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Apparently isolated CNS involvement in Erdheim-Chester disease: Case report
Giuseppe Romano1, Mario Cirillo2, Simona Bonavita1
1Second Division of Neurology, University of Campania "Luigi Vanvitelli", Naples, Italy.
Radiology Case Reports
|October 16, 2023
Summary
Erdheim-Chester disease, a rare condition, can affect the central nervous system, specifically the brainstem. Always check for multisystem issues when Erdheim-Chester disease is suspected.
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Erdheim-Chester disease is an extremely rare non-Langerhans cell histiocytosis.
- It typically affects multiple organ systems, but central nervous system (CNS) involvement can occur.
Observation:
- A case of a 48-year-old woman with Erdheim-Chester disease is presented.
- The disease manifested with apparently isolated central nervous system involvement, specifically affecting the brainstem.
Findings:
- The case highlights brainstem involvement as a potential presentation of Erdheim-Chester disease.
- This presentation may initially appear isolated to the CNS.
Implications:
- Clinicians should maintain a high index of suspicion for Erdheim-Chester disease in cases of CNS pathology, particularly with brainstem lesions.
- Thorough systemic evaluation is crucial to rule out or identify concurrent multisystem impairment, even when CNS findings seem isolated.
