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. 2007 Apr 25;92(8):708–711. doi: 10.1136/adc.2006.095380

Table 1 Clinical data.

Case number Initial clinical presentation DYT‐1 status Evolution of dystonia (functional impairment prior to DBS insertion) Age at GPi DBS insertion Preoperative BFMDRS Severity/ Disability Postoperative BFMDRS (6 months) severity/disability % Improvement in severity/disability (functional ability)
Case 1 F, aged 10 Negative Bilateral upper limb and 14 years 77/21 30/20 (34/20 61/5. Walked unaided;
years. truncal dystonia; unable to at 18 months) easier wheelchair
Handwriting walk, feed or wash unaided. use; self‐care skills
deterioration Schooling affected modestly improved
Case 2 M, aged 8 Positive Limbs, trunk and laryngeal 15 years 66/10 10/5 (8/3 at 85/50. Almost normal
years. muscles affected; severe 13 months) gait (in‐toe, right foot);
Dystonia right difficulties with walking, independent care skills
foot feeding and washing.
Schooling affected
Case 3 F, aged Negative Limbs, trunk, laryngeal 8 years; 103/28 36/10 (41/17 65/64. Walked and
18 months. and oromotor muscles revision at 36 months, used wheelchair;
Dystonia left affected; unable to use aged 9 years after battery independent care skills
foot wheelchair due to posture. due to lead change)
Crawled to move; unable displacement
to stand or walk. and
Schooling affected infection
Case 4 M, aged Negative Increased frequency of 10 years 85/17 62/9 (53/11 27/47. Paroxysmal
14 months. 1–2 min episodes. Wheel‐ at 12 months) dystonic episodes
Paroxysmal chair bound, unable to walk, eradicated, walked
dystonic feed or wash unaided. unaided; self‐care
episodes Schooling affected skills improved

BFMDRS, Burke, Fahn and Marsden Dystonia Rating Scale; DBS, deep brain stimulation; GPi, globus pallidus internus.