Profile of precipitating factors and its implication in 160 Indian patients with Moyamoya angiopathy

Abstract

Introduction Moyamoya angiopathy (MMA) has been known to manifest with myriad of neurological manifestations, often in association with various precipitating factors. This is the first study to systematically analyze the precipitating triggers to neurological symptoms done on the largest cohort of MMA in India. Methods A single-centered, cross-sectional observational study, recruiting 160 patients with consecutive angiographically proven MMA over a period of 5 years (2016–2021), was undertaken to evaluate the profile of immediate precipitating factors in temporal association to the neurological symptoms, along with their clinical and radiological characteristics. SPSS 25 was used for statistical analysis. Results Among the 160 patients (Adult-85, children-75), precipitating factors were seen in 41.3%, significantly higher in children (52%) than adults (31.8%) ( p value: 0.011). The commonest triggers included fever (18.8%), emotional stress (8.1%), heavy exercise and diarrhea (6.3% each). Cold bath triggered MMA symptoms in 1.3%. Fever ( p value: 0.008) and persistent crying ( p value: 0.010) triggered neurological symptoms more commonly in children than in adults. Amongst MMA patients with precipitating factors, the commonest MMA presentation included cerebral infarction type (37.9%) and TIA (31.8%). The majority of precipitating factors that preceded an ischemic event were BP-lowing ones (54.7%). Conclusion Neurological symptoms of MMA are commonly associated with several precipitating factors, including the lesser known triggers like cold bath. The frequency and profile precipitating factors varies with the age of presentation and type of MMA. It can serve as an early clue to the diagnosis of MMA and its careful avoidance can be largely beneficial in limiting the distressing transient neurological symptoms.

Headache Associated with Sexual Activity Unveiling Moyamoya Disease

“…These transient perfusion-dependent neurological symptoms in MMA often occur in close temporal association with the occurrence of precipitating factors, including heavy exercises and heightened emotional states, both of which may occur during sexual activity. [ 7 , 8 ] It has been long thought that orgasmic headache occur in the setting of trigeminovascular activation, leading to vasodilation from the release of inflammatory neuropeptide. [ 9 ] A similar mechanism leading to the accentuation of dilation of leptomeningeal collaterals may be underpinning the headache associated with sexual activity in our patient, wherein the physical exercise and heightened emotions associated with sexual activity acted as a precipitating factor.…”