MMN: From Immunological Cross-Talk to Conduction Block

Harschnitz, Oliver; Jongbloed, Bas A.; Franssen, Hessel; Straver, Dirk C.G.; Pol, W. Ludo van der; Berg, Leonard H van den

doi:10.1007/s10875-014-0026-3

Cited by 27 publications

(22 citation statements)

References 75 publications

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“…Patients with MMN showed the most marked reduction in CNF parameters, indicative of axonal loss. Although the electrophysiological finding of conduction block in motor but not sensory nerves is a hallmark of MMN,42 slow progressive axonal degeneration has been shown in the majority of MMN patients 43. Sensory impairment during the course of MMN with conduction block has been described previously.…”

Section: Discussionmentioning

confidence: 93%

Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy

Stettner

Hinrichs

Guthoff

et al. 2015

Ann Clin Transl Neurol

105

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ObjectiveThere is an unmet need for better diagnostic tools to further delineate clinical subsets of heterogeneous chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) to facilitate treatment decisions. Corneal confocal microscopy (CCM) is a noninvasive and reproducible nerve imaging technique. This study evaluates the potential of CCM as a diagnostic surrogate in CIDP and MMN.MethodsIn a cross‐sectional prospective approach, 182 patients and healthy controls were studied using CCM to quantify corneal nerve damage and immune cell infiltration.ResultsPatients with CIDP and MMN had a reduction in corneal nerve fiber (CNF) measures and an increase in corneal immune cell infiltrates. In CIDP, CNF parameters decreased with increasing duration of disease. The number of dendritic cells in proximity to CNFs was increased in patients with early disease and correlated with the degree of motor affection. A further reduction in CNF parameters and an increase in nondendritic cells were observed in patients with painful neuropathy. In CIDP patients with antineuronal antibodies the number of nondendritic cells was increased.InterpretationOur findings suggest that CNF loss may reflect severity of neuropathy and quantification of distinct cells around the CNF plexus may help in stratifying CIDP subtypes, clinical course, and disease activity. However, further longitudinal studies are required before CCM can be considered as a valid surrogate endpoint for patients with CIDP and MMN.

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Section: Discussionmentioning

confidence: 93%

Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy

Stettner

Hinrichs

Guthoff

et al. 2015

Ann Clin Transl Neurol

105

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“…MMN is an immune-mediated, pure motor neuropathy characterized by slowly progressive, typically asymmetric weakness of the limbs. [65][66][67][68] The characteristic EDX criteria for MMN are conduction block outside common sites of entrapment and normal sensory studies. 26,65,66 Extensive NCSs are sometimes needed to identify the conduction block or other features of demyelination.…”

Section: Resultsmentioning

confidence: 99%

“…MMN is an immune‐mediated, pure motor neuropathy characterized by slowly progressive, typically asymmetric weakness of the limbs . The characteristic EDX criteria for MMN are conduction block outside common sites of entrapment and normal sensory studies .…”

Section: Sonographic Abnormalities In Polyneuropathiesmentioning

confidence: 99%

Nerve ultrasound in polyneuropathies

et al. 2018

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Ultrasound can be used to visualize pathology in the peripheral nerves of patients with polyneuropathy. Nerve enlargement is the most frequent pathology, but other abnormalities, including abnormal nerve echogenicity and vascularity, are also encountered. This monograph presents an overview of the role of nerve ultrasound in the evaluation and management of both inherited and acquired polyneuropathies. A description of the sonographic techniques and common abnormalities is provided, followed by a presentation of typical findings in different neuropathies. Scoring systems for characterizing the presence and pattern of nerve abnormalities as they relate to different polyneuropathies are presented. Muscle Nerve 57: 716-728, 2018.

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“…They also had high titers of anti-GM1 IgM antibodies, which are rare in patients with lower motor neuron disease. 7 Patient 4 had a more diffuse pattern that affected his shoulder, upper arm, and hand. Nevertheless, the weakness was markedly asymmetric not only by differential involvement of forearm extensor muscles, which was common to all patients, but also by the extension of motor unit potential loss on needle examination, which was more severe in FDI than in APB.…”

Section: Discussionmentioning

confidence: 99%

“…Patients 2 and 3 both had a pure distal asymmetric motor presentation as described by Katz and co‐workers as being characteristic of MMN. They also had high titers of anti‐GM1 IgM antibodies, which are rare in patients with lower motor neuron disease . Patient 4 had a more diffuse pattern that affected his shoulder, upper arm, and hand.…”

Section: Discussionmentioning

confidence: 99%