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    SubjectsPediatricsNeuronal Ceroid Lipofuscinosis (Batten Disease) - EEG Pattern
    ClinicalPediatrics

    Neuronal Ceroid Lipofuscinosis (Batten Disease) - EEG Pattern

    1 MCQs in Pediatrics for NEET PG

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    A 4-year-old girl with progressive vision loss, developmental regression, and drug-resistant seizures is evaluated with EEG under standardized intermittent photic stimulation. The EEG shows the finding marked **B** in the diagram—a high-amplitude occipital polyspike-and-wave photoparoxysmal response elicited at unusually low flash frequencies of 1–3 Hz, with concurrent giant somatosensory and visual evoked potentials. Fundoscopy reveals optic atrophy, macular pigmentary changes, and attenuated retinal vessels. Axillary skin biopsy shows curvilinear cytoplasmic inclusions on electron microscopy. Which of the following diagnoses is most consistent with this pathognomonic EEG pattern and clinical presentation?

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