Congenital neurological disorders Quiz: Ace Your Exams

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A 4-week-old newborn boy is brought to the emergency department by his parents due to irritability and difficulty feeding over the past few days. He was born through normal vaginal birth and delivery to a 24-year-old primigravida woman who did not receive prenatal care. The patient’s family history is unremarkable. The patient’s temperature is 37.0°C (98.6°F), pulse is 70/min, respirations are 40/min, and blood pressure is 120/80 mmHg. On physical examination, frontal bossing and prominent scalp veins are noted. Generalized spasticity is present in all limbs. Examination of the skin shows a hair patch over the lower back with a gap under the skin. An MRI of the brain is obtained and shown below:  



Retrieved from: Wikimedia Commons    

Which of the following is the most likely diagnosis?  

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