title: Paraneoplastic limbic encephalitis with SOX1 and PCA2 antibodies and relapsing neurological symptoms in an adolescent with Hodgkin lymphoma.
abstract: BACKGROUND
  Immune cross-reactivity between malignant and normal tissues causes the rare, so called paraneoplastic syndrome (PS). In approximately 60% of the patients, various onconeural antibodies are detectable in the cerebrospinal fluid (CSF) and are associated with typical tumour entities.
  
  
  METHODS
  We report an unusual case of paraneoplastic limbic encephalitis (PLE) in a 17-year-old adolescent with classical Hodgkin lymphoma.
  
  
  RESULTS
  He presented with a variety of neurologic and neuropsychiatric symptoms, profound B-symptoms and typical MRI findings including hyperintense lesions with contrast enhancement in the medial temporal lobe and limbic system. Under immunosuppressive therapy and subsequently chemotherapy the neurological situation only temporarily improved and worsened again after interruption of immunosuppression several times. Thus, multiple courses of multidrug immunosuppressive therapy were administered. To date, five years after initial presentation, the young man is able to walk with walking aids and orthoses and is still on oral prednisolone therapy. Analyses of the CSF and serum revealed anti SOX-1 antibodies at initial presentation but PCA-2 antibodies seven months after diagnosis.
  
  
  CONCLUSIONS
  Neurologic and/or neuropsychiatric symptoms combined with typical MRI findings should raise the suspicion of PS and lead to further diagnostics for an underlying tumour even in children.
authors.affiliation: University of Duesseldorf, Medical Faculty, Department of Pediatric Oncology, Hematology and Clinical Immunology, Centre for Child and Adolescent Health, Duesseldorf, Germany. Electronic address: Marina.Kunstreich@med.uni-duesseldorf.de.
authors.forename: M
authors.initials: M
authors.lastname: Kunstreich
authors.affiliation: University of Duesseldorf, Medical Faculty, Department of Pediatrics and Neonatology, Centre for Child and Adolescent Health, Duesseldorf, Germany.
authors.forename: J H
authors.initials: JH
authors.lastname: Kreth
authors.affiliation: University of Duesseldorf, Medical Faculty, Department of Pediatric Oncology, Hematology and Clinical Immunology, Centre for Child and Adolescent Health, Duesseldorf, Germany.
authors.forename: P T
authors.initials: PT
authors.lastname: Oommen
authors.affiliation: University of Duesseldorf, Institute of Diagnostic and Interventional Radiology, Duesseldorf, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Schaper
authors.affiliation: University of Duesseldorf, Medical Faculty, Department of Pediatrics and Neonatology, Centre for Child and Adolescent Health, Duesseldorf, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Karenfort
authors.affiliation: University of Duesseldorf, Medical Faculty, Department of Neurology, Duesseldorf, Germany.
authors.forename: O
authors.initials: O
authors.lastname: Aktas
authors.affiliation: University of Duesseldorf, Medical Faculty, Department of Pediatrics and Neonatology, Centre for Child and Adolescent Health, Duesseldorf, Germany.
authors.forename: D
authors.initials: D
authors.lastname: Tibussek
authors.affiliation: University of Duesseldorf, Medical Faculty, Department of Pediatrics and Neonatology, Centre for Child and Adolescent Health, Duesseldorf, Germany.
authors.forename: F
authors.initials: F
authors.lastname: Distelmaier
authors.affiliation: University of Duesseldorf, Medical Faculty, Department of Pediatric Oncology, Hematology and Clinical Immunology, Centre for Child and Adolescent Health, Duesseldorf, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Borkhardt
authors.affiliation: University of Duesseldorf, Medical Faculty, Department of Pediatric Oncology, Hematology and Clinical Immunology, Centre for Child and Adolescent Health, Duesseldorf, Germany. Electronic address: Michaela.Kuhlen@med.uni-duesseldorf.de.
authors.forename: M
authors.initials: M
authors.lastname: Kuhlen
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D006689:Hodgkin Disease / Q000150:complications*; D006801:Humans; D020363:Limbic Encephalitis / Q000209:etiology* / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000276:immunology*; D055748:SOXB1 Transcription Factors / Q000276:immunology
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; C490906:SOX1 protein, human; D055748:SOXB1 Transcription Factors
keywords: Hodgkin lymphoma; Limbic encephalitis; PCA2; Paraneoplastic; SOX1