To investigate the presence and correlation of dysautonomia with the severity of hereditary spastic paraplegias (HSP). 

Hereditary spastic paraplegias (HSP) are a heterogeneous group of genetic diseases that affect the corticospinal tract. Although HSP present wide phenotypic and genotypic variability, little is known about the impact of its autonomic manifestations. 

Forty patients were selected and submitted to clinical evaluation using the SPRS (Spastic Paraplegia Rating Scale) and SCOPA-AUT (Scales for Outcomes in Parkinson's disease - autonomic questionnaire) scales. SPRS was applied to quantify the severity of spasticity and SCOPA-AUT to assess dysautonomia. A group of 43 controls was selected, matched in age and gender with patients with HSP. Results were considered statistically significant when p<0.05. 

The group of patients had significantly higher SCOPA-AUT scores than the control group (20 (17.75) vs 11 (16)); (p <0.001). When each SCOPA-AUT domain was evaluated, the gastrointestinal (p <0.001), urinary (p=0.031) and thermoregulatory (p=0.004) domains resulted in significantly higher scores in the patient group. Other domains showed no significant difference. Significantly higher scores were obtained in female patients, both in SCOPA-AUT (p=0.049) and in SPRS (p=0.049). There was a significant positive correlation between SCOPA-AUT and SPRS total scores (rho=0.389, p=0.015). 

Our results demonstrate that dysautonomia is significantly frequent in patients with HSP, especially in females. Dysautonomia directly impacts on the severity of HSP, and it is suggested that it be taken into account for the earliest diagnosis of the disease and as a target for its treatment.