The role of intrathecal baclofen in the management of primary and secondary dystonia in children

woon, Kim Gi; Tsegaye, Magnum; Vloeberghs, Michael

doi:10.1080/02688690701392899

Cited by 37 publications

(28 citation statements)

References 11 publications

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“…The patient was treated with a daily dosage of deferiprone just in between that used in recent pilot trials 15,16 . However, his baclofen dose was higher than that given in previous studies on ITB as a treatment for dystonia 5,19 . No adverse side effects of either drug was reported, aside from decreases in ferritin and transferrin saturation, which were not reflected in hematologic measurements such as MCV, RDW, or hemoglobin level.…”

Section: Discussionmentioning

confidence: 59%

“…Baclofen, a gamma-aminobutyric acid (GABA) receptor agonist, is one of the ‘mainstay drugs’ used to treat dystonia in patients with PKAN 4 . It is often administered via an intrathecal baclofen (ITB) pump, and has been recommended as a safe and effective treatment for dystonia 5 . ITB for the treatment of dystonia was first reported in 1991 6 .…”

mentioning

confidence: 99%

“…In a study of seven patients with PKAN treated with ITB, Albright et al found that although parents reported improvements in ease of care and severity of dystonia, there was no statistically significant difference in dystonia scores 8 . Woon et al found that treatment with ITB in PKAN patients improved vocalization, swallowing, drooling, fine motor skills, and ease of care 5 . More recently, intraventricular baclofen delivery has been used to treat PKAN with favorable outcomes.…”

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confidence: 99%

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Treatment of Classic Pantothenate Kinase–Associated Neurodegeneration with Deferiprone and Intrathecal Baclofen

Pratini¹,

Sweeters²,

Vichinsky³

et al. 2013

American Journal of Physical Medicine &Amp; Rehabilitation

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Neurodegeneration with brain iron accumulation (NBIA) describes a heterogeneous family of diseases characterized by high brain iron, particularly in the basal ganglia. The most common manifestation of childhood NBIA is classic Pantothenate Kinase-Associated Neurodegeneration (PKAN), a severe, progressive type of autosomal recessive neuroaxonal dystrophy characterized by early onset of symptoms (as opposed to atypical PKAN, with average onset age 14 years). There is currently no established therapy for the disease. Intrathecal baclofen has been reported to improve ease of care and dystonia in PKAN patients. Deferiprone, an iron chelator, has been shown to be safe and tolerable in PKAN patients, as well as effective in reducing brain iron accumulation as measured by MRI. This case report highlights the potency of combining ITB and oral deferiprone in a patient with classic PKAN. Although treatment with deferiprone alone was not attempted, this combination therapy appears to be more efficacious than treatment with only intrathecal baclofen.

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Section: Discussionmentioning

confidence: 59%

mentioning

confidence: 99%

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confidence: 99%

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Treatment of Classic Pantothenate Kinase–Associated Neurodegeneration with Deferiprone and Intrathecal Baclofen

Pratini¹,

Sweeters²,

Vichinsky³

et al. 2013

American Journal of Physical Medicine &Amp; Rehabilitation

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“…Another surgical option to consider for refractory disease is intrathecal baclofen (ITB) therapy. A number of case series have described beneficial outcomes for a variety of secondary dystonias, including those associated with cerebral palsy, traumatic brain injury, PKAN, and Wilson's disease 26, 97–101. In general, ITB appears to be most useful in treating spasticity, with an additional beneficial effect on lower extremity dystonic posturing that may reduce the incidence of a fixed orthopedic deformity.…”

Section: Surgically Intractable Secondary Dystonia: Importance Of Anamentioning

confidence: 99%

Surgical treatment for secondary dystonia

Tierney

Lozano

2012

Movement Disorders

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Surgical therapy for the secondary dystonias is generally perceived to be less effective than for primary disease. However, a number of case reports and small open series have recently appeared describing quite favorable outcomes following surgery for some nonprimary dystonias. We discuss surgical treatment options for this group of diverse conditions, including tardive dystonia, dystonic cerebral palsy, and certain heredodegenerative diseases in which deep brain stimulation and ablative lesions of the posteroventral pallidum have been shown to be effective. Other types of secondary dystonia respond less well to pallidal surgery, particularly when anatomical lesions of the basal ganglia are prominent on preoperative imaging. For these conditions, central baclofen delivery and botulinum toxin denervation may be considered. With optimal medical and surgical care, some patients with secondary dystonia have achieved reductions in disability and pain that approach those documented for primary dystonia.

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“…In addition to its role as a primary treatment modality, the effect of combined DBS and conventional surgical interventions for mixed movement disorder CP subtypes also remains undefined. In one study, Woon et al 52 treated 3 children with dystonia secondary to CP and suggested that DBS may be used to treat the primary functional disease while associated spasticity may be synergistically controlled by intrathecal baclofen infusion. Overall, these studies highlight the significant heterogeneity in movement impairments among children with CP.…”

Section: Spasticitymentioning

confidence: 99%

Current and future indications for deep brain stimulation in pediatric populations

Lipsman¹,

Ellis²,

Lozano³

2010

FOC

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Deep brain stimulation (DBS) has proven to be an effective and safe treatment option in patients with various advanced and treatment-refractory conditions. Thus far, most of the experience with DBS has been in the movement disorder literature, and more specifically in the adult population, where its use in conditions such as Parkinson disease has revolutionized management strategies. The pediatric population, however, can also be afflicted by functionally incapacitating neurological conditions that remain refractory despite the clinicians' best efforts. In such cases, DBS offers an additional treatment alternative. In this paper, the authors review their institution's experience with DBS in the pediatric population, and provide an overview of the literature on DBS in children. The authors conclude that DBS in children can and should be considered a valid and effective treatment option, albeit in highly specific and carefully selected cases.

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The role of intrathecal baclofen in the management of primary and secondary dystonia in children

Cited by 37 publications

References 11 publications

Treatment of Classic Pantothenate Kinase–Associated Neurodegeneration with Deferiprone and Intrathecal Baclofen

Treatment of Classic Pantothenate Kinase–Associated Neurodegeneration with Deferiprone and Intrathecal Baclofen

Surgical treatment for secondary dystonia

Current and future indications for deep brain stimulation in pediatric populations

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