Steroid-responsive multifocal demyelinating neuropathy with central involvement

Liguori, Rocco; Rizzi, Romana; Vetrugno, Roberto; Salvi, Fabrizio; Cevoli, Sabina; Montagna, Pasquale

doi:10.1002/(sici)1097-4598(199902)22:2<262::aid-mus16>3.0.co;2-1

Cited by 15 publications

(8 citation statements)

References 19 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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%

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

Rajabally

Chavada

2009

Muscle and Nerve

View full text Add to dashboard Cite

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.

show abstract

Section: Resultsmentioning

confidence: 99%

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

Rajabally

Chavada

2009

Muscle and Nerve

View full text Add to dashboard Cite

show abstract

“…Most examples of coexistent disease have been discovered at autopsy or by neuroimaging or electromyographic screening of patients with either known MS or CIDP. Some patients with both CNS and PNS symptomatology have, however, been described 1, 5, 7, 17, 21…”

mentioning

confidence: 99%

Spinal and cranial hypertrophic neuropathy in multiple sclerosis

et al. 2005

View full text Add to dashboard Cite

Two patients with multiple sclerosis developed symptomatic chronic inflammatory demyelinating polyneuropathy with massive spinal or cranial nerve hypertrophy revealed by neuroimaging. Sural nerve biopsy in one showed only moderate demyelination, axonal loss, and onion-bulb formation, illustrating dichotomy between severe proximal and milder distal nerve involvement. Patients with coexistent central and peripheral demyelination usually are symptomatic from dysfunction at one site or the other, but not from both. Our patients showed minimal response to steroids, intravenous immunoglobulin, or azathioprine. These cases suggest that the mechanism of disease in symptomatic central and peripheral demyelination may differ from that of disease in only one region, and that optimal therapy in this situation must be explored further.

show abstract

“…However, a growing body of evidence, consisting mainly of case reports and series, describe beneficial outcomes with IVG and corticosteroids. [76][77][78] The usual IVIG dose is 2 g/kg given over 2 to 5 days followed by monthly infusions of 0.4 to 2 g/kg. Alternative corticosteroid treatment regimens typically consist of initial high dose (1-1.5 mg/kg/day) oral prednisone or prednisolone, followed by a slow taper over several weeks to months.…”

Section: Treatment Of Lewis-sumner Syndromementioning

confidence: 99%

Treatment of Chronic Immune-mediated Neuropathies: Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Multifocal Motor Neuropathy, and the Lewis-Sumner Syndrome

Sederholm¹

2010

Semin Neurol

View full text Add to dashboard Cite

Current treatment approaches for the management of chronic immune-mediated peripheral neuropathies are reviewed, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN), and the Lewis-Sumner syndrome (LSS). A summary of existing evidence for commonly used treatment modalities, such as corticosteroids, intravenous immune globulin (IVIG), and plasma exchange is provided. Evidence for the use of additional immunosuppressant and immunomodulatory agents is also reviewed.

show abstract

Steroid-responsive multifocal demyelinating neuropathy with central involvement

Cited by 15 publications

References 19 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

Spinal and cranial hypertrophic neuropathy in multiple sclerosis

Treatment of Chronic Immune-mediated Neuropathies: Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Multifocal Motor Neuropathy, and the Lewis-Sumner Syndrome

Contact Info

Product

Resources

About