Comparison of IgM-MGUS and IgG-MGUS polyneuropathy

Simovic, Drasko; Gorson, Kenneth C.; Ropper, Allan H.

doi:10.1111/j.1600-0404.1998.tb00636.x

Cited by 58 publications

(22 citation statements)

References 38 publications

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“…10 However, the clinical phenotype and treatment response are dictated by the presence of IgM Kappa monoclonal gammopathy regardless of MAG status. Therefore, it remains uncertain whether there are any practical differences with regards to management, prognosis, or pathophysiology between MAG-positive and MAG-negative IgM-MGUS neuropathy cases 10, 132-135 CSF protein is elevated in most patients. Motor NCS demonstrate widespread symmetric slowing, most accentuated in distal sensory and motor nerves (Table 2).…”

Section: Distal Acquired Demyelinating Symmetric (Dads) Neuropathymentioning

confidence: 99%

Multifocal Motor Neuropathy, Multifocal Acquired Demyelinating Sensory and Motor Neuropathy, and Other Chronic Acquired Demyelinating Polyneuropathy Variants

2013

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Chronic acquired demyelinating neuropathies (CADP) are an important group of immune neuromuscular disorders affecting myelin. These are distinct from chronic inflammatory demyelinating polyneuropathy (CIDP). Classically, CIDP is characterized by proximal and distal weakness, large fiber sensory loss, elevated cerebrospinal fluid (CSF) protein content, demyelinating changes nerve conduction studies or nerve biopsy, and response to immunomodulating treatment. In this chapter we discuss CADP with emphasis on multifocal motor neuropathy (MMN), multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), distal acquired demyelinating symmetric (DADS) neuropathy and conclude with less common variants. While each of these entities has distinctive laboratory and electrodiagnostic features that aid in their diagnosis, clinical characteristics are of paramount importance in diagnosing specific conditions and determining the most appropriate therapies. Unlike CIDP, MMN is typically asymmetric and affects only the motor nerve fibers. MMN is a rare disease that presents chronically, over several years of progression affecting the arms are more commonly than the legs. Men are more likely than women to develop MMN. MADSAM should be suspected in patients who have weakness and loss of sensation in primarily one arm or leg which progresses slowly over several months to years. It is important in patient with multifocal demyelinating clinical presentation to distinguish MMN from MADSAM since corticosteroids are not effective in MMN where the mainstay of therapy is intravenous gammaglobulin (IVIg). DADS can be subdivided into DADS-M (associated woth M-protein) and DADS-I which is idioapthic. While DADS-I patients respond somewhat to immunotherapy, DADS-M patients present with distal predominant sensorimotor demyelinating neuropathy phenotype and are notoriously refractory to immunotherapies regardless of antibodies to myelin-associated glycoprotein (MAG). Our knowledge regarding pathogenesis, diagnosis and management continues to expand, resulting in improved opportunities for identification and treatment.

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Section: Distal Acquired Demyelinating Symmetric (Dads) Neuropathymentioning

confidence: 99%

Multifocal Motor Neuropathy, Multifocal Acquired Demyelinating Sensory and Motor Neuropathy, and Other Chronic Acquired Demyelinating Polyneuropathy Variants

2013

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“…Neurological examination shows abnormal vibration and joint position sense, decreased pain and temperature sensation, hypoactive muscle stretch reflexes, and if present, mild distal muscle weakness [27,28]. Postural tremors in the arms and palpable thickened nerves can be observed [25,28]. The neuropathy is classified as demyelinating by electrophysiological studies in most cases; however, nerve conduction studies can show features of both axonal degeneration and demyelination or less often predominantly axonal degeneration [26][27][28].…”

Section: Neuropathy Associated With Igm Mgusmentioning

confidence: 96%

“…The average age of symptom onset is between the sixth and seventh decade of life [37,38]. The clinical symptoms experienced by patients are predominantly sensory in nature and are similar to those of IgM MGUS neuropathy [25,28]. Progressive distal paresthesia, gait ataxia, and muscle weakness are typical.…”

Section: Neuropathy With Anti-myelin-associated Glycoprotein Antibodiesmentioning

confidence: 96%

“…In patients who have MGUS and peripheral neuropathy, however, the frequency of IgM, IgG and IgA are 48, 37 and 15%, respectively [24]. In fact, the prevalence of IgM among patients who have MGUS and neuropathy has been reported to be as high as 70% in some case series [25].…”

Section: Neuropathy Associated With Monoclonal Gammopathy Of Undetermmentioning

confidence: 97%

“…Furthermore, on electrophysiologic studies the neuropathy can be axonal, demyelinating, or mixed type [51,52]. Comparison of neuropathies associated with IgM and IgG/IgA MGUS in some studies has shown that these two groups are similar in the clinical characteristics, symptomology, neurophysiologic attributes, and severity [24,25,30]. Other studies, however, have demonstrated differences between these two groups including more severe worsening of neuropathic signs during longitudinal follow-up examination and abnormalities on nerve conduction studies in IgM MGUS [13,28].…”

Section: Neuropathy With Igg/iga Monoclonal Gammopathymentioning

confidence: 98%

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Dysimmune Neuropathy

Kwan¹

2009

Frontiers of Neurology and Neuroscience

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Dysimmune neuropathy is an etiologically heterogeneous entity with diverse clinical presentations. The underlying causes encompass several different benign and neoplastic syndromes. Peripheral nervous system manifestations are common, and in some cases, the initial symptom of the abnormal immune system. Early recognition of the immunologic disturbance or malignancy as the cause of neuropathy with appropriate diagnostic testing is necessary so that potentially effective therapies can be initiated. This review discusses evaluation, differential diagnosis, clinical findings, pathophysiology, and treatment of disease states with abnormal immunoglobulin production that are associated with peripheral neuropathies.

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