417 results match your criteria: "Multifocal Motor Neuropathy With Conduction Blocks"
Rev Med Inst Mex Seguro Soc
March 2024
Instituto Mexicano del Seguro Social. Centro Médico Nacional Siglo XXI, Hospital de Especialidades "Dr. Bernardo Sepúlveda", Servicio de Neurofisiología. Ciudad de México, México.
Background: Motor multifocal neuropathy is an immunemediated neuropathy characterized by progressive and asymmetric weakness of the distal extremities, without sensory symptoms, and an important feature of conduction blocks. The objective of this study is to comprehensively describe the nosological and pathogenic implications of this neurodegenerative disorder, given the unclear diagnosis of MMN and the significant challenges it poses.
Clinical Cases: We present three clinical cases with a chronic clinical presentation, in which neuroconduction studies were performed, revealing the presence of anti-IgM GM1 antibodies, consistent with the diagnosis of motor multifocal neuropathy.
Muscle Nerve
November 2024
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Introduction/aims: Multifocal motor neuropathy (MMN) is a rare disease for which epidemiological and clinical data are limited. We conducted a nationwide survey to determine disease prevalence, incidence, clinical profile, and current treatment status in Japan.
Methods: A nationwide survey was conducted in 2021 using an established epidemiological method.
Eur J Neurol
December 2024
Neuromuscular and Neuroimmunology Unit, IRCCS Humanitas Research Hospital, Milan, Italy.
Background And Purpose: This study was undertaken to compare the sensitivity and specificity of the 2010 European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria for multifocal motor neuropathy (MMN) with those of the American Association of Electrodiagnostic Medicine (AAEM).
Methods: Sensitivity and specificity of the two sets of criteria were retrospectively evaluated in 53 patients with MMN and 280 controls with axonal peripheral neuropathy, inflammatory demyelinating polyneuropathy, or amyotrophic lateral sclerosis. Comparison of the utility of nerve conduction studies with different numbers of nerves examined was also assessed.
Rinsho Shinkeigaku
September 2024
Department of Neurology, Shiga University of Medical Science.
A 26-year-old woman presented with a seven-month history of weakness in her left upper limb, progressing to difficulty lifting her arms within a few weeks. Her symptoms progressed with fluctuations. For the past three months, she has been unable to stand due to weakness in her proximal lower limbs.
View Article and Find Full Text PDFBrain Nerve
May 2024
Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.
Multifocal motor neuropathy (MMN), an acquired chronic progressive immune-mediated motor neuropathy, is characterized by asymmetrical distal upper limb muscle weakness and muscle atrophy without sensory impairment. Differentiation from amyotrophic lateral sclerosis is usually challenging, and electrophysiological studies show multifocal conduction blocks. Immunoglobulin (Ig)M GM1 antibodies are detected in approximately 50% of patients.
View Article and Find Full Text PDFMayo Clin Proc Innov Qual Outcomes
February 2024
Department of Neurology, Aarhus University Hospital, Aarhus, DK.
Multifocal motor neuropathy (MMN) is a rare immune-mediated motor neuropathy characterized by asymmetric weakness that preferentially affects distal upper limb muscles. The clinical features of MMN may be difficult to differentiate from motor neuron disease. Other conditions that may be mistaken for MMN include inclusion body myositis, chronic inflammatory demyelinating polyradiculoneuropathy, hereditary neuropathy with liability to pressure palsy, focal neuropathies, and radiculopathies.
View Article and Find Full Text PDFActa Neurol Taiwan
March 2024
Department of Neurology, Chang Gung Memorial Hospital, Keelung, 204, Taiwan; Collage of Medicine, Chang Gung University, Taoyuan, 333, Taiwan.
The typical presentation of multifocal motor neuropathy (MMN) is progressive asymmetric limb weakness. Cranial neuropathy is rare. We report a 28-year-old woman with cranial and bulbar palsies but with typical electrophysiological features of MMN by multifocal motor conduction blocks and serological markers of anti-ganglioside GM1 antibodies.
