Sequential prefrontal lobe volume changes and cognitive dysfunctions in children with Panayiotopoulos syndrome presenting with status epilepticus

• In PS with SE, prefrontal lobe growth decreased slightly soon after SE episodes.
• WISC and K-ABC scores in SE remained low and did not reach levels at seizure onset.
• Treatment to prevent SE may be required to achieve optimal prognosis in PS.

SummaryPanayiotopoulos syndrome (PS) is usually not associated with neurodevelopmental problems. However, neuropsychological impairments may also be present in at least some of the patients with PS. On the other hand, several degrees of neuronal damage due to status epilepticus (SE) may occur in the cortex. We prospectively measured frontal and prefrontal lobe volumes using three-dimensional magnetic resonance imaging (3D-MRI)-based volumetry in patients with PS with and without SE. Moreover, the neuropsychological outcome in relation to the presence of SE in children with PS is also discussed. We studied six patients with a final diagnosis of PS, including three cases with SE and cognitive impairments/behavioral problems (SE group) and three cases without SE (non-SE group). Serial 3D-MRI studies were performed five times (at onset of clinical symptoms and 1–4 years after onset) in both the SE and non-SE patients. All patients were studied with a set of Wechsler Intelligence Scale for Children, version III (WISC-III) or Wechsler Preschool and Primary Scale of Intelligence tests and the Kaufman Assessment Battery for Children (K-ABC). Growth of the frontal and prefrontal lobes was slightly decreased for some time after SE episodes in the SE patients. Moreover, the prefrontal-to-frontal lobe volume ratio was stagnant for some time after SE in the SE patients. The scores on the neuropsychological tests were decreased in the SE patients. Moreover, the average WISC and K-ABC scores in the SE group remained low and did not reach the levels of the initial examinations. Occurrence of SE in patients with PS at least in some patients may be associated with retarded prefrontal lobe growth, which was related to neuropsychological problems and ultimately, neuropsychological outcomes. Treatment management may be required to prevent SE as much as possible to achieve optimal prognosis in PS at least in some patients.