To report a novel treatment approach,
pallidotomy and
deep brain stimulation (DBS), in two sisters with dystonic storm due to Batten's disease. This study is based on long-term follow-up of two sisters, presenting with dystonic storm and their response to
pallidotomy and DBS. These sisters, who had visual loss,
seizures, and progressive psychomotor decline, experienced progressive disabling
abnormal movements culminating in dystonic storm at the age of 15 and 17 years, respectively. In addition to intubation and sedation, multiple medications, including
botulinum toxin injections and intrathecal
baclofen infusion were tried in both patients without any benefit. The old sister underwent bilateral
pallidotomy. Within 10 days postoperatively, there was marked improvement in dystonic storm. She was free of
abnormal movements for 9 months. Then she started having opisthotonus lasting 20 seconds to an hour several times/day, but over 6 years
abnormal movements are markedly improved, and not returned to pre-
pallidotomy level. The young sister underwent both bilateral
pallidotomy and DBS, 3 weeks apart. She was free of
abnormal movements for 7 months and able to maintain reduction in the
abnormal movements by adjusting DBS settings.
Pallidotomy and DBS should be considered in dystonic storm due to Batten's disease.