X-Linked Emery-Dreifuss Muscular Dystrophy (EMD / Emerin) - X-Linked Recessive Pedigree

A 21-year-old man presents with progressive upper-limb weakness, fixed flexion contractures of the elbows (unable to extend beyond 90°), marked limitation of neck flexion, and a humero-peroneal distribution of weakness. He has had two recent syncopal episodes during exercise. Cardiac evaluation reveals bradycardia (38 bpm), atrial standstill with junctional escape rhythm at 34 bpm, and intermittent complete atrioventricular block. His mother is clinically well but has occasional premature atrial complexes on screening ECG. His two maternal uncles had similar presentations; one died suddenly at age 29, the other received a pacemaker at age 32. His paternal uncle is entirely healthy. The pedigree pattern shown in the diagram at **B** is most consistent with which inheritance pattern?