Functional Neurosurgery for Secondary Dystonia: Indications and Long-Term Results
Dystonia is a movement disorder characterized by patterned, repetitive, phasic, or tonic sustained muscle contractions that produce abnormal, often twisting, postures or repetitive movements. When the disorder is genetic or the cause is unknown and dyston
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Abstract Dystonia is a movement disorder characterized by patterned, repetitive, phasic, or tonic sustained muscle contractions that produce abnormal, often twisting, postures or repetitive movements. When the disorder is genetic or the cause is unknown and dystonia is the sole feature, the disease is called primary or idiopathic, conversely secondary dystonia (SD) may be caused by various brain insults. Both primary dystonia and SD have been notorious for their poor response to medical treatment. Today, stereotactic neurosurgical procedures are offered to improve the disability and quality of life of patients who do not respond to medical therapy. However, SD shows less and more variable results than primary dystonia to neurosurgical procedures, the benefits of ablative or deep brain stimulation (DBS) procedures in basal structures being still subject to debate and much harder to fully appreciate. In this work, the authors show a 33-patient series with secondary dystonia, separating the statistic and clinical analysis into several etiology groups: perinatal insults, tardive syndromes, genetic syndromes, and posttraumatic. In these groups, we show the mean BFM score improvement in the different patient series, comparing our results with world literature, and finally propose a classification system for bettering the clinical approach in surgery decision when this is indicated. Keywords Secondary dystonia • Deep brain stimulation • Intrathecal baclofen • Brain motor circuits
Introduction Dystonia is a movement disorder characterized by patterned, repetitive, phasic, or tonic sustained muscle contractions that produce abnormal, often twisting, postures or repetitive movements [1, 2]. When the disorder is genetic or the cause is unknown and dystonia is the sole feature, the disease is called primary or idiopathic; conversely, secondary dystonia (SD) may be caused by various brain insults [2, 3] (Table 1). Both primary (especially generalized) and SD have been notorious for their poor response to medical treatment; stereotactic functional neurosurgical procedures are offered to improve the disability and quality of life of patients who do not respond to medical therapy. Nowadays, the preferred surgical target in primary dystonia patients is the globus pallidus internus (Gpi) [1, 2, 4–13], with encouraging results reported in the long term. In contrast to the overall good response in patients with idiopathic dystonia, patients with SD showed less and more variable results with functional neurosurgical brain Gpi procedures, the benefit of deep brain stimulation (DBS) in this target for SD being still subject to debate and much harder to fully appreciate [1, 2, 12, 13]. In this chapter, we show our surgical experience in secondary dystonia and our proposal to separate disease entities to a better systematic approach in these patients.
Materials and Methods
J.A.E. Martinez (*), O.A.E. Vidarte, and G.A. Uribe Movement Disorders and Pain Management Center, CIMAD, Calle 19 A # 82-14, Bogota, Colombia Marly Clinic, Bogota, C
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