Objective: The purpose of this study is to retrospectively review the results of bilateral pallidotomy in patients presenting with status dystonicus.

Background: Dystonias, third commonest movement disorders, are uncommon disorders characterized by sustained or intermittent muscle contractions causing abnormal movements or postures. Dystonias may be classified as “primary dystonia” or “isolated dystonia without evidence of central nervous system degeneration/insult” and “secondary dystonia” or “acquired/heredodegenerative dystonia”. Few patients with dystonia can present in status dystonicus, which is a neurological emergency. The patients in status dystonicus need mechanical ventilation and muscle relaxants. Many of these patients need an emergent surgical intervention to abate the status dystonicus.

Method: We retrospectively reviewed all the dystonia patients (less than 18 years) who presented to us in status dystonicus and underwent bilateral pallidotomy. Results of pallidotomy were recorded as were the complications resulting from surgical procedure.

Results: Three patients with a mean age of 16.3 years were included in the study. All the patients were male patients. All the patients had secondary dystonia. The various primary pathologies were Neurodegeneration with brain iron accumulation, Wilson’s and Hypoxic ischemic encephalopathy. Mean duration between the disease onset and surgical intervention was 2.9 years. Status dystonicus abated in all the three patients following bilateral pallidotomy. Mean hospital stay after pallidotomy was 19 days. One patient, who had Wilson’s disease, died in the eighth month of surgery due to liver failure. Other two patients had residual dystonia at the time of last follow up.

Conclusion: Emergent pallidotomy should be considered in the treatment of status dystonicus as it can be successfully abated with bilateral pallidotomy.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/role-of-bilateral-pallidotomy-in-status-dystonicus/