Between the MRA and the ED
A short read on adult moyamoya cases and the timeline that tends to decide them.
A composite of cases I see repeatedly. A 42-year-old woman presents to an emergency department with a focal seizure and a new headache. Her head CT shows an intraparenchymal hemorrhage (bleeding directly into the brain tissue). Buried in her chart from sixteen months earlier is the impression line of an MRA report (magnetic resonance angiography, an MRI sequence that maps the brain's arteries): moyamoya pattern; recommend cerebrovascular consultation. No such consultation ever occurred. The diagnosis appears in no progress note in the intervening period.
The cases that reach review on adult moyamoya rarely turn on the imaging. The arteries look the way they do, and a competent reader will say so. The cases turn on what fills the months between the day the disease is named and the day it announces itself.
That is the question this issue takes seriously. What does the chart between the MRA and the ED actually show, and what does its silence carry?
A defensible reconstruction of that interval is built from a small set of specific findings. Was the phenotype, ischemic (stroke from reduced blood flow) or hemorrhagic (stroke from bleeding), classified, or was the disease treated as a unified entity? Was referral to a center with cerebrovascular surgical capability offered, accepted, declined, or simply not made? Was cerebrovascular reserve, the brain's capacity to compensate when flow is challenged, measured before any decision to defer revascularization (surgery to route a new source of blood flow into the brain)? For ischemic-type disease, was antiplatelet therapy prescribed with an accurate understanding of what it does and does not do here? The pooled adult literature shows it lowers hemorrhagic stroke risk; it does not significantly lower ischemic stroke risk in this disease. For hemorrhagic-type disease, was the post-discharge blood-pressure plan tied to the diagnosis the patient carried, or did it read as generic post-bleed boilerplate? Was a surveillance plan documented in dates and modalities, or did the patient leave with "follow up as needed"?
The Primer in this month's Writings explains the medicine in detail, including the JAM trial and the gaps surrounding it. The point worth carrying into a file review is structural. In adult moyamoya, the analytical work begins with the timeline. Build that first, and the strengths and weaknesses of a given case tend to organize themselves against it. Some files that look strong in the complaint dissolve when weighed against a chart documenting a careful conversation, a refused referral, and a missed appointment. Others that look strong on the defense collapse against a chart in which the diagnosis was named once and never engaged.
The MRA shows the disease. The chart shows what was done with it. In adult moyamoya cases, the second record is the one worth reading first.
Tobias B. Kulik, MD, FAAN, CIME/ABIME, CPPS
CorteXion LLC · Scientia et Veritas
The Primer in this month's Writings
Adult Moyamoya: After the Diagnosis, Before the Event — cortexion.co/writings/adult-moyamoya
Primary source
Gonzalez NR, Amin-Hanjani S, Bang OY, et al. Adult Moyamoya Disease and Syndrome: Current Perspectives and Future Directions. A Scientific Statement From the American Heart Association/American Stroke Association. Stroke. 2023;54(10):e465–e479. doi:10.1161/STR.0000000000000443
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