'I am dead': Cotard syndrome and dementia.

Background: Cotard syndrome is a rare condition whose main feature is a nihilistic delusion ranging from the denial of body parts to that of the existence of one's own life or even the entire universe. Objectives: The aim of this article is to review the nosological significance of Cotard syndrome and to explore the disorder among patients with dementia. Methods: Medline and Google Scholar searches were conducted for relevant articles, chapters and books published before 2018. Search terms used included Cotard delusion, Cotard syndrome and dementia, nihilistic delusion. Publications found through this indexed search were reviewed for further relevant references. Results and conclusion: In this narrative review we emphasise the fact that cases of Cotard syndrome involving patients with dementia are reported quite infrequently. Published studies are limited to very short series or isolated cases. Clinicians are obliged to treat the disorder. They should be alert to the potential high risk inherent in this condition. Keypoints Cotard syndrome is a rare condition characterised by nihilistic delusions that may range from negation of existence of parts of the body to delusion of being dead. The prevalence and incidence of this rare syndrome are not known. Since Cotard's syndrome is conceptualised as part of an underlying disorder, several psychiatric and somatic diseases have been associated with the syndrome. The syndrome may occur in patients suffering from dementia.

Sexual disinhibition and dementia.

To describe inappropriate sexual behaviour (ISB) observed in patients with dementia, we conducted searches using the Cochrane Library, PubMed, and Web of Science to find relevant articles, chapters, and books published from 1950 to 2014. Search terms used included 'hypersexuality', 'inappropriate sexual behaviors', and 'dementia'. Publications found through this indexed search were reviewed for further relevant references. Sexuality is a human's need to express intimacy, but persons with dementia may not know how to appropriately meet their needs for closeness and intimacy due to their decline in cognition. Generally, the interaction among brain, physical, psychological, and environmental factors can create what we call ISB. The most likely change in the sexual behaviour of a person with dementia is indifference. However, ISB in dementia appear to be of two types--intimacy-seeking and disinhibited--that differ in their association with dementia type, dementia severity and, possibly, other concurrent behavioural disorder. Tensions develop from uncertainties regarding which, or when, behaviours are to be considered 'inappropriate' (i.e. improper) or abnormal. While most ISB occur in the moderate to severe stages of Alzheimer's dementia, they may also be seen in early stages of frontotemporal dementia because of the lack of insight and disinhibition. ISB are often better managed by non-pharmacological means, as patients may be less responsive to psychoactive therapies, but non-pharmacological interventions do not always stop the behaviour.

Clinical features associated with ictal osmophobia in migraine.

Olfactory hypersensitivity may occur during migraine attacks and has been found to be very specific for this form of headache. Aim of this study was to investigate if migraineurs with ictal osmophobia have particular clinical features comparing to patients without ictal osmophobia. We recruited 200 consecutive migraineurs. Other primary headaches comorbidity and migraine prophylaxis were exclusion criteria. Each patient was interviewed following a structured questionnaire including general features about migraine, depression and anxiety symptoms. Migraine triggers both spontaneously and selecting from a specific list. Allodynia during the migraine attack was measured using the Allodynia symptoms check-list 12 (ASC-12). Eighty four (42 %) patients are non-osmophobic vs. 116 patients (58 %) who are osmophobic. After a logistic regression analysis, pain intensity (OR 1.391; p = 0.008) and anxiety (OR 1.099; p = 0.047) were significantly higher while aura (OR 0.421; p = 0.028) is less frequent in osmophobic migraineurs. We found significant differences in clinical features of osmophobic patients in respect to non-osmophobic ones. Ictal osmophobia seems being related to a broader sensorial hypersensitivity that could lead to a more florid clinical presentation.

Sleep disturbances and dementia.

Sleep is a complex behavioural state, the ultimate functions of which remain poorly understood. It becomes more fragmented as we age, with more night-time awakenings and greater tendency for daytime sleep. The magnitude of disordered sleep among individuals affected by dementia has been clearly demonstrated, and disturbed sleep is a major clinical problem in dementia. Comorbid insomnia and other sleep disturbances are common in patients with neurodegenerative disorders, such Alzheimer's disease and other dementing disorders. How and when sleep problems manifest themselves can depend on the type of dementia involved as well as the stage of the dementia. However, differences in sleep pattern presentation show more variation during the initial stages of dementias than they do during the later stages. Effective, pragmatic interventions are largely anecdotal and untested.

