When Febrile Seizures Are Not Benign: An Unusual Cause of Dyke-Davidoff-Masson Syndrome

A 14-year-old girl presented with a history of recurrent seizures over the past year, varying in type-generalized tonic-clonic and right-sided focal seizures involving both upper and lower limbs. Birth and family history were unremarkable, and early developmental milestones were appropriate. However, her academic performance declined over the past 2 years, eventually leading her to drop out of school. At 7 months of age, she had an upper respiratory tract infection followed by febrile status epilepticus lasting 20 minutes and requiring 5 days of hospitalization. Neuroimaging, cerebrospinal fluid analysis, and blood cultures were normal. There were no focal neurological deficits at discharge. Antiepileptic medications were discontinued after two seizure-free years. Current examination showed mild right-sided spasticity with increased deep tendon reflexes and an extensor plantar response. Magnetic resonance imaging (MRI) revealed left frontal sinus hypertrophy, left calvarial thickening, left cerebral atrophy, and right-sided crossed cerebellar atrophy, suggestive of Dyke-Davidoff-Masson syndrome (DDMS) (Fig. 1). Electroencephalography (EEG) revealed intermittent sharp waves and background slowing over the left frontotemporal region. She was started on antiepileptic therapy, physiotherapy, and occupational and speech therapy.