Intrathecal Baclofen Therapy for Painful Muscle Spasms in a Patient with Friedreich’s Ataxia

Abstract

Friedreich’s ataxia (FA) is the most frequent hereditary ataxia syndrome, while painful muscle spasms and spasticity have been reported in 11–15% of FA patients. This report describes the successful management of painful spasms in a 65-year-old woman with FA via intrathecal baclofen (ITB) therapy following unsuccessful medical treatments. To our knowledge, this is the third reported case in the literature. Unfortunately, the pathophysiological characteristics of muscle spasms in FA are not well explored and understood while the therapeutic mechanisms of the different treatments are rather vague. Taking into consideration the suggested spinal atrophy in FA, the clinical resemblance of FA and chronic spinal injury muscle spasms, together with the rapid ITB therapy effectiveness in alleviating FA muscle spasms, we attempted to suggest a putative pathophysiological mechanism acting at the spinal level and possibly explained by the presence of independent spinal locomotor systems producing muscle spasms. Specifically, overexcitement of these centers, due to loss of normal regulation from upper CNS levels, may result in the uncontrolled firing of secondary motor neurons and may be the key to producing muscle spasms. However, further research under experimental and clinical settings seems to be necessary.

Advanced implantable drug delivery technologies: transforming the clinical landscape of therapeutics for chronic diseases

“…By contrast, SynchroMed™ II pumps have a fixed drug dose between rollers that cannot be finely tuned. Furthermore, the accuracy, efficacy, and safety of Prometra® pumps were demonstrated in patients for up to 12 months (Kalyvas et al 2014;Rauck et al 2010Rauck et al , 2013.…”

“…As previously mentioned, these pumps have movable components that control drug delivery, increasing the risk of device malfunctions. There are reports of cases in which the pump had to be explanted due to pump failures (Kalyvas et al 2014;Riordan and Murphy 2015;Sgouros et al 2010). These discoidal pumps are limited by their size and therefore necessitate catheters to deliver the drug to the site of interest.…”

“…These discoidal pumps are limited by their size and therefore necessitate catheters to deliver the drug to the site of interest. Given that most complications are attributable to catheter malfunctions, implants should be redesigned to omit the need for catheters (Ethans et al 2005;Kalyvas et al 2014;Miracle et al 2011;Stetkarova et al 2010). Even though abdominal subcutaneous placement of pumps clearly has limitations and challenges, the devices have had a positive impact on improving patient health and living conditions, outweighing the complications and reported adverse effects.…”