Moyamoya disease (MMD): pathology

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Moyamoya disease (MMD): pathology. This is hypertrophy of the lenticulostriate arteries caused by collateral vessels. Fully referenced neurology checklist.

Pathology

  • This is hypertrophy of the lenticulostriate arteries caused by collateral vessels
  • These appear on catheter angiography as moyamoya
  • The collaterals develop due to occlusion of the ACA, the proximal MCA, and the distal ICA
  • There are associated intracranial aneurysms in 8% of cases

Genetics

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Types

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Suzuki's grading

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Acronyms

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References

  1. Hallemeier CL, Rich KM, Grubb RL, et al. Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke 2006; 37:1490-1496.
  2. Yang H, Zhang L, Wang M, Wang J, Chen L, Lu H. Clinical features of and risk factors for intracranial aneurysms associated with moyamoya disease. Int J Stroke 2020 (Online ahead of print).
  3. Mertens R, Graupera M, Gerhardt H, et al. The genetic basis of moyamoya disease. Transl Stroke Res 2021 (Online ahead of print).
  4. Ihara M, Yamamoto Y, Hattori Y, et al. Moyamoya disease: diagnosis and interventions. Lancet Neurol 2022; 21:747-758.
  5. Fukui M. Current state of study on moyamoya disease in Japan. Surg Neurol 1997; 47:138-143.
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