Tabla I. Características demográficas y clínicas de los pacientes con diagnóstico de síndrome miasteniforme de Lambert-Eaton. |
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Paciente |
Edad en el momento del diagnóstico (años) |
Sexo |
Debilidad en los miembros inferiores inicial |
Hiporreflexia/arreflexia |
Hipotensión ortostática |
Boca seca/ojo seco |
Estreñimiento |
Impotencia |
Disnea |
Disartria |
Disfonía |
Disfagia |
Diplopía |
Pérdida de peso |
Neoplasia asociada |
Puntuación DELTA-P |
1 |
68 |
F |
X |
X |
X |
X |
X |
X |
X |
4 |
||||||
2 |
59 |
F |
X |
X |
X |
3 |
||||||||||
3 |
72 |
M |
X |
X |
X |
X |
X |
X |
X |
X |
4 |
|||||
4 |
35 |
F |
X |
X |
X |
X |
3 |
|||||||||
5 |
69 |
M |
X |
X |
X |
X |
X |
X |
2 |
|||||||
6 |
74 |
M |
X |
X |
X |
X |
X |
2 |
||||||||
7 |
16 |
F |
X |
X |
X |
X |
X |
1 |
||||||||
8 |
70 |
M |
X |
X |
X |
X |
X |
X |
x |
X |
X |
5 |
||||
9 |
58 |
M |
X |
X |
X |
X |
X |
X |
X |
4 |
||||||
10 |
59 |
F |
X |
X |
1 |
|||||||||||
11 |
68 |
M |
X |
X |
X |
X |
X |
X |
X |
X |
X |
X |
6 |
|||
12 |
73 |
F |
X |
X |
X |
X |
X |
X |
5 |
|||||||
13 |
70 |
F |
X |
X |
X |
X |
X |
2 |
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DELTA-P: Dutch-English LEMS Tumor Association Prediction. |
Tabla II. Detalle del tratamiento de los pacientes con diagnóstico de LEMS. |
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Paciente |
Fecha del diagnóstico |
IgIV en el diagnóstico |
Dosis de IgIV |
Fecha de inicio |
Piridostigmina |
Fecha de inicio de la piridostigmina |
Respuesta subjetiva a la piridostigmina |
3,4-DAP |
Fecha de inicio |
Respuesta |
1 |
15.08.2010 |
X |
27.03.2014 |
X |
||||||
2 |
25.01.2017 |
X |
10.04.2017 |
X |
X |
16.11.2017 |
X |
|||
3 |
12.03.2009 |
X |
14.04.2009 |
X |
||||||
4 |
22.05.2004 |
X |
13.05.2004 |
X |
06.10.2004 |
|||||
5 |
30.10.2013 |
X |
15.07.2012 |
X |
X |
17.04.2014 |
||||
6 |
15.08.2015 |
X |
01.09.2015 |
X |
X |
15.09.2015 |
||||
7 |
16.03.2009 |
X |
16.02.2009 |
X |
15.06.2011 |
X |
X |
22.07.2011 |
X |
|
8 |
20.08.2013 |
X |
2 g/kg |
24.08.2013 |
X |
20.08.2013 |
X |
|||
9 |
10.09.2007 |
X |
24.01.2008 |
X |
15.03.2008 |
|||||
10 |
19.09.2007 |
|||||||||
11 |
19.10.2018 |
X |
2 g/kg |
19.10.2018 |
X |
24.08.2018 |
X |
X |
15.03.2019 |
X |
12 |
05.02.2020 |
X |
2 g/kg |
06.02.2020 |
||||||
13 |
24.01.2020 |
X |
25.03.2020 |
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3,4-DAP: 3,4-diaminopiridina; IgIV: inmunoglobulina endovenosa; LEMS: síndrome miasteniforme de Lambert-Eaton. |
Lambert-Eaton myasthenic syndrome Introduction. Early diagnosis based on clinical findings, neurophysiological studies and serum antibody titres allows early initiation of symptomatic treatment and oncological 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. Key words. Lambert-Eaton myasthenic syndrome. LEMS. Neuromuscular plaque. P/Q-type voltage-gated calcium channels. Paraneoplastic syndrome. Small-cell lung carcinoma. |