antibodies.

J Peripher Nerv Syst 2014 Jun;19(2):180-2

Department of Clinical Neurosciences, Division of Neurology, Geneva University Hospital, Geneva, Switzerland.

Multifocal motor neuropathy (MMN) and anti-myelin-associated glycoprotein (anti-MAG)-associated neuropathy are clinically and electrophysiologically distinct entities. We describe a patient with characteristic features of both neuropathies, raising the possibility of an overlap syndrome. A 49-year-old patient reported a history of slowly progressive predominantly distal tetraparesis, with mild sensory deficits. Read More

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http://dx.doi.org/10.1111/jns5.12069DOI Listing
June 2014
41 Reads

Treatment of multifocal motor neuropathy with intravenous immunoglobulin.

Authors:
Carol Lee Koski

J Clin Immunol 2014 Jul 5;34 Suppl 1:S127-31. Epub 2014 Apr 5.

GBS/CIDP Foundation International, 104 ½ Forrest Avenue, Narberth, PA, 19072, USA,

Multifocal motor neuropathy (MMN) is a rare inflammatory, chronically progressive, unremitting disorder affecting the peripheral nervous system. Although the etiology of this condition is not known, high titers of IgM Ab to GM1 may serve as a biomarker for this disease. Clinical findings of motor weakness are associated with focal conduction blocks and with time, axonal destruction. Read More

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http://dx.doi.org/10.1007/s10875-014-0016-5DOI Listing
July 2014
7 Reads

Multifocal motor neuropathy.

Curr Opin Neurol 2013 Oct;26(5):503-9

National Referral Center for Rare Neuromuscular Diseases, Institut Hospitalo-Universitaire de Neurosciences, University Hospital Pitié-Salpêtrière, University Pierre et Marie Curie, Paris VI, Paris, France.

Purpose Of Review: Multifocal motor neuropathy (MMN) remains a difficult issue for neurologists, as its clinical and electrophysiological presentation may be atypical, and because no alternative treatment to periodic immunoglobulin infusions has been assessed in its long-term management. This review intends to summarize the most recent advances in the diagnosis and treatment of MMN.

Recent Findings: Recent reports have focused on atypical onset and unusual clinical presentation. Read More

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http://dx.doi.org/10.1097/WCO.0b013e328364cacaDOI Listing
October 2013
2 Reads

Multifocal motor neuropathy.

Handb Clin Neurol 2013 ;115:429-42

Department of Neurology, Wayne State University School of Medicine, Detroit, Michigan, USA.

Multifocal motor neuropathy (MMN) is a rare disorder in which the symptoms are caused by persistent conduction block lesions. The mononeuropathy multiplex progresses over time with increasing axonal loss. The cause of the conduction blocks and axonal loss are not completely understood but immune mechanisms are involved and response to intravenous immunoglobulin has been established. Read More

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http://dx.doi.org/10.1016/B978-0-444-52902-2.00024-2DOI Listing
April 2014
8 Reads

Multifocal motor neuropathy.

Presse Med 2013 Jun 25;42(6 Pt 2):e217-24. Epub 2013 Apr 25.

Hôpital de la Salpêtrière, Referral Center for Rare Neuromuscular Diseases, Bâtiment Babinski, Paris, France.

Multifocal motor neuropathy (MMN) is a chronic immune-mediated neuropathy that is particular for its asymmetric, multifocal, purely motor clinical presentation, often related to the distribution of individual nerves. Upper limbs are usually primarily and more severely affected, but lower limbs may be involved during the course of the disease. The hallmark of the disease is the presence, in electrophysiological studies, of persistent conduction blocks in the affected motor nerves, located outside the usual sites of nerve compression, contrasting with normal sensory nerve conduction velocities. Read More

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http://dx.doi.org/10.1016/j.lpm.2013.01.057DOI Listing
June 2013
2 Reads

Multifocal radiculoneuropathy during ipilimumab treatment of melanoma.

Muscle Nerve 2013 Sep 27;48(3):440-4. Epub 2013 Jul 27.

