Distinct behavioural profiles in frontotemporal dementia and semantic dementia

OBJECTIVE: To test predictions that frontotemporal dementia and semantic dementia give rise to distinct patterns of behavioural change. METHODS: An informant based semistructured behavioural interview, covering the domains of basic and social emotions, social and personal behaviour, sensory behaviour, eating and oral behaviour, repetitive behaviours, rituals, and compulsions, was administered to carers of 41 patients with semantic dementia and with apathetic (FTD-A) and disinhibited (FTD-D) forms of frontotemporal dementia. RESULTS: Consistent with prediction, emotional changes differentiated FTD from semantic dementia. Whereas lack of emotional response was pervasive in FTD, it was more selective in semantic dementia, affecting particularly the capacity to show fear. Social avoidance occurred more often in FTD and social seeking in semantic dementia. Patients with FTD showed reduced response to pain, whereas patients with semantic dementia more often showed exaggerated reactions to sensory stimuli. Gluttony and indiscriminate eating were characteristic of FTD, whereas patients with semantic dementia were more likely to exhibit food fads. Hyperorality, involving inedible objects, was unrelated to gluttony, indicating different underlying mechanisms. Repetitive behaviours were common in both FTD and semantic dementia, but had a more compulsive quality in semantic dementia. Behavioural differences were greater between semantic dementia and FTD-A than FTD-D. A logistic regression analysis indicated that emotional and repetitive, compulsive behaviours discriminated FTD from semantic dementia with 97% accuracy. CONCLUSION: The findings confirm predictions regarding behavioural differences in frontotemporal and semantic dementia and point to differential roles of the frontal and temporal lobes in affect, social functioning, eating, and compulsive behaviour.     

Problems family caregivers encounter in home care of patients with frontotemporal lobar degeneration

Aim: Frontotemporal dementia (FTD) is a degenerative dementia in which primary degeneration of the frontal region of the brain occurs. Because of the behavioral symptoms, the care of FTD patients has numerous problems. However, little has been clarified with regard to the actual care situation, especially in a family care setting. The aim of the present study was to elucidate the caregiver burden and problems associated with the care of FTD patients in home care settings. Methods: Two patients were diagnosed with FTD on the basis of the Lund and Manchester group criteria at the clinic for outpatients of a hospital located in Aichi Prefecture, Japan. Semi‐structured interviews were conducted with the family caregivers of the FTD patients. The content of the interview covered the patient course and any problems encountered in the home setting regarding activities of daily living (ADL), behavioral disorders and cognitive function. Results: These FTD patients had abnormal eating behaviors such as cramming of food into one's mouth and the abnormal manner of eating. They had to be fed bit by bit with total caregiver assistance. They were also overactive, restless and distractable, which subsequently caused problems with ADL‐assistance including extreme uncooperativeness toward their caregivers. Other behavioral symptoms associated with FTD, e.g., stereotypic behavior, distractability and high impulsivity, were also considerably burdening to the caregivers. Conclusion: The behavioral symptoms peculiar to FTD pose huge problems and heavy burden to the family caregiver. More resources should be allocated to specific needs of the FTD patients and their families.     

Preliminary findings: behavioral worsening on donepezil in patients with frontotemporal dementia.

OBJECTIVE: The objective of this study was to evaluate donepezil, an acetylcholinesterase inhibitor, in the treatment of frontotemporal dementia (FTD). METHODS: Twelve patients with FTD who received donepezil for six months were compared with 12 FTD controls on behavioral measures. RESULTS: The groups did not differ on most variables at baseline or at six months; however, the donepezil group had greater worsening on the FTD Inventory. Four treated patients had increased disinhibited or compulsive acts, which abated with discontinuation of the medication. CONCLUSION: There were no changes in global cognitive performance or dementia severity; however, a subgroup of patients with FTD can experience worsening of symptoms with donepezil.     

Fluvoxamine in the treatment of anxiety disorders

Fluvoxamine is a selective-serotonin reuptake inhibitor (SSRI) that has proved effective in large double-blind, randomized, controlled trials involving patients with social anxiety disorder (SAD), obsessive-compulsive disorder (OCD), and panic disorder. Improvements have also been demonstrated in patients with post-traumatic stress disorder, as well as those with a range of obsessive-compulsive spectrum disorders including binge eating disorder, bulimia nervosa, pathological gambling, and body dysmorphic disorder. Several well controlled studies have confirmed the efficacy of fluvoxamine in children and adolescents with OCD, SAD, and other anxiety disorders, and it was the first SSRI to be registered for the treatment of OCD in children. Fluvoxamine is well tolerated. In common with other SSRIs, the most frequently reported adverse event is nausea. Fluvoxamine does not cause sedation or cognitive impairment and is associated with a low risk of sexual dysfunction, suicidality, and withdrawal reactions. It is safe in overdose and has no significant effect on body weight or cardiovascular parameters.     

