Refractory status epilepticus due to SMART syndrome

Area of Science:

• Neurology
• Neuro-oncology
• Epileptology

Background:

• Stroke-like migraine attacks after radiation therapy (SMART) is a rare, late-onset complication following brain irradiation.
• The underlying pathophysiology of SMART syndrome remains poorly understood.

Observation:

• Three patients with a history of cranial radiation presented with recurrent neurological episodes mimicking stroke.
• Symptoms included headache, visual disturbances, altered awareness, and seizures, with some episodes evolving into generalized tonic-clonic seizures.
• Neuroimaging revealed focal cortical hyperperfusion, diffusion restriction, and gadolinium enhancement, suggestive of ictal activity.

Findings:

• Electroencephalography (EEG) demonstrated interictal epileptiform discharges or focal slowing.
• Ictal neuroimaging studies (PET, SPECT) indicated focal cortical hyperperfusion.
• The clinical presentation and neuroimaging findings strongly suggested status epilepticus as the cause of SMART syndrome episodes.

Implications:

• Status epilepticus is a potential explanation for the neurological episodes observed in SMART syndrome.
• While symptoms are often reversible, some patients may require aggressive interventions, including antiepileptic drugs, anesthesic coma, or corticosteroids, to prevent sequelae.
• This study highlights the importance of considering status epilepticus in the differential diagnosis of SMART syndrome.