A new consensus review from the VASCERN Neurovascular Diseases (NEUROVASC) Working Group, published in the European Journal of Neurology, offers an updated overview of headaches in Moyamoya angiopathy (MMA), a rare cerebrovascular condition associated with progressive stenosis of the internal carotid arteries.
The publication, titled “The Spectrum of Headaches in Moyamoya Angiopathy: From Mechanisms to Management Strategies—A Consensus Review from the NEUROVASC Working Group”, compiles current evidence and expert opinion to guide clinicians in recognising, classifying, and managing headaches in this patient population.
Read the full article in the European Journal of Neurology.
Understanding the spectrum of headache in Moyamoya angiopathy
Headache is a frequent but poorly characterised symptom in patients with Moyamoya angiopathy. The review highlights that headaches in MMA may mimic primary headache disorders such as migraine or tension-type headache, but can also arise from specific vascular mechanisms related to the disease itself.
The experts summarise proposed pathophysiological mechanisms, including altered cerebral haemodynamics, impaired cerebrovascular autoregulation, and formation of collateral vessels. These changes may contribute to both chronic and acute headache presentations.
Clinical features and diagnostic considerations
The article stresses that headache can occur both before and after revascularisation surgery, and may present independently of ischaemic or haemorrhagic events. Clinicians are encouraged to take a detailed history, as headache type, location, and associated symptoms vary widely.
The review proposes a classification approach distinguishing between “MMA-related” and “MMA-unrelated” headaches to improve diagnostic clarity and support patient-tailored management.
Current management strategies and research gaps
There are no specific evidence-based guidelines for the management of headache in Moyamoya angiopathy. The review discusses available therapeutic options, noting that antiplatelet therapy (particularly aspirin) may be beneficial in some cases, while vasoconstrictive drugs such as triptans should be used with caution or avoided.
The authors underline the need for further studies on the safety and efficacy of newer headache therapies, including CGRP-targeting agents and ditans, as data in Moyamoya patients are currently lacking. Surgical revascularisation, primarily indicated for stroke prevention, has been associated with headache improvement in some but not all patients.
Towards better clinical understanding
This publication represents a collective effort by the NEUROVASC Working Group to summarise the current state of knowledge and outline directions for future research. By gathering insights from European experts, the review aims to support neurologists and neurosurgeons in managing headache as part of the broader clinical picture of Moyamoya angiopathy.
Read the full open-access article: https://doi.org/10.1111/ene.70316