Department of Neurology Washington University School of Medicine, 600 South Euclid Avenue, St. Louis, Missouri, 63110, USA.

Introduction: Ipilimumab, a monoclonal anti-CTLA-4 antibody, is used to treat melanoma. Neuromuscular side effects, possibly autoimmune, may occur.

Methods: In this investigation we undertook a retrospective review of patient records. Read More

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http://dx.doi.org/10.1002/mus.23830DOI Listing
September 2013
3 Reads

Cranial nerve involvement as presenting sign of multifocal motor neuropathy.

J Clin Neurosci 2012 Dec 20;19(12):1733-5. Epub 2012 Sep 20.

Department of Neurosciences, Via P. Giardini, 1350, University of Modena and Reggio Emilia, 41010 Modena, Italy.

Multifocal motor neuropathy (MMN) is characterized by slowly progressive, predominantly distal, asymmetric limb weakness and partial conduction blocks (CB) of motor axons. Cranial nerve involvement and respiratory failure are uncommon. We report two patients who exhibited unilateral hypoglossal and abducens palsy as presenting signs. Read More

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http://dx.doi.org/10.1016/j.jocn.2011.12.030DOI Listing
December 2012
5 Reads

Immunosuppressant and immunomodulatory treatments for multifocal motor neuropathy.

Cochrane Database Syst Rev 2012 Apr 18(4):CD003217. Epub 2012 Apr 18.

Department of Neurology, National Neuroscience Institute, Singapore,

Background: Multifocal motor neuropathy is characterised by progressive, predominantly distal, asymmetrical limb weakness and usually multiple partial motor nerve conduction blocks. Intravenous immunoglobulin is beneficial but the role of immunosuppressive agents is uncertain. This is an update of a review first published in 2002 and previously updated in 2003, 2005 and 2008. Read More

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http://dx.doi.org/10.1002/14651858.CD003217.pub4DOI Listing
April 2012
10 Reads

Ultrasonography of MADSAM neuropathy: focal nerve enlargements at sites of existing and resolved conduction blocks.

Neuromuscul Disord 2012 Jul 16;22(7):627-31. Epub 2012 Apr 16.

Dept. of Neurology, Semmelweis University, Budapest, Hungary.

Using the emerging technique of peripheral nerve ultrasonography, multiple focal nerve swellings corresponding to sites of existing conduction blocks have been described in demyelinating polyneuropathies. We report two cases of multifocal acquired demyelinating sensory and motor neuropathy (MADSAM). In the first, multiple focal nerve enlargements were detected by ultrasound at sites of previous conduction blocks, well after complete clinical and electrophysiological resolution. Read More

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http://dx.doi.org/10.1016/j.nmd.2012.03.005DOI Listing
July 2012
6 Reads

[Multifocal motor neuropathy without conduction blocks and antibodies to GM1 gangliosides].

Zh Nevrol Psikhiatr Im S S Korsakova 2011 ;111(8 Pt 1):69-74

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December 2011

[Improvement of severe and intravenous immunoglobulin-dependent multifocal motor neuropathy with conduction block after long-term rituximab].

Rev Neurol (Paris) 2011 Dec 12;167(12):916-20. Epub 2011 Jul 12.

Centre de référence des maladies neuromusculaires et SLA, hôpital de l'Archet, 1, route Saint-Antoine-de-Ginestière, BP 3979, 06202 Nice, cedex 3, France.

Introduction: Some patients suffering from multifocal motor neuropathy with conduction blocks (MMNCB) are still disabled after treatment with intravenous immunoglobulin (IVIg).

Case Report: We report the benefits of a combination of rituximab (RTX) and IVIg in the case of a 72-year-old man with MMNCB.

Discussion: Despite an IVIg treatment, the patient had severe motor weakness of the four limbs which limited daily living activity. Read More

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http://linkinghub.elsevier.com/retrieve/pii/S003537871100234
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http://dx.doi.org/10.1016/j.neurol.2011.02.042DOI Listing
December 2011
5 Reads

[Autoimmune neuropathies: diagnosis, treatment, and recent topics].

