Binswanger's disease

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Binswanger's disease
Other namesSubcortical arteriosclerotic encephalopathy
Leucoaraiose.jpg
Specialty Neurology   OOjs UI icon edit-ltr-progressive.svg
Symptoms Cognitive impairment, ataxia, slowness of movement, problems with language, bradyphrenia

Binswanger's disease, also known as subcortical leukoencephalopathy and subcortical arteriosclerotic encephalopathy, [1] is a form of small-vessel vascular dementia caused by damage to the white brain matter. [2] White matter atrophy can be caused by many circumstances including chronic hypertension as well as old age. [3] This disease is characterized by loss of memory and intellectual function and by changes in mood. These changes encompass what are known as executive functions of the brain. [4] It usually presents between 54 and 66 years of age, and the first symptoms are usually mental deterioration or stroke. [5]

Contents

It was described by Otto Binswanger in 1894, and [6] Alois Alzheimer first used the phrase "Binswanger's disease" in 1902. [7] However, Jerzy Olszewski is credited with much of the modern-day investigation of this disease which began in 1962. [5] [8]

Signs and symptoms

Symptoms include mental deterioration, language disorder, transient ischemic attack, muscle ataxia, and impaired movements including change of walk, slowness of movements, and change in posture. These symptoms usually coincide with multiple falls, epilepsy, fainting, and uncontrollable bladder. [5] Because Binswanger's disease affects flow processing speed and causes impaired concentration, the ability to do everyday tasks such as managing finances, preparing a meal and driving may become very difficult. [3]

Neurological presentation

Binswanger's disease is a type of subcortical vascular dementia caused by white matter atrophy to the brain. However, white matter atrophy alone is not sufficient for this disease; evidence of subcortical dementia is also necessary. [9]

The histologic findings are diffuse, irregular loss of axons and myelin accompanied by widespread gliosis, tissue death due to an infarction or loss of blood supply to the brain, and changes in the plasticity of the arteries. The pathologic mechanism may be damage caused by severe atherosclerosis. The onset of this disease is typically between 54 – 66 years of age and the first symptoms are usually mental deterioration or stroke. [4]

The vessels that supply the subcortical white matter come from the vessels that support basal ganglia, internal capsule, and thalamus. It is described as its own zone by and susceptible to injury. Chronic hypertension is known to cause changes in the tension of the smooth wall vessels and changes in the vessel diameter. [3] Arterioles can become permeable resulting in compromise of the blood–brain barrier. [4] [10] It has been shown that Binswanger's disease targets the vessels in this zone of the subcortex, but spares the microcirculation's vessels and capillaries which may be attributed to a difference between Alzheimer's and Binswanger's disease. [11]

Psychological presentation

There is a difference between cortical and subcortical dementia. Cortical dementia is atrophy of the cortex which affects ‘higher’ functions such as memory, language, and semantic knowledge whereas subcortical dementia affects mental manipulation, forgetfulness, and personality/emotional changes. Binswanger's Disease has shown correlations with impairment in executive functions, but have normal episodic or declarative memory. Executive functions are brain processes that are responsible for planning, cognitive flexibility, abstract thinking, rule acquisition, initiating appropriate actions and inhibiting inappropriate actions, and selecting relevant sensory information. There have been many studies done comparing the mental deterioration of Binswanger patients and Alzheimer patients. It has been found in the Graphical Sequence Test that Binswanger patients have hyperkinetic perseveration errors which cause the patients to repeat motion even when not asked whereas Alzheimer patients have semantic perseveration because when asked to write a word they will instead draw an image depicting the word. [12]

Diagnosis

Binswanger's disease can usually be diagnosed with a CT scan, magnetic resonance imaging, and proton magnetic resonance spectrography in addition to clinical examination. Indications include infarctions, lesions, or loss of intensity of central white matter and enlargement of ventricles, and leukoaraiosis. A mini–mental state examination has been created to quickly assess cognitive impairment and serves as a screening test for dementia across different cultures. [13]

Imaging

Leukoaraiosis refers to the imaging finding of white matter changes that are common in Binswanger disease. However, leukoaraiosis can be found in many different diseases and even in normal patients, especially in people older than 65 years of age. [5]

There is controversy whether leukoaraiosis and mental deterioration actually have a cause and effect relationship. Research has shown that different types of leukoaraiosis can affect the brain differently, and that proton magnetic resonance spectroscopy would be able to distinguish the different types more effectively and better diagnose and treat the issue. [9] Because of this information, white matter changes indicated by magnetic resonance imaging or computerized tomography cannot alone diagnose Binswanger disease, but can aid to a bigger picture in the diagnostic process. There are many diseases similar to Binswanger's disease including CADASIL syndrome and Alzheimer's disease, which makes this specific type of white matter damage hard to diagnose. [5] Binswanger disease may be diagnosed by a team of experts including a neurologist and psychiatrist to rule out other psychological or neurological problems. [3]

