Neonatal hypoglycemic brain injury is a cause of infantile spasms

Yang, Guang; Zou, Li‐Ping; Wang, Jing; Shi, Xiaolan; Tian, Shuo; Yang, Xiaofan; Ju, Jun; Yao, Hongxiang; Liu, Yujie

doi:10.3892/etm.2016.3107

Cited by 17 publications

(12 citation statements)

References 32 publications

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“…To the best of our knowledge, there are no reports in the literature that describe an association between GSD and WS, although metabolic disorders can cause symptomatic WS. It is known that hypoglycemia is a cause of WS [1,[3][4]. In these cases, hypoglycemia was caused by hyperinsulinemia or brain injury confirmed by head magnetic resonance imaging during the neonatal period.…”

Section: Discussionmentioning

confidence: 99%

West syndrome associated with glycogen storage disease type 1b

Yoshida

Arai

Kasai

et al. 2019

E&S

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A girl born at 41 weeks of gestation experienced a seizure involving the extremities 12 hours after birth. Hypoglycemia was observed, although various tests failed to identify the cause of hypoglycemia. Thereafter, her development and weight gain were normal, and no hypoglycemia was observed. At five months of age, atypical spasms occurred concomitant with hypoglycemia. The patient was diagnosed with glycogen storage disease type 1b based on genetic testing. Hypsarrhythmia on electroencephalography (EEG) revealed West syndrome. The hypoglycemia were treated by continuous nasogastric tube feeding and neutropenia by injection of granulocyte-colony stimulating factor (G-CSF). The spasms and hypsarrhythmia did not improve by administration of oral antiepileptic drugs including vigabatrin. However, the symptoms improved with 0.011 mg/kg adrenocorticotropic hormone (ACTH) and G-CSF therapy, with no apparent adverse effects. ACTH was a safe treatment for an immunocompromised patient.

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Section: Discussionmentioning

confidence: 99%

West syndrome associated with glycogen storage disease type 1b

Yoshida

Arai

Kasai

et al. 2019

E&S

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“…There is an increasing concern with optimal cutoff values for diagnosis of hypoglycemia and initiation of therapy, as the plasma glucose concentration associated with neurological damage is still unknown [25], although said damage is known to be permanent [5]. Thus, the current trend would be to consider a diagnosis os hypoglycemia at relatively higher glucose levels (<2.6 mmol/L or 47 mg/dL) [27].…”

Section: Discussionmentioning

confidence: 99%

“…It is known that some neonates are more likely to develop hypoglycemia than others [4], and that low blood glucose levels can lead to permanent central nervous system damage [5]. The cut off level which should prompt treatment remains unclear, especially in asymptomatic neonates [6].…”

Section: Introductionmentioning

confidence: 99%

A Concept Map of Neonatal Hypoglycemia

G¹,

Acpm²,

Ad³

et al. 2017

Neonat Pediatr Med

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Objectives: To analyze current management of neonatal hypoglycemia and establish a concept map based on testimonials from physicians who use capillary blood glucose tests to guide management. Methods:This was an observational, descriptive, mixed qualitative and quantitative study. A questionnaire was administered to physicians, seeking to characterize their responses to neonatal hypoglycemia in pregnancy, labor and delivery, and the immediate neonatal period. Data collection was performed with two groups of Brazilian physicians: neonatologists (group GN) and pediatricians (group GP). A Likert-type scale was used to collect responses. The Mann-Whitney test and Fisher's exact test were used for statistical analysis of continuous and categorical variables respectively. Statistical significance was accepted at 5%. Principal components analysis with varimax rotation and Kaiser normalization was used to verify the structure of question/answer factors in the two groups of professionals. A concept map was constructed using the Cmap Tools Knowledge Kit, version 5.05.01. Results:Of the 98 questionnaires analyzed, 34.7% were completed by neonatologists (n=34/98) and 65.3% by pediatricians (n=64/98). Mean age in the two groups (GN and GP) was 42.4 years (SD: 11.78; 95% CI: 40.10, 44.88; p=0.597), with a significant difference in age in men (mean=50 years; SD: 10.35; 95% CI: 45.20, 54.90; p<0.001); 79.2% of respondents (n=78/98) were women. Overall, 32.7% of respondents (n=32/98) claimed they did not currently work with neonates. Respondents in GN had completed more postgraduate courses (p=0.38). The two groups responded similarly to all questions. The responses highlighted the importance of values lower than 40 mg/dL in the diagnosis of neonatal hypoglycemia, as well as the indication of intravenous infusion when glucose was below 40 mg/dL in symptomatic neonates. Immediate institution of periodic capillary blood glucose measurement was recommended for the following groups of neonates: those born to diabetic mothers, those with intrauterine growth restriction, small for gestational age, large for gestational age, preterm neonates, septic neonates, and those with birth asphyxia. Conclusions:Capillary blood glucose is part of routine neonatal management, especially preventive, in light of the possibility of neonatal hypoglycemia. Our findings highlight that, in high-risk gestational groups, the entire neonatal team should be focused on the risk of hypoglycemia. The development of management algorithms based on the use of peripheral blood glucose test strips has contributed to streamlining the management of neonatal hypoglycemia.

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“…Patients with persistent neonatal hypoglycemia and specific syndromic etiology such as those that may require pancreatectomy for persistent hyperinsulinemia may develop IS following variable latent time . In earlier studies, neonatal hypoglycemia (low blood glucose levels, <2.6 mmol/L) was reported in some of the IS patients in 2 long‐term population studies in Finland .…”

Section: Metabolic Errors In Organic Moleculesmentioning

confidence: 99%

“…Hypoglycemia may have profound effects on the brain where it is used as a primary energy source, even though the exact mechanism leading to IS has yet to be discovered. The effects may include but are not limited to mitochondrial energy deficiencies and increased susceptibility to hypoxic‐ischemic brain injuries, and may interfere with any of the proposed mechanisms of IS/WS development . The effects of hypoglycemia may be age specific.…”

Section: Metabolic Errors In Organic Moleculesmentioning

confidence: 99%

Metabolic etiologies in West syndrome

2018

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SummaryWest syndrome (WS) is an early life epileptic encephalopathy associated with infantile spasms, interictal electroencephalography (EEG) abnormalities including high amplitude, disorganized background with multifocal epileptic spikes (hypsarrhythmia), and often neurodevelopmental impairments. Approximately 64% of the patients have structural, metabolic, genetic, or infectious etiologies and, in the rest, the etiology is unknown. Here we review the contribution of etiologies due to various metabolic disorders in the pathology of WS. These may include metabolic errors in organic molecules involved in amino acid and glucose metabolism, fatty acid oxidation, metal metabolism, pyridoxine deficiency or dependency, or acidurias in organelles such as mitochondria and lysosomes. We discuss the biochemical, clinical, and EEG features of these disorders as well as the evidence of how they may be implicated in the pathogenesis and treatment of WS. The early recognition of these etiologies in some cases may permit early interventions that may improve the course of the disease.

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Neonatal hypoglycemic brain injury is a cause of infantile spasms

Cited by 17 publications

References 32 publications

West syndrome associated with glycogen storage disease type 1b

West syndrome associated with glycogen storage disease type 1b

A Concept Map of Neonatal Hypoglycemia

Metabolic etiologies in West syndrome

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