11th August 2024

Rare causes of vascular dementia

CADASIL

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by autosomal dominant inheritance of a mutation in the notch3 gene. There is typically a history of adult-onset migraine-like headaches and episodes of focal neurological symptoms, with an accumulation of subcortical infarcts resulting in dementia by the late forties.

Primary cerebral vasculitis

Primary cerebral vasculitis, isolated inflammation of cerebral vasculature, can cause a progressive dementia. It may present as cerebral amyloid-associated inflammation in some patients with cerebral amyloid angiopathy (CAA), presenting with headaches, seizures and progression of cognitive deficit. Neuroimaging can show microhaemorrhages, white matter changes and vasogenic oedema. Treatment is with immunosuppression.