European journal of neurology · 2026
Long-Term Outcomes in Stiff Person Spectrum Disorder.
СтатьяНазвание и аннотация: Перевод в очереди
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Mangioris Georgios, McKeon Andrew, Bower James H, Vorasoot Nisa, Dubey Divyanshu, Flanagan Eoin P, Pittock Sean J, Zekeridou Anastasia
Аннотация
BACKGROUND: Long-term outcome data for stiff person spectrum disorder (SPSD) remain limited and sustained response to treatment varies.
METHODS: In this retrospective cohort study, we included patients with probable or definite SPSD (Mayo Clinic criteria), evaluated at Mayo Clinic (12/1995-07/2024) with ≥ 5 years of follow-up after diagnosis. Primary outcome measures included the modified Rankin scale (mRS) and use of gait aids.
RESULTS: Fifty-four patients were included, 41 (76%) were female; median age at onset was 45 years (16-78). Most were antibody-positive (51 [94%]), with high-titer (serum, ≥ 20 nmol/L) GAD65-IgG in 45 (83%). Nine patients had a stiff-limb presentation (17%). The median interval from onset to diagnosis was 2 years (0-34), and median follow-up was 9 years (5-26). Most patients (46 [85%]) received immune therapy; 37 (82%) reported improvement. All patients had significant impairment (mRS ≥ 2) at nadir, with 31 (57%) losing functional independence (mRS ≥ 3) and 40 (75%) requiring bilateral gait assistance. The median time from onset to nadir was 6 years (0-39). At last follow-up, most (37 [69%]) were functionally independent (mRS ≤ 2), including 8 (15%) with minimal restrictions (mRS ≤ 1). Bilateral gait assistance was needed in 22 (41%). Among 40 patients with ≥ 10 years of follow-up, 22 (56%) had an mRS ≤ 2 and 25 (63%) required a gait aid at last follow-up. Poor outcome in this population was associated with bilateral gait assistance at nadir and older age (p < 0.05).
CONCLUSIONS: Although over half of SPSD patients lose functional independence at disease nadir, many regain independence and achieve well-controlled symptoms.
METHODS: In this retrospective cohort study, we included patients with probable or definite SPSD (Mayo Clinic criteria), evaluated at Mayo Clinic (12/1995-07/2024) with ≥ 5 years of follow-up after diagnosis. Primary outcome measures included the modified Rankin scale (mRS) and use of gait aids.
RESULTS: Fifty-four patients were included, 41 (76%) were female; median age at onset was 45 years (16-78). Most were antibody-positive (51 [94%]), with high-titer (serum, ≥ 20 nmol/L) GAD65-IgG in 45 (83%). Nine patients had a stiff-limb presentation (17%). The median interval from onset to diagnosis was 2 years (0-34), and median follow-up was 9 years (5-26). Most patients (46 [85%]) received immune therapy; 37 (82%) reported improvement. All patients had significant impairment (mRS ≥ 2) at nadir, with 31 (57%) losing functional independence (mRS ≥ 3) and 40 (75%) requiring bilateral gait assistance. The median time from onset to nadir was 6 years (0-39). At last follow-up, most (37 [69%]) were functionally independent (mRS ≤ 2), including 8 (15%) with minimal restrictions (mRS ≤ 1). Bilateral gait assistance was needed in 22 (41%). Among 40 patients with ≥ 10 years of follow-up, 22 (56%) had an mRS ≤ 2 and 25 (63%) required a gait aid at last follow-up. Poor outcome in this population was associated with bilateral gait assistance at nadir and older age (p < 0.05).
CONCLUSIONS: Although over half of SPSD patients lose functional independence at disease nadir, many regain independence and achieve well-controlled symptoms.