Brain Nerve 2011 Jun;63(6):549-55

Department of Neurology, Kinki University School of Medicine, Osaka, Japan.

Here, we have reviewed the clinical patterns, diagnostic paradigms, etiopathogenesis, and therapeutic strategies of autoimmune neuropathies such as Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and IgM paraproteinemic neuropathy. Antiganglioside antibodies are frequently present in the serum samples obtained during the acutephase of GBS and Miller Fisher syndrome (MFS), a subtype of GBS. Recently, we found that some patients with GBS and MFS have serum antibodies against antigenic epitopes formed by 2 different gangliosides (ganglioside complex). Read More

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June 2011
5 Reads

[Axonopathy in the pathogenesis of multiple sclerosis, peripheral diffuse and local motor neuropathies and motor neuron disease].

Zh Nevrol Psikhiatr Im S S Korsakova 2010 ;110(8):4-7

Two hundreds and seventy-six patients including 43 patients with multiple sclerosis, 24 - with acute inflammatory demyelinating polyneuropathy (AIDP), 144 - with chronic inflammatory demyelinating polyneuropathy (CIDP), 27 - with motor multifocal neuropathy (MMN), 38 - with lateral amyotrophic sclerosis (LAS) have been examined. Symptoms of axonal degeneration, manifested in denervation phenomena in both clinical and instrumental studies (electromyography, transcranial magnetic stimulation, MRT), were revealed in all groups of patients. The formation of excitation conduction blocks is an universal pathophysiological mechanism of the axonopathy development in AIDP, CIDP, MMN and LAS. Read More

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December 2010
1 Read

Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy.

Neurology 2010 Aug;75(9):818-25

Department of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht100, 3584 CX Utrecht, the Netherlands.

Objective: Identification and examination of all patients with multifocal motor neuropathy (MMN) in the Netherlands to document the clinical spectrum and response to IV immunoglobulin (IVIg) and to determine correlates of outcome.

Methods: A national cross-sectional descriptive study was performed. Ninety-seven patients were identified; 88 participated. Read More

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http://www.neurology.org/cgi/doi/10.1212/WNL.0b013e3181f0738
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http://dx.doi.org/10.1212/WNL.0b013e3181f0738eDOI Listing
August 2010
3 Reads

IVIG blocks complement deposition mediated by anti-GM1 antibodies in multifocal motor neuropathy.

J Neurol Neurosurg Psychiatry 2011 Jan 28;82(1):87-91. Epub 2010 Jul 28.

Department of Neurology, Niigata National Hospital, Niigata, Japan.

Background: The pathogenesis of multifocal motor neuropathy (MMN) has yet to be established. MMN patients often carry anti-GM1 IgM antibodies, suggesting an autoimmune process involving complement. Intravenous immunoglobulin (IVIG) is the first line treatment, but its action mechanism is unknown. Read More

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http://dx.doi.org/10.1136/jnnp.2010.205856DOI Listing
January 2011
2 Reads

A case of Lewis-Sumner syndrome showing dramatic improvement after plasma exchange.

J Korean Med Sci 2010 Jul 16;25(7):1101-4. Epub 2010 Jun 16.

Department of Neurology, Pusan National University School of Medicine, Busan, Korea.

We report a patient with Lewis-Sumner syndrome (LSS) who showed an improvement only with plasma exchange (PE). The patient, 32-yr old man, had progressive multifocal motor-sensory deficits with persistent, multiple conduction blocks and marked slowing of NCVs. Nerve pathology supported a diagnosis of demyelinating neuropathy by revealing marked loss of myelinated fibers with inter- and intrafascicular variation. Read More

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https://synapse.koreamed.org/DOIx.php?id=10.3346/jkms.2010.2
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http://dx.doi.org/10.3346/jkms.2010.25.7.1101DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2890894PMC
July 2010
5 Reads

Multifocal motor neuropathy: update on clinical characteristics, pathophysiological concepts and therapeutic options.

Eur Neurol 2010 11;63(4):193-204. Epub 2010 Feb 11.

Department of Neurology, University of Wuerzburg, Wuerzburg, Germany.

