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Neuromyelitis optica: disease onset in relationship with infections and vaccinations

Objectives: Neuromyelitis optica (NMO) is an uncommon but life-threatening demyelinating disorder. With the discovery of the NMO antibody in 2004, diagnostic criteria have been revised. Relationships with preceding infections and/or immunizations have been well studied in other demyelinating disorders with positive correlation with acute disseminated encephalomyelitis and negative correlation with multiple sclerosis (MS). There have been several case reports only linking specific vaccinations or infections as a precipitating factor for NMO, but this correlation has not been systematically studied in children. Methods: Charts were retrospectively reviewed for patients under 21 years old who presented to Cleveland Clinic in 2004 or after and were subsequently diagnosed with MS or NMO based on McDonald and Wingerchuk revised diagnostic criteria, respectively. Data collection included general demographics, clinical presentation, MRI, CSF, serum NMO antibody (NMOAb), treatment, and immunization record. Descriptive data, Fisher’s exact, and Wilcoxon sum rank test were used as appropriate. All tests were two-tailed and performed at a significance level of 0.05. SAS 9.2 software (SAS Institute, Cary, NC) was used for all analyses. Results: Nine NMO patients were identified with complete vaccination records obtained on six. Five were NMO antibody positive. Twelve control MS patients were matched for age and gender with no statistical difference between the groups. Symptom onset ranged from 7 to 16 years old in the NMO group (range 7 to 13.9 years for NMOAb positive and 9.5 to 15.8 years for NMOAb negative group). There was a predominance of females (7:2) and African-Americans (7:2). All NMO patients were treated with steroids, 4/9 with IVIG, and 6/9 (including all five NMOAb positive) were started on azathioprine. There was no correlation with disease onset and any vaccinations (hepatitis B, haemophilus influenzae type B, polio virus, diphtheria, tetanus, pertussis, pneumococcus, mumps, measles, or rubella). Six of 9 had preceding infections within one week of onset although statistical significance was not reached (p=0.08). Conclusion: Although sample size is small, our study suggests that there may be a correlation between NMO onset and preceding infections. There is no evidence of any childhood vaccinations as a precipitating factor for NMO. As the fundamental cause of the presumed autoimmunity in NMO remains elusive, further studies with larger sample size are needed.

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