American Academy of Neurology: Botulinum Toxin Injections in Musician’s Dystonia

Musician's DystoniaSpeaker: Stephen U. Schuele, MD, Consulting Neurologist, Department of Neurology and Medicine, The Cleveland Clinic Foundation, Cleveland, Ohio.

Results of an outcome survey of musicians with focal task-specific dystonia (FTSD) treated with injected botulinum toxin A (Botox®, Allergan) suggest that this approach can offer long-term benefit, independent of the musical instrument used.

Because FTSD in musicians often leads to the end of their careers, a study was performed to evaluate the initial and long-term benefits of the toxin. Between 1995 and 2002, musicians with FTSD at the Institute of Music Physiology and Musicians Medicine at the University of Music and Drama in Hanover, Germany, received botulinum toxin A injections. The initial average injected dose was 51.2 units, and the final dose was 40.1 units. A minimum of 2.2 and a maximum of 3.4 injections were administered.
Using telephones and a chart review, investigators surveyed 88 of 225 musicians with FTSD after treatment. Of the 88 musicians, 84 responded to the survey. Assessments included a rating of severity; an estimate of playing ability before and after injection, in percentages; and a rating of treatment response, from worse to none to mild to moderate to marked to remission. Initially, the investigators observed muscle involvement while the musicians were playing and then reported their findings in their charts.

A total of 74 men and 10 women participated in the study group. The group included 25 woodwind players, 25 guitarists, 20 keyboard players, 12 bowed-string musicians, and two brass instrumentalists. Their mean age was 45.9 years, and the mean duration of symptoms was 10.6 years.

As a group, the musicians subjectively rated their playing ability as significantly improved following botulinum toxin A treatment. In a self-rating of treatment response, 69% of the musicians indicated improvement and 45% rated the benefit as sufficient to improve their performance. Twenty-four musicians received ongoing treatment for an average of 36 months; another six patients showed long-lasting benefits without the need for further injections.
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Three patients with embouchure dystonia (of the lips and facial muscles) had a poor response. Overall, 30 of the 84 musicians either experienced persisting improvement or con­tinued to benefit from ongoing injections for an average of three years and up to six years.

Topiramate in Newly Diagnosed Epilepsy

Speaker: Elinor Ben-Menachem, MD, PhD, Professor, Department of Neurosciences, Sahgrenska Academy at Gote-borg University, and Director, Clinical Pharmacology and Epilepsy, Department of Clinical Neurosciences, Sahlgrenska University Hospital, Goteborg, Sweden.

Findings from two studies indicate that topiramate (Topa-max®, Johnson & Johnson) 100 mg/day is an effective dose in patients with newly diagnosed epilepsy; is at least as effective as the traditional first-line agents carbamazepine (Tegretol canadian, Novartis) and valproate (Depacon®, Abbott) for previously untreated patients with epilepsy; and is better tolerated.

A dose-controlled study enrolled 470 adults and children with epilepsy that was characterized by partial-onset or primary generalized tonic-clonic seizures. The patients were randomly assigned to take topiramate 50 mg or topiramate 400 mg daily, titrated at 50 mg from the first week to 400 mg at the sixth week. The significant between-group difference in time until the first seizure favoring the higher dose confirmed topira-mate’s efficacy as monotherapy. Furthermore, the significant difference between 100 mg/day and 25 mg/day during the dose-escalation phase identified topiramate 100 mg/day as an effective dose and an appropriate target dose for initial therapy in untreated epilepsy.

In a comparative monotherapy study, 613 adults and children with epilepsy, with no seizure type excluded, were randomly assigned to receive the following doses:

  • topiramate 100 mg/day (median duration, 544 days)
  • topiramate 200 mg/day (median duration, 378 days)
  • carbamazepine 600 mg/day (median duration, 434 days)
  • valproate 1,250 mg/day (median duration, 304 days)

Double-blind treatment continued until six months after the last patient was assigned to a therapy regimen or until a decision was made to change therapy by altering the dose or medication or by discontinuing the study medication entirely. The primary endpoint was the time to exit from the study (when a patient left). The secondary endpoints were (1) freedom from seizures during the last six months of the study and (2) the exit time.

There was no significant difference in efficacy between top-iramate 100 mg/day and 200 mg/day, as measured by the time until the first seizure. At most time points, however, the cumulative number of study exits was lower with topiramate than with valproate. Median retention times were longer with topiramate than with either of the comparator drugs. Topiramate 100 mg/day was better tolerated than topiramate 200 mg/day, carbamazepine 600 mg/day, or valproate 1,250 mg/day. Patient discontinuation rates were 19% with topiramate 100 mg/day, 28% with topiramate 200 mg/day, 25% with 600 mg/day, and 23% with valproate 1,250 mg/day.

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