title: Acute disseminated encephalomyelitis presenting as a brainstem encephalitis.
abstract: Acute disseminated encephalomyelitis (ADEM) is a disease characterized by inflammation and destruction of myelin. Acute hemorrhagic leukoencephalitis (AHLE) is a severe form of ADEM known for its particularly poor outcome. We present a case of a young Caucasian female who presented with drowsiness and slurred speech followed by rapid brainstem involvement resembling rhomboencephalitis. Despite multiple diagnostic tests and empiric therapy with immunosuppressants, immunoglobulins, and antimicrobials, she lost most brainstem reflexes within a few weeks and ultimately passed away. Magnetic resonance imaging (MRI) showed progression of lesions from the brainstem to eventually involve bilateral cerebral hemispheres. Autopsy and microscopic examination of the brain revealed several hemorrhagic lesions throughout the brain and rendered a diagnosis of AHLE. AHLE was initially described in 1941 and is thought to be autoimmune related, possibly related to cross reactivity between the immune system and CNS tissues like myelin. While a definitive inciting pathogen was not discovered, this case emphasizes the importance of considering AHLE in the differential diagnosis of patients with rapid loss of neurologic function and highlights an atypical presentation of ADEM/AHLE.
authors.affiliation: Department of Pathology, HSB 175J, 619 19th St. South, University of Alabama, Birmingham, AL 35249, United States. Electronic address: datherto@uab.edu.
authors.forename: Daniel S
authors.initials: DS
authors.lastname: Atherton
authors.affiliation: Department of Neurology, SC 350, 1720 2nd Ave. South, University of Alabama, Birmingham, AL 35294, United States.
authors.forename: Sarah R
authors.initials: SR
authors.lastname: Perez
authors.affiliation: Division of Infectious Diseases, 1201 11th Ave. South, University of Alabama, Birmingham, AL 35205, United States.
authors.forename: Nathan D
authors.initials: ND
authors.lastname: Gundacker
authors.affiliation: Division of Infectious Diseases, 1201 11th Ave. South, University of Alabama, Birmingham, AL 35205, United States.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Franco
authors.affiliation: Department of Neurology, SC 350, 1720 2nd Ave. South, University of Alabama, Birmingham, AL 35294, United States.
authors.forename: Xiaosi
authors.initials: X
authors.lastname: Han
mesh_terms: D000328:Adult; D001933:Brain Stem / Q000000981:diagnostic imaging*; D003937:Diagnosis, Differential; D004660:Encephalitis / Q000150:complications / Q000000981:diagnostic imaging*; D004673:Encephalomyelitis, Acute Disseminated / Q000150:complications / Q000000981:diagnostic imaging; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D004684:Leukoencephalitis, Acute Hemorrhagic / Q000150:complications / Q000000981:diagnostic imaging*
keywords: Acute disseminated encephalomyelitis; Demyelination; Hemorrhagic leukoencephalopathy; Rhomboencephalitis