1 MCQs in Pediatrics for NEET PG
A 9-year-old girl with progressive visual loss, cognitive decline, seizures, and myoclonic jerks is evaluated with EEG during photic stimulation. The pattern marked **A** in the diagram—a giant occipital spike-and-slow-wave response at 1–3 Hz stimulation frequency—is documented. Skin and conjunctival biopsy reveals lipofuscin inclusions with curvilinear profiles on electron microscopy. Genetic testing confirms CLN3 mutation. Which of the following best describes the pathophysiological basis of the distinctive photic EEG response marked **A** in this condition?
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