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Dementia praecox (a "premature dementia" or "precocious madness") is a disused psychiatric diagnosis that originally designated a chronic, deteriorating psychotic disorder characterized by rapid cognitive disintegration, usually beginning in the late teens or early adulthood. Over the years, the term "dementia praecox" was gradually replaced by "schizophrenia", which remains in current diagnostic use.
The term "dementia praecox" was first used in 1891 by Arnold Pick (1851–1924), a professor of psychiatry at Charles University in Prague. His brief clinical report described the case of a person with a psychotic disorder resembling hebephrenia. German psychiatrist Emil Kraepelin (1856–1926) popularised it in his first detailed textbook descriptions of a condition that eventually became a different disease concept and relabeled as schizophrenia. Kraepelin reduced the complex psychiatric taxonomies of the nineteenth century by dividing them into two classes: manic-depressive psychosis and dementia praecox. This division, commonly referred to as the Kraepelinian dichotomy, had a fundamental impact on twentieth-century psychiatry, though it has also been questioned.
The primary disturbance in dementia praecox was seen to be a disruption in cognitive or mental functioning in attention, memory, and goal-directed behaviour. Kraepelin contrasted this with manic-depressive psychosis, now termed bipolar disorder, and also with other forms of mood disorder, including major depressive disorder. He eventually concluded that it was not possible to distinguish his categories on the basis of cross-sectional symptoms.
Kraepelin viewed dementia praecox as a progressively deteriorating disease from which no one recovered. However, by 1913, and more explicitly by 1920, Kraepelin admitted that while there may be a residual cognitive defect in most cases, the prognosis was not as uniformly dire as he had stated in the 1890s. Still, he regarded it as a specific disease concept that implied incurable, inexplicable madness.
The symptoms of dementia vary across types and stages of the diagnosis. The most common affected areas include memory, visual-spatial, language, attention and problem solving. Most types of dementia are slow and progressive. By the time the person shows signs of the disorder, the process in the brain has been happening for a long time. It is possible for a patient to have two types of dementia at the same time. About 10% of people with dementia have what is known as "mixed dementia", which is usually a combination of Alzheimer's disease and another type of dementia such as frontotemporal dementia or vascular dementia. Additional psychological and behavioral problems that often affect people who have dementia include:
- Balance problems
- Tremor
- Speech and language difficulty
- Trouble eating or swallowing
- Memory distortions (believing that a memory has already happened when it has not, thinking an old memory is a new one, combining two memories, or confusing the people in a memory)
- Wandering or restlessness
- Perception and visual problems
- "Behavioral and psychological symptoms of dementia" (BPSD) almost always occur in all types of dementia. BPSDs may manifest as:
When people with dementia are put in circumstances beyond their abilities, there may be a sudden change to crying or anger (a ""catastrophic reaction"").
Psychosis (often delusions of persecution) and agitation/aggression also often accompany dementia.
Individuals with schizophrenia may experience hallucinations (most reported are hearing voices), delusions (often bizarre or persecutory in nature), and disorganized thinking and speech. The last may range from loss of train of thought, to sentences only loosely connected in meaning, to speech that is not understandable known as word salad. Social withdrawal, sloppiness of dress and hygiene, and loss of motivation and judgment are all common in schizophrenia.
Distortions of self-experience such as feeling as if one's thoughts or feelings are not really one's own to believing thoughts are being inserted into one's mind, sometimes termed passivity phenomena, are also common. There is often an observable pattern of emotional difficulty, for example lack of responsiveness. Impairment in social cognition is associated with schizophrenia, as are symptoms of paranoia. Social isolation commonly occurs. Difficulties in working and long-term memory, attention, executive functioning, and speed of processing also commonly occur. In one uncommon subtype, the person may be largely mute, remain motionless in bizarre postures, or exhibit purposeless agitation, all signs of catatonia. People with schizophrenia often find facial emotion perception to be difficult. It is unclear if the phenomenon called "thought blocking", where a talking person suddenly becomes silent for a few seconds to minutes, occurs in schizophrenia.
About 30 to 50 percent of people with schizophrenia fail to accept that they have an illness or comply with their recommended treatment. Treatment may have some effect on insight.
People with schizophrenia may have a high rate of irritable bowel syndrome but they often do not mention it unless specifically asked. Psychogenic polydipsia, or excessive fluid intake in the absence of physiological reasons to drink, is relatively common in people with schizophrenia.
