Diagnostic value of nerve biopsy for atypical chronic inflammatory demyelinating polyneuropathy: Evaluation of eight cases

Vallat, Jean‐Michel; Tabaraud, F; Magy, Laurent; Torny, F.; Bernet-Bernady, P; Macian, Francisco; Couratier, Philippe

doi:10.1002/mus.10348

Cited by 104 publications

(66 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Such results have been reported in other studies as well. In a study of 8 atypical CIDP patients, 1 had retained reflexes [16]. The retained or lively reflexes have been reported in multifocal motor neuropathy and 13% of patients with acute inflammatory demyelinating polyradiculoneuropathy [17].…”

Section: Discussionmentioning

confidence: 99%

A Comparison of Clinically Atypical with Typical Chronic Inflammatory Demyelinating Polyradiculoneuropathy

2007

View full text Add to dashboard Cite

Background: There is a paucity of prospective studies evaluating the atypical features of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Aims: To compare clinical and electrodiagnostic features of clinically typical and atypical CIDP patients. Methods: The patients with typical and atypical CIDP diagnosed according to the European Federation of Neurological Societies/Peripheral Nerve Society were included. The disability was graded on a 0–10 scale. Neurophysiological study included motor and sensory nerve conduction and F wave studies of both upper and lower limbs. The patients were treated with prednisolone with or without azathioprine. The outcome was evaluated at 6 months and improvement was defined as at least 2 grades improvement. Results: Eight out of 37 CIDP patients had atypical features, which included asymmetry in 2, dysautonomia in 3 and pure motor weakness, amyotrophic-lateral-sclerosis-like syndrome and distal weakness in 1 patient each. Tendon reflexes were retained in 3 patients. The mean duration of symptoms was 43 weeks in the typical and 30 weeks in the atypical group. The age, sex, disability, therapeutic response and neurophysiological features were similar in the 2 groups. Conclusion: About one fifth of CIDP patients may have atypical clinical features; however, their electrodiagnostic features and response to treatment are no different from typical CIDP.

show abstract

Section: Discussionmentioning

confidence: 99%

A Comparison of Clinically Atypical with Typical Chronic Inflammatory Demyelinating Polyradiculoneuropathy

2007

View full text Add to dashboard Cite

show abstract

“…et al, 2000; Vallat et al, 2003). The clinically distinct features concern the predominance of upper limb involvement and the asymmetrical and distal topography in LSS, even if asymmetry may also be encountered in CIDP.…”

mentioning

confidence: 99%

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

Viala¹

2004

Brain

185

140

View full text Add to dashboard Cite

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

show abstract

“…Overall, the clinical and laboratory findings were consistent with the diagnosis of CIDP according to the EFNS/PNS criteria [2]. We have previously presented some details of this patient (case 8 of Vallat et al [16]).…”

Section: Observationsmentioning

confidence: 64%

Natalizumab as a Disease-Modifying Therapy in Chronic Inflammatory Demyelinating Polyneuropathy - A Report of Three Cases

Vallat

Mathis²,

Ghorab³

et al. 2015

Eur Neurol

Self Cite

View full text Add to dashboard Cite

Background: Several treatments are available to treat the immune-mediated chronic inflammatory demyelinating polyneuropathy (CIDP). Among these treatments, intravenous immunoglobulins, corticosteroids and plasma exchanges are validated and widely used. A few immunosuppressive drugs have been tried, but they had little efficiency. Methods: We describe three CIDP patients treated by Natalizumab (acting against cellular adhesion and T-cell migration) after a failure of the validated treatments. Results: We observed a long-term improvement in one patient, a dramatic improvement over a significant duration in another patient and stabilization in the last one. Conclusion: This open label study provides evidence for the value of Natalizumab as second-line treatment for individual patients with a high dependency on waning efficacy of first-line therapies. CIDP is characterized by heterogeneity of clinical phenotypes, electrophysiological and pathological features, and various variable courses types of evolution. The different responses to drugs of our patients are consistent with some reported cases and may reflect the spectrum of lesional mechanisms and the molecular dysfunctions in CIDP.

show abstract

Diagnostic value of nerve biopsy for atypical chronic inflammatory demyelinating polyneuropathy: Evaluation of eight cases

Cited by 104 publications

References 29 publications

A Comparison of Clinically Atypical with Typical Chronic Inflammatory Demyelinating Polyradiculoneuropathy

A Comparison of Clinically Atypical with Typical Chronic Inflammatory Demyelinating Polyradiculoneuropathy

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

Natalizumab as a Disease-Modifying Therapy in Chronic Inflammatory Demyelinating Polyneuropathy - A Report of Three Cases

Contact Info

Product

Resources

About