Focal neuropathy preceding chronic inflammatory demyelinating polyradiculoneuropathy by several years

Verma, Abha; Tandan, Rup; Adesina, Adekunle; Pendlebury, William W.; Fries, Timothy J.; Bradley, Walter G.

doi:10.1111/j.1600-0404.1990.tb01011.x

Cited by 29 publications

(11 citation statements)

References 28 publications

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“…We identified 82 prior reports of patients with an initially pure upper‐limb form of asymmetric CIDP, or L‐SS, in 25 publications,3, 5–10, 12, 14, 17–20, 23, 28, 29, 31–33, 35, 36, 38–40, 42 including Lewis et al's 5 original patients (1982) 14. A summary of the clinical, biologic, immunologic, histologic, radiologic, and therapeutic characteristics of the 82 patients is provided in Table 3 (see supplementary file).…”

Section: Resultsmentioning

confidence: 99%

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Lewis–sumner syndrome of pure upper‐limb onset: Diagnostic, prognostic, and therapeutic features

Rajabally

Chavada

2009

Muscle and Nerve

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Lewis-Sumner syndrome (L-SS) represents the asymmetric variant of chronic inflammatory demyelinating polyneuropathy (CIDP). The characteristics and specificities of L-SS of pure upper-limb onset, as initially described by Lewis et al. [Multifocal demyelinating neuropathy with persistent conduction block. Neurology 32:958-964, 1982], have not been studied. We describe 8 such patients and review 82 previously reported cases. Distal involvement predominates and is mixed, sensory and motor from onset in only 50% of patients. Pain is a feature in about 20%. Subsequent lower-limb involvement occurs in <40% of cases. Electrophysiologically, upper-limb-onset L-SS is characterized by the presence of motor conduction blocks in arm nerves in about 90% of cases, and other demyelinating motor abnormalities are significantly less frequent. Cerebrospinal fluid (CSF) protein levels are raised in about 40% of cases and are moderate in most. Mildly raised anti-GM1 antibody titers are rare (<5%), but very high titers (> or =1:6400) have not been reported. Over 80% of treated patients respond, and intravenous immunoglobulins may be more effective than steroids. The prognosis is favorable in 40% of patients who eventually stabilize without treatment. We also reviewed 36 cases of other forms of L-SS, and present a further 2 cases. The upper-limb-onset variant is significantly less likely to spread to other limbs and may be less likely to have raised CSF protein levels. This could reflect a more localized disease process in upper-limb-onset L-SS. This variant may represent a separate entity, to be distinguished from other asymmetric forms of CIDP.

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Section: Resultsmentioning

confidence: 99%

“…We identified 36 other cases of L‐SS, with asymmetric lower‐limb, or mixed, simultaneous upper‐ and lower‐limb onset, from 12 studies,2, 9, 19, 23, 25, 27, 31, 36, 38–41 as shown in Tables 5 and 6. To these, we added 2 new patients from our clinic who presented with this form of L‐SS.…”

Section: Resultsmentioning

confidence: 99%

Lewis–sumner syndrome of pure upper‐limb onset: Diagnostic, prognostic, and therapeutic features

Rajabally

Chavada

2009

Muscle and Nerve

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“…Focal CIDP has also been reported with symptoms remaining restricted to one focal region for a prolonged period of time, 15 but may also precede the development of diffuse CIDP. 35 Focal sensory CIDP has been reported restricted to one upper limb for 30 years. 36 …”

Section: Cidp Phenotypic Variantsmentioning

confidence: 99%

Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype

Mathey

Park

Hughes

et al. 2015

J Neurol Neurosurg Psychiatry

355

297

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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, classically characterised by a slowly progressive onset and symmetrical, sensorimotor involvement. However, there are many phenotypic variants, suggesting that CIDP may not be a discrete disease entity but rather a spectrum of related conditions. While the abiding theory of CIDP pathogenesis is that cell-mediated and humoral mechanisms act together in an aberrant immune response to cause damage to peripheral nerves, the relative contributions of T cell and autoantibody responses remain largely undefined. In animal models of spontaneous inflammatory neuropathy, T cell responses to defined myelin antigens are responsible. In other human inflammatory neuropathies, there is evidence of antibody responses to Schwann cell, compact myelin or nodal antigens. In this review, the roles of the cellular and humoral immune systems in the pathogenesis of CIDP will be discussed. In time, it is anticipated that delineation of clinical phenotypes and the underlying disease mechanisms might help guide diagnostic and individualised treatment strategies for CIDP.

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“…Patients can present initially with involvement of single nerves that progress to confluent and symmetrical involvement. 94, 98 Up to one-half of patients with MADSAM may evolve into a typical pattern of generalized CIDP. 12 Many patients experience pain and paresthesias.…”

Section: Multifocal Acquired Demyelinating Sensory and Motor (Madsam)mentioning

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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Focal neuropathy preceding chronic inflammatory demyelinating polyradiculoneuropathy by several years

Cited by 29 publications

References 28 publications

Lewis–sumner syndrome of pure upper‐limb onset: Diagnostic, prognostic, and therapeutic features

Lewis–sumner syndrome of pure upper‐limb onset: Diagnostic, prognostic, and therapeutic features

Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype

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

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