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Epileptic nystagmus: description of a pediatric case with EEG correlation and SPECT findings
F Nicita1, L Papetti, A Spalice
1Department of Pediatrics, Child Neurology Division, Sapienza University of Rome, Rome, Italy.
Insights
Epileptic nystagmus (EN), linked to seizure activity, was effectively managed with topiramate in a young patient. Treatment led to seizure control and normalized brain activity patterns observed via EEG and SPECT scans.
Area of Science:
- Neurology
- Epileptology
- Neuroimaging
Background:
- Epileptic nystagmus (EN) is characterized by repetitive eye movements stemming from seizure activity.
- Understanding the neurobiological underpinnings of EN is crucial for effective diagnosis and treatment.
Observation:
- A pediatric patient presented with epileptic nystagmus and vertigo, with no anomalies on brain MRI.
- Ictal EEG revealed epileptic activity during horizontal, left-beating nystagmus.
- Ictal 99mTc-ECD SPECT identified active foci in prefrontal, parieto-temporo-occipital, and left parieto-insular-vestibular areas, with right hemisphere hypoperfusion.
Findings:
- Topiramate treatment resulted in complete seizure control within 14 days.
- Post-treatment EEG and SPECT scans showed disappearance of epileptic activity and improved cerebral perfusion.
- The case supports a cortical origin for saccadic epileptic nystagmus, involving temporo-occipital and frontal cortices.
Implications:
- This case highlights the efficacy of topiramate in managing complex epileptic nystagmus.
- It underscores the value of combined EEG and SPECT in diagnosing and monitoring EN.
- Findings reinforce the role of specific cortical regions in the generation of EN.
Abstract:
Epileptic nystagmus (EN) describes repetitive eye movements that result from seizure activity. We describe a patient with EN and vertigo first noted at the age of 4 yr and 10 mo. Brain MRI did not show anomalies. Ictal EEG recordings revealed epileptic activity during three episodes of horizontal, left-beating nystagmus not crossing the midline. Ictal 99mTc-ECD SPECT demonstrated the presence of active foci in multiple cerebral regions including bilateral prefrontal, bilateral parieto-temporo-occipital and the left parieto-insular-vestibular areas. A wide area of hypoperfusion was also evident in the right hemisphere, prevailing in the parieto-occipital regions and the medial prefrontal gyrus. Topiramate was started at a dose of 2 mg/kg/d with complete seizure control after 14 d. EEG and SPECT were repeated after a seizure-free period of 1 mo; disappearance of epileptic activity and modification of cerebral perfusion were evident. This case reaffirms the cortical origin and involvement of temporo-occipital and frontal cortex in the genesis of saccadic epileptic nystagmus. Rapid complete control of clinical events coincided with the normalization of EEG and improvement of the SPECT pattern.
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