Brain

Question 1

Moyamoya:
Definition
Epidemiology
Presentation

Answer 1

Def: idiopathic, progressive vasculopathy involving the terminal ICA and proximal COW.
Creates new vascular network (moyamoya vessels)
Ultimately leads to occlusionl

Epidemio: bimodal
-Childhood 5-10yo.
-Young adults 30-40yo.

Presentation:
Childhood: ischaemic event/ TIA/ headache
Adlult: haemorrhage/ TIA/ headache

Question 2

Causes of moyamoya sydrome?

Answer 2

-Syndrome: Downs, NF 1, TS, CTD
-Child: sickle cells
-Radiotherapy
-Infection/inflammation
-vasculopathies/thrombotic events

Question 3

Moyamoya
Morphology/Dx

Answer 3

Attenuation /narrowing of distal ICA/COW
tends to be bilateral
leads to multiple enlarged vessels in basal ganglia and basal cisterns

Dx:
-puff of smoke (net like vessels in BG)
-Ivy sign (slow flow from engorged pial vessels)
-Brush sign