View Article and Find Full Text PDFTher Adv Neurol Disord
August 2023
Department of Neurology with Institute of Translational Neurology, University of Münster, Albert-Schweitzer-Campus 1, Building A1, Münster 48149, Germany.
Autoantibodies against contactin-associated protein 2 (CASPR2) are usually associated with autoimmune encephalitis and neuromyotonia. Their association with inflammatory neuropathies has been described in case reports albeit all with distal symmetric manifestation. Here, we report a patient who developed distal arm paresis, dominantly of the right arm, over the course of 1 year.
View Article and Find Full Text PDFInt J Mol Sci
May 2023
Department of Neurology, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland.
The spectrum of immune-mediated neuropathies is broad and the different subtypes are still being researched. With the numerous subtypes of immune-mediated neuropathies, establishing the appropriate diagnosis in normal clinical practice is challenging. The treatment of these disorders is also troublesome.
View Article and Find Full Text PDFInt J Mol Sci
April 2023
Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, Glasgow G51 4TF, UK.
Dysfunction of the immune system can result in damage of the peripheral nervous system. The immunological mechanisms, which include macrophage infiltration, inflammation and proliferation of Schwann cells, result in variable degrees of demyelination and axonal degeneration. Aetiology is diverse and, in some cases, may be precipitated by infection.
View Article and Find Full Text PDFRinsho Shinkeigaku
April 2023
Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.
Objective: To clarify the clinical and long-term characteristics of multifocal motor neuropathy (MMN).
Methods: We retrospectively evaluated data from 8 consecutive MMN patients in Yamaguchi University Hospital from 2005 to 2020. Clinical information including dominant hand, occupations, hobbies, nerve conduction data, protein level in cerebrospinal fluid (CSF), responsiveness to intravenous immunoglobulin (IVIg) therapy as initial therapy as well as maintenance therapy were collected.
Rev Neurol
March 2023
Hospital Británico, Buenos Aires, Argentina.
Introduction: Multifocal motor neuropathy (MMN) is a chronic progressive immune-mediated neuropathy, predominantly involving upper limbs asymmetrically with electrophysiologic evidence of motor conduction block. The treatment of choice is immunoglobulin (Ig). Nevertheless, some patients may become resistant to treatment.
View Article and Find Full Text PDFBrain Sci
December 2022
Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II of Naples, 80131 Naples, Italy.
Chronic dysimmune neuropathies encompass a group of neuropathies that share immune-mediated pathomechanism. Chronic dysimmune antibody-related neuropathies include anti-MAG neuropathy, multifocal motor neuropathy, and neuropathies related to immune attack against paranodal antigens. Such neuropathies exhibit distinguishing pathomechanism, clinical and response to therapy features with respect to chronic inflammatory demyelinating polyradiculoneuropathy and its variants, which represent the most frequent form of chronic dysimmune neuropathy.
View Article and Find Full Text PDFIdeggyogy Sz
November 2022
University of Pécs, Medical School, Department of Neurology, Pécs, Hungary.
Background And Purpose: Multifocal motor neuropathy (MMN) is a rare, immune-mediated illness attacking ex-clusively motor nerves. It is known that oxidative stress is present in peripheral neuropathies, but it has not been investigated MMN.
Methods: We measured in our prospective study the L-arginine, symmetric and asymmetric dimethylarginine (SDMA, ADMA) serum concentrations of 10 patients and 10 controls before and after intravenous immunoglobulin treatment (IVIG), as markers of the L-arginine/NO pathway involved in chronic inflammation and oxidative stress.
Brain Commun
November 2022
Institute of Infection, Immunity and Inflammation, University of Glasgow, Glasgow G12 8TA, UK.
The involvement of the complement pathway in Guillain-Barré syndrome pathogenesis has been demonstrated in both patient biosamples and animal models. One proposed mechanism is that anti-ganglioside antibodies mediate neural membrane injury through the activation of complement and the formation of membrane attack complex pores, thereby allowing the uncontrolled influx of ions, including calcium, intracellularly. Calcium influx activates the calcium-dependent protease calpain, leading to the cleavage of neural cytoskeletal and transmembrane proteins and contributing to subsequent functional failure.
