Neurological sequelae of acute encephalopathy with febrile convulsive status epilepticus

OBJECTIVE: The clinical characteristics of neurological sequelae in patients with acute encephalopathy with febrile convulsive status epilepticus (AEFCSE) was elucidated. METHODS: We retrospectively reviewed 8 patients, which were admitted to our hospital from 2002 to 2011. RESULTS: In the subacute phase, transient neurological symptoms, such as dystonia (n = 3), choreoathetosis (n = 2), oral tendency (n = 5) and unilateral spatial neglect (n = 6), appeared from 3 weeks after onset. Then, severe intellectual disability (n = 7), attention deficit (n = 7), disturbance of communication skill (n = 7) and emotional disturbance (n = 2), persisted from one month after onset. Although seven patients resumed ambulatory abilities, six exhibited unstable gait without ataxia or muscular weakness. The neuroradiological findings on MRI corresponded to the clinical course. In the subacute phase, reversible bilateral signal changes were noted in the subcortical white matter (n = 8), caudate nuclei (n = 2), putamen (n = 1) and thalamus (n = 1). In the chronic phase, diffuse cortical atrophy, predominantly in the fronto-temporal lobes. Diffuse cortical atrophy suggested that the persistent neurological sequelae of AEFCSE represent cortical dysfunction. Therefore, we propose that the unstable gait in our patients was gait ataxia, being related to the frontal lobe dysfunction. CONCLUSIONS: These neurological findings of AEFCSE showed characteristic temporal changes, which should be considered in the development of rehabilitation programs.