Lambert-Eaton myasthenic syndrome

被引:0
|
作者
Hernandez, Micaela A. [1 ]
Kohler, Alejandro A. [1 ,2 ]
Marrodan, Mariano [1 ]
Lautre, Andrea [1 ,2 ]
Brand, Patricio [1 ,2 ]
Nogues, Martin [1 ,2 ]
Barroso, Fabio [1 ,2 ]
机构
[1] Fleni, Dept Neurol, Buenos Aires, DF, Argentina
[2] Fleni, Secc Enfermedades Neuromusculares, Buenos Aires, DF, Argentina
关键词
Lambert-Eaton myasthenic syndrome; LEMS; Neuromuscular plaque; P/Q-type voltage-gated calcium channels; Paraneoplastic syndrome; Small-cell lung carcinoma;
D O I
10.33588/rn.7303.2021140
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Introduction. Early diagnosis based on clinical findings, neurophysiological studies and serum antibody titres allows early initiation of symptomatic treatment and ontological screening. Reports of patients with LEMS in Latin America are scarce. Aim. This article aims to describe the characteristics of patients with LEMS from a private centre in Buenos Aires, Argentina, and to compare them with those of other series that have been published. Patients and methods. The medical records of 13 patients with LEMS with clinical findings, compatible electromyogram and/or positive antibodies were reviewed. Follow-up was performed until associated neoplasia was ruled out or confirmed according to the recommended algorithms. Results. Four patients were diagnosed with T-LEMS, two of them with small-cell lung carcinoma. Of the nine patients with NT-LEMS, five had a DELTA-P score of 3 and 4. Nine patients presented with the classic clinical triad from the onset of the disease. All patients had electromyogram findings compatible with presynaptic neuromuscular plaque defect. Of the total, 70% improved symptomatically with pyridostigmine. Conclusions. The clinical findings, together with compatible neurophysiological studies, are sufficient for the diagnosis of LEMS. The relationship between the DELTA-P score and the risk of small-cell lung carcinoma could not be replicated. Symptomatic treatment with pyridostigmine represents an effective therapeutic alternative.
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页码:96 / 100
页数:5
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