Much of the major research today is done on finding better and more efficient ways to diagnose this disease. Many researchers have divided the magnetic resonance imaging of the brain into different sections or quadrants. A score is given to each section depending on how severe the white matter atrophy or leukoaraiosis is. Research has shown that the higher these scores, the more of a decrease in processing speed, executive functions, and motor learning tasks. [14] [15] Other researchers have begun using computers to calculate the percentage of white matter atrophy by counting the hyper-intense pixels of the magnetic resonance images. These and similar reports show a correlation between the amount of white matter alterations and the decline of psychomotor functions, reduced performance on attention and executive control. [16] [17] One type of technology is called susceptibility weighted imaging (SWI) which is a magnetic resonance technique which has an unusually high degree of sensitivity and can better detect white matter alterations. [18]

Management

Binswanger's disease has no cure and has been shown to be the most severe impairment of all of the vascular dementias. [19] The successful management of vascular risk factors that contribute to poor perfusion in the brain is to treat the cause, such as chronic hypertension or diabetes. [20]

History

Binswanger in 1894 was the first to claim that white matter atrophy caused by 'vascular insufficiency' can result in dementia. He described a patient who had slow progression of dementia as well as subcortical white matter atrophy, ventricle enlargement, aphasia, hemianopsia, and hemiparesis. [9] He named this disease 'encenphailitis subcorticalis chronica progressive.' Binswanger did not conduct any microscopic investigations so many did not believe his findings and attributed the neural damage to neural syphilis. [3] Alzheimer in 1902 studied Binswanger's work with pathological evidence that concluded and supported Binswanger's ideas and hypotheses. Alzheimer renamed this disease Binswanger's disease. [4]

In the late 19th century vascular dementia was heavily studied, however by 1910 scientists were lumping Binswanger's disease with all other subcortical and cortical dementia and labeling everything senile dementia despite all previous research and efforts to distinguish this disease from the rest. In 1962 J. Olszewski published an extensive review of all literature about Binswanger's disease so far. He discovered that some of the information in the original reports was incorrect and that at least some of the patients studied in these cases probably had neurosyphilis or other types of dementia. Even with these errors, Olszewski concluded that Binswanger disease did exist as a subset of cerebral arteriosclerosis. [19] Yet again, in 1974 the term multi-infarct dementia was coined and all vascular dementia was grouped into one category. Because of this, the specific names of these types of this dementia, including Binswanger's disease were lost. [4] This was until 1992 when Alzheimer's diagnostic centers created specific criteria known as the Hachinski Ischemic Scale (after Dr. Vladimir Hachinski) which became the standard for diagnosing MID or vascular dementia. [21]

The complicated history of Binswanger's disease and that it was overlooked as a disease for many years means some patients may have been misdiagnosed with Alzheimer's disease. [9]

Related Research Articles

<span class="mw-page-title-main">White matter</span> Areas of myelinated axons in the brain

White matter refers to areas of the central nervous system (CNS) that are mainly made up of myelinated axons, also called tracts. Long thought to be passive tissue, white matter affects learning and brain functions, modulating the distribution of action potentials, acting as a relay and coordinating communication between different brain regions.

<span class="mw-page-title-main">Vascular dementia</span> Dementia resulting from stroke

Vascular dementia is dementia caused by a series of strokes. Restricted blood flow due to strokes reduces oxygen and glucose delivery to the brain, causing cell injury and neurological deficits in the affected region. Subtypes of vascular dementia include subcortical vascular dementia, multi-infarct dementia, stroke-related dementia, and mixed dementia.

<span class="mw-page-title-main">Caudate nucleus</span> Structure of the striatum in the basal ganglia of the brain

The caudate nucleus is one of the structures that make up the corpus striatum, which is part of the basal ganglia in the human brain. Although the caudate nucleus has long been associated with motor processes because of its role in Parkinson's disease, it also plays important roles in nonmotor functions, such as procedural learning, associative learning, and inhibitory control of action. The caudate is also one of the brain structures that compose the reward system, and it functions as part of the cortico-basal ganglia-thalamo-cortical loop.

<span class="mw-page-title-main">CADASIL</span> Medical condition

CADASIL or CADASIL syndrome, involving cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, is the most common form of hereditary stroke disorder, and is thought to be caused by mutations of the NOTCH3 gene on chromosome 19. The disease belongs to a family of disorders called the leukodystrophies. The most common clinical manifestations are migraine headaches and transient ischemic attacks or strokes, which usually occur between 40 and 50 years of age, although MRI is able to detect signs of the disease years prior to clinical manifestation of disease.

