Deep Brain Stimulation for Status Dystonicus: A Case Series and Review of the Literature

Abstract

Background: Status dystonicus (SD) is a rare and potentially life-threatening complication of primary or secondary dystonia, characterized by acute worsening of dystonic movements. There is no consensus regarding optimal treatment, which may be medical and/or surgical. Methods: We present our experience with pallidal deep brain stimulation (DBS) in 5 DYT1-positive patients with SD and provide a review of the literature to examine optimal management. Results: Of the 5 patients treated with pallidal DBS, all experienced postoperative resolution of their dystonic crisis within a range of 1-21 days. Long-term follow-up resulted in 1 patient returning to preoperative baseline, 3 patients improving from baseline, and 1 patient making a complete recovery. Of the 28 SD patients (including our 5 patients) reported in the literature who were treated with DBS or ablative surgery, 26 experienced cessation of their dystonic crisis with a return to baseline function and, in most cases, clinical improvement. Conclusion: DBS is an effective therapeutic modality for the treatment of SD. In addition to the long-term benefits of stimulation, early and aggressive treatment may improve the overall outcome.

Dystonic storm: a practical clinical and video review

“…Typical clinical scenarios for dystonic storm include DYT1 dystonia [20, 21], Wilson’s disease (typically after initiation of D-penicillamine) [13, 14], and cerebral palsy (CP) with infectious trigger [1, 6, 22]. Other scenarios for dystonic storm are listed in Table 2.…”

“…Nevertheless, with increased utilization of DBS in the past decade, the pendulum may be shifting away from this approach. An increasing number of reports have demonstrated excellent outcomes of GPi DBS and ITB in dystonic storm [10, 21, 24–37], especially in DYT1 generalized dystonia and other primary dystonia cases (Additional file 4: Video segment 4). Due to the dramatic impact on patient outcome, the modern trend is to advocate a tiered approach (based on diagnosis e.g.…”

“…Due to the dramatic impact on patient outcome, the modern trend is to advocate a tiered approach (based on diagnosis e.g. generalized DYT1 dystonia and severity) [5, 38] with consideration of GPi DBS or ITB therapy [21, 26–29, 39]. Patients in dystonic storm often improve immediately with DBS [8, 24], as opposed to the delayed benefit (weeks to months) of DBS in other forms of dystonia.…”

Deep brain stimulation treated dystonia‐trajectory via status dystonicus

“…Our series is the experience of a single center reporting on SD, with the longest follow‐up of a cohort, after SD. This study shows that SD is a lifetime threat . The spectrum of disorders that produce SD is large.…”

“…So far, there are only a few reports of evolution after SD with improvement of dystonia and even fewer on the resolution of SD for a longer period . Although algorithms have been proposed to treat SD, no consensus is available regarding the optimal strategy . Patients were treated with DBS for SD episodes following failure of medical management, usually transferred directly from remote hospital ICU to internal ICU.…”