Schizophrenia is often described in terms of positive and negative (or deficit) symptoms. "Positive symptoms" are those that most individuals do not normally experience, but are present in people with schizophrenia. They can include delusions, disordered thoughts and speech, and tactile, auditory, visual, olfactory and gustatory hallucinations, typically regarded as manifestations of psychosis. Hallucinations are also typically related to the content of the delusional theme. Positive symptoms generally respond well to medication.
"Negative symptoms" are deficits of normal emotional responses or of other thought processes, and are less responsive to medication. They commonly include flat expressions or little emotion, poverty of speech, inability to experience pleasure, lack of desire to form relationships, and lack of motivation. Negative symptoms appear to contribute more to poor quality of life, functional ability, and the burden on others than positive symptoms do. People with greater negative symptoms often have a history of poor adjustment before the onset of illness, and response to medication is often limited.
The validity of the positive and negative construct has been challenged by factor analysis studies observing a three dimension grouping of symptoms. While different terminology is used, a dimension for hallucinations, a dimension for disorganization, and a dimension for negative symptoms are usually described.
In the first stages of dementia, the signs and symptoms of the disorder may be subtle. Often, the early signs of dementia only become apparent when looking back in time. The earliest stage of dementia is called mild cognitive impairment (MCI). 70% of those diagnosed with MCI progress to dementia at some point. In MCI, changes in the person's brain have been happening for a long time, but the symptoms of the disorder are just beginning to show. These problems, however, are not yet severe enough to affect the person’s daily function. If they do, it is considered dementia. A person with MCI scores between 27 and 30 on the Mini-Mental State Examination (MMSE), which is a normal score. They may have some memory trouble and trouble finding words, but they solve everyday problems and handle their own life affairs well.
Simple-type schizophrenia is a sub-type of schizophrenia as defined in the International Classification of Diseases . It is not included in the current "Diagnostic and Statistical Manual of Mental Disorders" (DSM-5). Simple-type schizophrenia is characterized by negative ("deficit") symptoms, such as avolition, apathy, anhedonia, reduced affect display, lack of initiative, lack of motivation, low activity; with absence of hallucinations or delusions of any kind.
It has possibly the earliest onset compared to all other schizophrenias, considered to begin in some within childhood. Symptoms of "schizophrenia" "simplex" include an absence of will, impoverished thinking and flattening of affect. There is a gradual deterioration of functioning with increased amotivation and reduced socialization. It is considered to be rarely diagnosed and is a schizophrenia without psychotic symptoms.
In a study of patients in a Massachusetts hospital, persons suffering with "simple schizophrenia" were found to make attempts at reality fulfillment with respect to the more primitive needs; tending toward the achievement of fulfillment of these needs rather than engaging in fantasy as is typically found as a reaction to environmental stimuli by the psychotic person.
Schizophrenia is a mental disorder that is expressed in abnormal mental functions and disturbed behavior.
The signs and symptoms of childhood schizophrenia are nearly the same as adult-onset schizophrenia. Some of the earliest signs that a young child may develop schizophrenia are lags in language and motor development. Some children engage in activities such as flapping the arms or rocking, and may appear anxious, confused, or disruptive on a regular basis. Children may experience symptoms such as hallucinations, but these are often difficult to differentiate from just normal imagination or child play. It is often difficult for children to describe their hallucinations or delusions, making very early-onset schizophrenia especially difficult to diagnose in the earliest stages. The cognitive abilities of children with schizophrenia may also often be lacking, with 20% of patients showing borderline or full intellectual disability.
Very early-onset schizophrenia refers to onset before the age of thirteen. The prodromal phase, which precedes psychotic symptoms, is characterized by deterioration in school performance, social withdrawal, disorganized or unusual behavior, a decreased ability to perform daily activities, a deterioration in self-care skills, bizarre hygiene and eating behaviors, changes in affect, a lack of impulse control, hostility and aggression, and lethargy.
