Natural history in hereditary spastic paraplegias: real-world data from an Austrian cohort.

ABSTRACT:

Introduction

Hereditary spastic paraplegias (HSP) are rare inherited neurodegenerative disorders characterized by progressive lower limb spasticity and weakness. This study aimed to characterize an Austrian HSP cohort and prospectively assess disease progression using the Spastic Paraplegia Rating Scale (SPRS), addressing the knowledge gap regarding its longitudinal capabilities in a real-world setting.

Methods

Data from 126 patients were collected at the Center for Rare Movement Disorders Innsbruck. Baseline clinical data were available for 103 individuals. Follow-up extended up to 5 years (mean 2.3 ± 1.9). Disease severity was assessed with the SPRS, and longitudinal progression analyzed using generalized linear mixed models.

Results

The cohort (64.3% male, mean age

SUBMITTER: Amprosi M 

PROVIDER: S-EPMC12835074 | biostudies-literature | 2026 Jan

REPOSITORIES: biostudies-literature