Efficacy of fluvoxamine as a treatment for behavioral symptoms in frontotemporal lobar degeneration patients

Patients with frontotemporal lobar degeneration (FTLD) present a profound personality change, social misconduct, overeating, and stereotyped behavior. We examined the hypothesis that many of the behavioral symptoms of FTLD will respond to selective serotonin reuptake inhibitors (SSRIs). Sixteen FTLD patients were treated with an SSRI (fluvoxamine maleate) in an open 12-week trial. Treatment responses for stereotyped behavior and other neurobehavioral symptoms were evaluated by the Stereotypy Rating Inventory and the Neuropsychiatric Inventory. The behavioral symptoms, especially stereotyped behaviors of FTLD, significantly improved after treatment. Randomized, placebo- and other SSRI-controlled trials may improve available treatments.     

Compulsive buying and binge eating disorder--a case vignettes

Compulsive buying behaviour has recently received long overdue attention as a clinical issue. Aim of this report is to describe treatment of two female patients diagnosed with compulsive buying disorder in comorbidity with binge eating disorder. In both cases, criteria for diagnosing of other axis I or axis II disorder were not present. Fluvoxamine was used in pharmacotherapy, and psychodynamic psychotherapy as a psychotherapeutical approach. We conclude that fluvoxamine and psychodynamic psychotherapy may be effective in treatment of compulsive buyers in comorbidity with binge eating disorder.     

Behaviour in frontotemporal dementia, Alzheimer's disease and vascular dementia

OBJECTIVES: The study aimed to increase understanding of behavioural changes in frontotemporal dementia (FTD) and identify features that best differentiate FTD from Alzheimer's disease (AD) and cerebrovascular dementia (CvD). METHODS: A semi-structured questionnaire was administered to carers of 30 FTD, 75 AD and 34 CvD patients. RESULTS: Behavioural changes that strongly discriminated FTD from AD and to a lesser extent CvD were loss of emotions and insight, selfishness, disinhibition, personal neglect, gluttony and sweet food preference, wandering, motor and verbal stereotypies, loss of pain, echolalia and mutism. Irritability, hyposexuality and hypersomnia did not discriminate. Emotional, eating and stereotyped behaviours correctly classified 95% of patients using regression analysis. CONCLUSIONS: Behavioural characteristics accurately differentiate FTD from AD and CvD. The findings highlight the particular importance of affective change in FTD, and underline the role of the frontotemporal lobes in emotion.     

Neuropsychology of frontal type dementia]

The clinical conceptual change in frontal type dementia is reviewed in discussing its relationships to several related concepts such as Pick's disease, frontotemporal dementia (FTD), semantic dementia (SD) and frontotemporal lobar degeneration. We analyzed frontal type dementia selected from a consecutive series of our outpatients as to the details of neuropsychological symptoms, psychiatric symptoms, and abnormal behaviors. In our series of 143 patients with primary degenerative dementia, there were 16 cases of FTD and 6 cases of SD. Patients with two types of FTD and patients with SD were not distinguishable by neuropsychological examinations, behavioral abnormalities and psychiatric symptoms assessed with the Neuropsychiatric Inventory except for aphasia. The clinical picture of frontal type dementia involves frontal lobe symptoms such as disinhibition, apathy and stereotypy. Semantic memory loss for words, objects or faces suggestive of temporal lobe involvement developed only in patients with SD, and not in patients with FTD. Certain behavioral symptoms seen in frontal type dementia may respond to selective serotonin reuptake inhibitors. In care for patients with frontal type dementia, behavioral disturbances can be diminished and the quality of life can be improved by using their preserved procedural memory, pathological stereotypic behavior and stimulus-bound behavior such as utilization behavior and environmental dependency syndrome.     

