Fulminant amyotrophic lateral sclerosis manifesting in a young woman during pregnancy

Solodovnikova, Yuliia; Kobryn, Anastasiia; Ivaniuk, Alina; Сон, А. С.

doi:10.1007/s10072-021-05175-5

Cited by 4 publications

(3 citation statements)

References 9 publications

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“…In our cohort, the mean age of symptom onset was 37.81 years (± 6.83), with a median age of 38.00 years. In our study, the mean diagnostic delay was 12 (3–48) months, which was significantly shorter than in other reports in China ( 10 , 15 , 16 ). The mean baseline ALSFRS-R total score was 37.65 (± 5.63) (range 24–46), which indicated a relatively milder neurologic deficit.…”

Section: Discussioncontrasting

confidence: 73%

“…The few studies that evaluated the correlation between pregnancy or fertility and ALS progression have yielded contradictory results. Some case reports indicated rapid progression of ALS during pregnancy ( 7 , 10 , 25 , 26 ), while some argued that the patients were stable during the pregnancy and progressed rapidly after delivery ( 27 , 28 ). Therefore, we compared disease progression between sexes and in different subgroups based on temporal relationship between symptom onset and pregnancy.…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, the proportion of women diagnosed with ALS in close temporal proximity to their pregnancy, which in our study was defined as within 1 year (365 days) before or after the pregnancy (1 year before pregnancy was defined as the period between 395 and 30 days before conception) has also increased. However, whether pregnancy and fertility contribute to disease progression is unclear ( 7 – 10 ). Some studies have indicated that pregnancy could attenuate protection through elevated levels of estrogen and progesterone, which are associated with neuroprotection.…”

Section: Introductionmentioning

confidence: 99%

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Fertility Does Not Alter Disease Progression in ALS Patients of Childbearing Age: A Three Centers Retrospective Analysis in Southern China

et al. 2022

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BackgroundLimited data exists on the clinical features of patients with amyotrophic lateral sclerosis (ALS) during reproductive ages.ObjectiveOur study characterized the clinical features of ALS and the effects of pregnancy on disease progression in patients with ALS.MethodsWe performed a retrospective study of female patients with ALS in three ALS research centers in southern China from 2009 to 2021. Data regarding fertility status, and clinical and genetic features, were collected. Age-matched male patients with ALS served as controls. The patients were divided into the following two subgroups: patients with symptom onset within 1 year of pregnancy and patients with symptom onset over 1 year group after pregnancy.ResultsA total of 52 female and 52 matched male patients were enrolled. There were no differences in female and male patients in the mean age of symptom onset, the mean baseline ALSFRS-R score, or median reduction of ALSFRS-R score (p > 0.05). The mean age of first pregnancy was 25.57 ± 4.40) years. The mean age of first pregnancy in the over 1 year group was lower than that in the within 1 year group (p= 0.01). There was no difference in the median reduction of ALSFRS-R between the two subgroups. In the univariate analysis, diagnostic delay was highly correlated with the disease progression, with short delay representing rapid progress. No multicollinearity was found among every variable. In addition, 40.38% patients carried ALS-related gene variants. The proportion with gene mutations in the within 1 year group was higher than that in the over 1 year group (p < 0.01). Furthermore, SETX was the most frequently mutated gene in this cohort (16.67%) including 4 uncertain mutation.ConclusionPregnancy and fertility were not associated with disease progression. Diagnostic delay was correlated with disease progression in this cohort. In addition, SETX might be a gene of concern for ALS patients of childbearing age.

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Section: Discussioncontrasting

confidence: 73%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Fertility Does Not Alter Disease Progression in ALS Patients of Childbearing Age: A Three Centers Retrospective Analysis in Southern China

et al. 2022

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Clinical characteristics, course, and outcomes of amyotrophic lateral sclerosis overlapping with pregnancy: a systematic review of 38 published cases

Hamad,

Amer,

AL Mawla

et al. 2023

Neurol Sci

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Objectives Amyotrophic lateral sclerosis (ALS) is a rare and fatal neurodegenerative disease that can overlap with pregnancy, but little is known about its clinical characteristics, course, and outcomes in this context. This systematic review aimed to synthesize the current evidence on ALS overlapping with pregnancy. Methods We comprehensively searched four databases on February 2, 2023, to identify case studies reporting cases of ALS overlapping with pregnancy. Joanna Brigs Institute tool was followed to assess the quality of the included studies. Results Twenty-six articles reporting 38 cases were identified and included in our study. Out of the 38 cases, 18 were aged < 30 years. The onset of ALS was before pregnancy in 18 cases, during pregnancy in 16 cases, and directly after pregnancy in 4 cases. ALS progression course was rapid or severe in 55% of the cases during pregnancy, and this percentage reached 61% in cases with an onset of ALS before pregnancy. While ALS progression course after pregnancy was rapid or severe in 63% and stable in 37% of the cases. Most cases (95%) were able to complete the pregnancy and gave live birth. However, preterm delivery was common. For neonates, 86% were healthy without any complications. Conclusion While pregnancy with ALS is likely to survive and result in giving birth to healthy infants, it could be associated with rapid or severe progression of ALS and result in a worse prognosis, highlighting the importance of close monitoring and counselling for patients and healthcare providers.

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Spinal Cord Disorders

Preston,

Collins

2024

Obstetric Anesthesia and Uncommon Disorders

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Fulminant amyotrophic lateral sclerosis manifesting in a young woman during pregnancy

Cited by 4 publications

References 9 publications

Fertility Does Not Alter Disease Progression in ALS Patients of Childbearing Age: A Three Centers Retrospective Analysis in Southern China

Fertility Does Not Alter Disease Progression in ALS Patients of Childbearing Age: A Three Centers Retrospective Analysis in Southern China

Clinical characteristics, course, and outcomes of amyotrophic lateral sclerosis overlapping with pregnancy: a systematic review of 38 published cases

Spinal Cord Disorders

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