title: Stiff person syndrome.
abstract: Recognizing stiff person syndrome is clinically important. It is uncommon, characterized by body stiffness associated with painful muscle spasms, and varies in location and severity. It is subdivided into stiff trunk versus stiff limb presentation, and as a progressive encephalomyelitis. Stiff person-type syndrome also reflects a paraneoplastic picture. Most patients demonstrate exaggerated lumbar lordosis. Roughly 60% of patients have antiglutamic acid decarboxylase antibodies in the blood and the cerebrospinal fluid. The differential diagnosis includes many severe conditions. There are reports of response to muscle relaxants, immunosuppressants, intravenous gamma globulin, plasma exchange, a number of anticonvulsants, and botulinum toxin.
authors.affiliation: Department of Neurology, Tulane University School of Medicine, 1430 Tulane Avenue 8065, New Orleans, LA 70112, USA.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Ciccotto
authors.affiliation: Department of Neurology, Tulane University School of Medicine, 1430 Tulane Avenue 8065, New Orleans, LA 70112, USA.
authors.forename: Maike
authors.initials: M
authors.lastname: Blaya
authors.affiliation: Department of Neurology, Tulane University School of Medicine, 1430 Tulane Avenue 8065, New Orleans, LA 70112, USA. Electronic address: rkelley2@tulane.edu.
authors.forename: Roger E
authors.initials: RE
authors.lastname: Kelley
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D001905:Botulinum Toxins; D003937:Diagnosis, Differential; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology; D006801:Humans; D008875:Middle Aged; D009127:Muscle Rigidity / Q000188:drug therapy / Q000209:etiology*; D010951:Plasma Exchange; D013035:Spasm / Q000188:drug therapy / Q000209:etiology*; D016750:Stiff-Person Syndrome / Q000150:complications / Q000175:diagnosis / Q000276:immunology
chemical_list: D000927:Anticonvulsants; D001323:Autoantibodies; D001905:Botulinum Toxins; D005968:Glutamate Decarboxylase