Binswanger's disease

Last edited 05/2020

Binswanger's disease is a rare condition in which the subcortical white matter becomes acutely infarcted. Usually, it arises from hypertensive or atherosclerotic disease thickening perforating deep vessels and capillaries. There are usually associated lacunar or embolic strokes.

• Binswanger type has a progressive course characterized by extensive damage to the white matter, executive dysfunction, disruption of the blood-brain barrier, unstable gait and urinary incontinence
• clinical picture may resemble that of multi-infarct dementia except that in Binswanger's disease, major stroke is unusual, and examination shows:
  • characteristic wide-based gait - 'marche a petit pas'
  • pseudobulbar palsy
  • corticospinal signs
• one of the causes of gait disability and abulia in the elderly
  
  
• chronic hypertension is the most common cause - sustained hypertension causes the blood vessel lumen to narrow and the outer wall to develop fibrosis. Reduced cerebral blood flow leads to hypoxia
• this reduction in tissue oxygen causes an increase in hypoxia inducible factor-1alpha, which triggers the infiltration of macrophages from the systemic circulation and activation of endogenous microglia
• these inflammatory cells release proteases and free radicals
• produce disruption of the blood-brain barrier and breakdown of myelin, causing the characteristic pathology of Binswanger's disease

Periventricular areas of white matter disruption and gliosis are seen on CT or MRI scan. Imaging features:

• White matter hyperinteinsities on T2
• Brain atrophy (mild to moderate)
• Lacunar infarcts
• Micro-bleeds
• Enlarged perivascular spaces
• Intracranial atherosclerosis

Management:

• blood pressure control is the main therapy that most likely delays progression of the disease
• antiplatelet therapy, statins diet and exercise are also play an important role in the medical management of this condition.

Reference:

• Huisa NH, Rosenburg GA. Binswanger's disease: Diagnosis and Management. Expert Rev Neurother. 2014 Oct; 14(10): 1203-1213.