Dystonia Research from Movement Disorders Congress 2004

Subject: Dystonia Research from Movement Disorders Congress 2004

Date: 7/9/2004

E-MOVE reports from the Eight International Congress of Parkinson’s Disease and Movement Disorders, Rome June 14-17, 2004. Page (S) and poster (P) numbers are from Movement Disorders 2004;19 (supplement 9)

Long-term Outcomes from DBS in Dystonia (MovDis 2004)

Three studies report on the long-term outcome of deep brain stimulation of the GPi for dystonia in a total of 38 patients. In agreement with several previous studies, outcome was better in patients with primary dystonias, and less effective for those with secondary dystonias.

1. Long-term efficacy of pallidal DBS for treatment of medically intractable dystonia
M Tagliati, J Miravite, JL Shils, SB Bressman, R Saunders-Pullman, R Alterman
P935, S321-322

Bilateral GPi stimulators were implanted in 12 patients with primary dystonia (7 DYT1-positive) and 3 with secondary dystonia. Secondary dystonias were due to encephalitis or ischemic encephalopathy. All primary dystonia patients improved, with an average increase in Burke-Fahn-Marsden Dystonia Rating Scale score of 69% (range 32-97%), and a 50% decrease in disability. Benefits increased over time, with some evidence of a plateau at 12-18 months. Patients with secondary dystonias had an average improvement of 33%, with a 20% decrease in disability.

2. Long-term outcome of chronic GPi stimulation in dystonia: Sixteen cases
K Boetzel, B Bereznai, JH Mehrkens, U Steude
P264, S102

Two- to three-year follow-up of 16 patients receiving GPi stimulation for dystonia indicated excellent improvement in 6 patients and fair improvement in 5. Patients with secondary dystonia tended to do less well than those with primary dystonia.

3. Deep brain stimulation for dystonia: Outcome at long term follow-up (3 years or longer)
TJ Loher, A Kaelin-Lang, S Weber, JM Burgunder, JK Krauss
P186, S78

The authors report on 7 patients receiving GPi DBS for dystonia. Three of 4 patients with complex cervical dystonia continued to have sustained benefit for at least 4 years, while one returned to baseline. Benefit was also seen in 1 patient for 7 years with post-traumatic hemidystonia, in 1 patient for 3 years with generalized dystonia, and in 1 patient for 8 years with paroxysmal nonkinesogenic dystonia.

TorsinA Prevents Oxidative Cell Death (MovDis 2004)

Torsin-mediated neuroprotection against 6-OHDA toxicity in C. elegans
GA Caldwell, C Songsong, CC Gelwix, KA Caldwell
P227, S91

TorsinA and the roundworm analog TOR2 protect against excitotoxicity, an effect which is not shared by mutant torsinA, according to this study.

Caenorhabditis elegans overexpressed human torsinA or TOR2 under the control of a dopaminergic cell-specific promoter. 6-OHDA-induced cell death was suppressed by 70% in worms overexpressing normal torsins, versus only 30% in cells expressing mutant torsins.

Combined with a previous study showing that torsinA inhibits polyglutamine aggregation, these results suggest that “torsins play a critical role in protection against intracellular stressors,” according to the authors. A summary of the previous study is archived at http://www.mdvu.org/emove/article.asp?ID=645
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E-MOVE Editor: Richard Robinson, NASW, WE MOVE

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