This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (18)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Neuropathology  • Alert me on articles by topic  Social Bookmarking   What's this?

Correlation With Clinical Severity

Goran Rakocevic, MD; Raghavanpillai Raju, PhD; Marinos C. Dalakas, MD

Arch Neurol. 2004;61:902-904.

Background  Stiff-person syndrome (SPS) is an immune-mediated central nervous system disorder characterized by fluctuating muscle stiffness, disabling spasms, and heightened sensitivity to external stimuli. Up to 80% of patients with SPS have anti–glutamic acid decarboxylase (GAD) antibodies in the serum or cerebral spinal fluid (CSF). Whether these antibodies are clinically relevant and correlate with disease severity is unknown.

Objective  To correlate anti-GAD antibody titers in the serum and CSF of patients with SPS with the degree of clinical severity.

Design  Patients studied the last 6 years.

Setting  The Clinical Center of the National Institutes of Health, Bethesda, Md.

Patients  Sixteen patients with typical SPS and elevated serum anti-GAD antibody titers.

Interventions  Antibody titers in serum and CSF were measured by radioimmunoassay, and the intrathecal anti–GAD-specific IgG production was calculated.

Main Outcome Measures  Comparison of antibody titers with stiffness index and heightened sensitivity scores based on scales that reliably measure disease severity.

Results  The mean disease duration was 11 years (range, 5-30 years). The mean anti-GAD antibody titer in the serum was 51 500 U/mL (range, 24 000-200 000 U/mL); and in the CSF, 181 U/mL (range, 30-400 U/mL). A 10-fold increased intrathecal production of GAD-specific IgG antibodies was noted. No correlation was found between antibody titers in serum or CSF with disease severity. In 4 patients, the anti-GAD antibody titers measured serially during a 2-year period did not correlate with clinical fluctuations.

Conclusions  In patients with SPS, the anti-GAD antibody titers in serum and CSF do not correlate with disease severity or duration. Anti-GAD antibodies are an excellent marker for SPS, but monitoring their titers during the course of the disease may not be of practical value.

From the Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Md.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Spectrum of neurological syndromes associated with glutamic acid decarboxylase antibodies: diagnostic clues for this association
Saiz et al.
Brain 2008;131:2553-2563.
ABSTRACT | FULL TEXT  

Autoimmunity to GABAA-receptor-associated protein in stiff-person syndrome
Raju et al.
Brain 2006;129:3270-3276.
ABSTRACT | FULL TEXT  

Improvement with corticosteroids and azathioprine in GAD65-associated cerebellar ataxia.
McFarland et al.
Neurology 2006;67:1308-1309.
FULL TEXT  

Stiff person syndrome with cerebellar disease and high-titer anti-GAD antibodies.
Rakocevic et al.
Neurology 2006;67:1068-1070.
ABSTRACT | FULL TEXT  

Analysis of GAD65 Autoantibodies in Stiff-Person Syndrome Patients
Raju et al.
J. Immunol. 2005;175:7755-7762.
ABSTRACT | FULL TEXT  

Anti-glutamic acid decarboxylase antibodies in a patient with systemic lupus erythematosus and fibromyalgia symptoms
Taylor-Gjevre and Gjevre
Lupus 2005;14:486-488.
ABSTRACT  

GAD antibodies in stiff-person syndrome
Chang and Lang
Neurology 2004;63:1999-2000.
FULL TEXT