Caring for a Laboring Women With Amyotrophic Lateral Sclerosis

Abstract

A case presentation of a pregnant 32-year-old woman with advanced amyotrophic lateral sclerosis who presented in labor at 38+6 weeks gestation is described. Amyotrophic lateral sclerosis is a progressive neuromuscular disease that attacks nerve cells in the brain and spinal cord. It is usually diagnosed after the fourth decade of life, and is more commonly seen in men than women; thus, it is rarely found in the obstetric population. When the perinatal team is confronted with a pregnant woman with ALS,patient management can be a challenge.

Amyotrophic lateral sclerosis in pregnancy is associated with a vascular endothelial growth factor promoter genotype

“…In the 1950s Huston et al [24] reported the occurrence of ALS and pregnancy in 17 women in Guam, where the incidence was approximately 100 times greater than elsewhere, and the disease was found to be predominantly familial. Another 15 cases of ALS in pregnancy have been reported in the medical literature [5][6][7][8][9][10][11][12][13][14][15][16][17]. In one case of apparently sALS, Weiss and co-workers [15] reported an alanine for valine substitution in codon 4 in the SOD1 gene.…”

“…Two of the remaining 14 patients had a strong family history of ALS, but the genetic aspects of these cases were not investigated [8,10]. The median age at onset of the patients described so far in the literature [5][6][7][8][9][10][11][12][13][14][15][16][17], including our patients with ALS in pregnancy, is 31.5 years (range 21-42 years). This is in contrast with the median older age of patients described in the literature having the sporadic form of the disease [1,25], whereas it is closer to the earlier age of onset observed in the familial form, which may actually coincide with reproductive age [18].…”

“…Recently, ALS has been indicated as a syndrome characterized by degeneration of motor neurons presenting with different phenotypes, probably related to different pathogenetic mechanisms [4]. In this context, ALS during pregnancy has been reported since 1967 [5][6][7][8][9][10][11][12][13][14][15][16][17]. This association up to now has been considered rare and possibly coincidental as no pathogenic relationship between the disease and pregnancy has been demonstrated.…”

Revista Mexicana De Neurociencia Mayo 2018

“…1,2 La ELA durante el embarazo es poco común, sólo 18 casos se han descrito a nivel mundial. [3][4][5][6][7][8][9][10][11][12][13] En México, es el segundo caso reportado. 14 La serie más grande de esta asociación se describió en la isla de Guam en 1956 con una mayor prevalencia de la variante familiar de la enfermedad.…”