Nontumoral occipitotemporal epilepsy: localizing findings and surgical outcome

We describe a syndrome of medically intractable occipitotemporal epilepsy of nontumoral developmental origin and its treatment by surgery. From our epilepsy surgery database of 1988 to 1996, we selected all patients without neoplasm who had at least two characteristics localizing to the occipital lobe (clinical symptoms, interictal focus, ictal onset, or a lesion on magnetic resonance imaging scanning) and one to the temporal lobe (interictal spikes or seizure onset). We discuss seizure characteristics, electroencephalographic (EEG), magnetic resonance imaging, positron emission tomographic, and single-photon emission computed tomographic findings, pathological findings, surgical approach, outcome from resective surgery, and implications for pathophysiology. Sixty-nine percent of our 16 patients with occipitotemporal syndrome had neuronal migration disorder, suggesting a developmental etiology of this entity. Initial signs or symptoms suggested occipital lobe seizure onset in 13 of 16 patients. On scalp EEG, interictal spikes were localized to the temporal lobe in 9 and to the occipital lobe in 1; seizure onset was poorly localized. Intracranial EEG localized seizure onset to the area of temporo-occipital junction in 77% of patients. Positron emission tomography and single-photon emission computed tomography showed occipital and temporal or widespread deficits, and neuropsychological performance was diffusely abnormal. Surgical results were best with occipital and temporal resections, but sometimes satisfactory after occipital resection even with temporal (ipsilateral) EEG findings. Temporal resection with hippocampectomy uniformly failed to control seizures. An often refractory, probably developmental epileptic syndrome with regional occipitotemporal distribution can be diagnosed by a specific constellation of findings, which has implications for treatment and pathophysiology.