ABSTRACT
Case Presentation ...................................................................................................265 Differential Diagnosis ............................................................................................266 Diagnostic Approach .............................................................................................269 Treatment and Long-Term Outcome ......................................................................269 Neurobiology ......................................................................................................... 270 Clinical Pearls ........................................................................................................ 272 Selected Reading.................................................................................................... 272
An 8-year-old developmentally normal boy is brought to the clinic by his mother for episodes of staring and unresponsiveness. She reports that, as early as age 4 years, he would stare out the window of the car at the sun and blink repeatedly. When questioned about this behavior, he reported that “it felt good.” One particularly sunny day, while riding in the car, he continued to stare at the sun until he developed myoclonic jerking that ultimately progressed to whole-body tonic stiffening lasting several minutes. He was taken to a local emergency room where an EEG with photic stimulation evoked a second seizure. Shortly thereafter, he began to engage in an unusual stereotyped behavior. Throughout the day, he would look upwards at the sun or towards a light, and wave a hand in front of his face with ngers spread. As he did this, he would blink several times and then become unresponsive for a few seconds. After a large shiver, he would return immediately to baseline and resume his normal activity.
