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Parkinsonism induced by VNS in a child with double-cortex syndrome
Arthur Cukiert1, Pedro Paulo Mariani, Jose Augusto Burattini
1Epilepsy Surgery Program, Hospital Brigadeiro, São Paulo, Brazil. acukiert@uol.com.br
Insights
Vagus nerve stimulation (VNS) effectively reduced seizures in a child with epilepsy and double-cortex syndrome. However, increasing VNS intensity triggered parkinsonian symptoms, highlighting a direct effect on the basal ganglia.
Area of Science:
- Neurology
- Neurophysiology
Background:
- Epilepsy management often involves Vagus Nerve Stimulation (VNS) for refractory cases.
- Double-cortex syndrome is a rare neuronal migration anomaly associated with intellectual disability and epilepsy.
Observation:
- A 13-year-old female with refractory epilepsy and double-cortex syndrome underwent VNS therapy.
- VNS therapy led to a significant reduction in seizure frequency.
- Progressive increases in VNS intensity correlated with the emergence of parkinsonian symptoms, including tremor, rigidity, and gait disturbances.
Findings:
- The patient experienced reversible parkinsonian symptoms directly linked to VNS intensity.
- Discontinuation of VNS resulted in the complete resolution of extrapyramidal symptoms.
- This case suggests a direct impact of VNS on the basal ganglia function.
Implications:
- VNS may have previously unrecognized direct effects on basal ganglia function.
- Careful titration of VNS is crucial in patients with underlying neurological conditions.
- Further research is warranted to elucidate the neurobiological mechanisms underlying VNS-induced parkinsonism.
Abstract:
We describe a child with epilepsy associated with double-cortex syndrome in whom vagus nerve stimulation (VNS) generated parkinsonian symptoms. A 13-year-old girl presented with refractory secondary generalized epilepsy from the age of 6 years and mental retardation. Her electroencephalography (EEG) showed diffuse polyspike and wave discharges. Magnetic resonance imaging (MRI) showed double-cortex syndrome. She was submitted to extended callosal section at the age of 10 years, which yielded 50% seizure frequency reduction. She was submitted to VNS by the age of 12 years. As stimulation intensity was increased, there was appearance of extrapyramidal symptoms: She developed bilateral tremor and rigidity, and gait and postural disturbance. All symptoms disappeared 7-10 days after VNS was turned off. Several attempts to reactivate VNS led to the same results. During the periods when VNS was on she presented with marked seizure frequency reduction. This is the first report of a clinically evident direct effect of VNS on the basal ganglia.
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