Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin.

Donaghy, Michael; Mills, Kerry; Boniface, Simon; Simmons, Jonathan; Wright, Ingle; Gregson, N. A.; Jacobs, Jean M.

doi:10.1136/jnnp.57.7.778

Cited by 213 publications

(128 citation statements)

References 20 publications

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“…The European Federation of Neurological Societies/Peripheral Nerve Society criteria for diagnosing CIDP are based primarily on motor dysfunction 49. The good response of this entity to IVIg50 suggests that these patients may have pronounced inflammatory activity in this subgroup. Therefore of relevance we have shown that patients with motor affection had increased cell counts, particularly dendritic cells without fiber contacts.…”

Section: Discussionmentioning

confidence: 99%

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: 99%

Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy

Stettner

Hinrichs

Guthoff

et al. 2015

Ann Clin Transl Neurol

105

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“…Later, the association of this condition with high titres of IgM autoantibodies directed against GM1 ganglioside (Pestronk et al, 1988) and the description of numerous similar cases resulted in this being considered as a separate entity (Nobile-Orazio, 2001). Clarifying the position of LSS among these chronic immune-mediated neuropathies is of therapeutic relevance (Dalakas, 1999), since CIDP shows a beneficial response to plasma exchanges, intravenous immuno globulin (IVIg) and steroids (Hughes et al, 2001;Van Doorn and Garssen, 2002), whereas MMN responds to IVIg (Leger et al, 2001), but not to steroids or plasma exchanges (Pestronk et al, 1988;Donaghy et al, 1994).…”

Section: Introductionmentioning

confidence: 99%

Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome

Viala¹

2004

Brain

183

140

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SummaryLewis-Sumner syndrome (LSS) is a dysimmune peripheral nerve disorder, characterized by a predominantly distal, asymmetric weakness mostly affecting the upper limbs with sensory impairment, and by the presence of multifocal persistent conduction blocks. The nosological position of this neuropathy in relation to multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is still debated. We report the clinical, biological and electrophysiological features, the course and the response to treatment in 23 LSS patients. The initial symptoms started in the distal part of an upper limb in 70% of patients. They were sensorimotor in 65% and purely sensory in 35% of patients. A cranial nerve involvement was observed in 26% of patients and a distal limb amyotrophy in 52%. The CSF protein level was normal in 67% of patients and mildly elevated in the remainder. None had serum anti-GM1 antibodies. There were multiple motor conduction blocks (average of 2.87/patient), predominantly located in the forearm, whereas demyelinating features outside the blocked nerves were rare. Abnormal distal sensory potentials were found in 87% of patients. The electrophysiological pattern suggests a very focal motor fibre demyelination sparing the nerve endings, whereas sensory fibre involvement was widespread. The course was chronic progressive in 71% ofpatientsand relapsing-remitting inthe others. During the follow-up study (median duration of 4 years), half of the patients progressed with a multifocal pattern and the distribution of the motor deficit remained similar to the initial presentation. The other patients showed a progression to the other limbs, suggesting a more diffuse process. Fifty-four percent of the patients treated with intravenous immunoglobulin showedanimprovement, compared with 33% of the patients treated with oral steroids. Overall, 73% of patients had a positive response to immune-mediated therapy. LSS may be distinguished from MMN by the presence of sensory involvement, the absence of serum anti-GM1 antibodies and, in some cases, a positive response to steroids. In some of the patients in our study, LSS evolved into a more diffuse neuropathy sharing similarities with CIDP. Others had a clinical course characterized by a striking multifocal neuropathy, which suggests underlying mechanisms different from CIDP. Overall, whatever the clinical course, LSS responded to immunemediated treatment in a manner similar to CIDP.Keywords: Lewis-Sumner syndrome; multifocal acquired demyelinating sensory and motor neuropathy; multifocal motor neuropathy; chronic inflammatory demyelinating polyradiculoneuropathy Abbreviations: CB = conduction block; CIDP = chronic inflammatory demyelinating polyradiculoneuropathy; CMAP = compound muscle action potential; CV = conduction velocity; DL = distal latency; IVIg = intravenous immunoglobulin LSS = Lewis-Sumner syndrome; MMN = multifocal motor neuropathy; SNAP = sensory nerve action potential

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“…Steroid therapy is often effective in CIDP, whereas MMN patients do not improve and occasionally even deteriorate with steroid treatment [13,14,15,16,17,18] despite the fact that MMN is pathologically a demyelinating disorder [8]. In addition, plasma exchange is likewise ineffective in MMN [13, 14, 16, 19], although levels of IgM anti-GM1 antibodies are elevated in some patients with MMN [13,14,15,16,17, 20].…”

Section: Discussionmentioning

confidence: 99%

CD16+CD57– Natural Killer Cells in Multifocal Motor Neuropathy

et al. 2005

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We analyzed the CD16+CD57– lymphocyte subset, which is considered to have strong natural killer (NK) cell activity, in peripheral blood from patients with chronic immune-mediated neuropathies and patients with other neurological diseases. We found that the ratio of CD16+CD57– NK cells to total lymphocytes was increased in 4 of 6 patients with multifocal motor neuropathy (MMN) with persistent conduction block. Since the CD16 molecule is an Fc receptor for immunoglobulin G (IgG), high-dose intravenous immunoglobulin (IVIg) may interfere with CD16+CD57– NK cells via Fc receptor blockade. In addition, cyclophosphamide (Cy) is often used to suppress NK cells. Therefore, our findings may partly account for the effectiveness of IVIg or Cy, which is the current treatment of choice for MMN.

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Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin.

Cited by 213 publications

References 20 publications

Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy

Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy

Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome

CD16+CD57– Natural Killer Cells in Multifocal Motor Neuropathy

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