Erdheim-Chester disease presenting as meningitis with hypoglycorrhachia: A case report

Erdheim-Chester disease (ECD) commonly has neurologic manifestations but rarely presents with meningitis and hypoglycorrhachia. Here, were present a case of ECD with a clinical and laboratory presentation initially thought to be bacterial meningitis with sepsis.

We report a case of a 79-year-old with history of enigmatic bone pain and peritoneal nodules who presented with meningitis. After failure to improve on antibiotic therapy other etiologies of hypoglycorrhachia including sarcoid, tuberculosis, and fungal and carcinomatous meningitis were considered. However, no definite diagnosis could be made based on radiologic, serologic, microbiologic, and molecular testing and the patient failed to improve on empiric therapy including antibiotics, antifungals, and tuberculosis and steroid therapy.

Ultimately, autopsy revealed a new diagnosis of ECD manifesting as meningitis, a rare presentation of a rare disease.

Although only reported in one other case to our knowledge, ECD can present with meningitis with hypoglycorrhachia.