OBSOLETE: Binswanger disease

Binswanger disease, also known as subcortical arteriosclerotic encephalopathy or subcortical leukoencephalopathy, is a form of vascular dementia caused by widespread damage to the deep white matter of the brain. The condition results from chronic small vessel disease, typically associated with long-standing hypertension and atherosclerosis, which leads to progressive ischemic injury to the subcortical white matter. This Orphanet entry (ORPHA:1249) is marked as obsolete, as Binswanger disease is now generally classified under the broader category of cerebral small vessel disease or vascular dementia rather than being considered a distinct rare disease entity. The disease primarily affects the central nervous system, with progressive deterioration of white matter tracts in the brain. Key clinical features include slowly progressive cognitive decline, executive dysfunction, psychomotor slowing, gait disturbances, urinary incontinence, and mood changes including depression and apathy. Patients may also experience episodes of acute neurological deterioration related to small strokes. Brain imaging typically reveals extensive periventricular and deep white matter hyperintensities on MRI, along with lacunar infarcts and enlarged ventricles. There is no specific cure for Binswanger disease. Treatment focuses on managing underlying vascular risk factors, particularly hypertension, diabetes, and hyperlipidemia, to slow disease progression. Symptomatic management may include medications for mood disturbances, physical therapy for gait difficulties, and supportive care for cognitive impairment. The condition typically affects older adults and follows a progressive course, though the rate of decline varies among individuals.

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