Apathy and dementia. Nosology, assessment and management.

Apathy, characterized by lack of motivation and loss of initiative, is a non-cognitive symptom that affects a high proportion, but not all, of patients with all forms of dementia. To explore the phenomenon of apathy in people with dementia, we searched the PubMed and Google Scholar electronic databases for original research and review articles on apathetic behaviors in patients with dementia using the search terms "apathy, behavioral and psychological symptoms, dementia, Alzheimer's disease, Frontotemporal dementia, Dementia associated with Parkinson's disease, Huntington's disease, Vascular dementia". Some nosological aspects, neurobiological basis, and assessment of, as well as, potential benefits of non-pharmacologic and pharmacologic interventions for apathy in dementia are discussed. Greater understanding of apathy will improve the identification, intervention, and treatment of this ubiquitous and pernicious syndrome.

Wandering and dementia.

Wandering represents one of many behavioural problems occurring in people with dementia. To consider the phenomenon of wandering behaviour in demented patients, we conducted searches using Medline and Google Scholar to find relevant articles, chapters, and books published since 1975. Search terms used included 'wandering', 'behavioural and psychological symptoms', 'dementia', 'nursing', and 'elopements'. Publications found through this indexed search were reviewed for further relevant references. The term 'wandering' covers different types of behaviour, including aimless movement without a discernible purpose. It is associated with a variety of negatives outcomes. The aetiology of wandering is poorly understood and it remains an unsolved riddle. Wandering is an acutely distressing problem worldwide, both for the patients and caregivers, and it is a major reason for nursing home admission. Evidence on the effectiveness of pharmacological and non-pharmacological interventions is limited. It is possible that management of coexistent psychopathology would help to ameliorate this problematic behavioural disorder.

How aware are migraineurs of their triggers?

BACKGROUND: Broad discrepancies in the number of migraine triggers have been reported in several studies. Migraineurs do not seem to recognize easily headache triggers in clinical practice. OBJECTIVE: To evaluate how aware migraineurs are about their headache triggers. MATERIALS AND METHODS: We recruited 120 consecutive migraineurs. Each patient was first asked to report spontaneously any migraine trigger. Subsequently, the patient selected from a list of commonly known triggers. RESULTS: Ninety-seven patients (72.5%) spontaneously reported at least 1 migraine trigger, and 120 patients (100%) reported at least 1 migraine trigger selecting from a specific list of precipitants. The mean number of spontaneously identified triggers was 1.5 (±1.5), and the total number of triggers identified was 7.20 (±3.9). CONCLUSIONS: A relevant discrepancy between the number of spontaneously recognized triggers and the total number of triggers was found. This may suggest that migraineurs display poor awareness about headache triggers.

Triggers in allodynic and non-allodynic migraineurs. A clinic setting study.

BACKGROUND: Allodynia is considered a phenomenon of central sensitization that may lead to migraine transformation, lowering the attack threshold. Migraine triggers are factors that may induce headache attacks in susceptible individuals. We hypothesize that because allodynia decreases the migraine-attack threshold, allodynic migraineurs are more susceptible to triggers than the non-allodynic ones. OBJECTIVE: To determine if the number of headache triggers differs between migraineurs with no/mild allodynia and those with moderate/severe allodynia. MATERIALS AND METHODS: We recruited 120 consecutive migraineurs. Other primary headache comorbidity and migraine prophylaxis were exclusion criteria. Each patient was interviewed according a structured questionnaire including general features about migraine, depression, and anxiety symptoms. Patients reported any migraine trigger both spontaneously and by selecting from a specific list. Allodynia during the migraine attack was measured using the Allodynia Symptoms Check-List 12 (ASC-12): a cut-off of ASC-12 score of >2 defined allodynic patients; 3-5 score indicated mild allodynia; a 6-8 score moderate allodynia; and score of >8 severe allodynia. RESULTS: The total number of identified triggers was significantly and positively related to allodynia measured with ASC-12 (ρ(s) 0.33; P < .001). In a logistic regression model, allodynia independently influenced the risk to have a higher number of triggers. Moderate/severe allodynic patients had an odds ratio of 2.8 to report a number of triggers >7 in respect to non-/mild allodynic ones. CONCLUSIONS: Migraineurs with moderate/severe allodynia had more triggers than those with no/mild allodynia. It is unknown if those with moderate/severe allodynia are more susceptible to triggers, or repetitive stimulation of the trigeminal system by triggers resulted in moderate/severe allodynia.

