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Published on: October 21, 2014
Globus pallidus glial pigment and its changes with age and chronic illness in childhood
Floyd H Gilles1, C Jane Tavaré
1Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles and University of Southern California Keck School of Medicine, 90027, USA.
Insights
Glial lipopigment accumulates faster in the globus pallidus of children with cystic fibrosis compared to healthy children. Neuronal lipopigment was not observed in either group during childhood.
Area of Science:
- Neuroscience
- Pediatrics
- Pathology
Background:
- Glial lipopigment, or lipofuscin, is a cellular pigment that accumulates with age.
- In chronic childhood diseases, glial lipopigment deposition in the globus pallidus may differ from typical age-related accumulation.
- Neuronal lipopigment is typically observed later in life, but its presence in childhood is not well-documented.
Purpose of the Study:
- To compare the age-related accumulation of glial lipopigment in the globus pallidus between children with cystic fibrosis and healthy controls.
- To investigate the presence of neuronal lipopigment in the globus pallidus during childhood.
- To characterize the patterns and rates of glial pigment deposition.
Main Methods:
- Observational study comparing brain tissue from 37 children with cystic fibrosis (0-23 years) and 17 controls (0-18 years).
- Histochemical characterization of lipopigment.
- Regression models used to analyze pigment deposition rates.
Main Results:
- Intraglial pallidal pigment accumulated slowly in controls, appearing as globules and granular clusters.
- In cystic fibrosis, both pigment forms began accumulating earlier and at a significantly faster rate.
- An accelerated rate of accumulation was noted between 8 and 10 years of age in cystic fibrosis patients.
- No pallidal neuronal lipofuscin was detected in any child, regardless of health status.
Conclusions:
- Globus pallidus glial cells uniquely accumulate lipofuscin before neurons, even in childhood.
- Glial cells in the globus pallidus appear particularly susceptible to systemic effects of chronic illnesses like cystic fibrosis.
- The accelerated glial lipopigment deposition in cystic fibrosis suggests a heightened cellular stress or altered metabolic process.
Abstract:
Glial lipopigment appears in the globus pallidus without accumulating in neurons (except for late adolescence) in multiple chronic childhood diseases. In this observational study, we compared the age-related development of glial pigmentation in children with the chronic illness (cystic fibrosis) and children dying acutely. A secondary goal was to search for pallidal neuronal lipopigment in childhood. We recorded pigmentation in the brains of 37 consecutive cystic fibrosis children ranging in age from 0-23 yr and in 17 controls ranging in age from 0-18 yr. We characterized the lipofuscin histochemically and used several regression models to describe the mode of deposition. We observed that in the controls, intraglial pallidal pigment accumulated in 2 forms (relatively large globules and, separately, as clusters of fine granules) at a slow rate during childhood. In cystic fibrosis, both forms of pallidal glial pigment started accumulating at a younger age and were deposited far more rapidly. There was a further increase in the rate of accumulation between 8 and 10 yr of age. We did not encounter pallidal neuronal lipofuscin at any age. These observations are consistent with 2 propositions: 1) that globus pallidus glial cells are unique in their ability to accumulate lipofuscin before it accumulates in nearby neurons; and 2) that they are particularly susceptible to some systemic effect of this chronic illness.
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