The common drugs used are Trihexyphenidyl and Tetrabenazine. Most of the time, these drugs have very limited improvement. Over a period of time the disease continues to worsen, making it difficult for the person to continue performing the specific task. One of the other options is using Botulinum Toxin for the involved hand. However, the effect of this is for a limited duration, usually not lasting for more than 3-5 months. Over a period of time, the dose increases with the repeated injections and later on the symptoms fail to respond.
Surgery can be a very rewarding treatment for this disease. It is one of those diseases where surgical results are highly predictable. There are two different surgeries that can be performed. One is thalamotomy and the other deep brain stimulation of GPi. The thalamotomy involves a target called Ventalis oral nucleus of thalamus, a slightly different target from the conventional thalamotomy target. However, the target for DBS is the same that is used for other dystonias.
We have an experience of 11 patients, 10 who have undergone thalamotomy and one GPi stimulation for this disease, with excellent results that have been published in reputed journals
- Case story of thalamotomy
- Case story of GPi stimulation