Objective: To describe a consecutive series of 40 status dystonicus patients, identify major clinical characteristics and to document medical and surgical management.

Background: Characteristics and evolution of dystonia patients developping SD episodes are poorly documented. Status Dystonicus (SD) is a rare, severe, potentially life threatening condition reached in different types of dystonia, manifesting with intense and frequent episodes of dystonic spasms.

Methods: This is a cohort study approved by Comité de Protection des Personnes Sud Méditérranée I (RO – 2016/37). Forty patients with dystonia who developed SD were included and the following data collected: i) dystonia characteristics; ii) SD episodes, triggering factors, worsening modality towards SD, characteristics during SD); iii) dystonia specific pharmacological and ICU management and, long term evolution including recurrence of the SD episodes.Statistical analysis was performed with IBM SPSS Statistics Version 24 and IBM SPSS Modeler Version 18. 

Results:  Twenty one female (52,5%) and 19 male were inlcuded. Mean age at symptom onset was 5,5 years with 95% of pediatric onset (60%, age 0 to 2 years at onset). Mean disease duration at the first SD was 10,3 years. All but one adult presented with generalized dystonia. Other neurological features were combined in more complex neurological syndromes in 87,5% of them. Twenty-eight SD episodes previous to and 29 episodes under DBS were documented. Twenty-two out of the fourty SD patients necessitated ICU stay and four of them High Dependency Unit management. The most frequent general complications were fever (35,9%), altered sleep (92,3%), painful muscle spasms (76,9%) and weight loss (53,8%). A triggering factor could be documented in less than a half of the episodes. A total of 1332 days were spent in the ICU by the SD population, with a mean of 60,5 days by subject, when taken into consideration the 22 subjects who had a stay in ICU. Sedation inclued midazolam (n=18), du sufentanil (n=21), propofol (n=19). Twenty-five patients had de novo DBS indication to control SD (62,5%), and 9 patients necessitated additional DBS leads to control a SD episode. 

Conclusions:  SD represents a severe dystonia state necessitating frequently long term ICU management. New diagnostic criteria including specific and systemic complications should be considered. DBS remains the most efficient therapy, effective in 92,8% of SD cases in our series.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/history-and-management-of-status-dystonicus/