Cognitive performance in pure and complicated hereditary spastic paraparesis: a neuropsychological and neuroimaging study

The heterogeneous group of hereditary spastic paraparesis (HSP) is characterized by spastic paraparesis and was classified clinically into pure (pHSP) and complicated (cHSP) subtypes. Whereas cHSP is often associated with cognitive impairment, little is known about the cognitive performance in pHSP. Using a case-control study design, a cohort of 20 pHSP and 9 cHSP patients was assessed neuropsychologically. In the evaluation of working and episodic memory, attention, and executive functions, the cHSP patients showed highly significantly reduced scores in all cognitive domains tested here, whereas no pathological results were observed in the pHSP group. An additional correlation analysis between a 3D magnetic resonance imaging-based calculation of the global brain atrophy and the test performance revealed a strong association for the total HSP group but only weak correlations for the two HSP subtypes. This systematic assessment illustrated the different clinical character of cHSP and pHSP with respect to the cognitive profiles.