Binswanger's disease
Encyclopedia
Binswanger's disease is a form of small vessel vascular dementia caused by damage to the white brain matter
. White matter atrophy can be caused by many circumstances including chronic hypertension
as well as old age. 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. 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
first used the phrase "Binswanger's disease" in 1902. However, Olszewski is credited with much of the modern-day investigation of this disease which began in 1962.
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
. 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
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 create specific criteria known as the Hachinski's Ischemic Scale which became the standard for diagnosing MID or vascular dementia.
Because of the complicated history of Binswanger’s disease and the fact that it was overlooked as a disease at all for so many years, leads us to believe that many patients have been misdiagnosed as Alzheimer’s for years. This leads us to believe that Binswanger’s is more prominent in the population than once thought.
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 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 and 66 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 because it changes the tension of the smooth walls vessels and causes changes in the vessel diameter. Arterioles can become permeable resulting in compromise of the blood brain barrier. It has been shown that Binswanger’s disease targets the vessels in this zone of the subcortex, but spares the microvessel and capillaries which may be attributed to a difference between Alzheimer’s and Binswanger’s disease.
, but have normal episodic or declarative memory. Executive functions are brain processes that are responsible for planning, cognitive flexibility, abstract thinking, rule acquisition, initiation 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 preservation because when asked to write a word they will instead draw the object of the word.
, 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.
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.
or white matter atrophy.
(LA) are white matter changes that are common in Binswanger’s Disease. However, LA can be found in many different diseases and even in the general population, especially in people older than 65 years of age.
There is controversy whether LA and mental deterioration actually have a cause and effect relationship. Recent research is showing that different types of LA can affect the brain differently, and that proton MR spectroscopy would be able to distinguish the different types more effectively and better diagnosis and treat the issue. Because of this information, white matter changes indicated by a MRI or CT cannot alone diagnose Binswanger's disease, but can aid to a bigger picture in the diagnosis 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. Binswanger’s disease is best when diagnosed of a team by experts including a neurologist and psychiatrist to rule out other psychological or neurological problems. Because doctors must successfully detect enough white matter alterations to accompany dementia as well as an appropriate level of dementia, two separate technological systems are needed in the diagnosing process.
Other researchers have begun using computers to calculate the percentage of white matter atrophy by counting the hyper-intense pixels of the MRI. 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 recent 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 alternations.
Recently a Mini Mental Test (MMT) has been created to accurately and quickly assess cognitive impairment due to vascular dementia across different cultures. Binswanger’s disease has been shown to be the most severe impairment of all of the vascular dementia.
White matter
White matter is one of the two components of the central nervous system and consists mostly of myelinated axons. White matter tissue of the freshly cut brain appears pinkish white to the naked eye because myelin is composed largely of lipid tissue veined with capillaries. Its white color is due to...
. White matter atrophy can be caused by many circumstances including chronic hypertension
Hypertension
Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...
as well as old age. 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
Executive functions
The executive system is a theorized cognitive system in psychology that controls and manages other cognitive processes. It is responsible for processes that are sometimes referred to as the executive function, executive functions, supervisory attentional system, or cognitive control...
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
Otto Binswanger
Otto Ludwig Binswanger was a Swiss psychiatrist and neurologist who came from a famous family of physicians; his father was founder of the Kreuzlingen Sanatorium, and he was uncle to Ludwig Binswanger who was a major figure in the existential psychology movement...
in 1894, and Alois Alzheimer
Alois Alzheimer
Aloysius "Alois" Alzheimer, was a German psychiatrist and neuropathologist and a colleague of Emil Kraepelin. Alzheimer is credited with identifying the first published case of "presenile dementia", which Kraepelin would later identify as Alzheimer's disease....
first used the phrase "Binswanger's disease" in 1902. However, Olszewski is credited with much of the modern-day investigation of this disease which began in 1962.
