Arterial spin labeling reveals cerebral perfusion changes associated with involuntary movements in acute...

Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by a biphasic seizure pattern with distinct neuroimaging findings. While involuntary movements during the recovery phase may be a marker of an unfavorable neurological outcome, their relationship with cerebral perfusion changes remains poorly understood. Arterial spin labeling (ASL) is a non-invasive method of assessing changes in perfusion without using contrast agents that can provide valuable insight into the pathophysiology of the disease. We report herein the first case of AESD demonstrating a correlation between involuntary movements and concurrent abnormalities of cerebral perfusion on ASL.

A 13-month-old female patient was admitted for fever and status epilepticus. Magnetic resonance imaging (MRI) on day of illness (DOI) 6 revealed frontal lobe diffusion restriction on diffusion-weighted imaging (DWI) and hyperperfusion on ASL. ¹H-MR spectroscopy demonstrated metabolic changes consistent with excitotoxic encephalopathy. By DOI 20, myoclonic movements and ballism had developed, and ASL demonstrated a characteristic pattern of frontal hypoperfusion concurrent with basal ganglia hyperperfusion. The involuntary movements resolved over two months. Follow-up MRI performed 8 months after initial presentation revealed recovery of brain volume and improvement in perfusion abnormalities of the basal ganglia. At the age of 5 years, the patient demonstrated mild developmental delay with well-controlled epilepsy.

The perfusion abnormalities detected on ASL correlated with the involuntary movements of AESD, which suggests dysregulation of cerebral autoregulation propagation via cortico-basal ganglia circuits. ASL can provide valuable insight into the pathophysiology of AESD and may facilitate assessment of prognosis and therapeutic decision-making by enabling real-time monitoring of changes in cerebral perfusion during the recovery phase.