Rare form of encephalopathy evolving to dementia of vascular origin. Progressive chronic subcortical encephalitis. CADASIL syndrome or Altzheimer may be the causing factor. Arterial hypertension and diabetes are aggravating factors.

Clinical characteristics are:  

-        subacute onset after 50 years of age in a hypertensive patient, followed by an evolution over about five years on average, often  with repeated strokes;

-        subcortical dementia with frontal type behaviour, associated with a complex accumulation of focal neurological deficits, mainly pyramidal, but also pseudobulbar, extrapyramidal, loss of sphincter control, etc.; 

-        leukoencephalopathy with sclerohyalinosis, atheromatosis, ventricular dilatation and brain lesions

Updated: November 2019