Binswanger's Disease
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Binswanger's disease, also known as subcortical leukoencephalopathy and subcortical arteriosclerotic encephalopathy, is a form of small-vessel
vascular dementia 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 dement ...
caused by damage to the white brain matter. White matter atrophy can be caused by many circumstances including chronic
hypertension Hypertension, also known as high blood pressure, is a Chronic condition, long-term Disease, medical condition in which the blood pressure in the artery, arteries is persistently elevated. High blood pressure usually does not cause symptoms i ...
as well as old age.Giovannetti, T. Personal Interview. 16 October 2009 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 In cognitive science and neuropsychology, executive functions (collectively referred to as executive function and cognitive control) are a set of cognitive processes that support goal-directed behavior, by regulating thoughts and actions thro ...
of the brain. It usually presents between 54 and 66 years of age, and the first symptoms are usually mental deterioration or stroke. It was described by Otto Binswanger in 1894, and
Alois Alzheimer Alois Alzheimer ( , , ; 14 June 1864 – 19 December 1915) was a German psychiatrist, neuropathologist and colleague of Emil Kraepelin. He is credited with identifying the first published case of "presenile dementia", which Kraepelin later ide ...
first used the phrase "Binswanger's disease" in 1902. However, Jerzy Olszewski is credited with much of the modern-day investigation of this disease which began in 1962.


Signs and symptoms

Symptoms include mental deterioration, language disorder, transient ischemic attack, muscle
ataxia Ataxia (from Greek α- negative prefix+ -τάξις rder= "lack of order") is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes, and abnormalities in e ...
, and impaired movements including change of walk, slowness of movements, and change in posture. These symptoms usually coincide with multiple falls,
epilepsy Epilepsy is a group of Non-communicable disease, non-communicable Neurological disorder, neurological disorders characterized by a tendency for recurrent, unprovoked Seizure, seizures. A seizure is a sudden burst of abnormal electrical activit ...
, fainting, and uncontrollable bladder. 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.


Neurological presentation

Binswanger's disease is a type of subcortical
vascular dementia 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 dement ...
caused by
white matter White matter refers to areas of the central nervous system that are mainly made up of myelinated axons, also called Nerve tract, tracts. Long thought to be passive tissue, white matter affects learning and brain functions, modulating the distr ...
atrophy to the brain. However, white matter atrophy alone is not sufficient for this disease; evidence of subcortical dementia is also necessary. The histologic findings are diffuse, irregular loss of
axons An axon (from Greek ἄξων ''áxōn'', axis) or nerve fiber (or nerve fibre: see spelling differences) is a long, slender projection of a nerve cell, or neuron, in vertebrates, that typically conducts electrical impulses known as action pot ...
and
myelin Myelin Sheath ( ) is a lipid-rich material that in most vertebrates surrounds the axons of neurons to insulate them and increase the rate at which electrical impulses (called action potentials) pass along the axon. The myelinated axon can be lik ...
accompanied by widespread
gliosis Gliosis is a nonspecific reactive change of glial cells in response to damage to the central nervous system (CNS). In most cases, gliosis involves the proliferation or hypertrophy of several different types of glial cells, including astrocytes ...
, 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 Atherosclerosis is a pattern of the disease arteriosclerosis, characterized by development of abnormalities called lesions in walls of arteries. This is a chronic inflammatory disease involving many different cell types and is driven by eleva ...
. The onset of this disease is typically between 54 – 66 years of age and the first symptoms are usually mental deterioration or stroke. 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. Arterioles can become permeable resulting in compromise of the
blood–brain barrier The blood–brain barrier (BBB) is a highly selective semipermeable membrane, semipermeable border of endothelium, endothelial cells that regulates the transfer of solutes and chemicals between the circulatory system and the central nervous system ...
. It has been shown that Binswanger's disease targets the vessels in this zone of the subcortex, but spares the
microcirculation The microcirculation is the circulation of the blood in the smallest blood vessels, the microvessels of the microvasculature present within organ tissues. The microvessels include terminal arterioles, metarterioles, capillaries, and venules. ...
's vessels and
capillaries A capillary is a small blood vessel, from 5 to 10 micrometres in diameter, and is part of the microcirculation system. Capillaries are microvessels and the smallest blood vessels in the body. They are composed of only the tunica intima (the in ...
which may be attributed to a difference between Alzheimer's and Binswanger's disease.


