Multifocal acquired demyelinating sensory and motor neuropathy: The Lewis-Sumner syndrome

Saperstein, David; Amato, Anthony A.; Wolfe, Gil I.; Katz, Jonathan; Nations, Sharon P.; Jackson, Carlayne E.; Bryan, Wilson W.; Burns, Dennis K.; Barohn, Richard J.

doi:10.1002/(sici)1097-4598(199905)22:5<560::aid-mus2>3.0.co;2-q

Cited by 249 publications

(221 citation statements)

References 37 publications

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“…Persistent conduction blocks in motor and sensory fibers are typical. Similar to CIDP, there is a favorable response to plasmapheresis, steorids or intravenous immunoglobulin (IVIg), and CSF protein is increased in 60-80% of patients with MADSAM neuropathy (1,17). Our patient demonstrated the definitive clinical and electrophysiologic picture of MADSAM neuropathy, along with elevated CSF protein, contrast enhancement in cauda equina, and significant response to steroids.…”

Section: Discussionmentioning

confidence: 58%

Tumefactive Brain Demyelination Accompanying MADSAM Neuropathy

Erdener¹,

Temuçin²,

Söylemezoğlu³

et al. 2015

tnd

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Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is characterized by asymmetric multifocal motor and sensory loss and conduction blocks in peripheral nerves. Peripheral demyelinating diseases may be accompanied by demyelination in the central nervous system (CNS). In this report, we present a patient with MADSAM who had a solitary tumefactive demyelinating lesion in the brain. Neuroimaging was performed because of a visual field defect revealed a right parietooccipital lesion, which was initially misdiagnosed as a tumor. A pathologic examination showed that it was demyelinating in nature. The patient developed peripheral nervous symptoms two years later and was then diagnosed as having MADSAM. There was a prominent clinical and electrophysiologic response to steroid treatment. Tumefactive brain involvement has not previously been reported for MADSAM neuropathy, although it has been documented in a single case of typical chronic inflammatory demyelinating polyneuropathy (CIDP). CNS involvement should therefore be considered in patients with MADSAM. Keywords: Multifocal acquired demyelinating sensory and motor, neuropathy, tumefactive, brain, demyelination Multifokal edinilmiş demiyelinizan duysal ve motor (MADSAM) nöropati, asimetrik motor ve duysal kayıplar ve periferik sinirlerde ileti blokları ile karakterizedir. Periferik demiyelinizan hastalıklara santral sinir sisteminde de demiyelinizasyonun eşlik edebildiği iyi bilinmektedir. Bu raporda, beyinde tümefaktif tek bir demiyelinizan lezyonun bulunduğu bir MADSAM olgusu sunulmaktadır. Yeni gelişen görme alanı defekti nedeniyle yapılan nörogörüntüleme sonucunda sağ parietooksipital bölgede başlangıçta tümör olarak yanlış tanı alan bir lezyon saptanmıştır. Patolojik inceleme sonucunda bu lezyonun demiyelinizan nitelikte olduğu anlaşılmıştır. Bu bulgudan iki yıl sonra, hastada periferik nöropatiyi düşündüren semptomlar ortaya çıkmış ve hasta MADSAM tanısı almıştır. Steroid tedavisi ile belirgin klinik ve elektrofizyolojik yanıt elde edilmiştir. Beyinde tümefaktif demiyelinizan lezyonlar, daha önce bir kronik enflamatuvar demiyelinizan nöropati olgusunda bildirilmiş olmasına karşın, MADSAM için bu tür bir bildirimde bulunulmamıştır. MADSAM olgularında da bu nedenle santral sinir sistemi tutulumu olasılığı gözden geçirilmelidir.

