A 4-month-old boy of Ashkenazi Jewish descent presents to the pediatric clinic with his parents reporting an exaggerated startle response to loud sounds over the past 2 weeks. On examination, you note hypotonia and developmental regression. Fundoscopy reveals a cherry-red macular spot bilaterally. The condition marked **B** in the diagram is suspected. Which of the following biochemical findings would confirm this diagnosis?
A 14-month-old male infant of Ashkenazi Jewish ancestry presents with developmental regression, exaggerated startle response, loss of head control, hypotonia with brisk deep tendon reflexes, and bilateral cherry-red macular spots on fundoscopy. Enzyme assay shows absent hexosaminidase A activity with normal hexosaminidase B. Both parents are phenotypically normal and confirmed carriers. The pedigree pattern shown demonstrates **A** (autosomal recessive inheritance). Which of the following clinical and genetic features MOST STRONGLY supports this inheritance pattern in this family?
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