Botulinum toxin for hereditary spastic paraplegia: effects on motor and non-motor manifestations

Abstract

Motor and non-motor manifestations are common and disabling features of hereditary spastic paraplegia (HSP). Botulinum toxin type A (Btx-A) is considered effective for spasticity and may improve gait in these patients. Little is known about the effects of Btx-A on non-motor symptoms in HSP patients. Objective To assess the efficacy of Btx-A on motor and non-motor manifestations in HSP patients. Methods Thirty-three adult patients with a clinical and molecular diagnosis of HSP were evaluated before and after Btx-A injections. Results Mean age was 41.7 ± 13.6 years and there were 18 women. Most patients had a pure phenotype and SPG4 was the most frequent genotype. The Btx-A injections resulted in a decrease in spasticity at the adductor muscles, and no other motor measure was significantly modified. In contrast, fatigue scores were significantly reduced after Btx-A injections. Conclusion Btx-A injections resulted in no significant functional motor improvement for HSP, but fatigue improved after treatment.

Efficacy of a Combined Treatment of Botulinum Toxin and Intensive Physiotherapy in Hereditary Spastic Paraplegia

“…Our study also showed a significant improvement of the pain perceived by patients. Only one previous study has investigated the effectiveness of BoNT-A in HSP in terms of pain reduction, but showed no significant improvements (Servelhere et al, 2018). However, the same study did not combine the chemodenervation with PT and this may have mitigated the effects of the treatment.…”

“…The selective chemodenervation via intramuscular injection of botulinum toxin type A (BoNT-A) is widely used in the treatment of focal spasticity in patients with stroke, multiple sclerosis, spinal cord injuries and various neurological disorders (Dunne et al, 1995;Keren et al, 2000;Ward, 2008). There have been several reports on the use of botulinum toxin in HSP patients but with discrepancy of the various clinical protocols (Rousseaux et al, 2007;Hecht et al, 2008;Geva-Dayan et al, 2010;de Niet et al, 2015;Riccardo et al, 2016;Servelhere et al, 2018). Previous studies demonstrate the effectiveness of toxin injection at the level of triceps sural and/or of the adductors muscles as beneficial in reducing the spasticity and increasing the walking speed (Rousseaux et al, 2007;Hecht et al, 2008;de Niet et al, 2015).…”

“…However, functional improvements have been found only in a limited number of patients with a clinical diagnosis of HSP. The motor and non-motor effects of BoNT-A were described in 33 patients with definite HSP diagnosis (Servelhere et al, 2018), reporting improvement in terms of adductors tone reduction and perceived fatigue. The muscles more frequently inoculated were: triceps sural and adductors.…”

Use of botulinum toxin for movement disorders

“…BoNTs were rarely used and tested in studies for spasticity in progressive diseases. Ineffectiveness for motor enhancement in hereditary spastic paraplegics in a study, for example, is not enough to define the indication status for BoNTs in these patients 129…”

Health-Related Quality of Life Outcomes from Botulinum Toxin Treatment in Spasticity

“…Spasticity may deteriorate health-related quality of life (HR-Qol) through different mechanisms. Impairments in mobility, loss of self-dependence and need of nursing support, anxiety, and depression may reduce the self-perceived state of health and satisfaction in life [1,2]. In addition, recent studies identified additional modifiers of self-perceived HR-Qol in spasticity; one study in 62 children with cerebral palsy reported that spasticity affected gross motor functions [3], and that impaired motor function significantly deteriorated HR-Qol [3].…”