Abstract
IntroductionThe Hereditary Spastic Paraplegia (HSP) is a heterogeneous group of neurodegenerative disorders characterized by progressive spasticity and lower limbs (LL) weakness. There is no treatment to cure or halt the disease, except for symptomatic therapy. The use of botulinum toxin type A (BoNT-A) is one of the primary treatment for focal spasticity. Physiotherapy (PT) can help in maintaining residual functioning. We performed a retrospective study to evaluate the effect of the combined BoNT-A and intensive PT in patients with HSP.MethodsEighteen adult patients (50% females) with clinical diagnosis of HSP were recruited. Eleven patients had a genetic diagnosis of SPG4, 5, 7, 8, 11, 72. Patients were all autonomously deambulant or needed support. BoNT-A was injected in 36 LL in different spastic muscles under electromyographic guidance and followed by intensive PT sessions. Outcome measures included disease severity, motor functional measures, perceived pain self-report and quality of life. Assessments occurred at baseline, 1 and 3 months after BoNT-A injection.ResultsMost inoculated muscles were hamstrings, rectus femoris and gastrocnemius. We observed an improvement in muscle tone, in the gait velocity and distance length. Spastic Paraplegia Rating Scale was significantly reduced after treatment, in addition to improving pain and quality of life. These results were riconfirmed in 3 months time.ConclusionOur study indicates that combined treatment of BoNT-A and PT can lead to improvement of spasticity and quality of life in patients with HSP.
Highlights
The Hereditary Spastic Paraplegia (HSP) is a heterogeneous group of neurodegenerative disorders characterized by progressive spasticity and lower limbs (LL) weakness
The Hereditary Spastic Paraplegia (HSP) or Strumpell-Lorrain syndrome comprises a heterogeneous group of neurodegenerative disorders caused by axonal retrograde degeneration of the long corticospinal tracts (Fink, 2014; Lo Giudice et al, 2014; Klebe et al, 2015; de Souza et al, 2017)
HSP presents with progressive spasticity and weakness of the lower limbs (LL), reduced
Summary
The Hereditary Spastic Paraplegia (HSP) is a heterogeneous group of neurodegenerative disorders characterized by progressive spasticity and lower limbs (LL) weakness. The Hereditary Spastic Paraplegia (HSP) or Strumpell-Lorrain syndrome comprises a heterogeneous group of neurodegenerative disorders caused by axonal retrograde degeneration of the long corticospinal tracts (Fink, 2014; Lo Giudice et al, 2014; Klebe et al, 2015; de Souza et al, 2017). When the disease occurs in early childhood, the symptoms may not progress significantly for several years and decades; on the contrary, the late-onset HSPs might be associated with a more insidious worsening pattern (Harding, 1993). It has been reported that, while in the pure forms patients rarely resort to using the wheelchair but frequently walk with aids, in the complex forms the functional disability depends on the extent and severity of extra-motor involvement (Lo Giudice et al, 2014)
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