title: Anti-Hu paraneoplastic brainstem encephalitis caused by a pancreatic neuroendocrine tumor presenting with central hypoventilation.
abstract: Paraneoplastic neurological syndromes are rare autoimmune manifestations of malignancies associated with specific antibodies. Anti-Hu associated brainstem encephalitis, a well-described syndrome, usually presents subacutely with preferential involvement of the medulla. Anti-Hu antibodies target intraneuronal antigens and are therefore highly correlated with neurological syndromes when present concomitantly with a neoplasm. Reported is a case of anti-Hu brainstem encephalitis associated with a pancreatic neuroendocrine tumor (PNET) presenting with central hypoventilation. This is the first described case of brainstem encephalitis associated with a well-differentiated PNET as well as the first case of Anti-Hu antibodies associated with a PNET. There are no standardized protocols for the treatment of paraneoplastic brainstem encephalitis however, as in the present case, surgical resection and oncological treatment of the tumor is the first line treatment.
authors.affiliation: Department of Surgery, Columbia University Medical Center, NY, United States. Electronic address: mn2594@cumc.columbia.edu.
authors.forename: Marc
authors.initials: M
authors.lastname: Najjar
authors.affiliation: Department of Neurology, Columbia University Medical Center, NY, United States.
authors.forename: Andrew
authors.initials: A
authors.lastname: Taylor
authors.affiliation: Department of Surgery, Columbia University Medical Center, NY, United States.
authors.forename: Surbhi
authors.initials: S
authors.lastname: Agrawal
authors.affiliation: Department of Medicine, Division of Hematology/Oncology, Columbia University Medical Center, NY, United States.
authors.forename: Tito
authors.initials: T
authors.lastname: Fojo
authors.affiliation: Department of Neurology, Columbia University Medical Center, NY, United States.
authors.forename: Alexander E
authors.initials: AE
authors.lastname: Merkler
authors.affiliation: Department of Pathology, Division of Neuropathology, Memorial Sloan Kettering Cancer Center, NY, United States.
authors.forename: Marc K
authors.initials: MK
authors.lastname: Rosenblum
authors.affiliation: Department of Neurology, Columbia University Medical Center, NY, United States.
authors.forename: Laura
authors.initials: L
authors.lastname: Lennihan
authors.affiliation: Department of Surgery, Columbia University Medical Center, NY, United States.
authors.forename: Michael D
authors.initials: MD
authors.lastname: Kluger
mesh_terms: D004660:Encephalitis / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D007040:Hypoventilation / Q000175:diagnosis* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000150:complications / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology*; D020361:Paraneoplastic Syndromes, Nervous System / Q000175:diagnosis* / Q000209:etiology
keywords: Brainstem encephalitis; Pancreatic neuroendocrine tumor; Paraneoplastic syndrome