Deep Brain Stimulation (DBS) in Pediatric Dystonia: The Youngest Case of Neuromodulation for Refractory Acquired Dystonia and Status Dystonicus with 1-year Follow-up
Darshana Vijaywargiya1, Nienke Dosa2, Ai Sakonju2
1SUNY Upstate Medical University, 2SUNY Upstate Medical Center
Objective:
We present the youngest case of DBS placement for acquired dystonia with favorable outcome at 1-year-follow-up.
Background:
DBS was FDA-approved for pediatric dystonia in 2003. With smaller heterogeneous studies and lack of objective outcome measures, data on patient selection and programming protocols is limited. In general, patients aged 7 or older, with refractory genetic/acquired dystonia/status dystonicus are evaluated for intervention with best outcomes in monogenic dystonias. Limited responses are observed in acquired dystonias due to variability of brain pathologies and frequently co-existent spasticity.
Design/Methods:
A 4-year-old female with history of 31-week prematurity, hypoxic ischemic encephalopathy and Lennox Gastaut syndrome presented with episodes of cervical dystonia and opisthotonus. Neurological exam revealed global developmental delay, absent language, intact regard and light tracking, spastic quadriplegia, and severe truncal dystonia. These prolonged (>1 hour), frequent episodes evolved into status dystonicus that partially responded to dopamine replacement, clonidine and benzodiazepines. Coexistent sympathetic storming further complicated the course.
Results:
MRI brain revealed periventricular leukomalacia, and basal ganglia and corona radiata cystic encephalomalacia. EEG showed stable epileptic encephalopathy without electrographic correlate for dystonic episodes. CSF studies were unremarkable.
Recurring similar presentations failed to respond to baclofen, gabapentin and trihexyphenidyl causing severe scoliosis by the age of 5. Patient was evaluated by multidisciplinary team of neurosurgeons, physiatrists, movement disorders and child development specialists. DBS was recommended. Patient underwent DBS placement in bilateral globus pallidus internus with activation at 6.5 years age. She underwent scoliosis correction surgery a year later. Response to DBS was gradual but sustained with weaning of gabapentin and diazepam at 1-year-follow up.
Conclusions:
This case represents the youngest patient with DBS placement for refractory acquired dystonia and recurrent status dystonicus with continuing functional improvements after a year of activation. Alongside evolving literature, this case raises the possibility of better outcomes with earlier neuromodulation in acquired dystonias with coexistent epilepsies.
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