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Section: Discussionmentioning

confidence: 58%

Tumefactive Brain Demyelination Accompanying MADSAM Neuropathy

Erdener¹,

Temuçin²,

Söylemezoğlu³

et al. 2015

tnd

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“…Eine weitere differentialdiagnostisch zu berücksichtigende Form der erworbenen immunvermittelten demyelinisierenden Neuropathien stellt das LewisSumner-Syndrom dar (MADSAM/ multifocal aqcuired demyelinating sensory and motor neuropathy). Diese Erkrankung geht klinisch und elektrophysiologisch mit asymmetrischen multifokalen sensomotorischen Ausfällen einher, die wie die MMN eher den Verläufen einzelner peripherer Nerven folgt [3,25,26].…”

Section: Elektrophysiologie Und Diagnosestellungunclassified

Die multifokale motorische Neuropathie

Fischer¹,

Reimann²,

Schröder³

et al. 2002

Der Nervenarzt

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Multifocal motor neuropathy (MMN) is an acquired immune mediated motor neuropathy with characteristic clinical and neurographic features.Clinically, MMN is characterized by progressive, predominantly distal and asymmetrical limb weakness. Neurographic recordings demonstrate features of multifocal demyelination with or without conduction block. Sensory nerves are not affected. Due to strict diagnostic criteria,MMN may be underdiagnosed in patients with motor neuropathies. Since intravenous immunglobulins are an efficient therapy for MMN,clinical and electrophysiological differentiation from other neuromuscular disorders is mandatory to prevent progressive impairment of motor function. We present a patient with MMN and review the clinical, electrophysiological, and histological features. In addition,pathogenesis, differential diagnosis and treatment of MMN are discussed.

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“…When weakness is asymmetric, further classification hinges around the presence or absence of sensory signs. Asymmetric distal weakness without sensory loss is indicative of multifocal motor neuropathy (MMN) whereas sensory loss would suggest multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy (Lewis-Sumner syndrome) [21][22][23][24]. Up to half of the MMN patients also have serum GM-1 antibodies and most DADS patients have an IgM monoclonal protein in the serum, often accompanied by antibodies to myelin-associated glycoprotein (MAG).…”

Section: Cidp Clinical Featuresmentioning

confidence: 99%

“…Initial involvement is usually in the arms, with later spread to distal legs. Unlike MMN, MADSAM neuropathy is not associated with anti-GM1 antibodies [21,[25][26][27]. As in MMN and CIDP, nerve conduction studies in MADSAM neuropathy shows conduction block, temporal dispersion, prolonged distal latencies, slow conduction velocities, and delayed or absent F-waves in one or more motor nerves.…”

Section: Cidp Clinical Featuresmentioning

confidence: 99%

“…In contrast to MMN, sensory NCS are also abnormal at the outset. Though prospective randomized controlled studies have not been performed, retrospective series have demonstrated improvement in response to IVIg in treatment more than 70 % of MADSAM neuropathy patients [21,[25][26][27][28][29][30]. In contrast to MMN, most patients with MADSAM neuropathy have also shown response to corticosteroid treatment.…”

Section: Cidp Clinical Featuresmentioning

confidence: 99%

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Chronic Inflammatory Demyelinating Polyneuropathy

Encyclopedia of Pain

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Chronic Inflammatory polyneuropathies are an important group of neuromuscular disorders that present chronically and progress over more than 8 weeks, being referred to as chronic inflammatory demyelinating polyneuropathy (CIDP). Despite tremendous progress in elucidating disease pathogenesis, the exact triggering event remains unknown. Our knowledge regarding diagnosis and management of CIDP and its variants continues to expand, resulting in improved opportunities for identification and treatment. Most clinical neurologists will be involved in the management of patients with these disorders, and should be familiar with available therapies for CIDP. We review the distinctive clinical, laboratory, and electro-diagnostic features that aid in diagnosis. We emphasize the importance of clinical patterns that define treatment responsiveness and the most appropriate therapies in order to improve prognosis.

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Multifocal acquired demyelinating sensory and motor neuropathy: The Lewis-Sumner syndrome

Cited by 249 publications

References 37 publications

Tumefactive Brain Demyelination Accompanying MADSAM Neuropathy

Tumefactive Brain Demyelination Accompanying MADSAM Neuropathy

Die multifokale motorische Neuropathie

Chronic Inflammatory Demyelinating Polyneuropathy

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