Auditory hallucinations are the most common "positive symptom" in children. Positive symptoms have come to mean psychopathological disorders that are actively expressed, such as delusions, hallucinations, thought disorder etc.). A child's auditory hallucinations may include voices that are conversing with each other or voices that are speaking directly to the children themselves. Many children with auditory hallucinations believe that if they do not listen to the voices, the voices will harm them or someone else. Tactile and visual hallucinations seem relatively rare. The children often attribute the hallucinatory voices to a variety of beings, including family members or other people, evil forces ("the Devil", "a witch", "a spirit"), animals, characters from horror movies (Bloody Mary, Freddy Krueger) and less clearly recognizable sources ("bad things," "the whispers"). Command auditory hallucinations (also known as imperative hallucinations) were common and experienced by more than ½ of the group in a research at the Bellevue Hospital Center's Children's Psychiatric Inpatient Unit. And voices repeat and repeat: "Kill somebody!", "Kill her, kill her!". Delusions are reported in more than half of children with schizophrenia, but they are usually less complex than those of adults. Delusions often connected with hallucinatory experiences.. In a research delusions were characterized as persecutory for the most part, but some children reported delusions of control. Many said they were being tortured by the beings causing their visual and auditory hallucinations, some thought that if they disobeying their voices would cause them harm.
Some degree of thought disorder was observed in a test group of children in Bellevue Hospital. They displayed illogicality, tangentialiry (a serious disturbance in the associative thought process), and loosening of associations.
Negative ("deficit") symptoms in schizophrenia reflect mental deficit states such as apathy and aboulia, avolition, flattened affect, asthenia etc.
The history of disturbance in pseudodementia is often short and abrupt onset, while dementia is more often insidious. Clinically, people with pseudodementia differ from those with true dementia when their memory is tested. They will often answer that they don't know the answer to a question, and their attention and concentration are often intact, and they may appear upset or distressed. Those with true dementia will often give wrong answers, have poor attention and concentration, and appear indifferent or unconcerned.
Investigations such as SPECT imaging of the brain show reduced blood flow in areas of the brain in people with Alzheimer's disease, compared with a more normal blood flow in those with pseudodementia.
Mild and major neurocognitive disorders are usually associated with but not restricted to the elderly. Unlike delirium, conditions under these disorders develop slowly and are characterized by memory loss. In addition to memory loss and cognitive impairment, other symptoms include aphasia, apraxia, agnosia, loss of abstract thought, behavioral/personality changes, and impaired judgment. There may also be behavioral disturbances including psychosis, mood, and agitation.
Mild and major neurocognitive disorders are differentiated based on the severity of their symptoms. Previously known as dementia, major neurocognitive disorder is characterized by significant cognitive decline and interference with independence, while mild neurocognitive disorder is characterized by moderate cognitive decline and does not interfere with independence. To be diagnosed, it must not be due to delirium or other mental disorder. They are also usually accompanied by another cognitive dysfunction. For non-reversible causes of dementia such as age, the slow decline of memory and cognition is lifelong. It can be diagnosed by screening tests such as the Mini Mental State Examination (MMSE).
Pseudodementia is a phenotype approximated by a wide variety of underlying disorders (1). Data indicate that some of the disorders that can convert to a pseudodementia-like presentation include depression (mood), schizophrenia, mania, dissociative disorders, Ganser syndrome, conversion reaction, and psychoactive drugs (2). Although the frequency distribution of disorders presenting as pseudodementia remains unclear, what is clear is that depressive pseudodementia, synonymously referred to as depressive dementia(3) or major depression with depressive dementia (4), represents a major subclass of the overarching category of pseudodementia (4).
It has long been observed that in the differential diagnosis between dementia and pseudodementia, depressive pseudodementia appears to be the single most difficult disorder to distinguish from nosologically established "organic" categories of dementia(5), especially degenerative dementia of the Alzheimer type (6).
Depressive Pseudodementia is a syndrome seen in older people in which they exhibit symptoms consistent with dementia but the cause is actually depression.
Older people with predominant cognitive symptoms such as loss of memory, and vagueness, as well as prominent slowing of movement and reduced or slowed speech, were sometimes misdiagnosed as having dementia when further investigation showed they were suffering from a major depressive episode. This was an important distinction as the former was untreatable and progressive and the latter treatable with antidepressant therapy or electroconvulsive therapy or both. In contrast to major depression, dementia is a progressive neurodegenerative syndrome involving a pervasive impairment of higher cortical functions resulting from widespread brain pathology.
