Læknablaðið - 01.11.2023, Side 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