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 In cognitive science and neuropsychology, executive functions (collectively referred to as executive function and cognitive control) are a set of cognitive processes that support goal-directed behavior, by regulating thoughts and actions thro ...
, 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 Perseveration, in the fields of psychology, psychiatry, and speech–language pathology, is the repetition of a particular response (such as a word, phrase, or gesture) regardless of the absence or cessation of a stimulus. It is usually caused by ...
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.


Diagnosis

Binswanger's disease can usually be diagnosed with a
CT scan A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...
,
magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and ...
, and proton magnetic resonance spectrography in addition to clinical examination. Indications include infarctions, lesions, or loss of intensity of central
white matter White matter refers to areas of the central nervous system that are mainly made up of myelinated axons, also called Nerve tract, tracts. Long thought to be passive tissue, white matter affects learning and brain functions, modulating the distr ...
and enlargement of ventricles, and
leukoaraiosis 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 hyperintense, hype ...
. A
mini–mental state examination The mini–mental state examination (MMSE) or Folstein test is a 30-point questionnaire that is used extensively in clinical and research settings to measure cognitive impairment. It is commonly used in medicine and allied health to screen for d ...
has been created to quickly assess cognitive impairment and serves as a screening test for dementia across different cultures.


Imaging

Leukoaraiosis 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 hyperintense, hype ...
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. 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. 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 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'' gen ...
syndrome and Alzheimer's disease, which makes this specific type of white matter damage hard to diagnose. Binswanger disease may be diagnosed by a team of experts including a neurologist and psychiatrist to rule out other psychological or neurological problems. 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. 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. 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.


Management

Binswanger's disease has no cure and has been shown to be the most severe impairment of all of the vascular dementias. 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 Diabetes mellitus, commonly known as diabetes, is a group of common endocrine diseases characterized by sustained high blood sugar levels. Diabetes is due to either the pancreas not producing enough of the hormone insulin, or the cells of th ...
.


History

Binswanger in 1894 was the first to claim that white matter atrophy caused by 'vascular insufficiency' can result in
dementia Dementia is a syndrome associated with many neurodegenerative diseases, characterized by a general decline in cognitive abilities that affects a person's ability to perform activities of daily living, everyday activities. This typically invo ...
. He described a patient who had slow progression of dementia as well as subcortical white matter atrophy, ventricle enlargement,
aphasia Aphasia, also known as dysphasia, is an impairment in a person's ability to comprehend or formulate language because of dysfunction in specific brain regions. The major causes are stroke and head trauma; prevalence is hard to determine, but aph ...
,
hemianopsia Hemianopsia, or hemianopia, is a loss of vision or blindness ( anopsia) in half the visual field, usually on one side of the vertical midline. The most common causes of this damage are stroke, brain tumor, and trauma. This article deals only wi ...
, and
hemiparesis Hemiparesis, also called unilateral paresis, is the weakness of one entire side of the body (''wikt:hemi-#Prefix, hemi-'' means "half"). Hemiplegia, in its most severe form, is the complete paralysis of one entire side of the body. Either hemipar ...
. 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. 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. 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 Neurosyphilis is the infection of the central nervous system by '' Treponema pallidum'', the bacterium that causes the sexually transmitted infection syphilis. In the era of modern antibiotics, the majority of neurosyphilis cases have been report ...
or other types of dementia. Even with these errors, Olszewski concluded that Binswanger disease did exist as a subset of cerebral arteriosclerosis. 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. 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. 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 Alzheimer's disease (AD) is a neurodegenerative disease and the cause of 60–70% of cases of dementia. The most common early symptom is difficulty in remembering recent events. As the disease advances, symptoms can include problems wit ...
.


References

{{DEFAULTSORT:Binswanger's Disease Cerebrovascular diseases Cognitive disorders Neurological disorders Rare diseases