Multifocal acquired demyelinating sensory and motor neuropathy

Last update 23 Jan 2025

Basic Info

Synonyms

Lewis-Sumner syndrome, MADSAM, Multifocal acquired demyelinating sensory and motor neuropathy

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Introduction

Lewis-Sumner syndrome (LSS) is a rare acquired demyelinating polyneuropathy characterized by asymmetrical distal weakness of the upper or lower extremities and motor dysfunction with adult onset. It is considered to be a variant of chronic inflammatory demyelinating polyneuropathy.

100 Clinical Results associated with Multifocal acquired demyelinating sensory and motor neuropathy

100 Translational Medicine associated with Multifocal acquired demyelinating sensory and motor neuropathy

0 Patents (Medical) associated with Multifocal acquired demyelinating sensory and motor neuropathy

Literatures (Medical) associated with Multifocal acquired demyelinating sensory and motor neuropathy

01 Oct 2021·Current Opinion in Neurology

Atypical chronic inflammatory demyelinating polyradiculoneuropathy: recent advances on classification, diagnosis, and pathogenesis

Review

Author: Dentoni, Marta ; Doneddu, Pietro Emiliano ; Nobile-Orazio, Eduardo

Purpose of reviewIn recent years, there has been an intense debate in literature regarding the definition of the individual variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), their possible pathogenetic mechanisms, and impact in the diagnosis of CIDP.Recent findingsThe 2021 European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines revised the definition of the individual CIDP variants and implemented their diagnostic criteria. Diagnosis of atypical CIDP is challenging and misdiagnosis is frequent, leading to diagnostic delay and consequent greater accumulation of disability and treatment dependency. Recent studies suggest that patients with typical CIDP have an antibody-mediated mechanism of neuropathy whereas in those with Lewis--Sumner syndrome (LSS) neuropathy is preferentially mediated by macrophages and T cells.SummaryAlthough the validity of the 2021 EFNS/PNS diagnostic criteria for atypical CIDP is unknown, they will hopefully lead to greater uniformity in the selection of patients to be enrolled in future studies and to a greater diagnostic accuracy. New data are emerging on the possible pathological mechanisms of individual variants and this could result in the discovery of specific diagnostic biomarkers and new therapies.

01 Jan 2018·Clinical Neurophysiology

Ultrasound and MRI of nerves for monitoring disease activity and treatment effects in chronic dysimmune neuropathies – Current concepts and future directions

Review

Author: Grimm, Alexander ; Pham, Mirko ; Décard, Bernhard F

New imaging modalities like high-resolution-ultrasound (HRUS) and MR-Neurography (MRN) are increasingly used for the evaluation of the peripheral nervous system. The increasing knowledge on morphological changes observed in different neuropathies has led to a better understanding of underlying pathophysiological processes. The diagnosis of acquired chronic dysimmune neuropathies (CDN) like chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis-Sumner Syndrome (LSS) or multifocal motor neuropathy (MMN) can be challenging. The current diagnostic criteria and outcome parameters are mainly based on clinical and electrophysiological parameters. Especially in CDN cases with atypical presentation or during early disease stages, the diagnostic accuracy is low and standardized protocols for the evaluation of disease activity and treatment response are lacking. The establishment of combined diagnostic criteria for CDN including imaging modalities could help to improve the diagnostic accuracy, allow a better differentiation of subtypes and facilitate the follow-up of disease course. The appropriate selection of eligible patients and sensitive monitoring of treatment response is mandatory future in treatment trials. In this article, we briefly summarize the clinical presentations and pathophysiological concepts of different CDN like CIDP, LSS and MMN. Furthermore, this review focuses on the diagnostic value of HRUS/MRN and its potential role for the monitoring of disease activity.

Muscle & Nerve

Ultrasonographic abnormalities in clinically unaffected nerves of patients with nonvasculitic motor and sensorimotor mononeuropathies

Article

Author: Podnar, Simon

AbstractIntroduction/AimsDiagnostic criteria for multifocal motor neuropathy (MMN) and multifocal acquired demyelinating sensorimotor neuropathy (MADSAM) require the involvement of at least two peripheral nerves. However, many patients with very similar features have clinical involvement of only a single peripheral nerve, which may preclude their correct diagnosis and treatment. The present study aimed to present a cohort of such patients and discuss the role of ultrasonography (US) in their diagnosis.MethodsPatients with nonvasculitic immune‐mediated motor mononeuropathies (MM) and sensorimotor mononeuropathies (SMM) were recruited prospectively or identified from the electronic records. They were invited to comprehensive follow‐up visits consisting of clinical examination, electrodiagnostic (EDx), and US studies.ResultsTwenty‐four patients (13 men) were studied (11 with MM). The characteristics of MM and SMM patients were very similar to MMN and MADSAM, respectively. The US, in addition to a long‐swollen segment (average length, 20 cm) in the clinically affected nerve, revealed nerve swelling in, on average, six additional sites in clinically unaffected nerves.DiscussionIn patients with clinical and EDx involvement of only a single nerve, an US demonstration of multifocal peripheral nerve swelling points to a more widespread, probably dysimmune mechanism. Further studies are needed to evaluate the value of US as a supplementary method for the diagnosis of MADSAM and MMN in patients with clinical involvement of a single nerve.

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Multifocal acquired demyelinating sensory and motor neuropathy

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