Background: Currently, there is limited insight in to the function of the mesial frontal negative motor area (NMA) and the anatomic structures implicated in its function. Methods: We present a patient with a Rett-like phenotype, refractory frontal lobe epilepsy, and reflexogenic seizures in which backpatting induced atonic seizures with a semiology resembling the patient falling asleep. The patient underwent video EEG monitoring and ictal/interictal SPECT imaging capturing the reflexogenic seizures. Iterative reconstruction was performed, with images co-registered to previously acquired MRI with subtraction Ictal-Interictal imaging co-registered to MRI. Results: Interictally, the patient’s EEG showed a slow background and right frontal spikes. Ictally, the patient had numerous subclinical frontal seizures. The reflexogenic seizures had an ictal pattern at the vertex (Cz) with the ictal SPECT imaging, showing hyperperfusion in the right mesial frontal region, both paramedian precentral and postcentral gyri, and right basal ganglia. Conclusions: Our findings support the hypothesis that the negative motor area may be activated by the primary sensory cortex; moreover, the ictal SPECT now suggests involvement of the basal ganglia in the NMA’s function.
Epilepsy with myoclonic–atonic seizures (Doose syndrome): When video-EEG polygraphy holds the key to syndrome diagnosis
