Marianna Spatola1, Renaud Du Pasquier2, Myriam Schluep2, Axel Regeniter2. 1. From the University of Lausanne (UNIL) (M. Spatola), Lausanne, Switzerland; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) and Hospital Clinic (M. Spatola), Neuroimmunology Program, University of Barcelona, Barcelona, Spain; University Hospital of Lausanne (CHUV) (R.Du Pasquier, M. Schluep), Department of Clinical Neuroscience, Service of Neurology, Lausanne, Switzerland; and University Hospital of Basel (A.Regeniter), Department of Laboratory Medicine, Basel, Switzerland. spatola@clinic.ub.es. 2. From the University of Lausanne (UNIL) (M. Spatola), Lausanne, Switzerland; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) and Hospital Clinic (M. Spatola), Neuroimmunology Program, University of Barcelona, Barcelona, Spain; University Hospital of Lausanne (CHUV) (R.Du Pasquier, M. Schluep), Department of Clinical Neuroscience, Service of Neurology, Lausanne, Switzerland; and University Hospital of Basel (A.Regeniter), Department of Laboratory Medicine, Basel, Switzerland.
Abstract
OBJECTIVE: To establish the sensitivity and specificity of serum and CSF antibodies targeting the gangliosides GQ1b (GQ1bAb) in isolated ophthalmologic syndromes, such as acute ophthalmoplegia (AO) and optic neuritis (ON), caused by disorders other than Miller-Fisher syndrome (MFS). METHODS: We measured serum and CSF GQ1bAb in patients with MFS and with AO or ON caused by other disorders than MFS. RESULTS: Twenty-one patients with AO (21 serum, 9 CSF), 13 with ON (13 serum, 13 CSF), and 12 with MFS (12 serum, 10 CSF) were included in the study. There were no significant differences in age, sex, and CSF findings between the AO and MFS groups. Elevated serum GQ1b titers occurred in 11 of 12 patients with MFS but in only 1 of the 34 patients without MFS. Sensitivity was 92% (95% confidence interval [CI] 62%-100%) and specificity 97% (95% CI 85%-100%). In CSF, GQ1bAb were identified in 2 of 10 patients with MFS but in none with other disorders. Sensitivity was 20% (95% CI 2%-56%) and specificity 100% (95% CI 85%-100%). CONCLUSIONS: Increased serum GQ1bAb are highly specific for MFS. Measurement of GQ1bAb in CSF does not improve diagnosis. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that serum GQ1bAb accurately distinguish MFS from other disorders (sensitivity 92%, 95% CI 62%-100%; specificity 97%, 95% CI 85%-100%).
OBJECTIVE: To establish the sensitivity and specificity of serum and CSF antibodies targeting the gangliosides GQ1b (GQ1bAb) in isolated ophthalmologic syndromes, such as acute ophthalmoplegia (AO) and optic neuritis (ON), caused by disorders other than Miller-Fisher syndrome (MFS). METHODS: We measured serum and CSF GQ1bAb in patients with MFS and with AO or ON caused by other disorders than MFS. RESULTS: Twenty-one patients with AO (21 serum, 9 CSF), 13 with ON (13 serum, 13 CSF), and 12 with MFS (12 serum, 10 CSF) were included in the study. There were no significant differences in age, sex, and CSF findings between the AO and MFS groups. Elevated serum GQ1b titers occurred in 11 of 12 patients with MFS but in only 1 of the 34 patients without MFS. Sensitivity was 92% (95% confidence interval [CI] 62%-100%) and specificity 97% (95% CI 85%-100%). In CSF, GQ1bAb were identified in 2 of 10 patients with MFS but in none with other disorders. Sensitivity was 20% (95% CI 2%-56%) and specificity 100% (95% CI 85%-100%). CONCLUSIONS: Increased serum GQ1bAb are highly specific for MFS. Measurement of GQ1bAb in CSF does not improve diagnosis. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that serum GQ1bAb accurately distinguish MFS from other disorders (sensitivity 92%, 95% CI 62%-100%; specificity 97%, 95% CI 85%-100%).