ABSTRACT
Introduction: Eculizumab has been shown to be an effective and typically well-tolerated medication in the treatment of neuromyelitis optica spectrum disorder (NMOSD) in maintaining disease remission in patients who are aquaporin-4 water channel autoantibody (AQP4-IgG) seropositive. The efficacy of eculizumab in an acute relapse of NMOSD however is still under review. Case: We describe a 46 year-old female who presented with acute left monocular vision loss on a background of bilateral optic neuritis treated 15 years prior as suspected NMOSD. She had very poor vision from the right eye (6/60). On presentation she was not on any long-term immunosuppressive agents. Her serum was positive for AQP4-IgG and MRI brain and spine demonstrated areas of demyelination in the corpus callosum and thoracic spine. She was treated with high dose intravenous methylprednisolone and underwent plasmapheresis for five consecutive days, but continued to clinically deteriorate with ongoing blindness in her left eye (light perception only). She was subsequently administered eculizumab with weaning oral corticosteroids. Clinically her vision improved to counting fingers and she remains on maintenance eculizumab infusions in the community. At 3 months, there is a steady improvement but still significant loss of central vision from that eye. Conclusion: The utility of eculizumab in NMOSD may assist with treating acute episodes. This theoretically accords with the mode of action in inhibiting conversion of C5-C5a/b, perhaps arresting the acute inflammatory process in this disease. Given that disease burden and mortality in NMOSD is almost entirely related to relapses, increased use of eculizumab acutely could potentially aid recovery from an attack in very severe attacks, and therefore minimize immediate stepwise accrual of disability.
ABSTRACT
BACKGROUND: Prevalence of cardiovascular autonomic dysfunction (CAD) in multiple sclerosis (MS) varies between studies. Cardiac autonomic function is usually assessed by cardiovascular reflex tests. We hypothesized that MS is associated with CAD, quantifiable by non-invasive means including quantification of baroreceptor sensitivity (BRS) and heart rate variability. METHODS: In this study a comprehensive suite of cardiovascular autonomic tests based only on the spontaneous changes of heart rate and blood pressure was applied to 23 MS patients and age and gender-matched controls. From 5-min continuous non-invasive recording of the electrocardiogram and blood pressure, heart-rate, blood pressure, and autonomic function variables were calculated. Analysis included heart rate variability in the time domain, heart rate and blood pressure variability in the frequency domain, and baroreceptor sensitivity in both the time and frequency domain. RESULTS: BRS measured by the frequency technique in high frequency band was found to be significantly lower in MS (16⯱â¯9â¯ms/mmHg) compared to controls (29⯱â¯17â¯ms/mmHg) (pâ¯<â¯0.05). Also mean of BRS modulus in MS averaged 15⯱â¯8â¯ms/mmHg which is significantly lower compared to controls (25⯱â¯15â¯ms/mmHg) (pâ¯<â¯0.05). Systolic blood pressure variability in the high frequency band (0.15-0.5â¯Hz) was found to be significantly higher in the MS compared to controls (5.8⯱â¯16.7â¯mmHg2 vs. 1.3⯱â¯0.8â¯mmHg2) (pâ¯<â¯0.05). CONCLUSIONS: The results, using techniques novel to MS investigation, showed diminished baroreceptor reflex and impaired sympathetic function using frequency domain systolic blood pressure variability analysis.
