Inherited Ataxia and Intrathecal Baclofen for the Treatment of Spasticity and Painful Spasms

Abstract

Background: Intrathecal baclofen (ITB) treatment is considered a powerful tool in the management of severe spasticity in neurological conditions such as multiple sclerosis, cerebral palsy, and traumatic spinal cord and brain injury. Objectives: The objective of this study was to assess the effectiveness of the ITB in patients with inherited ataxia suffering from severe painful spasms and/or spasticity. Method: A total of 5 patients with spinocerebellar ataxia 3 or 7 or Friedreich’s ataxia were included in this observational multicenter study. The patients were interviewed and completed outcome measures assessing pain (The Brief Pain Inventory), fatigue (Fatigue Severity Scale), and life satisfaction (LiSAT-9) before and 1 year after the treatment. Spasticity (Modified Ashworth Scale) and spasm frequency (SPFS) were measured objectively for each patient. Results: The mean treatment time was 1.9 years. Evaluation of established standard forms revealed symptomatic relief from spasticity, spasms, pain, and fatigue in addition to improved body posture, sleep, and life satisfaction after ITB treatment. Conclusions: We report the potential beneficial effects of ITB treatment in patients with inherited ataxia who also suffer from spasticity/spasms. ITB treatment indication in neurological disorders allows for extension to the treatment of spasticity/ spasms in patients with hereditary ataxia.

From Pathogenesis to Novel Therapeutics for Spinocerebellar Ataxia Type 3: Evading Potholes on the Way to Translation

“…Since most trials have important limitations, as previously mentioned, these results on efficacy should be interpreted with caution Spasticity Botulinum toxin administration can be effective [195]. Alternative and/or complementary pharmacological therapies available are phenol blocks [196], baclofen [197], benzodiazepines [198], or gabapentin [199]…”

Targeting Ion Channels and Purkinje Neuron Intrinsic Membrane Excitability as a Therapeutic Strategy for Cerebellar Ataxia

“…Intrathecal baclofen (ITB) infusion is accepted as a treatment for spasticity (increased rigidity of muscle secondary to injury), where it has been shown to improve positioning and decrease muscle tone in targeted patients [ 158 ]. One study has shown ITB treatment has beneficial effects in patients with SCA3, SCA7, and Friedreich ataxia who have spasticity and muscle spasms [ 159 ]. From a molecular standpoint, baclofen functions as a GABA B receptor agonist in the central nervous system [ 160 ].…”