Status Epilepticus Amauroticus Revisited Ictal and Peri-ictal Homonymous Hemianopsia

Objective: To describe the clinical, electrographic, and radiographic features of status epilepticus amauroticus, or homonymous hemianopsia associated with partial status epilepticus, in 3 patients with subsequent resolution of radiographic abnormalities and visual deficits. Design: Case series. Setting: Rancho Los Amigos National Rehabilitation Center in Downey, California, and the Los Angeles County + University of Southern California Medical Center. Patients: One patient with a single remote seizure and 2 patients with symptomatic partial epilepsy all presented with homonymous hemianopsia. Intervention: Continuous electroencephalographic monitoring, magnetic resonance imaging, and antiepileptic medical therapy for status epilepticus. Main Outcome Measures: Neurologic examination, electroencephalography, and magnetic resonance imaging. Results: The association of homonymous hemianopsia and restricted diffusion on magnetic resonance imaging led to an initial diagnosis of ischemic infarction in 2 cases despite atypical diffusion-weighted imaging patterns. However, continuous electroencephalogram demonstrated focal epileptiform discharges in 2 cases and repetitive focal seizures in another, suggesting a diagnosis of status epilepticus amauroticus. Homonymous hemianopsia resolved in all 3 patients after escalation of the dosage of anticonvulsant therapy. Follow-up magnetic resonance imaging and electroencephalogram demonstrated complete or near-complete resolution of associated abnormalities. Conclusions: Status epilepticus amauroticus is an uncommon but important cause of homonymous hemianopsia, and it should be considered in any patient with a history of seizures, fluctuating visual symptoms, or atypical patterns of restricted diffusion involving the occipital cortex. Continuous electroencephalographic monitoring is an important diagnostic tool for the diagnosis of status epilepticus amauroticus, which may have a favorable prognosis when treated with aggressive anticonvulsant therapy.