Hemiconvulsive hemiplegia can occur in otherwise healthy children from prolonged unilateral seizures or status epilepticus resulting in unilateral hemispheric damage and hemiplegia and unilateral encephalopathy similar to HH as seen with MRI.53 The neuropathology of the affected hemisphere parallels our HH model with a widespread hypoxic-ischemia pathology pattern with vacuolization of the neurons and neuropil.54 Whereas some of these cases are associated with genetic seizure disorders (CACNA1A), they demonstrate that a severe seizure disorder can cause unilateral hemispheric encephalopathy. Here, CACNA1A is linked to epilepsy.