To evaluate the potential use of the Quality of Life in Neurological Disorders (Neuro-QoL) questionnaires for assessment of symptomatic burden and functional disability in stiff person syndrome spectrum disorders (SPSD).

SPSD are rare, disabling neurological disorders that profoundly impact patients’ social, emotional, and physical well-being. Common complaints outside of impaired mobility include anxiety, sleep disturbances, and fatigue. Despite improving quality of life being central to SPSD care, there remains an unmet need for standardized health-related quality of life measures.

Data were collected across 10 Neuro-QoL domains for a convenience sample of 19 patients. Demographics, clinical characteristics, and T25FW measures were also collected. Spearman correlation or one-sample t-tests (reference mean=50) were used to determine associations as appropriate.

Nineteen patients with median age 60 [IQR 55-65] participated (13 classic SPS, 5 SPS-plus, 1 PERM). Initial symptom onset was 12 [IQR 9, 20] years. Most patients were female (84%) and White (74%). SPSD patients scored lower in social participation (t=-4.0, p=0.001) and satisfaction (t=-3.7, p=0.002). They also showed mild upper extremity and moderate lower extremity (LE) functional impairments (t=-3.7, p=0.002; t=-5.1, p=0.000, respectively). There was a strong negative correlation between LE and T25FW (rho=-0.92, p=0.001 for unassisted T25FW and rho=-0.82, p=0.034 for T25FW with bilateral assistance). Participants also scored worse in stigma (t=2.5, p=0.02). No other statistically significant differences were observed.