title: Paraneoplastic stiff-person syndrome, heterotopic soft tissue ossification and gonarthritis in a HLA B27-positive woman preceding the diagnosis of Hodgkin's lymphoma.
abstract: Paraneoplastic neurologic syndromes associated with Hodgkin's lymphoma include the stiff-person syndrome. A case of stiff-person syndrome is reported who first presented with muscular hyperactivity and acute respiratory failure followed by heterotopic soft tissue ossification and acute seronegative gonarthitis. Initial improvement of a tetanus-like clinical picture was achieved with benzodiazepam given by continuous infusion for analgo-sedation to mechanically ventilate the patient followed by baclofen after successful weaning. The patient was HLA B27 positive and on conventional testing no autoantibodies were detected including anti-glutamic acid decarboxylase antibodies (anti-GAD). Months later in the absence of signs of stiff-person syndrome, mediastinal lymphadenopathy and pleural effusions developed which were diagnosed as classical Hodgkin's lymphoma that was successfully treated with polychemotherapy. No relapse of paraneoplastic neurologic syndromes was seen after two years of lymphoma remission. The case illustrates that stiff-person syndrome may precede the clinical appearance of symptomatic Hodgkin's lymphoma.
authors.affiliation: Department of Internal Medicine, Central Hospital of Bolzano, Lorenz Böhler Street 5, Bolzano, I-39100 Bolzano (BZ), Italy.
authors.forename: Bernd
authors.initials: B
authors.lastname: Gutmann
authors.forename: Claudio
authors.initials: C
authors.lastname: Crivellaro
authors.forename: Manfred
authors.initials: M
authors.lastname: Mitterer
authors.forename: Hermann
authors.initials: H
authors.lastname: Zingerle
authors.forename: Eduard
authors.initials: E
authors.lastname: Egarter-Vigl
authors.forename: Christian J
authors.initials: CJ
authors.lastname: Wiedermann
mesh_terms: D005260:Female; D020022:Genetic Predisposition to Disease; D015796:HLA-B27 Antigen / Q000032:analysis*; D006689:Hodgkin Disease / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D008875:Middle Aged; D009999:Ossification, Heterotopic / Q000209:etiology* / Q000235:genetics; D020370:Osteoarthritis, Knee / Q000209:etiology* / Q000235:genetics; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000235:genetics; D012074:Remission Induction; D012131:Respiratory Insufficiency / Q000209:etiology; D016750:Stiff-Person Syndrome / Q000209:etiology* / Q000235:genetics; D013997:Time Factors
chemical_list: D015796:HLA-B27 Antigen