Significant long-term improvements in spasticity
A long-term study of intrathecal baclofen has demonstrated significant improvement in severe spasticity in seven people with HSP. Improvements were noted for 2 to 3 years post implementation followed by stable walking and mobility functions for 4 to 5 years. Further progression was related to the progressive course of the disease, which varied for individuals.
Introduction: Treatment with intrathecal baclofen (ITB) is a therapeutic option in the management of severe spasticity in patients with hereditary spastic paraparesis (HSP). However, information on the impact of ITB on the natural course of disease, especially the effect of ITB on functional parameters over time is limited.
Materials and Methods: We evaluated seven patients with HSP retrospectively who were treated with an ITB device. The following parameters were measured before (pre-implantation) and after implantation (post-implantation) of the ITB device at steady state dosage of ITB and annually until last follow-up: modified Ashworth Scale, Reflex Scale, modified Rankin Scale, and Rivermead Mobility Index. The ITB dosages were assessed after reaching steady state as well as annually until last follow-up.
Results: The ITB device was implanted 13 ± 6 (range 9-16) years after diagnosis of HSP on average. Severe spasticity was controlled in all patients by a mean baclofen dosage of 188 ± 60 (range 145-230) μg per day at steady state post-implantation. The modified Ashworth Scale improved significantly from 3 (interquartile range [IQR] 3-3.25) to 1 (IQR 1-1.25; p = 0.046), as did the Reflex Scale from 5 (IQR 4.75-5) to 3 (IQR 2.75-3; p = 0.046) at steady state dosage of ITB. The modified Rankin Scale improved from 2 (IQR 2-2) to 1 (IQR 1-1.5; p = 0.083) and the Rivermead Mobility Index remained 14 (IQR 13.5-14 pre-implantation, IQR 14-14 post-implantation; p = 0.18). Post-implantation, spasticity improved for 2-3 years, followed by a stable phase of ambulatory and other mobility functions for 4-5 years. Thereafter, the maintenance or progressive loss of mobility depended on individual courses of the disease. No ITB-related severe side effects occurred.
Discussion: Our data further support the role of ITB in the treatment of severe spasticity in patients with deteriorated walking performance suffering HSP. ITB therapy may initially improve spasticity and stabilize mobility functions for the first 6-8 years in patients with HSP.
SOURCE: Front Neurol. 2019 Aug 23;10:901. doi: 10.3389/fneur.2019.00901. eCollection 2019. PMID: 31507512
Intrathecal Baclofen in Hereditary Spastic Paraparesis.
Pucks-Faes E1, Dobesberger J2, Hitzenberger G1, Matzak H1, Mayr A1, Fava E1, Genelin E1, Saltuari L1,3.
1 Department of Neurology, Hochzirl Hospital, Zirl, Austria.
2 Department of Neurology, Paracelsus Medical University, Salzburg, Austria.
3 Research Unit for Neurorehabilitation, Bolzano, Italy.
I’ve recently had an internal (baclofen) pump inserted. I know it won’t cure my HSP7 but it has made a big difference. I’m still going thru the process of getting the right dosage but I’m not getting near as tired as when I was on “oral” baclofen and I am walking easier.
The pump is not the answer for everyone.
I’m intrigued by this. The Baclofen pump has helped with your gait? I see a specialist every 6 weeks and it hasn’t been mentioned. Weird. I’m taking 80mg of Baclofen a day at the moment with no results
Editor’s Note: Spasticity in people with HSP varies along a continuum from mild to severe. Muscle weakness in people with HSP varies over a similar range. This is not the same as the variations that can occur because of season/air temperature, fatigue, stress and so on. The general spasticity/weakness profile varies from individual to individual. For those where spasticity is dominant, oral baclofen is a common choice, however it is not effective for everyone with HSP. In this scenario, another option is the intrathecal baclofen pump, where there is precision placement of a calculated dose via catheter into the cerebrospinal fluid in the lower spine. There is an assessment process to determine suitability for the pump. Talk with your specialist about the possibility. The evidence, both in this research study and in previous ones, is that this type of baclofen administration is effective in managing spasticity for people assessed as suitable for this treatment.