Acute infantile encephalopathy predominantly affecting the frontal lobe (AIEF): a European case.
Acute infantile encephalopathy predominantly affecting the frontal lobes (AIEF) has been described as a new entity, based on MRI findings (acute abnormal diffusion-weighted imaging signals in the frontal lobes followed by atrophy) and exclusion of other acute encephalopathies. Patients present with acute onset of fever, status epilepticus, and coma. Different causal mechanisms have been suggested such as localized viral infection, toxic insult due to cytokines, or postictal damage. Only children of Japanese descent have been described. We report the case of a Caucasian girl whose history and MRI findings were similar to the Japanese cases. She had a massive regression with verbal apraxia, while cognitive development was less affected; she initially presented with a cluster of complex partial seizures (and not convulsive status epilepticus), making epileptic or post anoxic-ischemic sequelae highly unlikely. The place of this proposed entity among other recently described acute encephalopathies with abnormal diffusion on MRI is discussed.
Jequier Gygax
M
M
Paediatric Neurology and Neurorehabilitation Unit, Department of Paediatrics, CHUV (Centre Hospitalier Universitaire Vaudois), Lausanne 1011, Switzerland. marine.jequier@chuv.ch
Deonna
T
T
Maeder
P
P
Mayor-Dubois
C
C
Roulet-Perez
E
E
D000208:Acute Disease; D005060:Europe / Q000453:epidemiology; D005260:Female; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000453:epidemiology / Q000473:pathology*; D065768:Lennox Gastaut Syndrome; D013036:Spasms, Infantile / Q000453:epidemiology / Q000473:pathology*