1978

DOI: 10.1212/wnl.28.12.1206

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Clinical features of subcortical arteriosclerotic encephalopathy (Binswanger disease)

Louis R. Caplan¹,

William C. Schoene²

Abstract: Subcortical arteriosclerotic encephalopathy, a chronic vascular dementia with hydrocephalus, was characterized pathologically in five patients by severe thickening of small vessels and by diffuse regions of white matter loss with gliosis. Lacunar infarcts were also present. The clinical picture in 11 patients was characterized by: (1) persistent hypertension and systemic vascular disease; (2) acute strokes; (3) subacute accumulation of focal neurologic symptoms and signs over weeks to months; (4) long plateau … Show more

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Binswanger's Disease1

Svd and Cognition1

Figure 1 Relative Survival In Patients With Mid With (O) Or1

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1983

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Cited by 264 publications

(106 citation statements)

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“…35,36 Diagnosis before death is now possible from clinical, imaging, and biochemical features; the typical patient with BD has an insidious onset suggestive of small strokes and clinical findings of executive dysfunction on neuropsychological testing, early onset of motor findings with prominent gait impairment, apathy, incontinence, and in the advanced stages, pseudobulbar findings with full-blown dementia. 37,38,39,40,41 There is generally a progressive course with some waxing and waning of symptoms. Vascular risk factors are common, particularly hypertension.…”

Section: Binswanger's Diseasementioning

confidence: 99%

Consensus statement for diagnosis of subcortical small vessel disease

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²

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et al. 2015

J Cereb Blood Flow Metab

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Vascular cognitive impairment (VCI) is the diagnostic term used to describe a heterogeneous group of sporadic and hereditary diseases of the large and small blood vessels. Subcortical small vessel disease (SVD) leads to lacunar infarcts and progressive damage to the white matter. Patients with progressive damage to the white matter, referred to as Binswanger's disease (BD), constitute a spectrum from pure vascular disease to a mixture with neurodegenerative changes. Binswanger's disease patients are a relatively homogeneous subgroup with hypoxic hypoperfusion, lacunar infarcts, and inflammation that act synergistically to disrupt the blood-brain barrier (BBB) and break down myelin. Identification of this subgroup can be facilitated by multimodal disease markers obtained from clinical, cerebrospinal fluid, neuropsychological, and imaging studies. This consensus statement identifies a potential set of biomarkers based on underlying pathologic changes that could facilitate diagnosis and aid patient selection for future collaborative treatment trials.

“…35,36 Diagnosis before death is now possible from clinical, imaging, and biochemical features; the typical patient with BD has an insidious onset suggestive of small strokes and clinical findings of executive dysfunction on neuropsychological testing, early onset of motor findings with prominent gait impairment, apathy, incontinence, and in the advanced stages, pseudobulbar findings with full-blown dementia. 37,38,39,40,41 There is generally a progressive course with some waxing and waning of symptoms. Vascular risk factors are common, particularly hypertension.…”

Section: Binswanger's Diseasementioning

confidence: 99%

Consensus statement for diagnosis of subcortical small vessel disease

¹

,

²

,

³

et al. 2015

J Cereb Blood Flow Metab

View full text Add to dashboard Cite

Vascular cognitive impairment (VCI) is the diagnostic term used to describe a heterogeneous group of sporadic and hereditary diseases of the large and small blood vessels. Subcortical small vessel disease (SVD) leads to lacunar infarcts and progressive damage to the white matter. Patients with progressive damage to the white matter, referred to as Binswanger's disease (BD), constitute a spectrum from pure vascular disease to a mixture with neurodegenerative changes. Binswanger's disease patients are a relatively homogeneous subgroup with hypoxic hypoperfusion, lacunar infarcts, and inflammation that act synergistically to disrupt the blood-brain barrier (BBB) and break down myelin. Identification of this subgroup can be facilitated by multimodal disease markers obtained from clinical, cerebrospinal fluid, neuropsychological, and imaging studies. This consensus statement identifies a potential set of biomarkers based on underlying pathologic changes that could facilitate diagnosis and aid patient selection for future collaborative treatment trials.

“…7 Markers of SVD are also associated with cognition in healthy older adults. 8 The question of whether structural damage plays a causal role in these deficits has been controversial, partly because the correlation between leukoaraiosis on T2-weighted MRI and cognitive performance is weak.…”

