Serial neuropsychological testing before and after hemispherectomy in a child with electrical status epilepticus in slow wave sleep

Electrical status epilepticus in slow wave sleep (ESES) is typically characterized by neuropsychological deterioration to varying extents. While the syndrome is self-limiting for some, for others it has a swiftly declining course that requires aggressive neurosurgical intervention. Here, we present a patient with ESES secondary to a large left middle cerebral artery stroke in utero who experienced behavioral and neuropsychological deterioration that rapidly progressed. Collectively, her neuropsychological status, EEGs, MRIs, and nature of her ESES warranted a left hemispherectomy to prevent further decline. The patient was seen for four neuropsychological evaluations. Over time, neuropsychological test scores did not adequately capture her degree of impairment and change. Behavioral observations during evaluations and parental reports played a critical role in determining her initial decline and mild post-surgical improvement. For all practitioners, this report illustrates one extreme of ESES. It also illustrates, particularly for neurologists and neurosurgeons, the importance of behavioral observations relative to test performances.