Autonomic dysfunction in people with neuromyelitis optica spectrum disorders

Abstract

Aims: To determine the difference in autonomic symptom burden measured with the Composite Autonomic System Score-31 (COMPASS-31) and presence of objective dysautonomia in people with neuromyelitis optica spectrum disorders (pwNMOSD) compared to people with multiple sclerosis (pwMS). Design/Methods: Twenty pwNMOSD and 20 pwMS, matched for age, sex, and disease duration, were enrolled. All patients completed the COMPASS-31. The quantification of cardiovascular autonomic dysfunction (CAD) was made using the two indices of the Composite Autonomic Scoring Scale (CASS): adrenergic index (AI) and cardiovagal index (CI). Results: In all pwNMOSD, COMPASS-31 was >0. Sympathetic dysfunction was present in 8 (40%), parasympathetic dysfunction in 10 (50%), and orthostatic hypotension in 6 (30%) pwNMOSD. This group of patients had higher frequency and level on the pupillomotor domain of the COMPASS-31 compared to pwMS ( p = 0.048 and p = 0.006, respectively). A binary logistic regression model showed that drop in diastolic blood pressure (dBP) during tilt-table test and normal function of autonomic nervous system, defined as AI = 0 and CI = 0, were independent predictors of pwNMOSD ( p = 0.042 and p = 0.029, respectively). If CAD was present, it was significantly worse in pwNMOSD compared to pwMS ( p = 0.003). Conclusion: Significant proportion of pwNMOSD experience dysautonomia, which seems to be different from dysautonomia observed in pwMS.

Frequency and characteristics of dysautonomic symptoms in multiple sclerosis: a cross-sectional double-center study with the validated Italian version of the Composite Autonomic Symptom Score-31

“…The underlying pathogenic mechanism remains poorly characterized but seems to be related to axonal loss along pupillomotor fibers consistent with the evidence of a correlation between efferent pathway shift and spinal cord atrophy [21]. Furthermore, Crnošija et al observed substantial differences in characteristics of pupillomotor disturbances comparing 20 matched patients with MS and neuromyelitis optica spectrum disorder (NMOSD) as assessed by COMPASS-31 [22]. While pupillomotor abnormalities in MS patients may reflect parasympathetic pupillary dysfunction with a relative increase of sympathetic dilatator tone [21,22], the more severe subtype of ON in NMOSD could determine either a permanent visual loss with consequently altered pupillary responses or an extensive involvement of the pupillary response system [22].…”

“…Furthermore, Crnošija et al observed substantial differences in characteristics of pupillomotor disturbances comparing 20 matched patients with MS and neuromyelitis optica spectrum disorder (NMOSD) as assessed by COMPASS-31 [22]. While pupillomotor abnormalities in MS patients may reflect parasympathetic pupillary dysfunction with a relative increase of sympathetic dilatator tone [21,22], the more severe subtype of ON in NMOSD could determine either a permanent visual loss with consequently altered pupillary responses or an extensive involvement of the pupillary response system [22]. We found no significant difference in COMPASS-31 total and single domain scores between patients with or without a positive history for myelitis.…”

Comparison of psychiatric disturbances in patients with multiple sclerosis and neuromyelitis optica

“…In our study, the HRV parameters were not significantly different between the MS and NMO groups. Although autonomic dysfunction is more severe in patients with NMO than in those with MS, [46] HRV did not reflect this difference. Moreover, the PO and NB findings were not significantly different between the MS and NMO groups.…”

Longitudinal assessment of autonomic nervous system in patients with first demyelinating event suggestive of multiple sclerosis

“…Cervical spinal cord lesions on the MRI were not associated with ANS dysfunction in a large cohort on pwCIS , whereas spinal cord atrophy correlated with ANS dysfunction in a heterogeneous population of pwMS , indicating that the way in which we measure spinal cord damage may be responsible for observed differences. Furthermore, in people with neuromyelitis optica spectrum disorder, in whom, unlike in pwMS, disability is totally dependent on the central nervous system locations of lesions, severe sympathetic dysfunction has been associated with spinal cord and/or brainstem lesions , which could explain the lack of association between spinal cord lesion in MS and worsening of ANS dysfunction.…”