Cerebral atrophy is a common feature of many of the diseases that affect the brain. Atrophy of any tissue means a decrement in the size of the cell, which can be due to progressive loss of cytoplasmic proteins. In brain tissue, atrophy describes a loss of neurons and the connections between them. Brain atrophy can be classified into two main categories: generalized and focal atrophy. Generalized atrophy occurs across the entire brain whereas focal atrophy affects cells in a specific location. If the cerebral hemispheres are affected, conscious thought and voluntary processes may be impaired.

In neurology, semantic dementia (SD), also known as semantic variant primary progressive aphasia (svPPA), is a progressive neurodegenerative disorder characterized by loss of semantic memory in both the verbal and non-verbal domains. However, the most common presenting symptoms are in the verbal domain. Semantic dementia is a disorder of semantic memory that causes patients to lose the ability to match words or images to their meanings. However, it is fairly rare for patients with semantic dementia to develop category specific impairments, though there have been documented cases of it occurring. Typically, a more generalized semantic impairment results from dimmed semantic representations in the brain.

<span class="mw-page-title-main">Perivascular space</span> Space surrounding blood vessels

A perivascular space, also known as a Virchow–Robin space, is a fluid-filled space surrounding certain blood vessels in several organs, including the brain, potentially having an immunological function, but more broadly a dispersive role for neural and blood-derived messengers. The brain pia mater is reflected from the surface of the brain onto the surface of blood vessels in the subarachnoid space. In the brain, perivascular cuffs are regions of leukocyte aggregation in the perivascular spaces, usually found in patients with viral encephalitis.

Logopenic progressive aphasia (LPA) is a variant of primary progressive aphasia. It is defined clinically by impairments in naming and sentence repetition. It is similar to conduction aphasia and is associated with atrophy to the left posterior temporal cortex and inferior parietal lobule. It is suspected that an atypical form of Alzheimer's disease is the most common cause of logopenic progressive aphasia.

Memory disorders are the result of damage to neuroanatomical structures that hinders the storage, retention and recollection of memories. Memory disorders can be progressive, including Alzheimer's disease, or they can be immediate including disorders resulting from head injury.

<span class="mw-page-title-main">Leukoencephalopathy</span> A term to describe all brain white matter diseases

Leukoencephalopathy is a term that describes all of the brain white matter diseases, whether their molecular cause is known or unknown. It can refer specifically to any of these diseases:

<span class="mw-page-title-main">Marchiafava–Bignami disease</span> Medical condition

Marchiafava–Bignami disease (MBD) is a progressive neurological disease of alcohol use disorder, characterized by corpus callosum demyelination and necrosis and subsequent atrophy. The disease was first described in 1903 by the Italian pathologists Amico Bignami and Ettore Marchiafava in an Italian Chianti drinker. In this autopsy, Marchiafava and Bignami noticed that the middle two-thirds of the corpus callosum were necrotic. It is very difficult to diagnose and there is no specific treatment. Until 2008 only around 300 cases had been reported. If caught early enough, most patients survive.

Mild cognitive impairment (MCI) is a neurocognitive disorder which involves cognitive impairments beyond those expected based on an individual's age and education but which are not significant enough to interfere with instrumental activities of daily living. MCI may occur as a transitional stage between normal aging and dementia, especially Alzheimer's disease. It includes both memory and non-memory impairments. The cause of the disorder remains unclear, as well as both its prevention and treatment, with some 50 percent of people diagnosed with it going on to develop Alzheimer's disease within five years. The diagnosis can also serve as an early indicator for other types of dementia, although MCI may remain stable or even remit.

<span class="mw-page-title-main">Leukoaraiosis</span> Type of white matter abnormality near the lateral ventricles

Leukoaraiosis is a particular abnormal change in appearance of white matter near the lateral ventricles. It is often seen in aged individuals, but sometimes in young adults. On MRI, leukoaraiosis changes appear as white matter hyperintensities (WMHs) in T2 FLAIR images. On CT scans, leukoaraiosis appears as hypodense periventricular white-matter lesions.

<span class="mw-page-title-main">Posterior cortical atrophy</span> Medical condition

Posterior cortical atrophy (PCA), also called Benson's syndrome, is a rare form of dementia which is considered a visual variant or an atypical variant of Alzheimer's disease (AD). The disease causes atrophy of the posterior part of the cerebral cortex, resulting in the progressive disruption of complex visual processing. PCA was first described by D. Frank Benson in 1988.

