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CSF protein profile in infantile spasms. Influence of etiology and ACTH or dexamethasone treatment
Insights
Infantile spasms in children are linked to increased blood-cerebrospinal fluid barrier permeability, particularly for albumin. Treatment with adrenocorticotropic hormone (ACTH) or dexamethasone normalized this permeability and reduced spasms.
Area of Science:
- Pediatric Neurology
- Neurochemistry
- Clinical Neuroscience
Background:
- Infantile spasms are a severe epilepsy syndrome in infants.
- The integrity of the blood-cerebrospinal fluid (CSF) barrier is crucial for brain health.
- Alterations in CSF protein profiles can indicate neurological dysfunction.
Purpose of the Study:
- To investigate blood-cerebrospinal fluid barrier function in infants with infantile spasms.
- To correlate CSF protein changes with etiology and treatment response.
- To assess the impact of adrenocorticotropic hormone (ACTH) and dexamethasone on barrier function.
Main Methods:
- Quantitative zone electrophoresis on agarose gel analyzed CSF proteins.
- 107 children (3-24 months) were studied: 50 with infantile spasms, 41 controls, 16 with meningitis.
- Infantile spasms group categorized by etiology: symptomatic, doubtful, cryptogenetic.
Main Results:
- Infants with infantile spasms showed increased blood-CSF barrier permeability, especially for albumin, before treatment.
- Severity of protein leakage correlated with etiological category (symptomatic > doubtful > cryptogenetic).
- Treatment with ACTH or dexamethasone for 2-11 weeks normalized barrier permeability and reduced hypsarrhythmia.
Conclusions:
- Blood-CSF barrier dysfunction is a significant finding in infantile spasms.
- Etiology influences the degree of barrier impairment.
- ACTH and dexamethasone effectively restore barrier integrity and are associated with clinical improvement.
Abstract:
CSF proteins in 107 children ranging from 3 to 24 months of age were analyzed by means of quantitative zone electrophoresis on agarose gel. Subjects included 50 children with infantile spasms, 41 children without CNS disease serving as controls, and 16 infants with acute aseptic meningitis who demonstrated the protein pattern of blood-CSF barrier disturbance. Children with infantile spasms were subdivided into several groups according to etiological categories: symptomatic (pre-, peri-, and postnatal), doubtful, and cryptogenetic. Before any treatment was started, these children showed the protein profile of increased permeability of the blood-CSF barrier, especially for albumin. There was an association between the severity of the changes and the etiological category. Changes were most marked in the symptomatic group, intermediate in the doubtful group, and slight in the cryptogenetic group. No child with infantile spasms of doubtful or unknown etiology revealed changes of the immunoglobulin-containing gamma fractions. Ten children who had received adrenocorticotropic hormone (ACTH) or dexamethasone for 2-11 weeks no longer showed any protein leakage into the CSF. The period of ACTH or dexamethasone treatment was characterized by the following findings: the disappearance or reduction of hypsarrhythmia; the reappearance of normal cerebrovascular permeability for protein; and the occurrence of reversible dilatation of the subarachnoid and intraventricular spaces.