History
BinswangerOtto Binswanger
Otto Ludwig Binswanger was a Swiss psychiatrist and neurologist who came from a famous family of physicians; his father was founder of the Kreuzlingen Sanatorium, and he was uncle to Ludwig Binswanger who was a major figure in the existential psychology movement...
in 1894 was the first to claim that white matter atrophy caused by 'vascular insufficiency' can result in dementia
Dementia
Dementia is a serious loss of cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging...
. He described a patient who had slow progression of dementia as well as subcortical white matter atrophy, ventricle
Ventricle
Ventricle may refer to:* Ventricle , the pumping chambers of the heart* Ventricular system in the brain* Ventricle of the larynx, a structure in the larynx* Stomach of the gastrointestinal tract...
enlargement, aphasia
Aphasia
Aphasia is an impairment of language ability. This class of language disorder ranges from having difficulty remembering words to being completely unable to speak, read, or write....
, hemianopsia
Hemianopsia
Hemianopia, or hemianopsia, is a type of anopsia where the decreased vision or blindness takes place in half the visual field of one or both eyes. In most cases, the visual field loss respects the vertical midline...
, and hemiparesis
Hemiparesis
Hemiparesis is weakness on one side of the body. It is less severe than hemiplegia - the total paralysis of the arm, leg, and trunk on one side of the body. Thus, the patient can move the impaired side of his body, but with reduced muscular strength....
. 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
Neurosyphilis is an infection of the brain or spinal cord caused by the bacterium Treponema pallidum. It usually occurs in people who have had untreated syphilis for many years, usually about 10 - 20 years after first infection.-Symptoms and signs:...
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
Multi-infarct dementia
Multi-infarct dementia is one type of vascular dementia. Vascular dementia is the second most common form of dementia after Alzheimer's disease in older adults. Multi-infarct dementia is thought to be an irreversible form of dementia, and its onset is caused by a number of small strokes or...
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 create specific criteria known as the Hachinski's Ischemic Scale which became the standard for diagnosing MID or vascular dementia.
Because of the complicated history of Binswanger’s disease and the fact that it was overlooked as a disease at all for so many years, leads us to believe that many patients have been misdiagnosed as Alzheimer’s for years. This leads us to believe that Binswanger’s is more prominent in the population than once thought.
Neural Presentation
Binswanger's disease is a type of subcortical vascular dementia caused by white matterWhite matter
White matter is one of the two components of the central nervous system and consists mostly of myelinated axons. White matter tissue of the freshly cut brain appears pinkish white to the naked eye because myelin is composed largely of lipid tissue veined with capillaries. Its white color is due to...
atrophy to the brain. However, white matter atrophy alone is not sufficient for this disease; evidence of subcortical dementia is also necessary.
The histologic
Histology
Histology is the study of the microscopic anatomy of cells and tissues of plants and animals. It is performed by examining cells and tissues commonly by sectioning and staining; followed by examination under a light microscope or electron microscope...
findings are diffuse, irregular loss of axons and myelin
Myelin
Myelin is a dielectric material that forms a layer, the myelin sheath, usually around only the axon of a neuron. It is essential for the proper functioning of the nervous system. Myelin is an outgrowth of a type of glial cell. The production of the myelin sheath is called myelination...
accompanied by widespread gliosis
Gliosis
Gliosis is a proliferation of astrocytes in damaged areas of the central nervous system . This proliferation usually leads to the formation of a glial scar....
, tissue death due to an infarction or loss of blood supply to the brain, and changes in the plasticity
Neuroplasticity
Neuroplasticity is a non-specific neuroscience term referring to the ability of the brain and nervous system in all species to change structurally and functionally as a result of input from the environment. Plasticity occurs on a variety of levels, ranging from cellular changes involved in...
of the arteries. The pathologic
Pathology
Pathology is the precise study and diagnosis of disease. The word pathology is from Ancient Greek , pathos, "feeling, suffering"; and , -logia, "the study of". Pathologization, to pathologize, refers to the process of defining a condition or behavior as pathological, e.g. pathological gambling....
mechanism may be damage caused by severe atherosclerosis
Atherosclerosis
Atherosclerosis is a condition in which an artery wall thickens as a result of the accumulation of fatty materials such as cholesterol...
