HEMICONVULSION-HEMIPLEGIA-EPILEPSY SYNDROME (HHE)

• Dravet syndrome - febrile hemiclonic status epilepticus can occur and may be followed by transient hemiparesis (Todd paresis) but the imaging findings of this syndrome are not seen.
• Sturge-Weber syndrome - focal motor status epilepticus can occur in a young infant and be followed by hemiparesis, however the typical skin and MRI features of Sturge-Weber syndrome are present.
• Rasmussen syndrome - this syndrome presents with focal seizures that gradually increase in frequency, followed by slowly progressive hemispheric atrophy and hemiparesis, usually over months-years.
• Focal status epilepticus and co-occurring hemiplegia due to other etiologies e.g. haemorrhagic or ischemic stroke, infection, mitochondrial disorders.