© Springer International Publishing Switzerland 2017
Ana Verdelho and Manuel Gonçalves-Pereira (eds.)Neuropsychiatric Symptoms of Cognitive Impairment and DementiaNeuropsychiatric Symptoms of Neurological Disease10.1007/978-3-319-39138-0_1010. Psychosis in Dementia
(1)
Norwegian National Advisory Unit for Ageing and Health, Vestfold Hospital Trust, Tønsberg, Norway
(2)
Faculty of Medicine, University of Oslo, Oslo, Norway
(3)
Center for Old Age Psychiatric Research, Innlandet Hospital Trust, Ottestad, Norway
Abstract
Psychotic symptoms, such as delusions and hallucinations, are rather common at all stages of dementia. The concept of psychosis in dementia (PiD) is elusive, and the symptomatology may overlap with cognitive symptoms, delirium and other neuropsychiatric symptoms such as agitation and depression.
Even though associations between biological markers and the occurrence of PiD exist, no clear causal factor has been identified. Also psychosocial and environmental factors may play a role in increasing the risk of PiD.
Rather few treatment trials have focused specifically on PiD. As for other types of neuropsychiatric symptoms in dementia, non-pharmacological options should be the first-line treatment strategy. The evidence for the effects of psychotropic drugs in the treatment of PiD is weak. The best available evidence is for the short-term use of antipsychotic drugs. However, these drugs are associated with a number of serious adverse events, so the treatment needs to be monitored carefully.
There is reason to believe that PiD defines a more severe phenotype of dementia, in terms of more rapid disease progression and greater dependency. Efforts to prevent or diminish these symptoms may benefit the patients and their caregivers considerably.
Keywords
PsychosisDelusionsHallucinationsDementiaAlzheimerNeuropsychiatric symptomsPsychotropic drug treatmentCase Vignette
Anna (82) lives in a small city in Norway. She was diagnosed with Alzheimer’s disease 4 years ago, and her husband says that the symptoms probably started 3 years before that. After a relatively stable phase covering the first 2 years after receiving the diagnosis, her situation has worsened quickly over the last year. For the last month, she has been disoriented in time and place; she has almost no short-term memory but is still able to recall incidents in her past. Her function in daily living activities is poor and she requires help with eating and grooming. She has become more aggressive and she is emotionally unstable, sometimes crying and other times appearing elated. At times she is hostile towards her husband and she accuses him of having an affair with several women working with the home service. The husband has noticed that she at times seems to see objects that he is not aware of, without her actually saying anything about it. He also tells us that his wife repeatedly talks about visitors in the house, mainly her mother, who has been dead for more than 20 years. He is exhausted and asks their physician and home service to do something about the situation.
Overview and General Definitions
In 1906 Alois Alzheimer described the case of Auguste Deter, who was admitted to the hospital where he was working, in 1901. Based on the neuropathological findings and the clinical picture, the disease was named Alzheimer’s disease in the 8th edition of Kraepelin’s authoritative textbook on psychiatry [1]. Prior to admission, Auguste Deter had over some time developed paranoid delusions in addition to cognitive symptoms. The first few days after admission, Alzheimer described symptoms such as emotional instability, paranoid delusions, screaming and possible hallucinations [2]. The case of Auguste Deter shows that right from the initial recognition of the condition, psychotic symptoms were recognised as symptoms occurring in the context of dementia or, more specifically, in Alzheimer’s disease. Strangely enough, Kraepelin did not mention these symptoms in his psychiatry textbook.
Psychosis in dementia (PiD) remains an elusive concept. Psychotic symptoms often seem to overlap with cognitive symptoms. If a person tells us that yesterday she had a visit from her mother, who has been deceased for many years, is she disoriented in time or is she psychotic? If a person looks out of the window and sees the house where she grew up, even though that place is 100 km away, is she disoriented to place or is she psychotic? If a person wants to fight with his mirror image, is he disoriented to person or is he psychotic?
Furthermore, psychotic symptoms in dementia often co-occur with other neuropsychiatric symptoms, such as agitation or anxiety, which makes it difficult to discern the specific psychotic symptoms.
Usually, we rely on verbal reports when we decide whether a person has hallucinations or delusions. However, with the progression of dementia, language impairments become more prominent, and the identification of psychotic symptoms has to be made from vocal signs or gestures, rather than language. This may cause both underestimation and overestimation of the extent of the psychotic symptoms.
