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Ictal PET in Ohtahara Syndrome With Hemimegalencephaly
Shambo Guha Roy1, Madhavi Tripathi, Manjari Tripathi
1From the *Department of Nuclear Medicine and PET/CT, All India Institute of Medical Sciences, New Delhi; and †Department of Neurology, CN Centre, All India Institute of Medical Sciences, New Delhi, India.
Insights
Ohtahara syndrome, a severe infantile epilepsy, often involves brain abnormalities. In this case, ictal FDG PET/CT imaging successfully pinpointed the seizure origin in a patient with hemimegalencephaly.
Area of Science:
- Neurology
- Pediatric Epilepsy
- Medical Imaging
Background:
- Ohtahara syndrome is a rare, severe form of infantile epilepsy.
- It is characterized by refractory seizures and specific electroencephalogram (EEG) patterns.
- Structural brain anomalies are frequently associated with this condition.
Observation:
- A 5-month-old female infant presented with intractable seizures.
- The patient was diagnosed with Ohtahara syndrome and concurrent hemimegalencephaly.
- Standard diagnostic workup included EEG and neuroimaging.
Findings:
- 18-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography (FDG PET/CT) was utilized during a seizure event (ictal).
- Ictal FDG PET/CT imaging effectively localized the epileptic focus within the brain.
- This imaging modality proved crucial for identifying the source of the refractory seizures.
Implications:
- FDG PET/CT can be a valuable tool for precisely localizing seizure foci in complex pediatric epilepsy syndromes like Ohtahara syndrome.
- Accurate seizure focus localization is essential for guiding potential therapeutic interventions.
- This case highlights the utility of advanced neuroimaging in managing challenging pediatric neurological disorders.
Abstract:
Ohtahara syndrome is one of the causes of infantile epilepsies, which presents with refractory seizures and characteristic EEG changes. It is often associated with structural anomalies in the brain. We report a case of 5-month-old girl with Ohtahara syndrome with hemimegalencephaly who presented with refractory seizures and ictal FDG PET/CT helped in localizing the seizure focus.
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