Binswanger's disease--revisited

Two decades ago, Binswanger's disease was an obscure disorder unfamiliar to the great majority of neurologists and of doubtful existence and dubious importance even to those who had heard of the entity. Before Schoene and I reported 1 the clinical features of five pathologically confirmed and six clinically diagnosed cases of what we called Binswanger's disease in Neurology in 1978, we first presented the material at a vascular research conference at the Massachusetts General Hospital. The senior clinical neurologists and neuropathologists in attendance (Drs. Raymond Adams, C. Miller Fisher, and E. Pierson Richardson) were skeptical of Binswanger's disease as an entity and of our cases as probable examples. Among our initial 11 cases, only two had had CT; the other clinically studied patients had had pneumoencephalography 1. During the past 15 years, the proliferation of neuroimaging and the advent of MRI have led to a dramatic change in neurologists' attitudes toward white matter ischemic disease. White matter abnormalities are ubiquitous in the elderly, but many patients with so-called leukoaraiosis do not have vascular dementia. The presence of these white matter abnormalities and their significance has brought Binswanger's disease onto center stage, but the disorder remains very controversial. Debated topics without clear agreement include the definition of the disorder and its appropriate name; the clinical diagnosis of the condition, especially separation from nonvascular causes of white matter changes; and its pathogenesis and treatment. Dementias, including vascular dementias, are an important public health problem that is becoming more common and important as the population ages. Microangiopathic vascular dementia of the Binswanger type is a common cause of vascular dementia and invalidism. During the past decade, much new information, many case reports, and several reviews 2-4 have been published. My intent is to review succinctly the major known features of Binswanger's disease, …