Headaches in Pediatric Moyamoya: Clinical Characteristics and Association with Ischemic Events
Salodin Al-Achkar1, Lisa Sun2
1School of Medicine, 2Pediatric Neurology, Johns Hopkins University School of Medicine
Objective:
Characterize headaches in pediatric moyamoya and determine whether their presence is associated with ischemic events.
Background:
Moyamoya is a rare and understudied progressive cerebral angiopathy characterized by chronic stenosis or a non-atherosclerotic occlusion of the internal carotid arteries, leading to the development of a collateral intracranial vasculature network. Pediatric moyamoya is a major cause of ischemic stroke. Headaches are a frequent symptom in pediatric moyamoya patients, yet their role in predicting ischemic events in pediatric moyamoya patients remains unclear.
Design/Methods:
We conducted a single-center retrospective cohort study of 81 pediatric moyamoya patients evaluated at the Johns Hopkins Hospital between 2003 and 2025. Demographic, clinical, and outcome data were extracted from electronic medical records. Headache history, timing, and relationship to diagnosis and ischemic events were assessed. Descriptive statistics were summarized as counts and percentages, and associations between headache and ischemic events (stroke or transient ischemic attack) were tested using Fisher’s exact test.
Results:
Headaches were highly prevalent in this cohort of pediatric moyamoya patients (82.7%) and commonly preceded diagnosis (46.2%). Among those with headaches preceding diagnosis, the headache prompted MRI imaging and subsequent diagnosis in only 27.8%. Headaches were often diagnosed as migraines (46.3%), severe (46.3%), and associated with photophobia (44.8%), phonophobia (35.8%), or nausea (35.8%). However, headaches were not associated with ischemic events (85.1% in headache group vs. 85.7% in no-headache group, p=1.0). Similarly, ischemic events did not occur more frequently in those with pre-diagnosis headaches (86.1% in pre-diagnosis headache group vs. 86.7% in no pre-diagnosis headache group; p=1.0).
Conclusions:
In this pediatric moyamoya cohort, headaches were common and sometimes facilitated diagnosis but were not associated with ischemic events. This suggests that while headache may trigger evaluation and early detection, their presence in a child with moyamoya should not necessarily heighten concern for ischemia.
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