Continuous muscle fiber activity, peripheral neuropathy, and thymoma

García‐Merino, Antonio; Cabello, Ana; Mora, Jesús; Liaño, H

doi:10.1002/ana.410290218

Cited by 61 publications

(14 citation statements)

References 12 publications

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“…6 10 26-30 32 34-36 Antibodies directed against voltage gated potassium channels have been reported in some patients with acquired neuromyotonia, and the node of Ranvier has been proposed as a possible target 10. The relation between anti-voltage gated potassium channel antibodies and thymoma is not clear, although some cases of neuromyotonia have been associated with thymoma 30. Other manifestations of Morvan’s fibrillary chorea may also have a paraneoplastic or autoimmune basis.…”

Section: Discussionmentioning

confidence: 99%

Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma

Lee¹,

Maselli

Ellis

et al. 1998

Journal of Neurology, Neurosurgery & Psychiatry

138

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Morvan's fibrillary chorea is a rare disease characterised by symptoms which include neuromyotonia, cramping, weakness, pruritis, hyperhidrosis, insomnia, and delirium. The first case of Morvan's fibrillary chorea to be associated with clinical manifestations of myasthenia gravis with thymoma, psoriasis, and atopic dermatitis is reported. Muscle histopathology disclosed chronic denervation and myopathic changes and in vitro electrophysiology demonstrated both presynaptic and postsynaptic defects in neuromuscular transmission. Serum antibodies to acetylcholine receptors, titin, N-type calcium channels, and voltage gated potassium channels were detected. Plasmapheresis, thymectomy, and long term immunosuppression induced a dramatic resolution of symptoms. The association of thymoma with other autoimmune disorders and autoantibodies, and prolonged and sustained remission with chronic immunosuppression, place Morvan's fibrillary chorea on the range of neurological diseases arising as a paraneoplastic complication of cortical thymomas. (J Neurol Neurosurg Psychiatry 1998;65:857-862) Keywords: Morvan's fibrillary chorea; neuromyotonia; thymoma; paraneoplastic La chorée fibrillaire was first coined by Morvan in 1890 when describing patients with multiple, irregular contractions of the long muscles, cramping, weakness, pruritis, hyperhidrosis, insomnia, and delirium.1 Additional cases of Morvan's fibrillary chorea have been reported, but only four cases are in the English literature, perhaps as a matter of semantics.2 3 Neuromyotonia and Isaacs' syndrome (continuous muscle fibre activity) are used interchangeably to refer to muscle twitching and cramping at rest, that is exacerbated with exercise. 4 It is caused by sustained or repetitive spontaneous muscle activity of peripheral nerve origin. Electromyography discloses spontaneous, repetitive motor unit or single fibre discharges firing in irregular rhythmic bursts at high intraburst frequencies. Myokymia, or spontaneous rippling and twitching movements of muscles, is a visible component of neuromyotonia and Isaacs' syndrome. 5 Morvan's fibrillary chorea is a syndrome that is characterised by not only neuromyotonia, but also dysautonomia and CNS dysfunction, often in the form of encephalopathy.The association of the disease with thymoma, tumour, autoimmune diseases, and autoantibodies suggests an autoimmune or paraneoplastic aetiology. Thymoma, prostate adenoma, and in situ carcinoma of the sigmoid colon, have been found in patients with Morvan's fibrillary chorea.2 3 In other patients, heavy metal intoxication with gold, mercury, and manganese, known stimulants of the immune system, has been implicated as a cause. [6][7][8] Anti-acetylcholine receptor (antiAChR) antibodies have been detected in patients with thymoma, but without clinical manifestations of myasthenia gravis.2 Raised CSF IgG concentrations and oligoclonal bands have been reported in patients with psychosis.3 9In addition, some of the individual components of Morvan's fibrillary chorea are sus...

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Section: Discussionmentioning

confidence: 99%

Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma

Lee¹,

Maselli

Ellis

et al. 1998

Journal of Neurology, Neurosurgery & Psychiatry

138

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“…Garcia-Merino et al [5] consider this syndrome as a paraneoplastic manifestation of a malignant tumour; recently there has been increasing interest in the hypotheses that consider the paraneoplastic syndromes to be the result of an autoimmune process towards shared antigens of the nervous system and of neoplastic cells. From the analysis of the data that have appeared in the literature in the last decade, an involvement of immunological mechanisms in NMT is inferred, since in 13 of the 17 described cases serum autoantibodies were found [2,5,7,8,13,16,17,20,21,26], and a good response to plasmapheresis was also reported [21]. Concerning the specificity of these autoantibodies, the most frequent ones are the anti-AChR Abs, which were found in 9 of 19 cases (including ours).…”

Section: Motor Conductionmentioning

confidence: 99%

“…NMT has been described in association with peripheral neuropathies [5,6,14,17,25], plexopathies secondary to radiotherapy [1], lung neoplasm [24], thymoma [5,7,17] myasthenia gravis [5,7,17] and plasmocytoma [26].…”

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confidence: 99%

“…NMT can be induced by penicillamine [18], and immune complexes were found in a patient with NMT [15]; NMT improves after plasmapheresis [21], and the presence of acetylcholine receptor (AchR) antibodies (Abs) were found in a high percentage of patients with NMT [5,7,17]. NMT can also be present in some cases as a paraneoplastic syndrome, as in the myasthenic syndrome of Lambert-Eaton in which an autoimmune mechanism is well established [11,19].…”

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Neuromyotonia, systemic lupus erythematosus and acetylcholine-receptor antibodies

et al. 1998

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“…Patienten mit Malignomen weisen in 10-20% subklinisch eine Muskelschwäche oder eine periphere sensible Polyneuropathie auf.Obwohl der Tumor selbst klinisch häufig bedeutsamer ist [8],ist das Erkennen von Paraneoplasien wichtig, da sie den eigentlichen raumfordernden Tumorsymptomen vorausgehen können [3,4,9].…”

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Polyneuropathie als einziges paraneoplastisches Syndrom bei malignem Thymom

et al. 2003

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Up to 40% of patients with malignant thymoma suffer from paraneoplastic symptoms (90% myasthenia, 10% other symptoms). A 55-year-old patient developed ascending symmetrical sensorimotor tetraparesis. A malignant thymoma without metastases was diagnosed 6 months later. Despite thymectomy followed by radiation and high-dose corticosteroid therapy, the polyneuropathy progressed. Six months after onset, the patient was bound to a wheelchair. Immunosuppressive therapy with cyclophosphamide was initiated, leading to marked remission. After ten cycles, the patient was able to walk independently with walking aids. After the sixth and tenth cycle, respectively, attempts to discontinue immunosuppression led to relapse. In several diagnostic workups, however, there was no tumour relapse. After 13 cycles, cyclophosphamide was replaced by immunoglobulins (0.4 g/kg per day i.v. for 5 days/month) due to progressive renal failure. The patient died just before the second course of this treatment. In conclusion, in the differential diagnosis of rapidly progressive polyneuropathy, a malignant thymoma should be considered, even in the absence of myasthenia. Immunosuppression with cyclophosphamide resulted in amelioration of symptoms in this patient.

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Continuous muscle fiber activity, peripheral neuropathy, and thymoma

Cited by 61 publications

References 12 publications

Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma

Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma

Neuromyotonia, systemic lupus erythematosus and acetylcholine-receptor antibodies

Polyneuropathie als einziges paraneoplastisches Syndrom bei malignem Thymom

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