What causes Binswanger's disease?

The primary cause is chronic small vessel ischemic disease, often due to long-standing hypertension, diabetes, or arteriosclerosis. These conditions lead to damage in the white matter of the brain, resulting in the characteristic symptoms.

What are the primary symptoms of Binswanger's disease?

Cognitive decline, motor impairment and gait disturbances, urinary incontinence or urgency, and psychiatric symptoms such as apathy, depression, or mood swings.

ISCHEMIC STROKE

Binswanger’s disease

David Goldemund M.D.
Updated on 11/08/2024, published on 18/02/2022

Binswanger’s disease is a progressive neurological disorder characterized by ischemic damage to the white matter and deep brain structures (basal ganglia and thalamus)
it is caused by small vessel disease and branch artery atherosclerosis
usually can be seen in individuals over 50 years of age
- typically observed in individuals with long-term and poorly controlled hypertension and other vascular risk factors
clinically characterized by a progressive motor and cognitive impairment (VCI)
synonyms used in the literature:
- Binswanger’s encephalopathy
- subcortical leukoencephalopathy
- multi-infarct or vascular dementia of the Binswanger type
- subcortical ischemic vascular disease
- subcortical arteriosclerotic encephalopathy (SAE)
- small vessel dementia

Etiology

arteriolopathy, atherosclerosis, thromboembolism
- mostly small penetrating artery disease
lesions are relatively symmetrical and diffuse – deep periventricular white matter lesions + lacunar infarcts in basal ganglia, thalamus, and brainstem (pons)

Arteriolopathy (arteriolosclerosis)

Clinical presentation

onset usually after the age of 50
“stepwise” progression (as opposed to the continuous progression of degenerative diseases)
- stepwise deterioration may occur as a result of cerebral infarction ⇒ lacunar syndromes
memory disorders, dysexecutive syndrome, emotional changes (apathy, abulia), depression, perseveration
- episodic or declarative memory is relatively preserved
quadripyramidal and pseudobulbar syndrome, gait disturbances (“parkinsonian gait”), falls
urinary urgency or incontinence
chronic arterial hypertension

Diagnostic evaluation

typical clinical presentation and imaging findings + vascular risk factors (especially hypertension)

Imaging methods

CT: symmetrical hypodensities in the deep white matter + lacunar infarcts + brain atrophy
MRI (FLAIR, T2): extensive periventricular and subcortical white matter lesions + subcortical lacunar infarcts + brain atrophy
- lesions are better visualized on MRI compared to CT
- scales have been developed to assess the extent of white matter lesions on MRI:
  - FAZEKAS
  - ARWMC

Binswanger´s disease (FLAIR, coronary plane)

Extensive leukoencephalopathy in Binswanger´s disease (FLAIR)

Differential diagnosis

CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy)
- typical lesions in the external capsule and pole of the temporal lobe
- more pronounced low signal intensity in the basal ganglia and dentate nuclei
normal pressure hydrocephalus (NPH)
- triad of symptoms: gait disturbance, sphincter insufficiency, and cognitive deterioration (similar to Binswanger’s disease)
- a rather fluent, gradual progression (whereas Binswanger’s disease usually has a stepwise course)
- ventricular dilatation in NPH x cortical atrophy in Binswanger’s disease
CNS vasculitis
Alzheimer’s disease (AD)
Dementia with Lewy bodies (DLB)
Frontotemporal dementia (FTD)

Management

Prevention of cerebrovascular disease

the disease is irreversible; therapy may slow down or halt its progression
antiplatelet or anticoagulant therapy may be considered to reduce the risk of thromboembolic events
aggressive treatment of arterial hypertension
aggressive treatment of other vascular risk factors (e.g., diabetes, hyperlipidemia, etc.)

Symptomatic therapy

symptomatic psychiatric therapy
- antidepressants
  - high prevalence of depression in individuals with Binswanger’s disease
  - selective serotonin reuptake inhibitors (SSRIs) are preferred
- anxiolytic drugs
  - choose medications that have minimal sedative effects to avoid worsening cognitive impairment
- hypnotic drugs
cognitive enhancers – cholinergic deficiency is suspected in VaD (vascular dementia)
- studies have shown some effects of both donepezil and rivastigmine (acetylcholinesterase inhibitors) Black, 2003][Birks, 2013]
physical and occupational therapy aims to:
- maintain mobility and reduce the risk of falls
- help patients adapt to physical limitations and learn techniques to maintain independence in daily activities for as long as possible