A missed diagnosis: acute encephalopathy with biphasic seizures and late reduced diffusion

Area of Science:

• Neurology
• Radiology
• Pediatrics

Background:

• Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a rare neurological disorder.
• Its pathogenesis remains uncertain, though infections are suspected etiologies.
• AESD presents with biphasic seizures and altered consciousness, followed by white matter diffusion restriction and cerebral atrophy.

Observation:

• A 15-year-old patient with pre-existing mental and motor retardation presented with symptoms consistent with AESD.
• Clinical presentation and neuroimaging findings were correlated for diagnosis.
• The patient's specific presentation and diagnostic process are detailed.

Findings:

• The case demonstrates the characteristic biphasic seizure pattern and altered consciousness in the acute phase.
• Subacute stage findings included restricted diffusion in the subcortical white matter on MRI.
• Long-term sequelae included cerebral atrophy, consistent with AESD progression.

Implications:

• This case contributes to understanding the rare AESD subtype, particularly in patients with developmental delays.
• It underscores the importance of clinicoradiological correlation for diagnosing AESD.
• Further research into AESD pathogenesis and effective management strategies is warranted.