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Clinico-Etiological Spectrum and Functional Outcomes of Children with Pre-Status Dystonicus and Status Dystonicus
Shridhar P Joshi1, Maya Thomas1, Sangeetha Yoganathan1
1Paediatric Neurology Unit, Departments of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.
Insights
Status dystonicus (SD) is a severe neurological emergency requiring hospitalization. Early identification of triggers and causes is crucial for managing this condition and improving patient outcomes.
Area of Science:
- Neurology
- Movement Disorders
- Pediatric Neurology
Background:
- Status dystonicus (SD) is a critical neurological emergency characterized by severe, generalized dystonia episodes.
- It necessitates urgent hospital admission and presents a complex clinico-etiological spectrum with potential for recurrence and lasting disabilities.
Purpose of the Study:
- To delineate the clinico-etiological spectrum, diagnostic findings, treatment strategies, and follow-up outcomes for pediatric patients experiencing pre-status dystonicus (pre-SD) and SD.
Main Methods:
- A retrospective cross-sectional study analyzed data from pediatric patients (<18 years) with pre-SD and SD admitted between January 2010 and December 2020.
- Severity was assessed using the Dystonia Severity Assessment Plan (DSAP) scale, and outcomes were evaluated with the modified Rankin Scale (mRS).
Main Results:
- Twenty-eight patients experienced 33 SD episodes, with a median onset age of 8.71 years.
- Etiologies included metabolic (Wilson's disease), genetic, structural, post-encephalitic sequelae, and immune-mediated causes. Wilson's disease and febrile illness were common triggers.
- Most patients required midazolam infusion and experienced residual dystonia; three patients died from refractory SD.
Conclusions:
- Prompt identification of triggers and etiology is vital for effective management of SD.
- Timely and appropriate interventions are essential to mitigate the severity and complications of this life-threatening movement disorder emergency.
Background:
Status dystonicus (SD) is a life-threatening movement disorder emergency characterized by increasingly frequent and severe episodes of generalized dystonia, requiring urgent hospital admission. The diverse clinico-etiological spectrum, high risk of recurrence, and residual disabilities complicate functional outcomes.
Aim:
We aim to describe the clinico-etiological spectrum, radiology, therapeutic options, and follow-up of patients with pre-status dystonicus (pre-SD) and SD.
Methodology:
A cross-sectional retrospective study was carried out in a tertiary care referral center. The clinical, laboratory, and radiology data of all patients aged less than 18 years with pre-SD and SD from January 2010 to December 2020 were collected. The Dystonia Severity Assessment Plan (DSAP) scale for grading severity and the modified Rankin Scale (mRS) for assessing outcome were used at the last follow-up visit.
Results:
Twenty-eight patients (male:female: 2.1:1) experiencing 33 episodes of acute dystonia exacerbation were identified. The median age at the onset of dystonia and SD presentation was 8.71 (range: 0.25-15.75) and 9.12 (range: 1-16.75) years, respectively. Four patients experienced more than one episode of SD. The etiological spectrum of SD includes metabolic (Wilson's disease-13, L-aromatic amino acid decarboxylase deficiency-one, and Gaucher's disease-one), genetic (neurodegeneration with brain iron accumulation-three and KMT2B and THAP 1 gene-related-one each), structural-three, post-encephalitic sequelae (PES)-four, and immune-mediated (anti-NMDA receptor encephalitis-one). Five patients had pre-SD (DSAP grade 3), and 23 patients had established SD (DSAP grade 4-17 and DSAP grade 5-six). The Rapid escalation of chelation therapy precipitated SD in 11 patients with Wilson's disease. Febrile illness or pneumonia precipitated SD in nine patients. Twenty-three episodes of SD required midazolam infusion in addition to anti-dystonic medications. The median duration of hospital stay was 10 days (range: 3-29). Twenty-three patients had resolution of SD but residual dystonia persisted, while two patients had no residual dystonia at follow-up. Three patients succumbed owing to refractory SD and its complications.
Conclusion:
Early identification of triggers, etiology, and appropriate management are essential to calm the dystonic storm.
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