Acquired neuromyotonia: superiority of plasma exchange over high-dose intravenous human immunoglobulin

Berg, J.S.P. van den; Engelen, B.G.M. van; Boerman, R.H.; Baets, M.H.V. de

doi:10.1007/s004150050419

Cited by 59 publications

(21 citation statements)

References 7 publications

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“…15 Neuromyotonia commonly responds to immunomodulatory treatment, 33 although it has been suggested that plasmapheresis is superior to intravenous immunoglobulins. 10,30 Morvan's syndrome has been reported to improve after plasmapheresis, 15,17,19,20 resection of an associated thymoma, and chronic immunosuppression, 5,15 but spontaneous recovery also may occur. 3 Intravenous immunoglobulin treatment was used as first-line treatment in our patient, but without success; plasmapheresis, which could have confirmed the presence of pathogenic humoral factors, was declined by our patient.…”

Section: Discussionmentioning

confidence: 95%

Morvan's syndrome: Clinical, laboratory, and in vitro electrophysiological studies

et al. 2004

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Morvan's syndrome is a rare disorder characterized by neuromyotonia, hyperhidrosis, and central nervous system dysfunction. We report a patient with features of this syndrome, but who initially presented with breathing difficulties. Concentric needle electromyography showed an abundance of myokymic and neuromyotonic discharges. Exercise tests and repetitive nerve stimulation showed a decrement-increment response of compound muscle action potentials. Antibodies against voltage-gated potassium channels were not detected on repeated testing, but the presence of oligoclonal bands in the cerebrospinal fluid (CSF) suggested an autoimmune etiology. At follow-up over 3 years, no cancer was found. Electrophysiological in vitro studies of effects of patient serum and CSF on rat nerves provided no evidence of altered voltage-gated sodium or potassium conductances. We conclude that putative humoral factors do not block ion channels acutely but may cause channel dysfunction with chronic exposure.

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Section: Discussionmentioning

confidence: 95%

Morvan's syndrome: Clinical, laboratory, and in vitro electrophysiological studies

et al. 2004

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“…Yet, the successful application of PE in neuromyotonia has so far only been described in case reports or small case series. 45 Some studies describe the elimination of autoantibodies or the normalization of peripheral nerve hyperexcitability after PE. 46 Another group reported on a patient who was not responsive to IVIG, but finally improved after treatment with PE.…”

Section: Plasma Exchange In Multiple Sclerosis Relapsesmentioning

confidence: 99%

“…46 Another group reported on a patient who was not responsive to IVIG, but finally improved after treatment with PE. 45 Plasma exchange in neuroimmunological disorders of the peripheral nerve…”

Section: Plasma Exchange In Multiple Sclerosis Relapsesmentioning

confidence: 99%

Plasmapheresis for neuroinflammatory disorders

Schröder

Linker

Gold

2010

Clinical & Exp Neuroim

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Nowadays, therapeutic plasma exchange (PE) is the most common apheresis procedure. Plasma is separated from corpuscular blood constitutents and replaced with a substitution fluid. Thus, PE is a non‐specific treatment modality. Its therapeutic effect is based on the removal of circulating, pathogenic immune factors, including autoantibodies. To date, PE is well established for several immune mediated neurological disorders. Whilst the first experience was acquired in acute life‐threatening conditions, such as the treatment of Guillain–Barré syndrome or myasthenic crisis, therapeutic success was later achieved in other chronic autoimmune diseases; PE was applied successfully in chronic inflammatory demyelinating polyneuropathy, paraproteinaemic polyneuropathy, stiff‐person syndrome and might also be tried in autoimmune diseases of paraneoplastic origin. From a novel aspect, PE was also established as an escalation therapy for steroid unresponsive relapses of multiple sclerosis, and thus has gained even more widespread attention. Humoral disease mechanisms dominate even more in neuromyelitis optica, as a subtype of MS, and usually respond to PE. Adding to its increasing application in clinical practice, the procedure is usually well tolerated. Possible adverse reactions of PE arise from the large‐size vascular access site, the use of replacement fluids and the need for anticoagulation. (Clin. Exp. Neuroimmunol. doi: 10.1111/j.1759‐1961.2010.0010.x, 2010)

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“…In einer Untersuchung wurde ein Patient aufgeführt, der auf IVIg nicht ansprach, anschließend aber nach PE eine klinische Verbesserung zeigte [107]. Mehrere Einzelfallberichte und kleine Fallserien (Evidenzklasse III/IV) berichteten eine deutliche klinische Besserung und Normalisierung der abnormen Spontanaktivität im EMG durch Elimination der Antikörper gegen K-Kanä-le mittels PE.…”

Section: Immunneuromyotonieunclassified

Einsatz von i. v. Immunglobulinen in der Neurologie

Stangel

Gold

2004

Nervenarzt

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Intravenous immunoglobulins (IVIg) have become an alternative immunomodulatory treatment for neurological autoimmune disorders. On the basis of several well conducted placebo-controlled trials evidence is available supporting the efficacy of IVIg. However, of all neurological diseases, IVIg is currently only licensed for Guillain-Barré syndrome. Thus, the off-label use of IVIg has to be focussed although an effective treatment must be made available for the patients, in particular when there is no other treatment alternative. The consensus paper presented here critically discusses the modern studies investigating the use of IVIg in neurological diseases. On the basis of this evidence recommendations for the treatment with IVIg were developed. To achieve a broad and valid consensus, numerous German speaking experts in the field were involved. It is expected that this manuscript will help to ascertain a rational use of IVIg in the future.

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Acquired neuromyotonia: superiority of plasma exchange over high-dose intravenous human immunoglobulin

Cited by 59 publications

References 7 publications

Morvan's syndrome: Clinical, laboratory, and in vitro electrophysiological studies

Morvan's syndrome: Clinical, laboratory, and in vitro electrophysiological studies

Plasmapheresis for neuroinflammatory disorders

Einsatz von i. v. Immunglobulinen in der Neurologie

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