Lipohyalinosis is a cerebral small vessel disease affecting the small arteries, arterioles or capillaries in the brain. Originally defined by C. Miller Fisher as 'segmental arteriolar wall disorganisation', it is characterized by vessel wall thickening and a resultant reduction in luminal diameter. Fisher considered this small vessel disease to be the result of hypertension, induced in the acute stage by fibrinoid necrosis that would lead to occlusion and hence lacunar stroke. However, recent evidence suggests that endothelial dysfunction as a result of inflammation is a more likely cause for it. This may occur subsequent to blood–brain barrier failure, and lead to extravasation of serum components into the brain that are potentially toxic. Lacunar infarction could thus occur in this way, and the narrowing – the hallmark feature of lipohyalinosis – may merely be a feature of the swelling occurring around it that squeezes on the structure.

<span class="mw-page-title-main">Hyperintensity</span> High intensity on MRI brain scans

A hyperintensity or T2 hyperintensity is an area of high intensity on types of magnetic resonance imaging (MRI) scans of the brain of a human or of another mammal that reflect lesions produced largely by demyelination and axonal loss. These small regions of high intensity are observed on T2 weighted MRI images within cerebral white matter or subcortical gray matter. The volume and frequency is strongly associated with increasing age. They are also seen in a number of neurological disorders and psychiatric illnesses. For example, deep white matter hyperintensities are 2.5 to 3 times more likely to occur in bipolar disorder and major depressive disorder than control subjects. WMH volume, calculated as a potential diagnostic measure, has been shown to correlate to certain cognitive factors. Hyperintensities appear as "bright signals" on an MRI image and the term "bright signal" is occasionally used as a synonym for a hyperintensity.

Subcortical dementias includes those diseases which predominantly affects the basal ganglia along with features of cognitive decline.

<span class="mw-page-title-main">Cerebral atherosclerosis</span> Medical condition

Cerebral atherosclerosis is a type of atherosclerosis where build-up of plaque in the blood vessels of the brain occurs. Some of the main components of the plaques are connective tissue, extracellular matrix, including collagen, proteoglycans, fibronectin, and elastic fibers; crystalline cholesterol, cholesteryl esters, and phospholipids; cells such as monocyte derived macrophages, T-lymphocytes, and smooth muscle cells. The plaque that builds up can lead to further complications such as stroke, as the plaque disrupts blood flow within the intracranial arterioles. This causes the downstream sections of the brain that would normally be supplied by the blocked artery to suffer from ischemia. Diagnosis of the disease is normally done through imaging technology such as angiograms or magnetic resonance imaging. The risk of cerebral atherosclerosis and its associated diseases appears to increase with increasing age; however there are numerous factors that can be controlled in attempt to lessen risk.

<span class="mw-page-title-main">Pathology of multiple sclerosis</span> Pathologic overview

Multiple sclerosis (MS) can be pathologically defined as the presence of distributed glial scars (scleroses) in the central nervous system that must show dissemination in time (DIT) and in space (DIS) to be considered MS lesions.

<span class="mw-page-title-main">Vladimir Hachinski</span> Canadian clinical neuroscientist

Vladimir Hachinski is a Canadian clinical neuroscientist and researcher based at the Schulich School of Medicine and Dentistry at Western University. He is also a Senior Scientist at London's Robarts Research Institute. His research pertains in the greatest part to stroke and dementia, the interactions between them and their joint prevention through holistic brain health promotion. He and John W. Norris helped to establish the world's first successful stroke unit at Sunnybrook Hospital in Toronto, and, by extension, helped cement stroke units as the standard of care for stroke patients everywhere. He discovered that the control of the heart by the brain is asymmetric, the fight/flight (sympathetic) response being controlled by the right hemisphere and the rest and digest (parasympathetic) response being controlled by the left hemisphere and damage to one key component can lead to heart irregularities and sudden death. This discovery has added fundamental knowledge to how the brain controls the heart and blood pressure and lays the foundation for helping prevent sudden death.

References

  1. ↑ van der Knaap, MS; Valk, J (1995). "Subcortical Arteriosclerotic Encephalopathy". Magnetic resonance of myelin, myelination, and myelin disorders (2nd ed.). Berlin, Heidelberg: Springer. doi:10.1007/978-3-662-03078-3_65. ISBN   978-3-662-03078-3.
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  11. ↑ Kitaguchi H, Ihara M, Saiki H, Takahashi R, Tomimoto H (May 2007). "Capillary beds are decreased in Alzheimer's disease, but not in Binswanger's disease". Neurosci Lett. 417 (2): 128–31. doi:10.1016/j.neulet.2007.02.021. PMID   17403574. S2CID   42743797.
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  20. ↑ "Binswanger's Disease". NIH. NINDS. July 25, 2022. Retrieved November 8, 2022.
  21. ↑ Hachinski VC, Iliff LD, Zilhka E, Du Boulay GH, McAllister VL, Marshall J, Russell RW, Symon L (September 1975). "Cerebral blood flow in dementia". Arch Neurol. 32 (9): 632–7. doi:10.1001/archneur.1975.00490510088009. PMID   1164215.
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