. The onset of this disease is typically between 54 and 66 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 because it changes the tension of the smooth walls vessels and causes changes in the vessel diameter. Arterioles can become permeable resulting in compromise of the blood brain barrier. It has been shown that Binswanger’s disease targets the vessels in this zone of the subcortex, but spares the microvessel and capillaries which may be attributed to a difference between Alzheimer’s and Binswanger’s disease.
Mental 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 functionsExecutive functions
The executive system is a theorized cognitive system in psychology that controls and manages other cognitive processes. It is responsible for processes that are sometimes referred to as the executive function, executive functions, supervisory attentional system, or cognitive control...
, but have normal episodic or declarative memory. Executive functions are brain processes that are responsible for planning, cognitive flexibility, abstract thinking, rule acquisition, initiation 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
Perseveration is the repetition of a particular response, such as a word, phrase, or gesture, despite the absence or cessation of a stimulus, usually caused by brain injury or other organic disorder. If an issue has been fully explored and discussed to a point of resolution, it is not uncommon for...
errors which cause the patients to repeat motion even when not asked whereas Alzheimer patients have semantic preservation because when asked to write a word they will instead draw the object of the word.
Symptoms
Symptoms include mental deterioration, language disorder, transient ischemic attackTransient ischemic attack
A transient ischemic attack is a transient episode of neurologic dysfunction caused by ischemia – either focal brain, spinal cord or retinal – without acute infarction...
, muscle ataxia
Ataxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
, and impaired movements including change of walk, slowness of movements, and change in posture. These symptoms usually coincide with multiple falls, epilepsy
Epilepsy
Epilepsy is a common chronic neurological disorder characterized by seizures. These seizures are transient signs and/or symptoms of abnormal, excessive or hypersynchronous neuronal activity in the brain.About 50 million people worldwide have epilepsy, and nearly two out of every three new cases...
, 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.
Diagnosis
Binswanger's disease can usually be diagnosed through a CT scan, MRI, and a proton MR spectrography. Indications include infarctions, lesions, or loss of intensity of central white matter and enlargement of ventricles, and leukoaraiosisLeukoaraiosis
Leukoaraiosis or periventricular white matter disease is a term for changes in the cerebral white matter that can be detected with high frequency by CT and MRI in aged individuals. It is a descriptive term for rarefaction of the white matter. It is also commonly referred to as white matter...
or white matter atrophy.
Presentation
LeukoaraiosisLeukoaraiosis
Leukoaraiosis or periventricular white matter disease is a term for changes in the cerebral white matter that can be detected with high frequency by CT and MRI in aged individuals. It is a descriptive term for rarefaction of the white matter. It is also commonly referred to as white matter...
(LA) are white matter changes that are common in Binswanger’s Disease. However, LA can be found in many different diseases and even in the general population, especially in people older than 65 years of age.
There is controversy whether LA and mental deterioration actually have a cause and effect relationship. Recent research is showing that different types of LA can affect the brain differently, and that proton MR spectroscopy would be able to distinguish the different types more effectively and better diagnosis and treat the issue. Because of this information, white matter changes indicated by a MRI or CT cannot alone diagnose Binswanger's disease, but can aid to a bigger picture in the diagnosis 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. Binswanger’s disease is best when diagnosed of a team by experts including a neurologist and psychiatrist to rule out other psychological or neurological problems. Because doctors must successfully detect enough white matter alterations to accompany dementia as well as an appropriate level of dementia, two separate technological systems are needed in the diagnosing process.
Technology
Much of the major research today is done on finding better and more efficient ways to diagnose this disease. Many researchers have divided the MRIs 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 MRI. 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 recent 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 alternations.
Recently a Mini Mental Test (MMT) has been created to accurately and quickly assess cognitive impairment due to vascular dementia across different cultures. Binswanger’s disease has been shown to be the most severe impairment of all of the vascular dementia.