title: Novel Presentation of Rosai-Dorfman Histiocytosis With a Prolonged Course of Cranial and Peripheral Neuropathies.
abstract: BACKGROUND
  Rosai-Dorfman disease is a form of histiocytosis affecting the systemic lymph nodes. Intracranial Rosai-Dorfman disease is rare and presents with extra-parenchymal or intraparenchymal proliferative mass lesions. Cranial neuropathy has not been reported in Rosai-Dorfman disease except when caused by mass effect by an adjacent lesion.
  
  
  METHODS
  We describe a girl with Rosai-Dorfman disease who presented with peripheral and multiple cranial neuropathies. Detailed clinical, immunologic, neurophysiology, imaging, and genetic studies were performed. She had a prolonged course but recovered fully after immune therapies. She had increased titers of striated muscle and smooth muscle antibodies. Imaging studies revealed contrast enhancement of cranial nerves and striated muscles. Demyelination was evident in the nerve twigs from muscle biopsy. Exome sequencing did not reveal a genetic mutation.
  
  
  CONCLUSIONS
  Most patients with Rosai-Dorfman disease have a benign course, but severe neurological dysfunction due to bulbar involvement and cranial and peripheral neuropathies may occur. Treatment with immunoglobulin and steroids may be of benefit.
authors.affiliation: Department of Pediatrics and Neurology, Wayne State University, Detroit, Michigan. Electronic address: Richa.tripathi@wayne.edu.
authors.forename: Richa
authors.initials: R
authors.lastname: Tripathi
authors.affiliation: Department of Pediatrics and Neurology, Wayne State University, Detroit, Michigan.
authors.forename: Fatema
authors.initials: F
authors.lastname: Serajee
authors.affiliation: Department of Pediatrics and Neurology, Wayne State University, Detroit, Michigan.
authors.forename: Huiyuan
authors.initials: H
authors.lastname: Jiang
authors.affiliation: Department of Pediatrics and Neurology, Wayne State University, Detroit, Michigan.
authors.forename: A H M Mahbubul
authors.initials: AHMM
authors.lastname: Huq
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D015618:Histiocytosis, Sinus / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D009132:Muscles / Q000473:pathology; D013116:Spinal Cord / Q000000981:diagnostic imaging
keywords: Rosai-Dorfman disease; chronic inflammatory demyelinating polyneuropathy; cranial neuropathy; histiocytosis