Non-convulsive status epilepticus revealing acute deterioration in AARS2 mutation-associated progressive leukoencephalopathy.
Meryem Tuba Sönmez, Hikmet Saçmaci
✦ AI-curated · Sources linked
Case in brief
A 41-year-old woman with AARS2 mutation-associated leukoencephalopathy presented with rapid cognitive decline and altered mental status. EEG revealed rhythmic frontal delta activity indicative of nonconvulsive status epilepticus (NCSE). Following antiseizure treatment, her consciousness improved significantly, and EEG abnormalities regressed. The case underscores the need to consider NCSE in patients with neurodegenerative disorders experiencing sudden clinical deterioration.
Diagnostic / clinical pearl
Clinicians should consider nonconvulsive status epilepticus in patients with neurodegenerative disorders who exhibit sudden changes in mental status, as it may be underdiagnosed in this population.