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Open Access 01-12-2022 | Myasthenia Gravis | Case report

Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report

Authors: Jinghong Zhang, Xusheng Huang, Qiang Shi

Published in: BMC Neurology | Issue 1/2022

Abstract

Background

Lambert-Eaton myasthenic syndrome (LEMS) is a type of paraneoplastic syndrome that may initially manifest itself with proximal weakness and gait abnormalities. Approximately up to 50% of LEMS patients have a primary autonomic dysfunction.

Case presentation

We present here a case of a 75-year-old male with symmetric proximal muscle weakness, dry mouth and constipation. The cutaneous response to scratch and upright tilt-table testing were positive. A repetitive nerve stimulation test showed that there was a decremental response of compound muscle action potential (CMAP) amplitude at 3 Hz while an incremental response at 20 Hz. The presence of antibodies against voltage-gated calcium channels (VGCC) confirmed the diagnosis. Because of the prominent symptom of autonomic disorder, the patient further underwent the test of skin sympathetic response (SSR). Lower amplitude and longer response duration were found in palms, while it evoked no response in soles.

Conclusions

In this case, we present the detailed results of SSR test on a patient suffering LEMS with autonomic disorder. Since autonomic dysfunction has a significant impact on clinical management and SSR test is an effective detection method, we recommend that SSR test be performed on patients with LEMS regularly.

Eaton LM, Lambert EH. Electromyography and electric stimulation of nerves in diseases of motor unit: observations on myasthenic syndrome associated with malignant tumors. J Am Med Assoc (JAMA). 1957;163(13):1117–24.CrossRef

Sally AW. Autonomic. Dysfunction in Lambert-Eaton myasthenic syndrome. Clin Auton Res. 2001;11(3):145–54.CrossRef

Kesner VG, Oh SJ, Dimachkie MM, Barohn RJ. Lambert-Eaton Myasthenic syndrome. Neurol Clin. 2018;36(2):379–94. https://doi.org/10.1016/j.ncl.2018.01.008.CrossRefPubMedPubMedCentral

Lorenzoni Paulo J, Scola Rosana H, Kamoi Kay CS, et al. Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases. Arq Neuropsiquiatr. 2010;68(6):849–54. https://doi.org/10.1590/S0004-282X2010000600004.CrossRefPubMed

O'Suilleabhain P, Low PA, Lennon VA. Autonomic dysfunction in the Lambert-Eaton myasthenic syndrome: serologic and clinical correlates. Neurology. 1998;50(1):88–93. https://doi.org/10.1212/WNL.50.1.88.CrossRefPubMed

Vetrugno R, Liguori R, Cortelli P, Montagna P. Sympathetic skin response: basic mechanisms and clinical applications. Clin Auton Res. 2003;13:256–314.CrossRef

Oneill JH, Murray NMF, Newsom-davis J. The Lambert-Eaton myasthenic syndrome. A review of 50 cases. Brain J Neurol. 1988;111(3):577–96.CrossRef

Titulaer MJ, Maddison P, Sont JK, et al. Clinical dutch-english Lambert-Eaton myasthenic syndrome (LEMS) tumor association prediction score accurately predicts small-cell lung Cancer in the LEMS. J Clin Oncol. 2011;29(7):902–8.CrossRef

Purroy García F, Comabella M, Raguer N, et al. Lambert-Eaton myasthenic syndrome associated with sarcoidosis. J Neurol. 2005;252(9):1127–8. https://doi.org/10.1007/s00415-005-0807-y.CrossRefPubMed

10.

Merino-Ramírez MÁ, Bolton CF. Review of the diagnostic challenges of Lambert–Eaton syndrome revealed through three case reports. Can J Neurol Sci. 2016;43(05):635–47. https://doi.org/10.1017/cjn.2016.268.CrossRefPubMed

11.

Yuzhou G, Benhong L, Hua D, et al. Manifestations of autonomic nervous system in Lambert-Eaton myasthenic syndrome (in Chinese). Chin J Neurol. 2011;44(12):841–3. https://doi.org/10.3760/cma.j.issn.1006-7876.2011.12.00.CrossRef

12.

Ellaway PH, Kuppuswamy A, Nicotra A, Mathias CJ. Sweat production and the sympathetic skin response: improving the clinical assessment of autonomic function. Auton Neurosci. 2010;155(1-2):109–14. https://doi.org/10.1016/j.autneu.2010.01.008.CrossRefPubMed

13.

Nazhel B, Ankan Z, Irkeç C, Karakc H. SSR abnormalities in chronic alcoholics. Addictive Behaviors. 2007;32(6):1290–4. https://doi.org/10.1016/j.addbeh.2006.08.00.CrossRef

14.

Vernino S, Hopkins S, Wang Z. Autonomic ganglia acetylcholine receptor antibodies, and autoimmune ganglionopathy. Auton Neurosci. 2009;146(1-2):3–7. https://doi.org/10.1016/j.autneu.2008.09.005.CrossRefPubMed

15.

Nikolić A, Perić S, Nišić T, et al. The presence of dysautonomia in different subgroups of myasthenia gravis patients. J Neurol. 2014;261(11):2119–27. https://doi.org/10.1007/s00415-014-7465-x.CrossRefPubMed

16.

Nalbantoglu M, Akalin MA, Gunduz A, Kiziltan M. Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis: a prospective study. Ideggyógy Sz. 2021;74(1-2):33–40.CrossRef

17.

Lennon VA, Kryzer TJ, Griesmann GE, et al. Calcium-Channel antibodies in the Lambert-Eaton syndrome and other Paraneoplastic syndromes. N Engl J Med. 1995;332(22):1467–75. https://doi.org/10.1056/nejm199506013322203.CrossRefPubMed

18.

Motomura M, Lang B, Johnston I, et al. Incidence of serum anti-P/Q-type and anti-N-type calcium channel autoantibodies in the Lambert-Eaton myasthenic syndrome. J Neurol Sci. 1997;147(1):35–42. https://doi.org/10.1016/s0022-510x(96)05303-8.CrossRefPubMed

19.

Vincent A. Immunology of disorders of neuromuscular transmission. Acta Neurol Scand. 2006;113(s183):1–7. https://doi.org/10.1111/j.1600-0404.2006.00605.x.CrossRef

20.

Wan EC, Gordon TP, Jackson MW. Autoantibodies to calcium channels in type 1 diabetes mediate autonomic dysfunction by different mechanisms in colon and bladder and are neutralized by antiidiotypic antibodies. J Autoimmun. 2008;31(1):66–72. https://doi.org/10.1016/j.jaut.2008.03.004.CrossRefPubMed

Title: Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report
Authors: Jinghong Zhang
Xusheng Huang
Qiang Shi
Publication date: 01-12-2022
Publisher: BioMed Central
Keywords: Myasthenia Gravis
Constipation
Lambert-Eaton Myasthenic Syndrome
Nerve Stimulation
Constipation
Published in: BMC Neurology / Issue 1/2022
Electronic ISSN: 1471-2377
DOI: https://doi.org/10.1186/s12883-022-02625-1

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report

Abstract

Background

Case presentation

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case presentation

Conclusions

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