Back to Search
Start Over
Erdheim-Chester disease of brain parenchyma without any systemic involvement: A case report and review of literature.
- Source :
-
Neuropathology : official journal of the Japanese Society of Neuropathology [Neuropathology] 2024 Feb; Vol. 44 (1), pp. 59-67. Date of Electronic Publication: 2023 Jun 26. - Publication Year :
- 2024
-
Abstract
- Erdheim-Chester disease is a non-Langerhans cell histiocytosis syndrome characterised by histiocytic infiltration of different organs and systems in the body. Erdheim-Chester disease with isolated central nervous system (CNS) involvement causes diagnostic difficulties due to the absence of systemic findings and may result in misdiagnosis and inaccurate treatment choices. The case discussed in this report exemplifies how challenging it is to diagnose Erdheim-Chester disease with isolated CNS involvement. This case, which presented with progressive pyramidocerebellar syndrome, was clinically and radiologically resistant to all immunosuppressive and immunomodulatory treatments administered. The presence of false negative results in repeated histopathological investigations and the absence of evidence for systemic disease hindered the diagnosis and treatment work-up. In this study, we reviewed and discussed the prominent features of the presented case in light of the relevant literature.<br /> (© 2023 Japanese Society of Neuropathology.)
Details
- Language :
- English
- ISSN :
- 1440-1789
- Volume :
- 44
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Neuropathology : official journal of the Japanese Society of Neuropathology
- Publication Type :
- Review
- Accession number :
- 37357975
- Full Text :
- https://doi.org/10.1111/neup.12930