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A 16-Year-Old Boy Is Being Evaluated for Difficulty Walking

question 434

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A 16-year-old boy is being evaluated for difficulty walking.  On physical examination, he has thoracic scoliosis and multiple small scars on his hands.  When asked about these, the boy replies, "I frequently burn my hands by accident because I can't feel the heat."  Neurologic examination reveals 1+ biceps reflexes and 3+ patellar reflexes bilaterally, as well as decreased muscle strength in the bilateral upper and lower extremities.  His hand muscles are atrophic, and position and vibration senses in the feet are also impaired.  Which of the following is the most likely cause of this patient's problems?


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