Clinical presentation of prodromal frontotemporal dementia

BACKGROUND: Misrecognition of symptoms in the early stages of frontotemporal dementia (FTD) frequently contributes to diagnostic delay. Three frameworks have been proposed for the clinical identification of prodromal FTD: (1) cognitive profiling, (2) the presence of behavioral/psychiatric symptoms in the absence of memory complaints, and (3) a combined approach of cognitive, behavioral, and neuroimaging features. OBJECTIVE: To evaluate current conceptual frameworks for the clinical recognition of prodromal FTD with current empirical evidence. METHOD: We performed a comprehensive PsychINFO and MEDLINE database search to identify articles investigating the prodromal symptoms of FTD. CONCLUSIONS: The 3 frameworks capture important aspects of the clinical picture of prodromal FTD but require further refinement. The prodromal stage of FTD is characterized by both cognitive and behavioral features. Diagnostic accuracy will likely be improved by considering a combination of cognitive and behavioral features, because some features overlap with prodromes for Alzheimer's disease and vascular dementia.     

You stole my food! Eating alterations in frontotemporal dementia

ABSTRACT

Patients with different types of dementia may exhibit pathological eating habits, including food fads, hyperphagia, or even ingestion of inanimate objects. Several findings reveal that such eating alterations are more common in patients with frontotemporal dementia (FTD) than other types of dementia. Moreover, eating alterations may differ between the two variants of the disease, namely the behavioral variant and semantic dementia (SD). In this review, we summarized evidences regarding four areas: eating and body weight alterations in FTD, the most common assessment methods, anatomical correlates of eating disorders, and finally, proposed underlying mechanisms. An increasing understanding of the factors that contribute to eating abnormalities may allow first, a better comprehension of the clinical features of the disease and second, shed light on the mechanism underlying eating behaviors in the normal population.     

Initial complaints in frontotemporal lobar degeneration

AIMS: Frontotemporal lobar degeneration (FTLD) is probably underrecognized. The goal of this study was to investigate initial complaints of both patients and their caregivers at first specialist referral. Also, we tried to assess whether misrecognition of symptoms contributed to diagnostic delay. METHODS: The case notes of all patients diagnosed with FTLD at the VU University Medical Center, Alzheimer Center of Amsterdam, The Netherlands, since 1998 were retrospectively reviewed. Only patients of whom detailed information of first specialist referral was available were included. The diagnosis of FTLD was based on the clinical diagnostic criteria of Neary and Snowden, supported by ancillary investigations. RESULTS: Forty-six patients with FTLD were included. Twenty-one patients had frontotemporal dementia (FTD), 17 semantic dementia (SD) and 8 progressive nonfluent aphasia (PA). The majority of the FTD patients presented without complaints or with somatic complaints and nearly a quarter of them expressed memory complaints. The presenting complaints of most of their caregivers differed from the patients' complaints and often consisted of cognitive complaints. In SD and PA, language problems but also forgetfulness were presented. Misrecognition of the initial symptoms in some cases seemed to have contributed to diagnostic delay. CONCLUSION: Presenting complaints in FTLD can be misleading. In our cohort, memory complaints occurred relatively often. A multidisciplinary approach, including a structured behavioral interview, is important to recognize symptoms of FTLD.     

Two cases of adult Down syndrome treated with selective serotonin re-uptake inhibitor for behavior disorders

Adult patients with Down syndrome show psychological symptoms and early senility. Improving their environment and dealing with their complaints and stress should first address their behavioral problems, such as self-injury, depression, aggression and outbursts. Pharmacological treatment may also be tried for behavioral disorders. Individuals with Down syndrome demonstrate neurotransmitter changes such as the loss of acetylcholine, norepinephrine, and serotonin (5-HT) with increasing age. Selective serotonin re-uptake inhibitor (SSRI) is effective for depression and panic disorders. We report here the effect of SSRI in two adult male patients with Down syndrome, 35 and 47 years of age. Self-injury in one case and aggression and outbursts in another improved after 1 week of fluvoxamine treatment, suggesting the effects of SSRI for behavioral disorders of adult Down syndrome.     

A longitudinal study of behavior in frontotemporal dementia and primary progressive aphasia.

OBJECTIVE: To evaluate the construct validity of the Frontal Behavioral Inventory (FBI) and to describe the evolution of the behavioral abnormalities of the behavioral and aphasic presentations of frontotemporal dementia (FTD) by means of a 3-year longitudinal study. BACKGROUND: The FBI is a standardized behavioral questionnaire useful in the diagnosis and quantification of the personality and behavior disorder FTD. METHOD: Patients who had three consecutive yearly assessments with the FBI were selected, 12 with the behavioral variant of FTD (FTD-bv) and 14 with Primary Progressive Aphasia (PPA). RESULTS: FBI scores rose as the disease progressed in both the FTD-bv and PPA groups over the 3 years of testing. Initial mean FBI scores of the FTD-bv group were above the cutoff for FTD as established for this diagnosis with previous standardization. By the third year, the mean FBI score of PPA patients was also above the established cutoff for FTD. CONCLUSIONS: The outcome of the study demonstrates that the FBI is sensitive to changes in behavior and personality in both variants of FTD. The FBI can be used to describe the evolution of symptoms and the course of the illness of Pick complex patients who present initially with FTD-bv or who present with PPA and subsequently develop the behavioral disorder.     

