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Læknablaðið - 01.11.2023, Qupperneq 28

Læknablaðið - 01.11.2023, Qupperneq 28
512 L ÆKNABL AÐIÐ 2023/109 R A N N S Ó K N E N G L I S H S U M M A R Y A 50 year old man with progressive weakness in lower limbs Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare neurological disorder caused by autoimmune antibodies attacking the presynaptic neuromuscular junction, in some cases caused by underlying cancer. The main clinical finding is fluctuating weakness of the extremities and a triad of symtoms can help physicians suspect the disease. A key to the diagnosis are the electrophysiological abnormalities seen in this group of diseases. Treatment with symtomatic and/or immunosuppressive therapy is important as well as a workup for possible malignancy. This article identifies the clinical features, diagnosis and treatment of this uncommon disease. doi 10.17992/lbl.2023.11.768 Brynhildur Thors1 Bjarni Guðmundsson2 1Neurological department Karolinska University Hospital, Stockholm 2Neurology department Landspitali-University Hospital. Correspondence: Brynhildur Thors, bthors@gmail.com Key words: Lambert-Eaton Myasthenic Syndrome, proximal weakness, neuromuscular junction disorder, repetitive nerve stimulation, paraneoplastic phenomenon. Heimildir 1. Dourado E, Bottazzi F, Cardelli C, et al. Idiopathic inflammatory myopathies: one year in review 2022. Clin Exp Rheumatol 2023; 41: 199­231. 2. Galiero R, Caturano A, Vetrano E, et al. Peripheral Neuropathy in Diabetes Mellitus: Pathogenetic Mechanisms and Diagnostic Options. IJMS 2023; 24: 3554. 3. London ZN. A Structured Approach to the Diagnosis of Peripheral Nervous System Disorders. CONTINUUM: Lifelong Learning in Neurol 2020; 26: 1130­60. 4. Zalewski NL, Lennon VA, Lachance DH, et al. P/Q­ and N­type calcium­channel antibodies: Oncological, neurological, and serological accompaniments: Neuronal Calcium­Channel Abs. Muscle Nerve 2016; 54: 220­7. 5. Schoser B, Eymard B, Datt J, et al. Lambert–Eaton myasthenic syndrome (LEMS): a rare autoimmune presynaptic disorder often associated with cancer. J Neurol 2017; 264: 1854­63. 6. Muñiz­Castrillo S, Vogrig A, Honnorat J. Associations between HLA and autoimmune neurological diseases with autoantibodies. Autoimmun Highlights 2020; 11: 2. 7. McGrogan A, Sneddon S, de Vries CS. The Incidence of Myasthenia Gravis: A Systematic Literature Review. Neuroepidemiol 2010; 34: 171­83. 8. Kesner VG, Oh SJ, Dimachkie MM, et al. Lambert­Eaton Myasthenic Syndrome. Neurologic Clin 2018; 36: 379­94. 9. Ebright MJ, Li SH, Reynolds E, et al. Unintended consequences of Mayo paraneoplastic evaluations. Neurology 2018; 91: e2057­66. 10. Titulaer MJ, Klooster R, Potman M, et al. SOX Antibodies in Small­Cell Lung Cancer and Lambert­Eaton Myasthenic Syndrome: Frequency and Relation With Survival. JCO 2009; 27: 4260­7. 11. Titulaer MJ, Maddison P, Sont JK, et al. Clinical Dutch­English Lambert­Eaton Myasthenic Syndrome (LEMS) Tumor Association Prediction Score Accurately Predicts Small­Cell Lung Cancer in the LEMS. JCO 2011; 29: 902­8. 12. Morren JA, Li Y. Myasthenia gravis: Frequently asked questions. CCJM 2023; 90: 103­ 13. 13. Sanders DB, Juel VC, Harati Y, et al. 3,4­diaminopyridine base effectively treats the weakness of Lambert­Eaton myasthenia. Muscle Nerve 2018; 57: 561­8. 14. Weinberg DH. Lambert­Eaton myasthenic syndrome: Treatment and prognosis. med­ ilib.ir/uptodate/show/5134 ­ september 2023. 15. Weingarten TN, Araka CN, Mogensen ME, et al. Lambert­Eaton myasthenic syndrome during anesthesia: a report of 37 patients. J Clin Anesthes 2014; 26: 648­53. 16. Cautionary drugs. Myasthenia Gravis Foundation of America. myasthenia.org/ MG­Community/Cautionary­Drugs ­ september 2023. 17. Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: Executive summary. Neurol 2016; 87: 419­25. Síðumúli 16 I 108 Reykjavík I Sími 580 3900 I fastus.is Vscan Air CL og SL eru lítil og nett ómskoðunartæki sem hvort um sig hafa tvo mismunandi ómhausa sem skila kristaltærum myndum beint í spjaldtölvu eða síma. Nánari upplýsingar og ráðgjöf veita: Guðmundur Hreiðarsson: gh@fastus.is – S: 891 6109 Erna Dís Brynjúlfsdóttir: erna@fastus.is – S:843 3919 Vscan Air SL Tveir ómhausar: „Sector-phased“ ómhaus, til að meta ástand hjarta, og linear ómhaus. Vscan Air CL Tveir ómhausar: Abdominal og linear ómhaus . HealthCo Nýtt Vscan Air

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