Computed tomography, magnetic resonance imaging and pathological correlations in a case of Binswanger's disease

The results of 3 computed tomography (CT) examinations carried out over a 7 year period and of a post-mortem magnetic resonance (MR) study showed aspects of a white matter disease in a hypertensive patient suffering from vascular dementia. Histopathology revealed the primary cause of dementia to be a white matter degeneration sparing the U fibers. Rarefaction of both the myelin sheaths and the axons was present together with severe thickening of the medullary arteries. These findings support the existence of Binswanger's disease (BD) as a distinct variety of arteriosclerotic dementia. CT and MR imaging are valuable aids for diagnosis. However, since there are many other causes of CT and MR demonstrated diffuse white matter degeneration in the elderly, a conclusive diagnosis of BD requires pathological confirmation.     

Pathological findings correlated with magnetic resonance imaging in subcortical arteriosclerotic encephalopathy (Binswanger''s disease).

Formalin-fixed brain slices from four cases of subcortical arteriosclerotic encephalopathy in which a firm diagnosis could be made both clinically and pathologically have been studied by magnetic resonance imaging (MRI). The slices were subsequently embedded in paraffin-wax or celloidin and sections were cut in the same plane as the MRI slices. There was a good correlation between the extent and severity of the abnormal MRI signal and the pathological changes. Areas of diffuse MRI abnormality corresponded with areas of axonal and myelin loss with gliosis, and small "lacune"-like lesions corresponded with lacunar infarcts histologically. Sparing of the subcortical U-fibres was seen histologically and on MRI. The abnormal signal probably originates from increased tissue water attributable to gliosis and an expanded extracellular space.     

Evaluation of dementia in subcortical arteriosclerotic encephalopathy (Binswanger's disease)

Summary Sixteen patients with a clinical diagnosis of subcortical arteriosclerotic encephalopathy (Binswanger's disease) were studied. Mild or moderate dementia was found in 14 cases, with characteristic disturbances of concentration, and psychic retardation with apathy. Neuropsychological studies revealed that signs of diffuse lesions in the frontal lobes dominated. Impairment of memory was significant in all cases. Although intellectual impairment in subcortical arteriosclerotic encephalopathy resembled subcortical dementia, some significant differences were found.     

Gait disorder of subcortical arteriosclerotic encephalopathy: Binswanger's disease

The abnormality of gait that may occur in patients with subcortical arteriosclerotic encephalopathy (Binswanger's disease) is described in 12 patients in whom difficulty walking was the presenting symptom. Their gaits had elements of both Parkinsonism and ataxia, which were most marked in 4 patients unable to stand unaided. The difficulty in using their legs to walk was out of proportion to that of other movements of the lower limbs when lying or seated. In contrast, upper limb mobility and facial expression were relatively preserved. Attention is drawn to the similarity of this gait pattern to that of some patients with hydrocephalus, frontal lobe lesions, and "senile" disorders of gait. A possible mechanism for this Parkinsonian-ataxia in these various conditions may be their propensity to damage the afferent and efferent interconnections of the leg areas of the motor and supplementary motor areas of the cerebral cortex with, the cerebellum and basal ganglia.     

Subcortical arteriosclerotic encephalopathy: a clinical and radiological investigation.

Subcortical arteriosclerotic encephalopathy (Binswanger's type) was diagnosed in a group of patients with hypertension or arteriosclerosis, who showed acute and subacute neurological deficits, dementia, reduced cerebral blood flow, and white matter low attenuation with mild atrophy and infarcts as the predominant CT scan features. This set of clinical and radiological criteria could be used to make the diagnosis in life, as confirmed neuropathologically in one patient.     

The role of plasma hyperviscosity in subcortical arteriosclerotic encephalopathy (Binswanger's disease)

Summary Five haemorheological variables (haematocrit, plasma viscosity, red cell aggregation, red cell deformability and yield shear stress) and the plasma fibrinogen concentration were determined in blood samples from 21 untreated patients with subcortical arteriosclerotic encephalopathy (SAE, Binswanger's disease). The results were compared with those obtained in blood samples from 40 untreated patients with lacunar infarcts and from 275 healthy control subjects without vascular risk factors. Pathological (solid body) flow behaviour was detected in the blood of both groups of patients (SAE and lacunar infarcts). However, highly elevated plasma viscosity was a consistent feature only of SAE. The authors present the hypothesis that the high plasma viscosity in patients with SAE may account for the progressive degeneration of cerebral white matter. It is postulated that microcirculatory abnormalities due to microrheological changes may be one of the many missing links in the pathophysiology of SAE.     