Childhood schizophrenia was not directly added to the DSM until 1968, when it was added to the DSM-II, which set forth diagnostic criteria similar to that of adult schizophrenia. "Schizophrenia, childhood type" was a DSM-II diagnosis with diagnostic code 295.8. It's equivalent to "schizophrenic reaction, childhood type" (code 000-x28) in DSM-I (1952). "Schizophrenia, childhood type" was successfully removed from the DSM-III (1980), and in the Appendix C they wrote: "there is currently no way of predicting which children will develop Schizophrenia as adults". Instead of childhood schizophrenia they proposed to use of "infantile autism" (299.0x) and "childhood onset pervasive developmental disorder" (299.9x).
In the DSM-III-R (1987), DSM-IV (1994), DSM-IV-TR (2000), DSM-5 (2013) there are no "childhood schizophrenia". The rationale for this approach was that since the clinical picture of adult schizophrenia and childhood schizophrenia is identical, childhood schizophrenia should not be a separate disorder.
In schizophrenia's "development and course" in the DSM-5 they wrote:
The diagnosis of schizophrenia was often given to children who by today’s standards would be diagnosed as having of autism or pervasive developmental disorder. This may be because the onset of schizophrenia is gradual, with symptoms relating developmental difficulties or abnormalities appearing before psychotic symptoms.
The presenting symptom of dementia with Lewy bodies is often cognitive dysfunction, though dementia eventually occurs in all individuals with DLB. In contrast to Alzheimer's disease (AD), in which memory loss is the first symptom, those with DLB first experience impaired attention, executive function, and visuospatial function, while memory is affected later. These impairments present as driving difficulty, such as becoming lost, misjudging distances, or as impaired job performance. In terms of cognitive testing, individuals may have problems with figure copying as a result of visuospatial impairment, with clock-drawing due to executive function impairment, and difficulty with serial sevens as a result of impaired attention. Short-term memory and orientation to time and place remain intact in the earlier stages of the disease.
While the specific symptoms in a person with DLB may vary, core features include: fluctuating cognition with great variations in attention and alertness from day to day and hour to hour, recurrent visual hallucinations (observed in 75% of people with DLB), and motor features of Parkinson's disease. Suggestive symptoms are rapid eye movement (REM)-sleep behavior disorder and abnormalities detected in PET or SPECT scans. REM sleep behavior disorder (RBD) often is a symptom first recognized by the patient's caretaker. RBD includes vivid dreaming, with persistent dreams, purposeful or violent movements, and falling out of bed. Benzodiazepines, anticholinergics, surgical anesthetics, some antidepressants, and over-the-counter drug cold remedies may cause acute confusion, delusions, and hallucinations.
Tremors are less common in DLB than in Parkinson's disease. Parkinsonian features may include shuffling gait, reduced arm-swing during walking, blank expression (reduced range of facial expression), stiffness of movements, ratchet-like cogwheeling movements, low speech volume, sialorrhea, and difficulty swallowing. Also, DLB patients often experience problems with orthostatic hypotension, including repeated falls, fainting, and transient loss of consciousness. Sleep-disordered breathing, a problem in multiple system atrophy, also may be a problem.
One of the most critical and distinctive clinical features of the disease is hypersensitivity to neuroleptic and antiemetic medications that affect dopaminergic and cholinergic systems. In the worst cases, a patient treated with these medications could become catatonic, lose cognitive function, or develop life-threatening muscle rigidity. Some commonly used medications that should be used with great caution, if at all, for people with DLB, are chlorpromazine, haloperidol, or thioridazine.
Visual hallucinations in people with DLB most commonly involve perception of people or animals that are not there, and may reflect Lewy bodies or AD pathology in the temporal lobe. Delusions may include reduplicative paramnesia and other elaborate misperceptions or misinterpretations. These hallucinations are not necessarily disturbing, and in some cases, the person with DLB may have insight into the hallucinations and even be amused by them, or be conscious they are not real. People with DLB also may have problems with vision, including double vision, and misinterpretation of what they see, for example, mistaking a pile of socks for snakes or a clothes closet for the bathroom.
Paranoid schizophrenia manifests itself in an array of symptoms. Common symptoms for paranoid schizophrenia include auditory hallucinations (hearing voices) and paranoid delusions (believing everyone is out to cause the sufferer harm). However, two of the symptoms separate this form of schizophrenia from other forms.