Multifocal motor neuropathy (MMN) is an acquired immune-mediated neuropathy characterized by chronic or stepwise progressive asymmetrical limb weakness without sensory deficits. The upper extremities are more often affected than the lower extremities with distal paresis dominating over proximal paresis. Important diagnostic features are persistent multifocal partial conduction blocks (CBs) and the presence of high-titer anti-GM1 serum antibodies. Read More

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http://dx.doi.org/10.1159/000282734DOI Listing
July 2010
5 Reads

[Differential diagnosis of multifocal motor neuropathy and related diseases].

Klin Med (Mosk) 2009 ;87(9):54-7

Multifocal motor neuropathy (MMN) is a rare disease of the peripheral nervous system pathogenetically related to local demyelinization and formation of excitation conduction blocks. MMN affect only those nerves and their segments that comprise excitation conduction blocks. Such blocks have a persistent character and show a mosaic pattern over motor fibres which accounts for the specific clinical picture of MMN. Read More

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December 2009
2 Reads

Do patients having a decrease in SNAP amplitude during the course of MMN present with a different condition?

J Neurol 2009 Nov 30;256(11):1876-80. Epub 2009 Jul 30.

Centre de référence pour Maladies NeuroMusculaires et SLA, Hôpital Archet 1, CHU de Nice, route saint Antoine de Ginestière, BP3079, 06202 Nice Cedex 3, France.

A decrease in sensory nerve action potentials (SNAP) amplitude has been recently reported in some patients during the course of multifocal motor neuropathy with conduction blocks (MMNCB). It is not known if those patients have different clinical expression and disability when compared with typical MMNCB. Clinical, biological and electrophysiological assessments were performed in 15 patients fitting the diagnosis criteria of MMNCB, including normal SNAP amplitude at initial examination. Read More

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http://link.springer.com/10.1007/s00415-009-5217-0
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http://dx.doi.org/10.1007/s00415-009-5217-0DOI Listing
November 2009
6 Reads

A case of spontaneously recovering multifocal motor neuropathy with conduction blocks (MMNCB) during anti-TNF alpha therapy for ankylosing spondylitis.

Clin Rheumatol 2009 Aug 7;28(8):993-5. Epub 2009 Apr 7.

Department of Rheumatology, S. Chiara Hospital, Trento, Italy.

We report the case of a 54-year-old patient affected by ankylosing spondylitis who developed multifocal motor neuropathy with conduction blocks after 8 months of infliximab treatment. TNF alpha antagonist therapy has been associated with the development of both central nervous system and peripheral nervous system disorders, mainly of the demyelinating type. To our knowledge, this seems to be the second reported case of infliximab-related typical multifocal motor neuropathy with conduction blocks in which no other underlying causes of neuropathy were present. Read More

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http://dx.doi.org/10.1007/s10067-009-1174-3DOI Listing
August 2009
2 Reads

Immunosuppressant and immunomodulatory treatments for multifocal motor neuropathy.

Cochrane Database Syst Rev 2009 Jan 21(1):CD003217. Epub 2009 Jan 21.

Department of Neurology, National Neuroscience Institute, 11 Jalan Tan, Tock Seng, Singapore, Singapore, 308433.

Background: Multifocal motor neuropathy is characterised by progressive, predominantly distal, asymmetrical limb weakness and usually multiple partial motor nerve conduction blocks. Intravenous immunoglobulin is beneficial but the role of immunosuppressive agents is uncertain.

Objectives: To provide the best available evidence from randomised controlled trials on the role of immunosuppressive agents for the treatment of multifocal motor neuropathy. Read More

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http://dx.doi.org/10.1002/14651858.CD003217.pub3DOI Listing
January 2009
3 Reads

Lewis-sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features.

Muscle Nerve 2009 Feb;39(2):206-20

Neuromuscular Clinic, Department of Neurology, University Hospitals of Leicester, Leicester LE5 4PW, UK.