Binswanger's disease (Part I): The clinical recognition of subcortical arteriosclerotic encephalopathy in elderly neuropsychiatric patients

The role of vascular disorders in the pathogenesis of dementia has been controversial. Recent studies suggest that subcortical arteriosclerotic encephalopathy (Binswanger's disease), a disorder of white-matter demyelination associated with narrowing of penetrating medullary arteries and arterioles may affect more than 5% of the population over age 65 years. In part I of this paper, the authors present clinical examples and review the clinical literature, including clinical course and radiologic features. Differential diagnosis and treatment options are reviewed. In part II of this paper we will discuss theories of pathogenesis of subcortical arteriosclerotic encephalopathy and implications for the nosology of dementia.     

Subcortical arteriosclerotic encephalopathy: a clinical and radionuclide brain scintiscan study

Thirty Chinese patients with clinical and computed tomographic (CT) features of subcortical arteriosclerotic encephalopathy (SAE) were reviewed. It is concluded that the features were sufficiently characteristic for SAE to be considered as a sub-category of cerebrovascular disease and an identifiable cause of dementia in the elderly. Sixteen patients in this series underwent radioisotope brain scintiscan, in which the dynamic radionuclide scintiangiography (RSA) correlated with the clinical course. An increased mean plateau/peak value in the hemispheric curves is seen in the deteriorating patients in contrast to the stable patients.     

White matter lucencies on computed tomography, subacute arteriosclerotic encephalopathy (Binswanger's disease), and blood pressure

Of 1,643 cranial computed tomography (CT) scans done in a primary-tertiary care private hospital over a 1-year period, 11 (0.67%) showed diffuse confluent white matter lucencies of less than 30 Hounsfield units. By retrospective analysis, at least 4 of the 11 were demented. Of these, 3 had clinical evidence of Binswanger's disease--characterized by progressive dementia, incontinence, variable pseudobulbar signs, and acute and subacute motor deficits. Two additional patients suffered only transient ischemic attacks or lacunar strokes; 2 had syncope; 1 had multiple sclerosis. The remaining patients were neurologically asymptomatic. In this small retrospective series, the severity of CT changes did not distinguish the patients with clinical Binswanger's syndrome from neurologically less symptomatic patients. Ten of the eleven patients had disordered blood pressure regulation--hypertension, labile systolic pressure, orthostatic hypotension, or a combination of these factors. The severity of CT changes correlated more clearly with blood pressure instability than with clinical encephalopathy. Asymptomatic adult patients with unexplained CT white matter hypodensity and blood pressure disorders may, however, be at risk for the development of subsequent subacute arteriosclerotic encephalopathy.     

Subcortical arteriosclerotic encephalopathy (Binswanger): computerized tomography

The subcortical arteriosclerotic encephalopathy of Binswanger is characterized clinically by hypertension, dementia, spasticity, syncope, and seizures. It is usually diagnosed pathologically by the finding in white matter of diffuse demyelination or foci of necrosis plus arteriosclerotic and hypertensive vasculopathy. We present a case in which the diagnosis was made on the basis of the clinical course and a computerized tomogram which demonstrated extensive white matter degeneration. Postmortem examination confirmed both the diagnosis and the extent of the degeneration as shown by CT scan.     

Subcortical arteriosclerotic encephalopathy (Binswanger''s disease): a report of five patients

Five patients with variable clinical symptoms were diagnosed as having--subcortical arteriosclerotic encephalopathy (Binswanger disease) based on the presence of lacunar infarcts in basal ganglia, various abnormalities of subcortical white matter and severe thickening and hyalinization of penetrating arteries and arterioles. One case had a classical clinical picture while in the others the course of the disease was short and was associated with severe systemic abnormalities. The variability of the clinical features, the identify of "classical" clinical symptoms with other forms of cerebral arteriosclerosis, the similarity between "atypical" cases and other entities, and the high frequency of associated conditions makes it difficult to characterize the clinical pathological entity called subcortical arteriosclerotic encephalopathy.     

