Τίτλος:
Intrathecal Baclofen Therapy for Painful Muscle Spasms in a Patient with Friedreich's Ataxia
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
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. © 2018 S. Karger AG, Basel. Copyright: All rights reserved.
Συγγραφείς:
Kalyvas, A.V.
Drosos, E.
Korfias, S.
Gatzonis, S.
Themistocleous, M.
Sakas, D.E.
Περιοδικό:
Stereotactic and Functional Neurosurgery
Λέξεις-κλειδιά:
baclofen; botulinum toxin; diazepam; baclofen; central muscle relaxant, aged; Article; case report; Caucasian; clinical article; clinical effectiveness; clinical evaluation; clinical outcome; disease severity; drug treatment failure; electromyography; female; flexor muscle; follow up; Friedreich ataxia; gastrocnemius muscle; genetic screening; hearing impairment; human; latent period; low drug dose; motor nerve conduction; muscle spasm; mutism; paraplegia; pathophysiology; postoperative period; somatosensory evoked potential; spinal cord atrophy; spine injury; tibialis anterior muscle; unsteadiness; walking difficulty; complication; Friedreich ataxia; intraspinal drug administration; pain; spasticity, Aged; Baclofen; Female; Follow-Up Studies; Friedreich Ataxia; Humans; Injections, Spinal; Muscle Relaxants, Central; Muscle Spasticity; Pain