One criterion for separating paranoid schizophrenia from other types is delusion. A delusion is a belief that is held strongly even when the evidence shows otherwise. Some common delusions associated with paranoid schizophrenia include, “believing that the government is monitoring every move you make, or that a co-worker is poisoning your lunch." In all but rare cases, these beliefs are irrational, and can cause the person holding them to behave abnormally. Another frequent type of delusion is a delusion of grandeur, or the “fixed, false belief that one possesses superior qualities such as genius, fame, omnipotence, or wealth." Common ones include, “the belief that you can fly, that you're famous, or that you have a relationship with a famous person."
Another criterion present in patients with paranoid schizophrenia is auditory hallucinations, in which the person hears voices or sounds that are not really present. The patient will sometimes hear multiple voices and the voices can either be talking to the patient or to one another. These voices can influence the patient to behave in a particular manner. Researchers at the Mayo Foundation for Medical Education and Research provide the following description: “They [the voices] may make ongoing criticisms of what you’re thinking or doing, or make cruel comments about your real or imagined faults. Voices may also command you to do things that can be harmful to yourself or to others." A patient exhibiting these auditory hallucinations may be observed "talking to them" because the person believes that the voices represent people who are present.
Early diagnosis is critical for the successful treatment of schizophrenia.
The DSM-IV criteria (note there may be new criteria for the condition under the DSM-5) for the diagnosis of schizophrenia require the presence of symptoms for certain periods of time. A person must exhibit two or more core symptoms for a minimum of one month, such as delusions, hallucinations, disorganized speech, grossly disorganized or catatonic behavior, or negative symptoms. There also must be significant impairment for the person at work, with academic performance, interpersonal relationships, and/or the ability to take care of oneself. These symptoms must continue for a minimum of six months, with the first symptoms continuing for at least one month. Paranoid schizophrenia is differentiated by the presence of hallucinations and delusions involving the perception of persecution or grandiosity in one's beliefs about the world.
People with paranoid schizophrenia are often more articulate or "normal" seeming than other people with schizophrenia, such as disorganized schizophrenia (hebephrenia)-afflicted individuals. The diagnosis of paranoid schizophrenia is given based on the presence of bizarre delusions or hallucinations that defy the natural laws of basic logical thought processes, or thought disorders and withdrawal due to these thoughts and delusions. The paranoid subset of schizophrenia tends to have a better prognosis than other subtypes (disorganized and simple–type in particular), as the intellect and personality are relatively preserved, thus enabling a greater degree of cognitive and interpersonal functioning.
With the removal of the subtypes of schizophrenia in DSM-5, paranoid schizophrenia will no longer be used as a diagnostic category. If a person is exhibiting symptoms of schizophrenia, including symptoms of paranoid type, they will simply be diagnosed with schizophrenia and will be treated with antipsychotics based on their individual symptoms.
Delirium can be caused by the worsening of previous medical conditions, substance abuse or withdrawal, mental illness, severe pain, immobilization, sleep deprivation and hypnosis.
Other common causes that may increase the risk of delirium include infections of urinary tract, skin and stomach, pneumonia, old age, and poor nutrition.
The main symptoms of paraphrenia are paranoid delusions and hallucinations. The delusions often involve the individual being the subject of persecution, although they can also be erotic, hypochondriacal, or grandiose in nature. The majority of hallucinations associated with paraphrenia are auditory, with 75% of patients reporting such an experience; however, visual, tactile, and olfactory hallucinations have also been reported. The paranoia and hallucinations can combine in the form of “threatening or accusatory voices coming from neighbouring houses [and] are frequently reported by the patients as disturbing and undeserved". Patients also present with a lack of symptoms commonly found in other mental disorders similar to paraphrenia. There is no significant deterioration of intellect, personality, or habits and patients often remain clean and mostly self-sufficient. Patients also remain oriented well in time and space.
Paraphrenia is different from schizophrenia because, while both disorders result in delusions and hallucinations, individuals with schizophrenia exhibit changes and deterioration of personality whereas individuals with paraphrenia maintain a well-preserved personality and affective response.
Pseudosenility also reversible dementia is a condition where older people are in a state of memory loss, confusion, or disorientation that may have a cause other than the ordinary aging process. Generally, the term "reversible dementia" is used to describe most cases. A more specific term "Pseudodementia" is referring to "behavioral changes that resembler those of the progressive degenerative dementias, but which are attributable to so-called functional causes".
