Acute infantile encephalopathy predominantly affecting the frontal lobe (AIEF): a European case

Area of Science:

• Neurology
• Pediatrics
• Radiology

Background:

• Acute infantile encephalopathy predominantly affecting the frontal lobes (AIEF) is a recently identified neurological disorder.
• Diagnosis relies on MRI showing frontal lobe diffusion abnormalities and subsequent atrophy, excluding other causes.
• Previous cases were exclusively reported in children of Japanese descent.

Observation:

• This report presents a Caucasian girl with clinical and MRI findings consistent with AIEF.
• The patient exhibited significant verbal apraxia and regression, with less impact on overall cognitive development.
• Initial presentation involved complex partial seizures, not convulsive status epilepticus.

Findings:

• The case expands the known ethnic and clinical spectrum of AIEF.
• The presentation challenges previously suggested etiological mechanisms like postictal damage.
• MRI findings of diffusion-weighted imaging signals and atrophy in the frontal lobes were key diagnostic indicators.

Implications:

• This case suggests AIEF may occur in diverse ethnic populations.
• Further research is needed to elucidate the varied causes of AIEF.
• Understanding AIEF's unique presentation aids in differentiating it from other acute encephalopathies with abnormal MRI diffusion.