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Treatment of secondary dystonia with a combined stereotactic procedure: long-term surgical outcomes

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Abstract

Objective

There is some debate about the effects of pallidal deep brain stimulation (DBS) or lesioning on secondary dystonia. We applied a multimodal method to maximize the treatment effects of deep brain stimulation in patients with secondary dystonia.

Methods

Between March 2003 and January 2009, four patients underwent bilateral globus pallidus internus (GPi) DBS and six patients underwent bilateral GPi DBS plus unilateral thalamotomy for treatment of cerebral palsy (CP). Among the patients with secondary dystonia without CP, five were also treated by DBS. We classified patients with generalized secondary dystonia with cerebral palsy into group I and patients with focal dystonia without CP into group II. Clinical outcome assessments were based on Burke-Fahn-Marsden Dystonia Rating Scale movement and disability scores. Heath-related quality of life was assessed with a 36-item short-form general health survey questionnaire preoperatively and at the last follow-up.

Results

The movement and disability scores of group I-A had improved by 32.0% (P = 0.285) and 14.3% (P = 0.593), respectively, at the last follow-up compared with baseline. The movement and disability scores of group I-B had improved by 31.5% and 0.18% at the last follow-up compared with baseline, respectively. In comparison with patients in group I-A, patients in group I-B showed a significant improvement in movement scores for the contralateral arm (P = 0.042). Group II patients showed a marked improvement in movement and disability scores of 77.7% (P = 0.039) and 80.0% (P = 0.041), respectively.

Conclusions

We demonstrated that DBS plus unilateral ventralis oralis thalamotomy for CP patients with fixed states in the upper extremities is useful not only to treat secondary dystonic movement but also to improve quality of life. In group II patients with post-traumatic dystonia and tardive dyskinesia, we achieved excellent clinical outcomes using a stereotactic procedure.

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Acknowledegments

The authors would like to thank Eun Jeong Kwon (RN) for clinical data acquisition and assistance with this paper. This study was supported by a faculty research grant of Yonsei University College of Medicine for 2009 (6-2009-0063).

Conflicts of interest

None.

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Authors and Affiliations

  1. Department of Neurosurgery, Severance Hospital, Brain Korea 21 Project for Medical Science, Brain Research Institute, Yonsei University College of Medicine, 134 Shinchon-Dong, Seodaemoon-Gu, 120-752, Seoul, Korea

    Joo Pyung Kim, Won Seok Chang & Jin Woo Chang

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  1. Joo Pyung Kim
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  2. Won Seok Chang
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  3. Jin Woo Chang
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Correspondence to Jin Woo Chang.

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Comment

In this interesting paper the authors report their experience in a small population of adult patients with secondary dystonia (with or without CP), their surgical approach consisted of GPi/DBS and GPi/DBS plus Vo thalamotomy. In group I (CP-dystonia) there were ten patients: I-A (four patients underwent bilateral DBS/GPi) and I-B (six patients underwent GPi/DBS plus Vo thalamotomy). Group II consisted of secondary dystonia without CP.

In group I, patients treated with GPi/DBS plus Vo thalamotomy experienced an improvement of the movement and disability scores of 31.5% and 0.18% and patients with GPi/DBS had improvement of 32% and 14.3%. In group II (all patient were treated with DBS/GPi), there was a significant improvement in movement and disability scores of 77% and 80% respectively. The authors should be congratulated for their relatively novel and aggressive approach to secondary dystonia, a condition traditionally very difficult to treat and for their good surgical results and lack of complications.

Secondary dystonia is a severely disabling condition that is very difficult to treat (and to correctly diagnose). In several cases, secondary dystonia is associated with CP with clinical presentation during the paediatric age. One of the major problems associated with secondary dystonia is the timing of treatment and the overall assessment of the clinical condition.

In these patients there are generally two sets of problems:

1. Dystonic movement (ballistic and choreic movement are present in several cases as well)

2. Spasticity (with consequent orthopaedic deformities if the spasticity is not addressed timely)

In an ideal situation, secondary dystonia (with or without CP) should be considered for treatment at presentation in a integrated functional neurosurgical service that address both the movement disorder (dystonia) and the spasticity. Realistic functional goals should be determined and a variety of medical/surgical interventions (DBS, lesioning, intrathecal baclofen, SDR, DREZ lesioning, Botox, etc.) should be considered and tailored to the individual patient [4–7].

Treatment of this condition in adulthood is problematic and to a certain degree sub-optimal (neurological deficit and limb deformities are already well established), there is a tendency to address either the movement disorder component by means of DBS or lesioning or the spasticity (but very rarely both).

In group I the authors used a novel approach, combining a Vo lesioning with DBS/GPi. Cooper [1] introduced Vo thalamotomy for dystonia with an improvement rate of 69% and several authors reported variable and inconsistent results with this tecnique [2,3], the combination of DBS/GPi with unilateral Vo thalamotomy may be a solution for cases of secondary dystonia in which DBS alone is not sufficient to address the dystonic movement.

Articles reporting surgical treatment for secondary dystonia and CP dystonia are uncommon in the current literature; both the surgical approach and the result represent a valuable contribution to the existing literature on this difficult and frustrating clinical condition.

Jibril Osman Farah

Liverpool, UK

References

1. Cooper IS (1976) 20 years follow up study of the neurosurgical treatment of dystonia musculorum deformans. In: Eldridge R, Fahn S (eds) Dystonia: advances in neurology. Raven Press , New York, pp 423–452.

2. Yamashiro K, Tasker RR (1993) Stereotactic thalamotomy for dystonic patients. Stereotact Funct Neursurg 60(1–3):81–85

3. Cardoso F et al (1995) Outcome after stereotactic thalamotomy for dystonia and hemiballismus. Neurosurgery 36(3):501–508

4. Sindou M, Abbott R, Keravel Y (1991) Neurosurgey for spasticity: a multidisciplinary approach. Springer, New York

5. Vidailhet M et al (2009) Bilateral Pallidal deep brain stimualtion stimulation for the treatment of patients with dystonia-choreoathetosis cerebral palsy: a prospective pilot study. Lancet Neurol (8):709–717

6. Steinbok P (2007) Selective dorsal rhizotomy for spasticity cerebral palsy: a review. Child Nerv Syst 23(9):981–990

7. Motto et al (2008) Effect of intrathecal baclofen for dystonia in children with cerebral palsy and the use of functional scales. J. Pediatri Orthop 28(2):213–217

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Kim, J.P., Chang, W.S. & Chang, J.W. Treatment of secondary dystonia with a combined stereotactic procedure: long-term surgical outcomes. Acta Neurochir 153, 2319–2328 (2011). https://doi.org/10.1007/s00701-011-1147-6

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