Identification of the inflammatory cells in the central nervous system of patients with adrenoleukodystrophy

Diane Griffin, H. W. Moser, Q. Mendoza, T. R. Moench, Ann B. Moser

Research output: Contribution to journalArticle

Abstract

Adrenoleukodystrophy is a disorder of long-chain fatty acid metabolism associated with adrenal cortical insufficiency and central nervous system demyelination. The central nervous system disease is unusual in that it is abrupt in onset and accompanied by a considerable infiltration of mononuclear inflammatory cells. To determine the nature of these inflammatory cells, immunocytochemical staining was carried out on the mononuclear cells in the brain and cerebrospinal fluid of patients with adrenoleukodystrophy. Monoclonal antibodies to T lymphocytes (T11), the helper/inducer (T4) and cytotoxic/suppressor (T8) subsets of T lymphocytes, B lymphocytes (B1), and monocyte/macrophages (M1 or esterase) were used. Mononuclear cells in the perivascular cuffs of autopsy material from 4 patients were, on average, 59% T cells, 34% T4 cells, 16% T8 cells, 24% B cells, and 11% monocyte/macrophages. Cerebrospinal fluid from 8 of 10 patients had increased IgG concentrations. Mononuclear cells in the cerebrospinal fluid of 6 patients with active disease were, on average, 61% T cells, 40% T4 cells, 16% T8 cells, 3% B cells, and 18% monocyte/macrophages. This distribution of cells is similar to that found in the central nervous system during a cellular immune response and suggests the possibility that one component of this disease is immunologically mediated.

Original languageEnglish (US)
Pages (from-to)660-664
Number of pages5
JournalAnnals of Neurology
Volume18
Issue number6
StatePublished - 1985

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Adrenoleukodystrophy
Central Nervous System
CD8-Positive T-Lymphocytes
CD4-Positive T-Lymphocytes
Cerebrospinal Fluid
Monocytes
B-Lymphocytes
Macrophages
T-Lymphocytes
Adrenal Insufficiency
Central Nervous System Diseases
T-Lymphocyte Subsets
Demyelinating Diseases
Esterases
Helper-Inducer T-Lymphocytes
Cellular Immunity
Autopsy
Fatty Acids
Immunoglobulin G
Monoclonal Antibodies

ASJC Scopus subject areas

  • Neuroscience(all)

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Identification of the inflammatory cells in the central nervous system of patients with adrenoleukodystrophy. / Griffin, Diane; Moser, H. W.; Mendoza, Q.; Moench, T. R.; Moser, Ann B.

In: Annals of Neurology, Vol. 18, No. 6, 1985, p. 660-664.

Research output: Contribution to journalArticle

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