In the late 1990s, the concept of behavioural and psychological symptoms of dementia (BPSD) was heavily promoted [3]. The aim of this effort was to highlight the importance of noncognitive symptoms in dementia, which are associated with a number of negative outcomes for the person with dementia, for their caregivers and for society generally. However, an unfortunate side effect of this initiative has been the tendency to view BPSD as a homogenous symptom group, lumping together aggression, disinhibition, aberrant motor behaviour and psychotic symptoms under terms like challenging behaviour, disruptive behaviour or simply agitation. This may have caused an erroneous view that psychotic symptoms are similar to other BPSD in terms of aetiology or treatment approaches. In a number of research studies, including clinical trials, the outcome has been a sum score of an assessment scale for BPSD (or neuropsychiatric symptoms (NPS)), such as the Neuropsychiatric Inventory (NPI) scale [4] or the Behavioural Pathology in Alzheimer’s Disease (BEHAVE-AD) scale [5], which makes interpretation of the results difficult.
Diagnosis
How to Diagnose PiD According to DSM-V or ICD-10
In ICD-10 [6] a fifth character may be used to specify psychotic symptoms in dementia in Alzheimer’s disease (F00), vascular dementia (F01), dementia in diseases classified elsewhere (F02) or unspecified dementia (F03). This may be x1 for other symptoms, predominantly delusional, and x2 for other symptoms, predominantly hallucinatory. Hence, a person with late-onset Alzheimer’s disease with prominent delusions may receive the diagnosis F00.11. In DSM-V the term dementia, which was used in DSM-IV, has been replaced by the term major neurocognitive disorder [7]. An extra character may be used to distinguish between “with or without behavioural disturbance”.
Diagnostic criteria for psychosis in Alzheimer’s disease have been suggested [8]. They require that hallucinations or delusions are present in a person who meets all the criteria of Alzheimer’s disease and that they have been present for at least 1 month and be of a severity that causes disruption in the patients’ or others’ functioning. The symptoms should not have been present prior to the onset of AD. The authors state that the criteria may also be valid for other types of dementia, provided that the criteria of dementia subtype, e.g. vascular dementia or Lewy body dementia, are adjusted accordingly.
Differential Diagnosis
Psychotic symptoms are highly prevalent in delirium, and delirium is common in dementia. It can be difficult to distinguish psychotic symptoms caused by the delirium from the psychotic symptoms caused by dementia. Diagnosing delirium is, however, of key importance as it is a reversible condition with usually distinct aetiological factors. The abrupt onset usually seen in delirium might be of help in the diagnostic process.
Some authors challenge the view that delusions in persons with dementia should be classified as a psychotic symptom, as they sometimes represent reality and are neither firm nor incontrovertible [9]. In clinical practice, one might find it easier to base a diagnosis of PiD on delusions of persecution than on symptoms which could be understood as cognitive impairment. Furthermore, an observation of a person with dementia currently suffering from hallucinations might be more reliable than a person with dementia’s report of having seen or heard something that cannot be confirmed by others, e.g. having seen a deceased family member in the room or having been talking to a person who has not been present for years.
Clinical Characteristics
The most common psychotic symptoms in dementia are delusions and hallucinations. The Schneiderian first-rank psychotic symptoms commonly encountered in persons with schizophrenia (e.g. thought withdrawal, thought insertion, thought broadcasting, hearing voices talking to each other or commenting on the patient’s action) are very rare in dementia. Delusions in dementia are typically non-bizarre and simple and paranoid in character, like ideas about infidelity, abandonment, poisoning or theft. The content of the delusional thoughts is usually conceivable, which underlines the need for a comprehensive assessment of the symptoms. Sometimes things really are stolen or the food might taste strange in the nursing home. Language disturbances, which are common, particularly in severe dementia, may make it difficult to interpret delusions. Other types of delusions that are rather common in dementia include misidentifications, beliefs about living in another person’s home, thinking that family members have been replaced by identical-looking imposters (Capgras’ syndrome) or duplicated or experiencing people on the television to be present in the room.