Periodic EEG activity in subcortical arteriosclerotic encephalopathy (Binswanger's type)

Serial EEGs with early and progressive bilateral periodic activity were recorded from a patient with clinically "atypical" but pathologically confirmed subcoritcal arteriosclerotic encephalopathy (Binswanger's type). This disease should be considered as a diagnostic possibility when periodic EEG activity is encountered, particularly when dementia, hypertension, and focal neurologic signs coexist. At times, white matter lesions may contribute to the production of periodic EEG activity.     

Subacute sclerosing panencephalitis with atypical onset: clinical, computed tomographic, and magnetic resonance imaging correlations

Clinical, computed tomographic (CT), and magnetic resonance imaging (MRI) correlations of subacute sclerosing panencephalitis with an atypical onset are presented in three children. In all three patients, the disease began similarly, with unilateral neurologic deficit followed by gnosis, praxis, and memory dysfunction corresponding to massive one-sided lesions. The first patient demonstrated right frontal-lobe syndrome and polymorphic extrapyramidal hyperkinesias; MRI showed a large high-signal lesion in the right frontal lobe, while CT was normal. The second patient displayed a disease onset with left-sided hemiparkinsonism and involuntary movements correlating to the MRI finding, ie, a massive rightsided occipitotemporoparietal subcortical lesion. An acute, stroke-like episode represented the first symptom in the third child. CT visualized cerebral, mainly left-sided cortical atrophy. In all three children, CT and MRI revealed significantly progressing brain atrophy at the disease's latest stages. We discuss the role of MRI in detecting early pathologic changes in children with subacute sclerosing panencephalitis.     

Binswanger's disease (Part II): Pathogenesis of subcortical arteriosclerotic encephalopathy and its relation to other dementing processes

Subcortical arteriosclerotic encephalopathy (SAE) is a common though infrequently recognized dementia of the elderly. The unique vascular anatomy of the subcortical white matter and central brain stem probably predisposes those regions to chronic ischemia and incomplete infarction in the presence of various cardiovascular and hemodynamic insults. Recent studies have begun to define the risk factors for SAE, and others have shown it to be a condition frequently comorbid with the dementias of Alzheimer's disease, the multi-infarct state, and normal pressure hydrocephalus. Recent research into the etiologies of these disorders suggest certain pathogenetic links between them, strongly implying that they are not neatly distinct disease entities, as is commonly believed, and accounting for some of the overlap between these dementing illnesses seen clinically.     

Subcortical arteriosclerotic encephalopathy (Binswanger''s type) and cortical infarcts in a young normotensive patient.

A 49-year-old normotensive man died after a series of strokes, slowly evolving dementia and personality change occurring over a period of 23 years. CT scan showed large infarcts involving the cortex and white matter of the temporo-occipital areas, small subcortical infarcts and low attenuation in the white matter of the frontal and parietal lobes. Neuropathological examination revealed large cortical and small subcortical infarcts corresponding to the radiological findings as well as degeneration/demyelination of central white matter corresponding to the areas of low attenuation seen on CT. The basic underlying pathological process was hyaline arteriosclerosis and atheroma which diffusely affected the small intracerebral arteries and to a lesser extent the arteries of the circle of Willis. Though usual because of the absence of hypertension, the very early age at onset of the syndrome and the presence of large cortical infarcts this case illustrates the clinical, radiological and neuropathological features of subcortical arteriosclerotic encephalopathy (Binswanger's type).     

Computed tomography and magnetic resonance imaging of the brain in hypoglycaemic encephalopathy

A diabetic man suffering from a hypoglycaemic attack presented to our hospital. Magnetic resonance imaging (MRI) findings were grossly abnormal and difficult to interpret. The computed tomographic appearance simulated multiple infarctions. The MRI appearance simulated a space occupying lesion. Literature review of the radiology and the pathophysiology of hypoglycaemic brain damage is provided. Comparison between our findings and previous reports is also given.