Cognitive correlates of negative symptoms in behavioral variant frontotemporal dementia: implications for the frontal lobe syndrome.

Although both behavioral disturbances and executive impairments of patients with the behavioral variant frontotemporal dementia (bvFTD) seem to depend on early neurodegenerative damages to the prefrontal cortex, the relationship between these two distinct clinical features has been only partially established and represents the focus of the current preliminary neuropsychological study. Ten subsequent bvFTD patients underwent a neuropsychiatric assessment with the Frontal Behavior Inventory and a neuropsychological battery focused on prefrontal functions. Significant correlations were found only between negative symptoms and measures of prevalent medial prefrontal functioning, i.e. decision making under ambiguity (Iowa gambling task) (r = -0.887; p = 0.018) and affective theory of mind (reading the mind in the eyes task) (r = -0.982; p = 0.017). This finding could preliminary support a "frontal lobe syndrome" hypothesis for negative symptoms of bvFTD patients, as proposed for negative symptoms of schizophrenia; the small sample size represents a limit and empirical findings need replication in larger samples of bvFTD patients.

Behavioral and psychological symptoms of dementia: factor analysis and relationship with cognitive impairment.

BACKGROUND/AIMS: Behavioral and psychological symptoms of dementia (BPSD) affect almost all people with dementia at some stage during the progression of disease. The current study aimed at replicating previous findings on BPSD clustering in behavioral sub-syndromes. METHODS: One hundred and sixty-six consecutive outpatients with dementia attending a dementia outpatient clinic were enrolled and evaluated with the Neuropsychiatric Inventory and the Mini Mental State Examination. RESULTS: BPSD were reported in 157 patients included in the final sample: 140 patients (89.17%) had Alzheimer's disease (AD), while 17 patients had frontotemporal dementia or vascular dementia. The most frequent BPSD were depression in the whole sample and irritability in the AD subgroup. The severity of cognitive impairment was predicted by two BPSD in the whole sample (apathy and motor disturbance) and only by nighttime behavior in the AD subgroup. Factor analyses identified 4 factors, accounting for about 60% of variance in the whole group and in the AD subgroup of patients. CONCLUSIONS: The current study confirmed findings of previous studies on BPSD clustering, regarding the number of BPSD clusters and the total variance explained. This study has some limitations, including the small number of non-ADs and its monocentric character.

Alzheimer's Disease and Neurosyphilis: Meaningful Commonalities and Differences of Clinical Phenotype and Pathophysiological Biomarkers.

BACKGROUND: Neurosyphilis-associated cognitive and behavioral impairment- historically coined as "general paralysis of the insane"- share clinical and neuroradiological features with the neurodegenerative disease spectrum, in particular Alzheimer's disease (AD). Anatomopathological similarities have been extensively documented, i.e., neuronal loss, fibrillary alterations, and local amyloid-ß deposition. Consequently, accurate classification and timely differential diagnosis may be challenging. OBJECTIVE: To describe clinical, bio-humoral, brain MRI, FDG-PET, and amyloid-PET features in cases of neurosyphilis with an AD-like phenotypical presentation, as well as clinical outcome in terms of response to antibiotic therapy. METHODS: We selected the studies comparing patients with AD and with neurosyphilis associated cognitive impairment, to investigate candidate biomarkers classifying the two neurological diseases. RESULTS: The neuropsychological phenotype of general paralysis, characterized by episodic memory impairment and executive disfunction, substantially mimics clinical AD features. Neuroimaging often shows diffuse or medial temporal cortical atrophy, thus contributing to a high rate of misdiagnosis. Cerebrospinal fluid (CSF)-based analysis may provide supportive diagnostic value, since increased proteins or cells are often found in neurosyphilis, while published data on pathophysiological AD candidate biomarkers are controversial. Finally, psychometric testing using cross-domain cognitive tests, may highlight a wider range of compromised functions in neurosyphilis, involving language, attention, executive function, and spatial ability, which are atypical for AD. CONCLUSION: Neurosyphilis should be considered a potential etiological differential diagnosis of cognitive impairment whenever imaging, neuropsychological or CSF features are atypical for AD, in order to promptly start antibiotic therapy and delay or halt cognitive decline and disease progression.