View Article and Find Full Text PDFSkeletal Radiol
June 2023
Magnetic Resonance Department, Inscanner SL, C/San Pedro Poveda, 10. 03010, Alicante, Spain.
Lewis-Sumner syndrome (LSS) is an atypical variant of chronic inflammatory demyelinating polyneuropathy characterized by an asymmetric sensory-motor neuropathy with multifocal distribution. The diagnosis is typically clinical and electrophysiological but in some cases might be challenging causing a significant therapeutic delay. Diffusion tensor imaging (DTI) has been progressively used for the in vivo assessment of peripheral nerves integrity.
View Article and Find Full Text PDFNeurology
January 2023
From the Nerve-Muscle Unit (V.L., M.T., A.V.), Neurology Service, Department of Clinical Neurosciences, Lausanne University Hospital and University of Lausanne; and Dermatology Service (G.B., E.G.), Lausanne University Hospital and University of Lausanne, Switzerland.
A 39-year-old man presented with an asymmetric distal weakness and loss of sensitivity sequentially affecting both lower extremities and the left upper limb. Nerve conduction studies showed a multifocal sensory and motor axonal neuropathy, and a pseudo-conduction block of the right fibular nerve, the whole being consistent with a mononeuropathy multiplex. An uncommon etiology was found after an extensive workup.
View Article and Find Full Text PDFJ Peripher Nerv Syst
December 2022
Department of Neurology, School of Medicine at Ribeirão Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
The distal hereditary motor neuropathies (dHMN) encompass a group of peripheral nervous system disorders characterized by progressive distal predominant weakness and wasting, usually in a length-dependent pattern. The classical neurophysiological pattern is a motor axonal neuropathy with chronic distal denervation/reinnervation on needle examination. Conduction block (CB) and temporal dispersion (TD) are electrophysiological features classically associated with acquired demyelinating neuropathies.
View Article and Find Full Text PDFCureus
May 2022
Department of Neuroscience, University of the East Ramon Magsaysay Memorial Medical Center, Quezon City, PHL.
Multifocal motor neuropathy (MMN) is a progressive, multifocal weakness, which typically begins and predominates in the upper extremities with the absence of a sensory deficit and a hallmark electrophysiologic finding of conduction block. We describe a case of an adult male with MMN who developed both cranial nerve involvement and vocal cord paralysis. The patient presented with left shoulder weakness without sensory loss followed by hoarseness of voice and later developed tongue deviation and wasting of the left sternocleidomastoid and left trapezius muscle.
View Article and Find Full Text PDFBioengineering (Basel)
January 2022
S.C. Neurologia I, Dipartimento di Neuroscienze, Universitá di Torino, 10124 Torino, Italy.
A deconvolution method is proposed for conduction block (CB) estimation based on two compound muscle action potentials (CMAPs) elicited by stimulating a nerve proximal and distal to the region in which the block is suspected. It estimates the time delay distributions by CMAPs deconvolution, from which CB is computed. The slow afterwave (SAW) is included to describe the motor unit potential, as it gives an important contribution in case of the large temporal dispersion (TD) often found in patients.
View Article and Find Full Text PDFCochrane Database Syst Rev
January 2022
Department of Neurology, Amsterdam Neuroscience, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands.
Background: Multifocal motor neuropathy (MMN) is a rare, probably immune-mediated disorder characterised by slowly progressive, asymmetric, distal weakness of one or more limbs with no objective loss of sensation. It may cause prolonged periods of disability. Treatment options for MMN are few.
View Article and Find Full Text PDFJ Peripher Nerv Syst
March 2022
MRC Centre for Neuromuscular Diseases, National Hospital of Neurology and Neurosurgery, Queen Square, University College London Hospitals NHS Foundation Trust, London, UK.
Medicine (Baltimore)
November 2021
Department of Neurology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan.
Introduction: Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is an asymmetric immune-related neuropathy with conduction block. We report 2 MADSAM cases with detailed clinical, electrophysiological, and sonography profiles.
Patient Concerns And Diagnosis: Two cases presented with patchy sensorimotor impairment in both clinical and electrophysiological findings.