Binswanger's disease (subcortical atherosclerotic encephalopathy)

Introduction

The original description of this disorder is credited to OL Binswanger (1894) who described post-mortem white matter changes in 8 cases of slowly progressive dementia with focal neurologic signs.

Etiology

• chronic ischemia of deep white matter
• hypertension may play a role

Epidemiology

• rare
• generally occurs in patients 50-60 years of age

Pathology

• gross pathology
  • focal & diffuse loss of myelin in deep white matter with reactive changes
  • subcortical arcuate fibers are largely unaffected
  • changes are mostly in temporal & occipital cortex
  • compensatory ventricular dilatation may occur in severe cases
  • myelin destruction may resemble plaques of multiple sclerosis (MS)
• histopathology
  • rarefaction of tissue
  • lipid-containing macrophages
  • gliosis
• pathophysiology
  • demyelination due to reduced perfusion (ischemia)
  • large vessel atherosclerosis (circle of Willis)
  • lacunar infarcts (small penetrating vessels {arterioles})
• valvular heart disease

Clinical manifestations

• slowly progressive dementia (3-5 years)
• focal neurologic deficits
• pseudobulbar palsy
• psychiatric disturbances
• gait disturbance
• hypertension or hypotension
• urinary incontinence
• speech impairment
• ataxia

Radiology

• computed tomography (CT)
  • low density in periventricular white matter
  • changes not specific; common in patients > 60 years
• magnetic resonance imaging (MRI)

Management

• no specific treatment; treatment is symptomatic
• treat hypertension, hypotension, depression, arrhythmias

More general terms

• multi-infarct dementia
• leukoencephalopathy

Patient information

Binswanger's disease patient information