Multifocal inflammatory demyelinating neuropathy

Berg-Vos, Renske M. Van den; Berg, Leonard H van den; Franssen, Hessel; Vermeulen, M.; Witkamp, Theo D.; Jansen, Gerard H.; Es, Hendrik W. van; Kerkhoff, Henk; Wokke, John H. J.

doi:10.1212/wnl.54.1.26

Cited by 149 publications

(128 citation statements)

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“…Hyperintensity and hypertrophy of cervical nerves and nerve roots using T2‐weighted short tau inversion recovery (STIR) magnetic resonance imaging (MRI) techniques have been described in patients with various types of demyelinating inflammatory neuropathies, including multifocal motor neuropathy (MMN) (Van Es et al., 1997), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) (Adachi et al., 2011; Sinclair et al., 2011; Van Es et al., 1997), Lewis‐Sumner syndrome (LSS) (Rajabally, Knopp, Martin‐Lamb, & Morlese, 2014; Van den Berg‐Vos et al., 2000), and polyneuropathy associated with IgM monoclonal gammopathy (Eurelings, Notermans, Van de Donk, & Lokhorst, 2001). Abnormalities on MR imaging of the brachial plexus are an argument for inflammatory neuropathies, particularly when nerve conduction studies are not fully conclusive.…”

Section: Introductionmentioning

confidence: 99%

“…Comparative studies of brachial plexus MRI have not been performed. A number of smaller studies have mainly suggested that asymmetry is associated with MMN or LSS rather than CIDP, but others could not confirm this (Rajabally et al., 2014; Van den Berg‐Vos et al., 2000; Van Es et al., 1997). Moreover, it is not known whether MRI abnormalities predict disease course or response to treatment.…”

Section: Introductionmentioning

confidence: 99%

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Brachial plexus magnetic resonance imaging differentiates between inflammatory neuropathies and does not predict disease course

et al. 2017

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ObjectiveThe main objective of this study was to evaluate the correlation between the distribution of brachial plexus magnetic resonance imaging (MRI) abnormalities and clinical weakness, and to evaluate the value of brachial plexus MRI in predicting disease course and response to treatment in multifocal motor neuropathy (MMN), Lewis‐Sumner syndrome (LSS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).MethodsSixty‐seven patients with an inflammatory neuropathy diagnosed at our tertiary referral center for neuromuscular diseases had undergone bilateral T2‐weighted short tau inversion recovery (STIR) MRI of the brachial plexus. We obtained clinical follow‐up data and scored all MRIs for abnormalities and the symmetry of their distribution.ResultsBrachial plexus MRI abnormalities were detected in 45% of the patients. An abnormal MRI did not predict disease course in terms of patterns of weakness, sensory disturbances or response to treatment. Within the spectrum of radiological abnormalities, asymmetrical clinical syndromes, MMN and LSS were significantly associated with asymmetrical radiological abnormalities, whereas symmetrical abnormalities predominated in CIDP (p < .001, phi 0.791).ConclusionT2 STIR brachial plexus MRI abnormalities correspond with the distribution of neurological deficits in inflammatory neuropathies, but do not correlate with specific clinical characteristics, response to treatment or disease course.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Brachial plexus magnetic resonance imaging differentiates between inflammatory neuropathies and does not predict disease course

et al. 2017

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“…127 No. 9 # Guarantors of Brain 2004; all rights reserved et al, 1999), multifocal inflammatory demyelinating neuropathy (Van den Berg-Vos et al, 2000), multifocal acquired demyelinating sensory and motor neuropathy (Saperstein et al, 1999), and motor and sensory demyelinating mononeuropathy multiplex (Oh et al, 1997), underlying the difficulty of defining the nosological position of LSS among the dysimmune neuropathies, such as CIDP and multifocal motor neuropathy (MMN) (Parry, 1999). The term CIDP was coined by Dyck and co-workers in 1982 to describe an acquired peripheral neuropathy thought to be of immunological origin (Dyck et al, 1982).…”

Section: Introductionmentioning

confidence: 99%

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

Viala¹

2004

Brain

185

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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“…16,17,[46][47][48][49][50][51][52][53] In patients with CIDP, proximal symmetrical weakness and general areflexia are common, whereas weakness in MMN is asymmetrical and distal, and reflexes are only poor or absent in affected limbs. A remitting and relapsing course or a progression of symptoms in weeks is common in CIDP but not in MMN.…”

