Fine hand motor dysfunction in a girl with epileptic encephalopathy with spike-and-wave activation in sleep (EE-SWAS): A case report

Background Regression in cognitive, behavioral, or psychiatric functioning is the cardinal symptom of developmental and/or epileptic encephalopathy with spike and wave activation in sleep (D/EE-SWAS). Motor regression has also been reported but is very rare. We describe the case of a girl with EE-SWAS who developed fine motor dysfunction localized in the right hand. Case presentation A 4-year-old girl presented with focal seizure of the right upper extremity. Interictal EEG showed localized epileptic discharges predominantly in the left central area, and she was initially diagnosed with focal epilepsy. The seizures ceased completely after administration of levetiracetam and carbamazepine. However, she gradually developed clumsiness of the right hand. Long-term video EEG monitoring revealed electrical status epilepticus in sleep (ESES), predominantly in the left central area, and single photon emission computed tomography (SPECT) showed increased perfusion in the left central area. Subsequently a diagnosis of EE-SWAS was made. Adjustment of the anti-seizure medication led to disappearance of the ESES pattern on her sleep EEG, and the fine motor dysfunction in the right hand resolved completely. Conclusion Previous studies have suggested that specific neuropsychiatric regressions may depend on the cortical area involved by the epileptic activity of each ESES in patient with D/EE-SWAS. The present case clearly highlights the link between epileptiform activity and focal motor dysfunction in D/EE-SWAS.