The "New York Times" reports that illnesses such as the flu and hydrocephalus, as well as side-effects to common medications, can produce symptoms in the elderly that are difficult to distinguish from ordinary dementia caused by aging. However, if the real cause of the effects is caught early enough, the effects can be reversed. According to studies cited in Cunha (1990), approximate 10% to 30% of patients who have exhibited symptoms of dementia might have a treatable or reversible pathologic process to some extent.
Paraphrenia (from – beside, near + φρήν – intellect, mind) is a mental disorder characterized by an organized system of paranoid delusions with or without hallucinations (the positive symptoms of schizophrenia) without deterioration of intellect or personality (its negative symptom).
This disorder is also distinguished from schizophrenia by a lower hereditary occurrence, less premorbid maladjustment, and a slower rate of progression. Onset of symptoms generally occurs later in life, near the age of 60. The prevalence of the disorder among the elderly is between 0.1 and 4%
Paraphrenia is not included in the DSM-5; psychiatrists often diagnose patients presenting with paraphrenia as having atypical psychoses, delusional disorder, psychoses not otherwise specified, schizoaffective disorders, and persistent persecutory states of older adults. Recently, mental health professionals have also been classifying paraphrenia as very late-onset schizophrenia-like psychosis.
In the Russian psychiatric manuals paraphrenia (or paraphrenic syndrome) is the last stage of development of paranoid schizophrenia. "Systematized paraphrenia" (with systematized delusions i. e. delusions with complex logical structure) and "expansive-paranoid paraphrenia" (with expansive/grandiose delusions and persecutory delusions) are the variants of paranoid schizophrenia (). You see sometimes "systematized paraphrenia" with delusional disorder ().
Alcohol-related dementia presents as a global deterioration in intellectual function with memory not being specifically affected, but it may occur with other forms of dementia, resulting in a wide range of symptoms. Certain individuals with alcohol-related dementia present with damage to the frontal lobes of their brain causing disinhibition, loss of planning and executive functions, and a disregard for the consequences of their behavior. Other types of alcohol-related dementia such as Korsakoff's Syndrome cause the destruction of certain areas of the brain, where changes in memory, primarily a loss of short term memory, are the main symptom. Most presentations of alcohol dementia are somewhere along the spectrum between a global dementia and Korsakoff's psychosis, and may include symptoms of both.
Individuals affected by alcohol-related dementia may develop memory problems, language impairment, and an inability to perform complex motor tasks such as getting dressed. Heavy alcohol abuse also damages the nerves in arms and legs, i.e. peripheral neuropathy, as well as the cerebellum that controls coordination thereby leading to the development of cerebellar ataxia. These patients frequently have problems with sensation in their extremities and may demonstrate unsteadiness on their feet.
Alcohol-related dementia can produce a variety of psychiatric problems including psychosis (disconnection from reality), depression, anxiety, and personality changes. Patients with alcoholic dementia often develop apathy, related to frontal lobe damage, that may mimic depression. People with alcoholism are more likely to become depressed than people without alcoholism, and it may be difficult to differentiate between depression and alcohol dementia.
Agitation often accompanies dementia and often precedes the diagnosis of common age-related disorders of cognition such as Alzheimer's disease (AD). More than 80% of people who develop AD eventually become agitated or aggressive.
Dementia with Lewy bodies (DLB) is a type of dementia that worsens over time. Additional symptoms may include fluctuations in alertness, visual hallucinations, slowness of movement, trouble walking, and rigidity. Excessive movement during sleep and mood changes such as depression are also common.
The cause is unknown. Typically, no family history of the disease exists among those affected. The underlying mechanism involves the buildup of Lewy bodies, clumps of alpha-synuclein protein in neurons. It is classified as a neurodegenerative disorder. A diagnosis may be suspected based on symptoms, with blood tests and medical imaging done to rule out other possible causes. The differential diagnosis includes Parkinson's and Alzheimer's.
At present there is no cure. Treatments are supportive and attempt to relieve some of the motor and psychological symptoms associated with the disease. Acetylcholinesterase inhibitors, such as donepezil, may provide some benefit. Some motor problems may improve with levodopa. Antipsychotics, even for hallucinations, should generally be avoided due to side effects.
DLB is the most common cause of dementia after Alzheimer's disease and vascular dementia. It typically begins after the age of 50. About 0.1% of those over 65 are affected. Men appear to be more commonly affected than women. In the late part of the disease, people may depend entirely on others for their care. Life expectancy following diagnosis is about eight years. The abnormal deposits that cause the disease were discovered in 1912 by Frederic Lewy.
