Acute Infantile Encephalopathy Predominantly Affecting the Frontal Lobes

To establish a novel subtype of acute infantile encephalopathy, the clinical and radiologic features of nine infants with acute encephalopathy involving the bilateral frontal lobes were examined. These patients had convulsive status epilepticus with hyperpyrexia followed by a prolonged impairment of consciousness for 2-20 days. After the recovery of consciousness, all the patients manifested regression of verbal function and lack of spontaneity. Some of them also exhibited stereotypic movements, instability of mood, or catalepsy. Transient postictal edema in both frontal lobes was suggested by diffusion-weighted magnetic resonance imaging. Attenuated cerebral perfusion in the frontal lobes was demonstrated by single-photon emission computed tomography at the tenth day after onset or subsequently. Serial studies disclosed atrophic changes in the frontal lobes. All patients manifested regression or retardation of motor and verbal functions. The recovery of intellectual deficit was slower and less prominent than that of motor dysfunction. These unique features suggest that the frontal lobes are the focus of this novel subtype of acute encephalopathy, which we propose to call acute infantile encephalopathy predominantly affecting the frontal lobes.