Frontotemporal lobar degeneration: a study in Japan

Frontotemporal lobar degeneration is the most common form of cortical dementia occurring in the presenium after Alzheimer's disease. We analyzed two types of frontotemporal dementia (FTD) and semantic dementia (SD) selected from a consecutive series of outpatients based on neuropsychological symptoms, psychiatric symptoms and abnormal behavior. In our series of 134 patients with primary degenerative dementia, there were 16 cases of FTD and 6 cases of SD. Patients with subgroups of FTD and patients with SD were distinguishable only by the presence of aphasia in the latter group. They were not distinguishable from one another by other neuropsychological examinations, behavioral abnormalities or psychiatric symptoms assessed with the Neuropsychiatric Inventory.     

Impulsive and compulsive behaviors among Danish patients with Parkinson's disease: Prevalence,depression, and personality

IntroductionDopaminergic medication administered to ameliorate motor symptoms of Parkinson's disease is associated with impulse control disorders, such as pathological gambling, hypersexuality, compulsive buying, and binge eating. Studies indicate a prevalence of impulse control disorders in Parkinson's disease of 6–16%.ObjectiveTo estimate the prevalence of impulsive and compulsive behaviors among Danish patients with Parkinson's disease and to explore the relation of such behavioral disorders to depression and personality.Methods490 patients with Parkinson's disease (303 males), identified through the National Danish Patient Registry, were evaluated with: 1) the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease; 2) the Geriatric Depression Scale; and 3) the NEO-Personality Inventory.Results176 (35.9%) patients reported impulsive and compulsive behaviors sometime during Parkinson's disease (current symptoms in 73, 14.9%). Hereof, 114 (23.3%) reported multiple behavioral symptoms. Patients with behavioral symptoms were significantly younger, were younger at PD onset, had longer disease duration, displayed more motor symptoms, and received higher doses of dopaminergic medication than patients without behavioral symptoms. Furthermore, they reported significantly more depressive symptoms and scored significantly higher on neuroticism and lower on both agreeableness and conscientiousness than patients without behavioral symptoms.ConclusionA history of impulsive and compulsive behaviors are common in Danish patients with Parkinson's disease and have clinical correlates that may allow identification of patients at risk for developing these behaviors.     

Frontotemporal dementia

Frontotemporal dementia (FTD) is a unique neurodegenerative disease that can be differentiated from Alzheimer's disease and other diseases that result in cognitive complaints. The primary anatomic focus of degeneration determines the clinical presentation, which can vary from aphasia to behavioral symptoms. Expanding knowledge about the genetics and biochemistry of FTD may lead to specific treatments.     

A retrospective study of SSRI treatment in adolescent anorexia nervosa: insufficient evidence for efficacy

Although selective-serotonin-reuptake-inhibitors (SSRI) have been of limited efficacy in the treatment of eating disorder psychopathology and comorbid symptoms of malnourished patients with anorexia nervosa (AN), there is recent data suggesting that SSRI may play a role in preventing relapse among weight-restored patients. Though some previous studies included patients in late adolescence, the vast majority of investigated subjects have been adults. The aim of our retrospective study was to assess the effects of SSRI treatment in partially weight-restored children and adolescents with AN. Thirty two females with AN (mean 14.5+/-1.4 years) were investigated three times during inpatient treatment and at 3- and 6-month follow-up for BMI, eating disorder psychopathology, depressive symptomology, and obsessive-compulsive symptomology. Medication history during inpatient and outpatient treatment was reconstructed at the 6-month follow-up. Nineteen patients received SSRI treatment, while 13 subjects were non-medicated. In comparison to the non-SSRI group, the SSRI group had similar BMI and obsessive-compulsive scores, but higher levels of core eating disorder psychopathology and depressive symptoms at the start of medication. Rates of re-admissions were similar in both groups (SSRI group: 36%, non-SSRI group: 31%, Phi: p=0.72). Repeated measures ANOVA revealed no significant group with time interactions for BMI-SDS (p=0.84), core eating disorder symptoms (ANIS, p=0.79), depression (DIKJ, p=0.75), and obsessive-compulsive (CY-BOCS, p=0.40) scores indicating minimal or no effects of SSRI medication on the course of these variables. In conclusion, our results challenge the efficacy of SSRI medication in the treatment of eating disorder psychopathology as well as depressive and obsessive-compulsive comorbidity in adolescent AN. Clinicians should be chary in prescribing SSRI in adolescent AN unless randomized controlled trials have proofed the benefit of these drugs.     

