Referral to National Highly Specialised Care (NHV) in Moyamoya

For emergencies, contact the neurosurgery emergency service at the hospital switchboard: 018-611 00 00.

For referral in elective cases, fax the referral to 018-611 49 04 and link radiological images.

About Moyamoya

Moyamoya disease and syndrome (MM) is characterised by a progressive narrowing of the brain vessels at the base of the skull which can lead to both cerebral infarctions and haemorrhages, affecting both children (most commonly under 10 years of age) and adults (most commonly 30-45 years of age). Spontaneous vascularisation is induced to compensate for the reduction in blood flow caused by the constrictions. On a vascular X-ray, the newly formed vessels have a typical appearance similar to puffs of smoke (moyamoya=puff of smoke in Japanese). The course of the disease varies from slow deterioration with a few occasional mild ischaemic episodes to rapid progression with very severe bleeding or cerebral infarction. In addition to symptoms such as paralysis and speech difficulties, there is also a risk of impaired cognitive functions. Moyamoya is 10 times more common in Asia than in the West.

Moyamoya is hereditary in Asia but not in the West. 

Treatment

Medical treatment with antiplatelet drugs alone is rarely sufficient to prevent long-term progression, while surgical treatment with revascularisation operations significantly reduces the risk of deterioration.

The blood supply can be improved with direct revascularisation surgery using a 'by-pass' and/or the 'burr hole' technique, where multiple holes are drilled in the skull to allow blood vessels to grow from the outside into the brain through the holes. The choice of method is made on an individual basis.

Our care organisation and working methods

Patients are admitted for urgent treatment from both Uppsala University Hospital and other referring hospitals, or are treated electively by referral from various sources for planned assessment and treatment. Urgent cases are usually treated in the neurointensive care unit (NICU), the neurointermediate care unit (NICU) or the paediatric intensive care unit (PICU). Cases not requiring intensive care are mainly treated in the neurosurgery, neurology, stroke departments or the paediatric neurology department of the children's hospital. Non-urgent cases are largely managed on an outpatient basis or interactively with the local hospital.

All cases are managed within the multidisciplinary neurovascular team led by vascular neurosurgeons. Two senior vascular neurosurgeons with specific expertise in MM (Associate Professor Anders Lewén and Professor Per Enblad) are responsible for the multidisciplinary care of patients in terms of investigation, assessment, treatment decision, surgical treatment, and follow-up, as well as for care programmes and R&D. Required competences that do not regularly attend the weekly neurovascular multidisciplinary rounds are involved when necessary. International expertise can also be consulted if necessary.

For national and regional patients, the primary investigation is carried out at the local hospital using routine radiological methods, as well as parts of the recommended follow-up. The images are transferred to the MM team in Uppsala for assessment and decision/recommendation on further processing. More highly specialised examinations, such as vascular X-rays, imaging of cerebral blood flow with MRI and PET and testing of the haemodynamic blood flow reserve with the Diamox test, which are needed both urgently and in the follow-up are carried out at Uppsala University Hospital.

Care programmes, quality monitoring and R&D

The treatment is based on a standardised care programme and the results of the treatment are continuously evaluated in various ways. Research and education (R&D) is a natural and important part of the organisation.

Research articles on Moyamoya

Editorial: Moyamoya disease - natural history and therapeutic challenges '

Teodor Svedung Wettervik, Markus Fahlström, Johan Wikström, Per Enblad and

Anders Lewén. 
Editorial, Front. Neurol. - Endovascular and Interventional Neurology, 2023, In press.

ASL-MRI-guided evaluation of multiple burr hole revascularization surgery in Moyamoya diseas e.

Lewén A, Fahlström M, Borota L, Larsson EM, Wikström J, Enblad P.

Acta Neurochir (Wien), 2023.

Evaluation of single-delay arterial spin labeling-based spatial coefficient of variation and histogram-based parameters in relation to cerebrovascular reserve in patients with Moyamoya disease.

Fahlström M, Svedung Wettervik T, Enblad P, Lewén A, Wikström J. 
Front Neurol, 2023.

Variable Temporal Cerebral Blood Flow Response to Acetazolamide in Moyamoya Patients Measured Using Arterial Spin Labeling.  

Fahlström M, Wikström J, Borota L, Enblad P, Lewén A. 
Front Neurol, 2021.

High Intravascular Signal Arterial Transit Time Artifacts Have Negligible Effects on Cerebral Blood Flow and Cerebrovascular Reserve Capacity Measurement Using Single Postlabel Delay Arterial Spin-Labeling in Patients with Moyamoya Disease.  

Fahlström M, Lewén A, Enblad P, Larsson EM, Wikström J. 
AJNR Am J Neuroradiol, 2020.

Cerebrovascular Reserve in Moyamoya Disease: Relation to Cerebral Blood Flow, Capillary Dysfunction, Oxygenation, and Energy metabolism.

Svedung Wettervik T, Fahlström M, Wikström J, Lewén A, Enblad P. 
Frontiers in Neurology, section Endovascular and Interventional Neurology. In press.