Subcortical arteriosclerotic encephalopathy

Subcortical arteriosclerotic encephalopathy (SAE), also known as Binswanger disease or small vessel dementia, refers to slowly progressive, exclusively white-matter, multi-infarct dementia

A genetically transmitted form of the disease is known as familial arteriopathic leukoencephalopathy or CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) 3.

Patients usually present with subcortical dementia symptoms including forgetfulness, personality and emotional changes.

Clinical criteria for diagnosis are as follows:

  • marked subcortical microangiopathic lesions at MR imaging
  • a negative family history for strokes, early cognitive impairment, or psychiatric disorders in first- and second-degree relatives
  • documented arterial hypertension: systolic values higher than 160 mm Hg, diastolic values higher than 95 mm Hg, or both, measured at several occasions 5

Pathologically,  relatively symmetrical and diffuse bilateral deep periventricular white matter lesions are associated with severe arteriosclerosis of the small penetrating arteries which are thickened, lipohyalinised, stenotic, or even occluded. In most patients, multiple lacunar infarcts are also present in the basal ganglia, thalami, and pons 3

CT

Diffuse, incompletely symmetrical hypodensities are present in deep white matter, especially they are prominent in the frontal lobes and the centrum semiovale 3.

MRI

MRI changes are much more striking, consisting of subcortical and periventricular lesions visible on FLAIR, T2-weighted, and proton-density sequences. 

The areas are irregular, commonly grouped around the frontal and occipital horns, and in the centrum semi ovale. 

Moderate, generalised cerebral atrophy is invariably present, and lacunar infarcts in the basal ganglia and thalami are common 3.

It was first described in 1894 by Otto Ludwig Binswanger (1852-1929), a Swiss psychiatrist and neurologist 4

CADASIL: classically, lesions involve anterior temporal and superior frontal lobes which are uncommonly involved in SAE. Also arcuate fibers are more likely to be affected in CADASIL. Low signal intensity of the basal ganglia and dentate nuclei of the cerebellum are more pronounced in CADASIL compared to the SAE 5

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Article information

rID: 7275
Section: Pathology
Synonyms or Alternate Spellings:
  • Subcortical arteriosclerotic encephalopathy (SAE)
  • Leukoariaosis
  • Encephalopathia subcorticalis progressiva
  • Subcortical arteriosclerotic encephalopathy
  • Binswanger disease
  • Binswanger's disease

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Cases and figures

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    Case 1: Binswanger disease (small vessel dementia)
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    Case 2: Binswanger disease
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