Section: Svd and Cognitionmentioning

confidence: 99%

Imaging Small Vessel Disease

¹

2010

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Abstract-Small vessel disease is a major contributor to the growing burden of cognitive impairment and dementia. In addition to diagnosis, MRI techniques provide a means to investigate mechanisms of cognitive decline. Studies that incorporate diffusion tensor MRI show that variance in cognitive performance is largely accounted for by alterations in brain structure. Unresolved questions about the link between structure and function include: (1) the relative importance of a small number of strategic lesions versus the cumulative effect of multiple lesions; (2) the underlying basis for the characteristic profile of cognitive impairment, with selective deficits of executive function, processing speed and working memory. This update focuses on MRI approaches to these problems and techniques to analyze spatial distribution of damage in relation to the networks that subserve major cognitive functions. Lesion mapping and voxel-based analysis, and the application of diffusion tensor MRI tractography to reconstruct critical white matter projections, are highly promising approaches for improving understanding of the relationship between structure and function and the mechanisms of cognitive decline. (Stroke. 2010;41[suppl 1]:S154-S158.)Key Words: CADASIL syndrome Ⅲ diffusion-weighted imaging Ⅲ lacunes Ⅲ leukoaraiosis Ⅲ vascular cognitive impairment Ⅲ white matter disease C erebral small vessel disease (SVD) refers to a spectrum of clinical, cognitive, and behavioral abnormalities linked to pathology of small penetrating arteries and arterioles in the brain. 1 In its common sporadic form, SVD occurs in the context of vascular risk factors, notably hypertension and diabetes mellitus. 2 Growth in interest in SVD has been interwoven with advances in noninvasive brain imaging. The advent of computed tomography in the early 1970s 3 led to renewed interest in Binswanger disease, which until this period had been considered rare. As part of this renaissance, the term leukoaraiosis was coined to describe the diffuse changes seen on computed tomography. 4 The spectrum of classical lesions now recognized on MRI includes leukoaraiosis, lacunae, and more recently cerebral microbleeds. 5 Cognitive impairment is a leading cause of morbidity in SVD. Characteristic lesions on MRI may support a diagnosis of vascular cognitive impairment. 6 However, MRI techniques can also help elucidate the mechanisms of cognitive decline. This update focuses on this issue, reviewing briefly the contribution of MRI to date, outlining remaining puzzles, and providing 2 examples of state-of-the-art approaches to solve them. SVD and CognitionCognitive impairment was recognized as a prominent feature in early clinical series. 7 Markers of SVD are also associated with cognition in healthy older adults. 8 The question of whether structural damage plays a causal role in these deficits has been controversial, partly because the correlation between leukoaraiosis on T2-weighted MRI and cognitive performance is weak. 9 However, the fact that the presence of leukoaraisi...

“…13 The latter is linked to systemic hypertension and thought to be caused by white matter hypoperfusion secondary to local vasculopathy. 14 The possibility of non-CT-imaged damage to periventricular corticospinal fibers can be raised. Only 1 patient displayed periventricular white matter hypodensities on CT scan.…”

Section: Figure 1 Relative Survival In Patients With Mid With (O) Ormentioning

confidence: 99%

Urinary and gait disturbances as markers for early multi-infarct dementia.

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et al. 1987

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In a retrospective study of 84 outpatients with multiinfarct dementia, urinary and gait disturbances were found in 50% and 27%, respectively, and often preceded dementia and discrete stroke-like episodes by more than 5 years. Compared to patients without urinary disturbance, those with urinary dysfunction were predominantly male and more behaviorally impaired, but were similar in age, cognitive score, depression score, computerized tomography findings, and relative survival. Compared to patients without gait disturbance, those with gait abnormality had a higher Hachinski iscbemic score and depression score and were more behaviorally impaired. Urinary and gait abnormalities may be markers for cerebrovascular disease and vascular dementia even in the absence of frank stroke. Damage to bifrontal outflow tracts may be the common pathophysiological mechanism underlying the behavioral and motor symptoms characteristic of vascular dementias. 4 There is presently no accepted treatment for this disease, but there is some rationale for the prevention of MID via the control of risk factors for stroke and by the prophylactic administration of aspirin to patients at risk. Identification of clinical markers for early MID might allow early recognition of highrisk groups so that prophylactic treatment might prevent or delay the development of this fulminant disorder.We therefore examined the prevalence of various risk factors for stroke and vascular disease in our patients with clinical MID. Patients with MID may have a long history of gait or urinary disturbances preceding overt dementia and discrete cerebrovascular events, and we sought to investigate this relation in a retrospective series. Subjects and MethodsThe Geriatric Evaluation Service at The Burke Rehabilitation Center in White Plains, New York, is an outpatient dementia clinic that has studied more than 700 patients over the last 6 years, as described previously.3 All patients had a complete medical, neurological, psychiatric, and neuropsychological evaluation, with laboratory work, brain computerized tomography (CT), and EEG to rule out treatable causes and a modified Hachinski score 6 of 2 6. Patients thought to have a mixed dementia or a multiinfarct state coexistent with Alzheimer's disease were excluded, as were patients with dementias of other etiologies such as head trauma, alcohol abuse, or metabolic encephalopathies. Each patient's record was reviewed for 1) risk factors for and manifestations of vascular disease, 2) family history of hypertension, diabetes mellitus, coronary artery disease, myocardial infarction, and cerebrovascular accident, 3) manifestations of cerebrovascular disease such as stroke, carotid disease or surgery, and transient ischemic attacks (TIA's), 4) neurologic history and examination, 5) scores for ischemic dementia (modified Hachinski score 6 ), cognitive impairment ("mental status quotient" 7 ), behavioral impairment (Haycox score 8 ), and depression (Hamilton score), and 6) brain CT findings.Subgroups with urinary and/or gait dis...

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