In dementia, visual hallucinations are more common than auditory hallucinations. This is opposite to what is normally the case in other psychotic disorders, such as schizophrenia or paranoid psychosis. In dementia with Lewy bodies (DLB), visual hallucinations are particularly prominent, and they are included among the core diagnostic features [10]. In the early stages of DLB, hallucinations are typically well formed and detailed, and the patient often shows some level of insight about the perceptions not representing reality. As the disease progresses, more complex hallucinations, both visual and auditory, may occur. Auditory hallucinations are rarely commenting or commanding voices, but rather sounds, individual words or phrases. Musical hallucinations have been described.
Other types of hallucinations, such as tactile hallucinations or olfactory hallucinations, are not common and may be an indication of the co-occurrence of delirium.
Other forms of psychotic symptoms, such as disorganised speech or motor activity, are rarely reported in the context of PiD. However, one should bear in mind that the scales (inventories) most commonly used to assess psychotic symptoms in dementia (NPI, BEHAVE-AD) focus entirely on hallucinations or delusions. A more in-depth investigation of other types of psychotic symptoms in dementia might be warranted. This may in particular apply to disorganised motor behaviour, which is one of the key clinical domains of psychosis in schizophrenia. Disorganised motor behaviour is highly prevalent in dementia but is often categorised as a type of agitation, distinct from psychosis.
Symptom Course
Early research indicated that hallucinations and delusions would come and go in a rather unpredictable fashion, but this view has since been challenged [11]. Recently, several reports have indicated that delusions and hallucinations are persistent, often lasting from 3 months to more than 1 year. In a large nursing home study, 69 % of the patients who displayed dementia with psychosis at baseline still had psychotic symptoms 1 year later [12].
Psychosis seems to be associated with a more severe phenotype of dementia. The cognitive decline is more rapid, and dependence is higher in dementia with psychosis than in dementia without psychosis. Furthermore, it seems that the higher rate of cognitive decline is apparent also before the onset of psychosis [13]. It should be noted that antipsychotic drugs, which are often used to treat PiD, are also associated with increased cognitive impairment. Other adverse outcomes of PiD are increased burden on family and professional caregivers, earlier institutionalisation, greater functional impairment and increased mortality risk [14].
Epidemiology
With the increasing prevalence of dementia, PiD will soon become the second most prevalent psychotic disorder, second only to schizophrenia [15].
However, prevalence estimates for PiD vary widely. A comprehensive review reported prevalence rates for delusions from 9 to 63 % (median 36 %) and for hallucinations from 4 to 41 % (median 18 %) [16]. This probably reflects different sample characteristics and different cutoffs for defining the condition. Misidentification symptoms, which are quite common in dementia, are in some studies classified as delusions, but not in others. Psychotic symptoms are more frequent among inpatients (e.g. in nursing homes) than in outpatient settings (e.g. memory clinic samples). Population-based studies have reported prevalence rates of around 22 % for delusions and 13 % for hallucinations [17]. Five-year period prevalence rates were around 60 % for delusions and nearly 40 % for hallucinations [18]. Even higher prevalence rates are found among the oldest group of the elderly (85+) [19].
In general, prevalence rates based on clinical criteria seem to give lower figures than rates based on assessment scales.
Psychosis and Dementia Severity
Whereas psychosis can be the debut symptom of dementia, the prevalence is normally higher in the later stages of dementia. Cross-sectional studies have found a robust relationship between psychosis and dementia severity [16]. However, findings from longitudinal studies are mixed [20, 21]. They indicate that psychotic symptoms are most common in the middle phase of dementia. In severe dementia the prevalence of dementia tends to decrease, but this may be caused by diminished ability to verbalise the symptoms by the patient.
Aetiology
Biological Factors
Most of the knowledge on the genetic component of dementia comes from research on Alzheimer’s disease and psychosis (AD + P). Three different cohort studies show that psychosis in Alzheimer’s disease aggregates in families. The heritability is estimated to be between 30 and 61 %. A stricter definition of AD + P, requiring multiple symptoms and persistence, strengthens the familial aggregation. A genome-wide association study has produced some indication that genes that have been linked with schizophrenia also may be implicated in psychosis in Alzheimer’s disease. In linkage studies, specific loci on several chromosomes, such as chromosomes 2, 7, 8 and 15, have been linked to AD + P. ApoE status, which is strongly associated with increased risk of AD, does not seem to be associated with an increased risk of AD + P [22].