Lewis-Sumner syndrome (L-SS) represents the asymmetric variant of chronic inflammatory demyelinating polyneuropathy (CIDP). The characteristics and specificities of L-SS of pure upper-limb onset, as initially described by Lewis et al. [Multifocal demyelinating neuropathy with persistent conduction block. Read More

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http://dx.doi.org/10.1002/mus.21199DOI Listing
February 2009
14 Reads

Autoimmune-mediated polyneuropathy triggered by borrelial infection?

Muscle Nerve 2008 Jun;37(6):781-5

Department of Neurology, Ludwig-Maximilians University, Marchioninistr. 15, 81377 Munich, Germany.

A patient with proven borrelial infection of the central nervous system (CNS) and progressive weakness of the arms was treated with antibiotics. Although the initially elevated CXCL13 concentration in the cerebrospinal fluid decreased, indicating effective treatment of the infection, weakness progressed. Investigation revealed multiple nerve conduction blocks and the presence of GM1 antibodies, suggesting a multifocal motor neuropathy; the patient improved on treatment with intravenous immunoglobulins. Read More

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http://dx.doi.org/10.1002/mus.20929DOI Listing
June 2008
2 Reads

[Nerve conduction blocks and peripheral neuropathies].

J Soc Biol 2006 ;200(4):307-21

Service de Physiologie-Explorations Fonctionnelles, Hôpital Henri Mondor, Créteil Cedex, France.

A motor nerve conduction block is defined as a reduction of either amplitude or area of the compound motor action potential elicited by proximal vs. distal motor nerve stimulation. The pathophysiological mechanisms leading to a figure of conduction block include segmental demyelination, recent axonal interruption, or various axonal excitability abnormalities due to ion channel dysfunction or membrane potential changes. Read More

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September 2007
2 Reads

[Inflammatory demyelinating neuropathies: classification, evolution and prognosis].

J Soc Biol 2006 ;200(4):301-6

Service de Neurologie, Hôpital Henri Mondor, Créteil Cedex, France.

Inflammatory demyelinating neuropathies can be classified according to the topography of the nervous lesion. Acute and chronic polyradiculoneuritis are characterized by diffuse and multifocal, but predominantly proximal lesions, multifocal motor and sensory-motor neuropathies with persistent conduction blocks are restricted to some nerve trunks, while neuropathies due to monoclonal IgM with anti-MAG (Myelin Associated Glycoprotein) activity show distal and symmetric distribution. The clinical characteristics of inflammatory demyelinating neuropathies vary according to the type of neuropathy. Read More

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September 2007
9 Reads

[Long-term follow-up of multifocal motor neuropathy with conduction block under intravenous immunoglobulin].

Rev Neurol (Paris) 2007 Jan;163(1):82-8

Service de Neurologie, CHU de Nice.

Introduction: Multifocal motor neuropathy with conduction block is an immune-mediated motor neuropathy, which usually responds to intravenous immunoglobulin. However, efficacy of long-term intravenous immunoglobulin is controversial. Our aim was to establish the long-term effects of intravenous immunoglobulin therapy on clinical and neurophysiological outcome measures and to determine the criteria predicting a good response to long-term intravenous immunoglobulin treatment. Read More

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January 2007
3 Reads

Triple-stimulation technique in multifocal neuropathy with conduction block.

Muscle Nerve 2007 May;35(5):632-6

Department of Neurology and Neuromuscular Diseases, CHU La Timone, 264 rue Saint-Pierre, 13385 Marseilles, France.

In patients with multifocal neuropathy with conduction block (CB), CBs located between the root and Erb's point are not detected in nerve conduction studies. We therefore examined whether the triple-stimulation technique (TST) might provide a useful means of detecting CB proximal to Erb's point. Clinical assessments, extensive nerve conduction studies (NCS), conventional transcranial magnetic stimulation, and TST were performed on 10 patients with multifocal motor neuropathy with CB (MMNCB) and 6 patients with Lewis-Sumner syndrome. Read More

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http://dx.doi.org/10.1002/mus.20742DOI Listing
May 2007
3 Reads

[Multifocal motor neuropathies with conduction block: long-term follow-up of ten patients treated with IVIg].

