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Volker M. Tronnier and Wolfgang Fogel

D ystonia is a neurological syndrome characterized by involuntary, sustained, patterned, and often repetitive contractions of opposing muscles that causes twisting or spasmodic movements or abnormal postures. 8 Primary dystonia is usually a hereditary disease, for which the gene locus has already been determined. 19 In juvenile-onset primary dystonia, approximately 72% of patients have the tendency to develop generalized dystonia, in contrast with 5% of patients with adult-onset dystonia. Especially in the former patients, medical treatment is often

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A. Leland Albright

T he use of ITB for dystonia was first reported in 1991, in a case report of an 18-year-old boy with generalized dystonia who improved at a dosage of 825 μg baclofen/day. 7 Since then, several authors have reported that administration of ITB improves secondary dystonia. 5 , 8 , 10 In 2001, we described 86 children treated with ITB for dystonia, 71% of whom had cerebral palsy. 1 Their dystonia scores decreased significantly (p < 0.005) and remained decreased in 92% of cases at a mean follow-up period of 29 months. Since then, we have used ITB to treat

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Juei-Jueng Lin, Ging-Yau Lin, Chunhsi Shih, Shinn-Zong Lin, Dar-Cheng Chang and Chan-Chian Lee

P harmacotherapeutic effects on patients with generalized dystonia are often disappointing. Although surgical therapy with thalamotomy can alleviate clinical symptoms in some patients, there are risks of complications and adverse effects, particularly when the surgery is bilateral. 1, 3 In recent years, there have been a few reports showing successful application of posteroventral pallidotomy (PVP) in the treatment of patients with dystonia. 6, 9 The results showed that surgical therapy could significantly ameliorate dystonic movements and improve motor

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Dan Piña-Fuentes, Martijn Beudel, Simon Little, Jonathan van Zijl, Jan Willem Elting, D. L. Marinus Oterdoom, Martje E. van Egmond, J. Marc C. van Dijk and Marina A. J. Tijssen

A ccording to the latest expert consensus, dystonia is clinically defined as “a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both.” 2 Dystonia can be caused by numerous etiologies, including acquired and different biochemical, cellular, or genetic substrates. The similarity of the clinical dystonia phenotype, however, suggests a unifying common pathophysiological pathway at a functional or network level. 4 A currently leading hypothesis on its neurophysiological

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A. Leland Albright, Margaret J. Barry, Michael J. Painter and Barbara Shultz

C erebral palsy (CP) occurs in 1.5 to 2.5 of 1000 live births and is associated with movement disorders such as spasticity, athetosis, and dystonia. Dystonia is characterized by sustained muscle contractions, causing twisting and repetitive movements or abnormal postures. 12 It frequently impairs function and may be painful. Dystonia is classified by the patient's age at onset, anatomical distribution, and cause. It may be an idiopathic, independent disorder or secondary to other conditions. Dystonia affects 15 to 25% of persons with CP, in whom it is

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A. Leland Albright and Susan S. Ferson

S ince 1991, ITB has been used to treat dystonia, particularly dystonia secondary to CP or traumatic brain injury, when Narayan et al. 15 reported its use in an 18-year-old man with generalized dystonia. During the 1990s, several investigators reported the use of ITB to treat primary and secondary dystonia, including generalized dystonia, hemidystonia, and focal dystonia. Improvement was observed in 28 of the 38 patients from 10 different studies. 1 , 6 , 8 , 10–13 , 16 , 17 , 19 In 2001, we reported the use of ITB to treat severe generalized dystonia in

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S. Elizabeth Zauber, Nidhi Watson, Cynthia L. Comella, Roy A. E. Bakay and Leo Verhagen Metman

D eep brain stimulation of the GPi can successfully treat PD and dystonia. Pallidal stimulation can also worsen akinesia in patients with PD, depending on where along the ventro-dorsal axis stimulation is applied. 1 , 5 We report the unexpected finding that DBS of the GPi induced significant parkinsonism in a patient with craniocervical dystonia who did not have preexisting bradykinesia. Case Report History This 42-year-old man developed progressive craniocervical dystonia over a 3-month period. The initial symptom was blepharospasm

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Atsushi Umemura, Jurg L. Jaggi, Carol A. Dolinskas, Matthew B. Stern and Gordon H. Baltuch

D ystonia is a clinical syndrome characterized by an involuntary muscle contraction causing a sustained twisted or abnormal posture. In generalized dystonia, symptoms begin in an arm or a leg and advance, spreading to involve the trunk and the rest of the body, and some patients become severely disabled despite medical treatment. Most cases of dystonia occur in the absence of an identified cause or structural lesion in the nervous system, and they are considered to be hereditary and are classified as primary or idiopathic dystonia. Most cases of early

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Shouyan Wang, Xuguang Liu, John Yianni, Alex L. Green, Carole Joint, John F. Stein, Peter G. Bain, Ralph Gregory and Tipu Z. Aziz

D ystonia is a clinical syndrome characterized by twisting, abnormal postures, repetitive movements, and pain resulting from sustained muscle contractions. Chronic DBS of the GPI is becoming a favored treatment for dystonia in patients unresponsive to medical intervention. 9 , 10 , 12 , 19 , 23 Note, however, that outcomes have varied greatly in terms of both overall effectiveness and time required for a treatment response. 5 , 19 , 23 This variation is probably due to our limited understanding of the pathophysiology of dystonia, despite various attempts

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Robert J. Bollo, Judith L. Gooch and Marion L. Walker

S econdary dystonia is a prominent feature in 15%–25% of patients with CP and is often refractory to medical therapy. 4 , 18 Secondary generalized dystonia is also a common sequela of traumatic and anoxic brain injury. 5 , 18 Narayan et al. 19 first described the use of intrathecal baclofen to treat secondary generalized dystonia in 1991. Since then, multiple studies have demonstrated its efficacy for children with secondary dystonias. 4 , 5 , 8 , 10 , 18 , 21 , 26 , 27 Albright and colleagues 5 demonstrated that a more cranial location of the