Intrathecal baclofen for intractable axial dystonia

Narayan, Raj K.; Loubser, Paul G.; Jankovic, Joseph; Donovan, William H.; Bontke, Catherine F.

doi:10.1212/wnl.41.7.1141

Cited by 134 publications

(71 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Scoliosis is a constant finding in severe forms of dystonia such as dystonia musculorum deformans, and also in other forms of generalized dystonia [39,40]. Sometimes, scoliosis is the first sign of a dystonia and the deformity progression can be controlled after treating the dystonia, for example, with L-dopa [41,42].…”

Section: Discussionmentioning

confidence: 99%

Motor cortical hyperexcitability in idiopathic scoliosis: could focal dystonia be a subclinical etiological factor?

et al. 2009

View full text Add to dashboard Cite

The aetiology of idiopathic scoliosis (IS) remains unknown; however, there is a growing body of evidence suggesting that the spine deformity could be the expression of a subclinical nervous system disorder. A defective sensory input or an anomalous sensorimotor integration may lead to an abnormal postural tone and therefore the development of a spine deformity. Inhibition of the motor cortico-cortical excitability is abnormal in dystonia. Therefore, the study of cortico-cortical inhibition may shed some insight into the dystonia hypothesis regarding the pathophysiology of IS. Paired pulse transcranial magnetic stimulation was used to study corticocortical inhibition and facilitation in nine adolescents with IS, five teenagers with congenital scoliosis (CS) and eight healthy age-matched controls. The effect of a previous conditioning stimulus (80% intensity of resting motor threshold) on the amplitude of the motor-evoked potential induced by the test stimulus (120% of resting motor threshold) was examined at various interstimulus intervals (ISIs) in both abductor pollicis brevis muscles. The results of healthy adolescents and those with CS showed a marked inhibitory effect of the conditioning stimulus on the response to the test stimulus at interstimulus intervals shorter than 6 ms. These findings do not differ from those reported for normal adults. However, children with IS revealed an abnormally reduced cortico-cortical inhibition at the short ISIs. Cortico-cortical inhibition was practically normal on the side of the scoliotic convexity while it was significantly reduced on the side of the scoliotic concavity. In conclusion, these findings support the hypothesis that a dystonic dysfunction underlies in IS. Asymmetrical cortical hyperexcitability may play an important role in the pathogenesis of IS and represents an objective neurophysiological finding that could be used clinically.

show abstract

Section: Discussionmentioning

confidence: 99%

Motor cortical hyperexcitability in idiopathic scoliosis: could focal dystonia be a subclinical etiological factor?

et al. 2009

View full text Add to dashboard Cite

show abstract

“…29 Narayan et al have described postoperative SD in an adolescent with CP who required muscle paralysis and ventilatory support. 33 Although numerous drugs failed, the patient showed a remarkable response to ITB, which allowed him to be weaned off ventilation and eventually discharged. 33 However, ITB is not without complications, which include catheter migration, withdrawal syndromes, and overdosage, 7,32 although the last problem is less likely with our setup.…”

Section: Discussionmentioning

confidence: 99%

“…15 Tetrabenazine and benzhexol may be efficacious, but the majority of patients who receive these drugs require sedation and further intervention. 15,29 Previous reports have found ITB to be efficacious in this setting, 18,30,33 but evidence to the contrary also exists. 29 Narayan et al have described postoperative SD in an adolescent with CP who required muscle paralysis and ventilatory support.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Indwelling intrathecal catheter with subcutaneous abdominal reservoir: a viable baclofen delivery system in severely cachectic patients

Waqar

Ellenbogen²,

Kumar³

et al. 2014

PED

View full text Add to dashboard Cite

Intrathecal baclofen (ITB) is a reversible treatment that reduces muscle tone to ameliorate spasticity and dystonia in patients with cerebral palsy (CP). The resulting decrease in energy expenditure allows patients to gain much-needed weight, albeit temporarily. Modern techniques require sufficient abdominal musculature and subcutaneous fat to permit the implantation of an indwelling pump. In patients with extremely low muscle bulk, visceral pumps may be impractical or impossible, with increased risks of dehiscence and infection. The authors describe a variation of the classical procedure in a young patient with severe cachexia. A 10-year-old boy with spastic-dystonic quadriplegic CP was admitted to the neuromedical unit. Numerous drug trials had failed, and surgical intervention was deemed necessary but was complicated by his cachectic body habitus. The authors inserted a lumbar intrathecal catheter and subcutaneously tunneled it to the anterolateral abdomen, where it was connected to a subcutaneous injection port. Baclofen was continuously infused into the subcutaneous port using a noncoring needle connected to an external pump. The needle and line were changed every 5 days to minimize the risk of sepsis. Although other techniques, such as intraventricular baclofen delivery, have been described, these are largely dependent upon sufficient musculature to support a visceral pump. A subcutaneous injection port system represents an alternative approach that reduces the risk of sepsis and may be better tolerated in cachectic patients.

show abstract

“…The first successful use of ITB was described at Baylor College of Medicine in a patient with refractory axial dystonia [102]. ITB may be particularly efficacious in patients with predominant lower limb dystonia and associated spasticity.…”

Section: Intrathecal Baclofenmentioning

confidence: 99%

Treatment of Dystonia

Thenganatt

Jankovic

2014

Neurotherapeutics

View full text Add to dashboard Cite

Selecting the appropriate treatment for dystonia begins with proper classification of disease based on age, distribution, and underlying etiology. The therapies available for dystonia include oral medications, botulinum toxin, and surgical procedures. Oral medications are generally reserved for generalized and segmental dystonia. Botulinum toxin revolutionized the treatment of focal dystonia when it was introduced for therapeutic purposes in the 1980s. Surgical procedures are available for medication-refractory dystonia, markedly affecting an individual's quality of life.

show abstract

Intrathecal baclofen for intractable axial dystonia

Abstract: Following spinal instrumentation for scoliosis, a patient developed intractable axial dystonia refractory to conservative medical management. We describe the successful treatment of this dystonia with a continuous infusion of intrathecal baclofen and the subsequent long-term management.

Cited by 134 publications

References 0 publications

Motor cortical hyperexcitability in idiopathic scoliosis: could focal dystonia be a subclinical etiological factor?

Motor cortical hyperexcitability in idiopathic scoliosis: could focal dystonia be a subclinical etiological factor?

Indwelling intrathecal catheter with subcutaneous abdominal reservoir: a viable baclofen delivery system in severely cachectic patients

Treatment of Dystonia

Contact Info

Product

Resources

About