Botulinum Toxin Benefits Many PSP Patients With Dystonia

Daniel M. Keller, PhD

June 18, 2013

BARCELONA, Spain — For patients with progressive supranuclear palsy (PSP), botulinum toxin A injections relieve disabling blepharospasm and neck and hand dystonia, a relatively large retrospective study shows. About three quarters of patients with PSP actually developed dystonia during the treatment.

"The best benefit [was in] patients with blepharospasm — about 80% of patients with blepharospasm had benefit. When you look at the neck dystonia, it's about 70% had benefit, less in orofacial dystonia hand dystonia," Diana Apetauerová, MD, director of the Movement Disorders Program at the Lahey Health and Medical Center in Burlington, Massachusetts, told Medscape Medical News here at the 22rd Meeting of the European Neurological Society (ENS).

During a 14-year period, researchers identified 67 patients with PSP in the medical center's movement disorder clinic. Of the 67, 49 (73%) had at least 1 form of dystonia. The mean age was 77 years and the mean duration of PSP was 6.7 years.

The most prevalent sites were hand dystonia (31 patients), blepharospasm (n = 30), and neck (n = 24) dystonia, followed by orofacial (n = 19), foot, arm, and trunk dystonia. Fifteen of the 49 patients had a single form of dystonia, and the rest had multiple forms, including 8 patients with 5 types. The mean time from PSP diagnosis to dystonia was 3.7 years (range, 1 to 9 years).

Twenty-two (45%) patients received botulinum toxin A injections for dystonia, with blepharospasm the most commonly treated type.

Table. Effectiveness of Botulinum Toxin A for Dystonias in PSP

Type of Dystonia Proportion of All Treated Patients (n = 22) (%) Proportion of Patients Who Benefited (%)
Blepharospasm 55 93
Neck 23 80
Hand 23 60
Orofacial 18 50
Foot 4.5 100

 

Dr. Apetauerová said the findings indicate a high incidence of dystonia in patients with PSP. Although botulinum toxin A was least effective for orofacial and foot dystonias, she nonetheless feels that it should be tried for these conditions.

"I think as a clinician very often we struggle with treatment options," she said. Patients are often diagnosed with PSP and are told it is an untreatable disease, she added, "and I think we have to be very cautious making that statement, and we have to really concentrate on symptomatic treatment, and I think this is one example where the symptomatic treatment can improve quality of life."

Dr. Apetauerová advised that these treatments are probably not for patients with infections of the eyes, which are not uncommon because of closure of the eyes. She recommended waiting a couple of weeks until after the infection clears up. "And I'm always careful with patients who have severe swallowing difficulties, and I would probably not inject them in both sternocleidomastoid muscles because they would develop worsening of swallowing issues," she said.

John O'Dwyer, MD, MB, consultant neurologist and director for dystonia and botulinum at St. George's Hospital Health Care Trust in London, United Kingdom, commented to Medscape Medical News that botulinum toxin A can benefit some patients with PSP by alleviating pain and improving posture and position.

"It's nice to see how many patients seem to benefit, particularly with hand injection and foot injection because they were often in the literature in the past quoted as being very difficult or refractory to [botulinum]," he noted. "My personal experience is the patients do get relief from pain if they have painful dystonia as well."

Dr. O'Dwyer said that oral medications tend to be limited by their adverse effects in patients with PSP, and patients with dementia and confusion may be more difficult to treat with oral medications.

Botulinum toxin A "can give them back some function, make them more comfortable, make the carers' lives easier in terms of managing the patient who has severe postures, such as a very cramped hand where the fingers could be open and prevent fungal infections in the skin and improve basic skin hygiene," he advised.

Dr. Apetauerová noted that limitations of the study were the relative lack of longitudinal data on response to botulinum toxin A, loss to follow-up, or patient deaths.

There was no commercial funding for the study. Dr. Apetauerová and Dr. O'Dwyer, who was not involved in the study, have disclosed no relevant financial relationships.

23rd Meeting of the European Neurological Society (ENS). Abstract P729. Presented June 10, 2013.

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