Section: Differential Diagnosismentioning

confidence: 99%

“…23,46 Another disease entity that has similarities with MMN (and CIDP) is the Lewis-Sumner syndrome (table 1). [48][49][50][51][52][53] Patients with this syndrome have an asymmetrical sensory or sensorimotor demyelinating neuropathy that can be localised to one arm or leg for several years, sometimes associated with neuropathic pain or focal nerve tenderness. Nerve-conduction studies are necessary to diagnose the syndrome and can help to differentiate it from MMN because low action-potential amplitudes in distal sensory nerves are found in many patients with the Lewis-Sumner syndrome, but not in patients with MMN.…”

Section: Differential Diagnosismentioning

confidence: 99%

Multifocal motor neuropathy

Wokke¹,

Doorn²,

Hoogendijk³

et al. 2013

Neuromuscular Disease a Case-Based Approach

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In 1985, Parry and Clarke 1 studied nerve conduction in two patients with a lower-motor-neuron syndrome characterised by progressive, asymmetric, predominantly distal weakness without sensory loss and found conduction block. Soon afterwards, others reported patients with similar characteristics. 2,3 Weakness in patients with a lower-motor-neuron syndrome with conduction block was shown to be reversible with immunomodulating therapy and associated with high titres of IgM antibodies to GM1 ganglioside. 4 Hence, a separate disorder emerged: multifocal motor neuropathy (MMN). [4][5][6][7][8][9][10][11] The disorder was thought to be immune mediated, possibly through IgM antibodies that bind gangliosides on human peripheral nerves. 12 The differential diagnosis of MMN includes motor-neuron disease 13,14 and demyelinating neuropathies.15-17 Diagnosis of MMN is supported by the finding of motor but not of sensory abnormalities on nerve-conduction studies. [18][19][20][21][22][23] Whether conduction block must be present for the diagnosis of MMN is debatable. Various open and placebo-controlled studies have shown that treatment with high-dose intravenous immunoglobulin leads to improvement of muscle strength. Intravenous immunoglobulin is now the treatment of first choice in MMN. [24][25][26][27] Many clinical and electrophysiological studies have improved our understanding of MMN in the past 15 years, but the disease mechanisms underlying weakness in MMN are poorly understood. Clinical featuresMMN is characterised by slowly progressive weakness and muscle atrophy that develops gradually over several years. 8,11,22,23,[28][29][30][31] More men than women are affected, at a ratio of 2·6. The mean age at onset is 40 years, with a range of 20-70 years. 9,22,32 In almost 80% of patients, the first symptoms occur between age 20 years and 50 years. The most common initial symptoms are wrist drop, grip weakness, and foot drop. Weakness develops asymmetrically and is more prominent in the arms than in the legs. 22,23 In most patients with onset in the legs, the abnormalities also eventually affect the arms and become the most prominent.33 Symptoms and signs in the distal muscles prevail for a long time, but eventually weakness in proximal muscle groups of the arms, but not of the legs, may develop. 32,33 Weakness is typically more pronounced than the degree of atrophy suggests.11,22 Nevertheless, atrophy of affected muscles can be substantial in patients with a long disease duration. Other motor symptoms include muscle cramps and fasciculations in about two-thirds of patients. 3,19,21 Myokymia has been reported occasionally. 3,19,21 Tendon reflexes are commonly reduced in affected regions, although these are rarely brisk in the arms. 4,21,22,28,30 Single cases of cranial-nerve involvement have been reported. 21,[34][35][36] Respiratory failure due to unilateral or bilateral phrenic-nerve palsy can occur, even at the beginning of the disorder. 35,37,38 Some patients report feelings of paraesthesia or numbness but sensory l...

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Multifocal inflammatory demyelinating neuropathy

Abstract: The authors propose calling this neuropathy "multifocal inflammatory demyelinating neuropathy" and considering it as a distinct clinical entity to facilitate early diagnosis of this treatable disorder.

Cited by 149 publications

References 30 publications

Brachial plexus magnetic resonance imaging differentiates between inflammatory neuropathies and does not predict disease course

Brachial plexus magnetic resonance imaging differentiates between inflammatory neuropathies and does not predict disease course

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

Multifocal motor neuropathy

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