Differentiating the different dementia syndromes can be challenging, due to the frequently overlapping clinical features and related underlying pathology. In particular, Alzheimer's dementia often co-occurs with vascular dementia.
People with vascular dementia present with progressive cognitive impairment, acutely or subacutely as in mild cognitive impairment, frequently step-wise, after multiple cerebrovascular events (strokes). Some people may appear to improve between events and decline after more silent strokes. A rapidly deteriorating condition may lead to death from a stroke, heart disease, or infection.
Signs and symptoms are cognitive, motor, behavioral, and for a significant proportion of patients also affective. These changes typically occur over a period of 5–10 years. Signs are typically the same as in other dementias, but mainly include cognitive decline and memory impairment of sufficient severity as to interfere with activities of daily living, sometimes with presence of focal neurologic signs, and evidence of features consistent with cerebrovascular disease on brain imaging (CT or MRI). The neurologic signs localizing to certain areas of the brain that can be observed are hemiparesis, bradykinesia, hyperreflexia, extensor plantar reflexes, ataxia, pseudobulbar palsy, as well as gait and swallowing difficulties. People have patchy deficits in terms of cognitive testing. They tend to have better free recall and fewer recall intrusions when compared with patients with Alzheimer's disease. In the more severely affected patients, or patients affected by infarcts in Wernicke's or Broca's areas, specific problems with speaking called dysarthrias and aphasias may be present.
In small vessel disease, the frontal lobes are often affected. Consequently, patients with vascular dementia tend to perform worse than their Alzheimer's disease counterparts in frontal lobe tasks, such as verbal fluency, and may present with frontal lobe problems: apathy, abulia, problems with attention, orientation, and urinary incontinence. They tend to exhibit more perseverative behavior. VaD patients may also present with general slowing of processing ability, difficulty shifting sets, and impairment in abstract thinking. Apathy early in the disease is more suggestive of vascular dementia.
Rare genetic disorders which result in vascular lesions in the brain have other patterns of presentation. As a rule, they tend to present earlier in life and have a more aggressive course. In addition, infectious disorders, such as syphilis, can lead to arterial damage, strokes, and bacterial inflammation of the brain.
It is important to rule out infection and other environmental causes of agitation, such as disease or other bodily discomfort, before initiating any intervention. If no such explanation is found, it is important to support caregivers and educate them about simple strategies such as distraction that may delay the transfer to institutional care (which is often triggered by the onset of agitation).
FTD is traditionally difficult to diagnose due to the heterogeneity of the associated symptoms. Signs and symptoms are classified into three groups based on the functions of the frontal and temporal lobes:
- Behavioural variant frontotemporal dementia (BvFTD) is characterized by changes in social behavior and conduct, with loss of social awareness and poor impulse control.
- Semantic dementia (SD) is characterized by the loss of semantic understanding, resulting in impaired word comprehension, although speech remains fluent and grammatically faultless.
- Progressive nonfluent aphasia (PNFA) is characterized by progressive difficulties in speech production.
However, the following abilities in the person with FTD are preserved:
- Perception
- Spatial Skills
- Memory
- Praxis
In later stages of FTD, the clinical phenotypes may overlap. FTD patients tend to struggle with binge eating and compulsive behaviors. These binge eating habits are often associated with abnormal eating behavior including overeating, stuffing oneself with food, changes in food preferences (cravings for more sweets, carbohydrates), eating inedible objects and snatching food from others. Recent findings from structural MRI research have indicated that eating changes in FTD are associated with atrophy (wasting) in the right ventral insula, striatum, and orbitofrontal cortex.
Patients with FTD show marked deficiencies in executive functioning and working memory. Most FTD patients become unable to perform skills that require complex planning or sequencing. In addition to the characteristic cognitive dysfunction, a number of primitive reflexes known as frontal release signs are often able to be elicited. Usually the first of these frontal release signs to appear is the palmomental reflex which appears relatively early in the disease course whereas the palmar grasp reflex and rooting reflex appear late in the disease course.
In rare cases, FTD can occur in patients with motor neuron disease (MND) (typically amyotrophic lateral sclerosis). The prognosis for people with MND is worse when combined with FTD, shortening survival by about a year.