Authors:
D Cros K Drake

Rev Neurol (Paris) 2006 Jun;162 Spec No 1:3S46-3S50

Massachusetts General Hospital, Boston, USA.

Unlabelled: Multifocal motor neuropathy (MMN) with conduction block responds to high-dose i.v. polyvalent immunoglobulins (IVIg) over the short term, but several studies have demonstrated a long-term increase in the degree of axonal degeneration and the number of conduction blocks, factors indicating a poor prognosis. Read More

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June 2006
3 Reads

Nerve conduction studies in polyneuropathy: practical physiology and patterns of abnormality.

Acta Neurol Belg 2006 Jun;106(2):73-81

Department of Neurology, Neuromuscular Research Group, Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, The Netherlands.

Nerve conduction studies are an essential part of the work-up of peripheral neuropathies. Many neuropathic syndromes can be suspected on clinical grounds, but optimal use of nerve conduction study techniques (in combination with needle electromyography) allows diagnostic classification and is therefore crucial to understanding and separation of neuropathies. Multifocal motor neuropathy, for example, may clinically present as ALS. Read More

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http://www.actaneurologica.be/acta/download/2006-2/05-Franse
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June 2006
5 Reads

[Multifocal motor neuropathy with persistent conduction blocks. A chronic immune-mediated neuropathy].

Presse Med 2006 Apr;35(4 Pt 2):720-7

Centre de Référence Maladies Neuromusculaires Rares, CHU Pitié-Salpêtrière, Paris.

Multifocal motor neuropathy with persistent conduction blocks was first specifically identified in 1986. Its major criterion is conduction blocks in motor nerves only. Clinically, this is a multifocal, thus asymmetric, neuropathy that begins and predominant touches upper limbs; it especially affects men after the age of 50 years and has a chronic course with relapses. Read More

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April 2006
3 Reads

Childhood-onset multifocal motor neuropathy with conduction blocks.

Neurology 2006 Mar;66(6):922-4

Department of Child Neurology, National Neurological Institute C. Besta, Milan, Italy.

Multifocal motor neuropathy (MMN) is an acquired disorder with onset in adulthood. The authors describe a patient with a slowly progressing distal upper limb motor neuropathy since age 6 years, in whom definite conduction blocks in upper limbs, outside common entrapment sites, and no sensory involvement were consistent with MMN. IV immunoglobulin treatment produced marked muscle strength improvement and conduction block disappearance. Read More

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http://www.neurology.org/cgi/doi/10.1212/01.wnl.0000203346.2
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http://dx.doi.org/10.1212/01.wnl.0000203346.25068.3fDOI Listing
March 2006
2 Reads

Prediction of response to IVIg treatment in patients with lower motor neurone disorders.

Eur J Neurol 2006 Feb;13(2):135-40

Friedrich-Baur-Institute, Munich, Germany.

The distinction between multifocal motor neuropathy, treatable by intravenous immunoglobulins (IVIg), and degenerative motor neurone disorders is often difficult. To find predictive factors for the response to IVIg treatment, 40 consecutive patients with pure lower motor neurone disorders (LMND) were prospectively examined. They all received at least two times IVIg (2 g/kg bodyweight). Read More

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http://doi.wiley.com/10.1111/j.1468-1331.2006.01142.x
Publisher Site
http://dx.doi.org/10.1111/j.1468-1331.2006.01142.xDOI Listing
February 2006
2 Reads

Multifocal motor neuropathy.

Curr Opin Neurol 2005 Oct;18(5):567-73

consutation de Pathologie Neuro-Musculaire, Babinski Building, Salpêtrière Hospital, Paris, France.

Purpose Of This Review: To conduct a critical review of recent studies on the clinical and therapeutic aspects of multifocal motor neuropathy, and to analyse their implications for patient management.

Recent Findings: Recent studies have contributed to defining the specific position of multifocal motor neuropathy within the spectrum of chronic immune-mediated polyneuropathies. One study compared features of this condition with multifocal acquired demyelinating sensory and motor neuropathy, while others have focused on pathological alterations at the site of conduction blocks. Read More

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October 2005
2 Reads