Year: 2016
Type: conference-abstract
Abstract: We present an unusual case of a 37-year old male who presented with a three-week history of diplopia, vertigo, left facial numbness, and left facial droop. MRI demonstrated a contrast enhancing mass at the left pontomedullary junction and extending into the cerebellum. Biopsy was consistent with crystal-storing histiocytosis, which is characterized by abnormal cytoplasmic aggregation of immunoglobulin crystals in histiocytes. To our knowledge, this is the first case of crystal-storing histiocytosis described in the brainstem and cerebellum and one of only a handful described in the central nervous system. This rare disorder is usually associated with underlying lymphoplasmacytic disorders or less commonly inflammatory or infectious disease. However, none of these have been identified thus far in this case. The patient received a short oral steroid course with resultant radiologic normalization. We review the crystal-storing histiocytosis literature and propose continued regular surveillance for the development of lymphoplasmacytic disease.
Source: Neuro-Oncology
Authors: Justin Low, James Wang, Margaret Flanagan, C. Dirk Keene, David Louis, Gordana Juric-Sekhar, Maciej Mrugala, Gwen Garden
Language: English
Subfield: Physiology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W2588200287