Biomaterial Database

Multifocal motor neuropathy. 2018

Katie Beadon, and Raquel Guimarães-Costa, and Jean-Marc Léger

National Referral Center for Neuromuscular Diseases, Institut Hospitalo-Universitaire (IHU) de Neurosciences, University Hospital Pitié Salpêtrière, Paris, France.

Multifocal motor neuropathy (MMN) has specific clinical and electrophysiologic features but can be difficult to diagnose if cases are not typical. Intravenous immunoglobulin (IVIg) remains the core initial and long-term treatment. In this review, recent advances in the diagnosis, monitoring and treatment of MMN are discussed. The pathology of MMN likely depends on immune-mediated attack of the nodes of Ranvier and paranodal regions leading to conduction block. Antiganglioside antibodies are present in over 50% of patients. The sensitivity of antibody detection can be improved by testing for GM1/galactocerebroside (GM1/GalC) complexes. Complement activation plays a key role in the pathophysiology of MMN. Subcutaneous immunoglobulins are an efficacious alternative to IVIg for maintenance therapy in MMN. Complement inhibitor eculizumab may be a potential future treatment, but further studies are necessary. The European Federation of Neurological Societies (EFNS)/Peripheral Nerve Society (PNS) guidelines for the diagnosis of MMN are currently widely used but probably need revision. Nerve ultrasound and plexus/nerve MRI can be helpful in diagnostic dilemmas. Monitoring of disease and response to treatment may improve using disease-specific evaluation scales such as MMN-Rasch-built overall disability scale. Further research into the pathophysiology of MMN is necessary to direct future treatment strategies.

UI	MeSH Term	Description	Entries
D007155	Immunologic Factors	Biologically active substances whose activities affect or play a role in the functioning of the immune system.	Biological Response Modifier,Biomodulator,Immune Factor,Immunological Factor,Immunomodulator,Immunomodulators,Biological Response Modifiers,Biomodulators,Factors, Immunologic,Immune Factors,Immunological Factors,Modifiers, Biological Response,Response Modifiers, Biological,Factor, Immune,Factor, Immunological,Factors, Immune,Factors, Immunological,Modifier, Biological Response,Response Modifier, Biological
D007166	Immunosuppressive Agents	Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging.	Immunosuppressant,Immunosuppressive Agent,Immunosuppressants,Agent, Immunosuppressive,Agents, Immunosuppressive
D011115	Polyneuropathies	Diseases of multiple peripheral nerves simultaneously. Polyneuropathies usually are characterized by symmetrical, bilateral distal motor and sensory impairment with a graded increase in severity distally. The pathological processes affecting peripheral nerves include degeneration of the axon, myelin or both. The various forms of polyneuropathy are categorized by the type of nerve affected (e.g., sensory, motor, or autonomic), by the distribution of nerve injury (e.g., distal vs. proximal), by nerve component primarily affected (e.g., demyelinating vs. axonal), by etiology, or by pattern of inheritance.	Polyneuropathy, Acquired,Polyneuropathy, Critical Illness,Polyneuropathy, Familial,Polyneuropathy, Inherited,Polyneuropathy, Motor,Acquired Polyneuropathies,Acquired Polyneuropathy,Critical Illness Polyneuropathies,Critical Illness Polyneuropathy,Familial Polyneuropathies,Familial Polyneuropathy,Inherited Polyneuropathies,Inherited Polyneuropathy,Motor Polyneuropathies,Motor Polyneuropathy,Polyneuropathies, Acquired,Polyneuropathies, Critical Illness,Polyneuropathies, Familial,Polyneuropathies, Inherited,Polyneuropathies, Motor,Polyneuropathy
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D016472	Motor Neuron Disease	Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)	Anterior Horn Cell Disease,Familial Motor Neuron Disease,Lateral Sclerosis,Motor Neuron Disease, Lower,Motor Neuron Disease, Upper,Lower Motor Neuron Disease,Motor Neuron Disease, Familial,Motor Neuron Disease, Secondary,Motor System Disease,Primary Lateral Sclerosis,Secondary Motor Neuron Disease,Upper Motor Neuron Disease,Lateral Scleroses,Lateral Scleroses, Primary,Lateral Sclerosis, Primary,Motor Neuron Diseases,Motor System Diseases,Neuron Disease, Motor,Neuron Diseases, Motor,Primary Lateral Scleroses,Scleroses, Lateral,Scleroses, Primary Lateral,Sclerosis, Lateral,Sclerosis, Primary Lateral