Peri-ictal prolonged atrial fibrillation after generalized seizures: description of a case and etiopathological considerations.

Cardiac rhythm changes are not uncommon in connection with seizures and should be considered and recognized given their potentially harmful consequences including Sudden Unexpected Death in Epilepsy (SUDEP). The most well known are ictal tachycardia and bradycardia. However, other potentially dangerous peri-ictal arrhythmias have been reported. Brief atrial fibrillation episodes, never longer than 2 min, have rarely been described in connection with seizures. We report the case of a patient who presented with two generalized tonic-clonic seizures associated with prolonged atrial fibrillation. Extensive non-invasive cardiac investigations failed to disclose cardiac abnormalities, and after proper antiepileptic drug treatment the patient had neither further seizures nor cardiac events in an 18-month follow-up. Our case, to our knowledge, is the first report of prolonged (more than 1 h) peri-ictal atrial fibrillation.

Dangerous passion: Othello syndrome and dementia.

Jealousy is a complex emotion that most people have experienced at some time in life; pathological jealousy refers primarily to an irrational state. Othello syndrome is a psychotic disorder characterized by delusion of infidelity or jealousy; it often occurs in the context of medical, psychiatric or neurological disorders. At least 30% of cases in the literature show a neurological basis for their delusion of infidelity, although its biological basis is not fully understood. The purpose of this paper is to examine the phenomenon of pathological jealousy in people with dementia. We searched the electronic databases for original research and review articles on Othello syndrome in demented patients using the search terms 'Othello syndrome, morbid jealousy, pathological jealousy, delusional disorders, dementia'. Convictions about the partner's infidelities may form the content of psychopathological phenomena, such as delusions. Delusional jealousy is a frequent problem in dementia. Coexistent delusions and hallucinations are frequent. The violence in demented patients suffering from this syndrome is well documented and forensic aspects are highlighted. There are no systematic researches about the clinical characteristics of Othello syndrome in persons suffering from dementia, but only case reports and it is not possible to differentiate or compare differences of delusional jealousy across the various type of dementia or distinguish the syndrome in demented patients from the syndrome in other psychiatric disorders. Frontal lobe dysfunction may be called into question in delineating the cause of the delusional jealousy seen in Othello syndrome.

Bilateral thalamic infarct caused by spontaneous vertebral artery dissection in pre-eclampsia with HELLP syndrome: a previously unreported association.

Cerebrovascular accidents are not rare during pregnancy and the postpartum period. Pre-eclampsia is a common condition that is characterized by proteinuria and de novo hypertension that may be complicated by hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Spontaneous cervical artery dissection has been rarely reported in the postpartum period but never in association with HELLP syndrome. We describe a case of pre-eclampsia and HELLP syndrome complicated in the postpartum period by bilateral thalamic infarct as result of left vertebral artery dissection. We speculated about the possible common etiopathologic mechanisms involved in this previously unreported association.

Postpartum headache due to spontaneous cervical artery dissection.

Postpartum headache is quite common and often related to potentially ominous cerebrovascular accidents. As illustrated in previously published reports, spontaneous cervical artery dissection is a rare but possible cause of headache in the postpartum. We provide 2 additional cases to the 19 described so far, including the first ever report of migraine with aura-like symptoms. Additionally, we summarize the literature and we speculate about the possible etiopathological mechanism underlying this condition.

Categorising a problem: alcohol and dementia.

Alcoholism is a chronic relapsing disorder that can include extended periods of abstinence followed by relapse to heavy drinking. Decades of evidence have clearly shown that long-term, chronic ethanol exposure produces brain damage in humans. The article aims to review the relationship between alcohol use and dementia. Medline and Google Scholar searches were conducted for relevant articles, chapters and books published until 2019. Search terms used included alcohol consumption, alcohol-related dementia, alcohol use disorders, chronic alcoholism, dementia. Publications found through this indexed search were reviewed for further relevant references. Alcohol acts on the central nervous system via both direct and indirect effects, frequently a combination of the two. There is consensus that alcohol contributes to the acquisition of cognitive deficits in late life. However, there are doubts regarding the aetiopathogenesis, nosological status and prevalence of alcohol-related dementia and still, there is much debate over how much alcohol consumption will lead to alcohol-related dementia.

