Central nervous system involvement in the erythrophagocytic disorders of infancy: the role of cerebrospinal fluid neopterins in their differential diagnosis and clinical management

ABSTRACT ABSTRACT: In two children with familial erythrophagocytic lymphohistiocytosis accompanied by neurologic symptoms, total neopterin concentrations in cerebrospinal fluid were 200

times higher than in controls and 10 to 20 times higher than in five children with presumed neurologic disease due to primary viral infections (human immunodeficiency virus, herpes simplex,

measles) of the CNS. In one child with familial erythrophagocytic lymphohistiocytosis, clinical remission was accompanied by a fall in neopterin concentrations to normal; in a second child,

who died, total neopterin concentrations remained high. In two other children with a diagnosis of infection-associated hemophagocytic syndrome without any neurologic disturbance, neopterin

concentrations were also elevated but only to 10 times the concentrations in controls. Total neopterin concentrations in cerebrospinal fluid provide a measure of the severity of macrophage

infiltration and activation within the CNS, and are useful in assessing the need for intensive chemotherapy and monitoring the response to treatment. SIMILAR CONTENT BEING VIEWED BY OTHERS

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NEUROBORRELIOSIS Article Open access 31 May 2024 ARTICLE PDF AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Departments of Child Health, United Kingdom D W Howells, I Smith & K Hyland *

Immunology. Institute of Child Health, 30 Guilford Street, WCIN IEH, London, United Kingdom S Strobel & R J Levinsky Authors * D W Howells View author publications You can also search

for this author inPubMed Google Scholar * S Strobel View author publications You can also search for this author inPubMed Google Scholar * I Smith View author publications You can also

search for this author inPubMed Google Scholar * R J Levinsky View author publications You can also search for this author inPubMed Google Scholar * K Hyland View author publications You can

also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Howells, D., Strobel, S., Smith, I. _et al._ Central

Nervous System Involvement in the Erythrophagocytic Disorders of Infancy: The Role of Cerebrospinal Fluid Neopterins in Their Differential Diagnosis and Clinical Management. _Pediatr Res_

28, 116–119 (1990). https://doi.org/10.1203/00006450-199008000-00008 Download citation * Received: 23 August 1989 * Accepted: 08 March 1990 * Issue Date: 01 August 1990 * DOI:

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