Complex compulsive behaviour in the temporal variant of frontotemporal dementia

Objective As metabolic and structural changes in frontotemporal-subcortical pathways have been reported in patients with obsessive-compulsive disorders, we investigated the correlation between complex compulsive behaviour (CCB) and the distribution of atrophy in a group of 90 patients with frontotemporal dementia (FTD). Methods CCB was defined as complex, intentional, and time consuming repetitive behaviour, which was distinguished from simple compulsive behaviour (SCB), such as verbal and motor repetitions and utilisation behaviour. Cortical atrophy on CT and/or MRI was semi-quantitatively assessed in frontal, temporal, parietal and occipital regions, and the pattern of atrophy was compared between patients with and without CCB or SCB. Linear measures were used to establish the presence of caudate atrophy (bicaudate ratio) and ventricular enlargement (bifrontal ratio). Results CCB was reported in 18 (21 %) and SCB in 53 (61 %) FTD patients. Frontotemporal atrophy was present in 64 patients (74 %), and predominant temporal atrophy in 23 (26 %). The pattern of atrophy was asymmetric in 25 patients (29 %). Logistic regression analysis showed that temporal lobe atrophy (p < 0.005), as well as asymmetry of atrophy (p < 0.05) were independently associated with CCB, after adjusting for age at onset, gender, duration of symptoms at the time of imaging, severity of atrophy, and bicaudate and bifrontal ratio. No relationship was found between the presence of SCB and the distribution of atrophy, although patients with SCB tended to have more caudate atrophy (p < 0.1). Conclusion Temporal lobe atrophy appears to mediate CCB in patients with FTD, especially if asymmetry of atrophy is present. Future studies with quantitative and volumetric measurements of the cortical and subcortical structures may further clarify the aetiology of CCB in FTD. Received: 29 November 2000, Received in revised form: 8 March 2001, Accepted: 25 March 2001     

Management of frontotemporal dementia in mental health and multidisciplinary settings

Abstract

Diagnosis of frontotemporal dementia (FTD) in the mental health setting and issues pertaining to longitudinal care of this population in a specialist clinic are reviewed. FTD is often misdiagnosed as a psychiatric disorder, most commonly as a mood disorder. FTD has features that overlap with those of major depression, mania, obsessive–compulsive disorder and schizophrenia. We describe these features and how to differentiate FTD from these psychiatric disorders. This paper also describes practical issues in the management of FTD, specifically the issues that clinicians, patients and their families face in managing this disease. Areas of clinical care along the continuum are explored; FTD care involves collaborative management of symptoms and disability, and assisting patients and families in adapting to the disease.     

Frontal behavioral syndromes in Prader-Willi syndrome

BACKGROUND: Prader-Willi syndrome (PWS) is a genetically determined neurodevelopmental disorder presenting with behavioral problems including hyperphagia, emotional aberration, and compulsion-like behaviors. This combination of behavioral problems is likely to be caused by damage to the orbitofrontal cortices and anterior temporal lobes or to circuits involving them. OBJECTIVES: To investigate the prevalence of eating and non-eating behavioral disturbances in PWS by using assessment tools developed originally for patients with frontotemporal dementia and with frontal lobe injury. METHOD: The questionnaire consisted of 35 questions related to three categories of behavior: eating behaviors (including four domains: appetite, food preference, eating habits, and other oral behaviors), stereotypy (including four domains: roaming, speaking, movements, and daily rhythm), and collecting behaviors. It was administered in Japan to the parents of 250 individuals aged 1-42 years with a clinical diagnosis of PWS. RESULTS: The prevalence rates of symptoms in all categories were high. Each domain involved in eating behaviors was significantly correlated with stereotypy and collecting behaviors. The prevalence rates and severity scores of some eating and non-eating behaviors were higher in the older groups. CONCLUSION: Abnormal eating behaviors, stereotyped behaviors, and collecting behaviors were common in the PWS subjects. There was also a potential link between abnormal eating and non-eating behaviors related to frontal behavioral syndromes. It is likely that these behavioral abnormalities reflect dysfunction of the orbitofrontal cortices and anterior temporal lobes.