A case of idiopathic low CSF pressure headache presenting as cough headache.

Idiopathic low cerebrospinal fluid (CSF) pressure headache is considered to be one that worsens within 15 min of sitting-up or standing-up, accompanied by at least one of the following: neck stiffness, tinnitus, hypacusia, photophobia and nausea. Several reports suggest that a substantial number of idiopathic low CSF pressure cases do not present typical clinical symptoms and that a considerable clinical variability exists. We report the case of an idiopathic low CSF pressure presenting as a cough headache.

Is that schizophrenia or frontotemporal dementia? Supporting clinicians in making the right diagnosis.

Schizophrenia (SCH) and frontotemporal dementia (FTD) are neurobehavioral syndromes characterized by a profound alteration in personal and social conduct. Differential diagnosis between SCH and FTD remains a challenge. In this short narrative review, we summarize evidences regarding similarities and differences between these disorders to support clinicians in making the right diagnosis. Reports of FTD misdiagnosed as schizophrenia or schizophrenia-like psychosis are frequently reported in the literature. The behavioural variant of FTD (bvFTD) along with familial FTD characterized by delusions and hallucinations represent the medical conditions that best illustrate overlaps between psychiatry and neurology. Neuropsychological patterns of core deficits and anatomical and physiological brain alterations primarily concur in differencing such disorders while additional research on genetic alterations and their reflection on clinical phenotypes should be implemented in the near future. In some cases, a correct diagnosis should be made within an interdisciplinary clinical setting by complementary competences and follow-up visits to evaluate pathology evolution.

Daily functioning and dementia.

Dementia is characterized by a decline in memory, language, problem-solving and in other cognitive domains that affect a person's ability to perform everyday activities and social functioning. It is consistently agreed that cognitive impairment is an important risk factor for developing functional disabilities in patients with dementia. Functional status can be conceptualized as the ability to perform self-care, self- maintenance and physical activity. A person with dementia usually requires help with more complex tasks, such as managing bills and finances, or simply maintaining a household. Good functional performance is fundamental for elderly people to maintain independency and avoid institutionalization. The purpose of this review is to describe functional changes in demented patients, evaluating the variability in subgroups of dementias.

Demência é caracterizada por declínio na memória, linguagem, resolução de problemas e de outros domínios cognitivos que afetam a capacidade de realização de atividades cotidianas e atividades sociais. É consensual que o comprometimento cognitivo é um importante fator de risco para o desenvolvimento de incapacidades funcionais em pacientes com demência. O status funcional pode ser conceituado como a capacidade de realizar autocuidado, automanutenção e atividade física. Uma pessoa com demência geralmente requer ajuda para tarefas mais complexas, como gerenciar contas e finanças, ou simplesmente realizar atividades domésticas. Um bom desempenho funcional é fundamental para que os idosos mantenham a independência e evitem a institucionalização. O objetivo desta revisão é delinear alterações funcionais em pacientes com demência, valorizando os subgrupos variados de demências.

A complication of coronavirus disease 2019: delirium.

COVID-19 is predominantly a respiratory disease. However, some cases exhibit other features including Central Nervous System symptoms. In the older adult, COVID-19 may present with atypical symptoms, including delirium and its complications. The objective of this study is to describe the relationship between the new type of coronavirus infection and delirium. Systematic research (Cochrane Library and PubMed) was carried out (only upper time limit: April 2020). Publications found through this indexed search were reviewed and manually screened to identify relevant studies. Search terms used included "COVID-19, Delirium, Dementia, Intensive Care Unit". We manually added articles identified through other sources (i.e., key journals). Older people are at the greatest risk from COVID-19. If infected, they may present delirium. Moreover, it is not exclusive to older people. Delirium is not inevitable; rather, it is preventable. Delirium prevention programs are even more crucial in the era of COVID-19 and cannot be allowed to wither despite the challenges of integrating delirium prevention with COVID-19 care. An acute change in condition, behaviour, or mental status should prompt a delirium screen. As regards the treatment, it is advisable to use non-pharmacological interventions first where possible. Medication may be needed for patients with agitation where there is intractable distress or high risk to self/others.