title: Antiglycine receptor-related stiff limb syndrome in a patient with chronic lymphocytic leukaemia.
abstract: We report a 61-year-old man presenting with rapidly progressive stiffness and painful muscle spasms in the lower extremity muscles. The patient was diagnosed with chronic lymphocytic leukaemia (CLL) approximately a year before symptom onset. Electromyography displayed continuous motor unit activity and immunocytochemistry showed a positive staining for antiglycine receptor (anti-GlyR) antibodies. The clinical course was complicated by autonomic instability and cardiac arrest, but stabilised under continuous therapy with plasma exchange and symptomatic treatment with baclofen and clonazepam. Anti-GlyR antibodies induce rare, but severe, variants of stiff person syndrome that can be of paraneoplastic origin and life threatening due to autonomic dysfunction.
authors.affiliation: Department of Neurology, Heinrich-Heine-University, Duesseldorf, Germany. Angelika.derksen@uniduesseldorf.de
authors.forename: Angelika
authors.initials: A
authors.lastname: Derksen
authors.forename: Mark
authors.initials: M
authors.lastname: Stettner
authors.forename: Winfried
authors.initials: W
authors.lastname: Stöcker
authors.forename: Rüdiger J
authors.initials: RJ
authors.lastname: Seitz
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000276:immunology*; D001418:Baclofen / Q000627:therapeutic use; D002998:Clonazepam / Q000627:therapeutic use; D004576:Electromyography; D018682:GABA Agents / Q000627:therapeutic use; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications*; D008297:Male; D010257:Paraneoplastic Syndromes / Q000276:immunology* / Q000628:therapy; D010951:Plasma Exchange; D018009:Receptors, Glycine; D016750:Stiff-Person Syndrome / Q000276:immunology* / Q000628:therapy
chemical_list: D001323:Autoantibodies; D018682:GABA Agents; D018009:Receptors, Glycine; D002998:Clonazepam; D001418:Baclofen