Central neurogenic hyperventilation in an adult with multisystem Langerhans cell histiocytosis

Abstract We present a case of a 46-year-old male with a history of biopsy-proven pulmonary Langerhans cell histiocytosis (LCH) who presented with shortness of breath and progressive neurologic decline. Neurologic examination suggested both posterior fossa and bilateral supratentorial dysfunction without papilledema and arterial blood gas revealed a respiratory alkalosis. Magnetic resonance imaging of the brain was suggestive of central nervous system (CNS)-LCH. The patient was diagnosed with central neurogenic hyperventilation (CNH) secondary to CNS-LCH. He was treated with intravenous morphine and lorazepam without improvement in his respiratory alkalosis. He received two doses of immunoglobulin and empiric chemotherapy with vinblastine and prednisone for the LCH. This case illustrates one of a few reported cases of CNH due to suspected CNS LCH.