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Binswanger's disease (subcortical atherosclerotic encephalopathy)
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:
1) chronic ischemia of deep white matter
2) hypertension may play a role
Epidemiology:
1) rare
2) generally occurs in patients 50-60 years of age
Pathology:
1) gross pathology
a) focal & diffuse loss of myelin in deep white matter with reactive changes
b) subcortical arcuate fibers are largely unaffected
c) changes are mostly in temporal & occipital cortex
d) compensatory ventricular dilatation may occur in severe cases
e) myelin destruction may resemble plaques of multiple sclerosis (MS)
2) histopathology
a) rarefaction of tissue
b) lipid-containing macrophages
c) gliosis
3) pathophysiology
-> demyelination due to reduced perfusion (ischemia)
a) large vessel atherosclerosis (circle of Willis)
b) lacunar infarcts (small penetrating vessels {arterioles})
4) valvular heart disease
Clinical manifestations:
1) slowly progressive dementia (3-5 years)
2) focal neurologic deficits
3) pseudobulbar palsy
4) psychiatric disturbances
5) gait disturbance
6) hypertension or hypotension
7) urinary incontinence
8) speech impairment
9) ataxia
Radiology:
1) computed tomography (CT)
a) low density in periventricular white matter
b) changes not specific; common in patients > 60 years
2) magnetic resonance imaging (MRI)
Management:
1) no specific treatment; treatment is symptomatic
2) treat hypertension, hypotension, depression, arrhythmias
Interactions
disease interactions
General
leukoencephalopathy
multi-infarct dementia
References
- Greenfield's Neuropathology, 5th ed, Adams JH & Duchen LW (eds),
Oxford University Press, New York, 1992
- National Institute of Neurological Disorders and Stroke (NINDS)
NINDS Binswanger's Disease Information Page
https://www.ninds.nih.gov/disorders/all-disorders/binswangers-disease-information-page