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Query: UMLS:C0085632 (apathy)
 4,089 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In patients with the frontal variant of frontotemporal lobar degeneration (fv-FTLD), behavioral abnormalities may vary from apathy with motor slowness (apathetic form) to disinhibition with agitation (disinhibited form). These clinical presentations may be related to specific regional cerebral dysfunction and to deficit in the serotoninergic system. We studied cerebral glucose uptake using (18)F-fluorodeoxyglucose and positron emission tomography in 18 patients fulfilling clinical criteria for fv-FTLD and showing, respectively, an apathetic or disinhibited behavioral syndrome. In eight of these patients, we also evaluated the 5-hydroxytryptamine-2A receptor cerebral receptor distribution with [(11)C]MDL and positron emission tomography. We found a reduction of frontal glucose metabolism in the whole group of fv-FTLD patients. Apathetic syndrome was associated with a prevalent dorsolateral and frontal medial hypometabolism, whereas the disinhibited syndrome demonstrated a selective hypometabolism in interconnected limbic structures (the cingulate cortex, hippocampus/amygdala, and accumbens nucleus). The in vivo measurements of [(11)C]MDL indicated a significant reduction of 5-hydroxytryptamine-2A receptors in orbitofrontal, frontal medial, and cingulate cortices. These (18)F-fluorodeoxyglucose positron emission tomography changes can be considered as specific functional markers of the different behavioral presentations in fv-FTLD. The serotoninergic system dysfunction provides a rationale for therapeutic trials with selective serotonin reuptake inhibitors.
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PMID:Glucose metabolism and serotonin receptors in the frontotemporal lobe degeneration. 1566 60

The aim of this study was to investigate whether a brief neuropsychological battery consisting of a limited number of cognitive tests and an evaluation of the behavioural domains intended to discriminate between frontotemporal dementia (fv-FTD) and Alzheimer's disease (AD), constitutes a useful instrument for making a differential clinical diagnosis between these two pathologies. Nineteen fv-FTD and 39 AD patients were compared on cognitive tasks (assessing memory, executive functions, language and constructional praxis) and on the NPI behavioural assessment. A stepwise discriminant analysis was performed to identify the linear combination of cognitive and behavioural measures able to best discriminate between the two groups. One test for each of the investigated cognitive domains (Delayed Prose Recall, FAS verbal fluency, Boston naming test, Rey's Figure A Copy) and the four subscales of the Neuropsychiatry Inventory (NPI) which best differentiated between fv-FTD and AD patients (apathy, disinhibition, euphoria, aberrant motor behaviour) were used. The analysis selected Rey's Figure A Copy, FAS verbal fluency and NPI apathy subscale as the best discriminants between fv-FTD and AD patients. The final equation assigned 73.7% of the fv-FTD patients and 94.7% of the AD patients to the correct diagnostic group. A validation study conducted on a new independent sample of 11 fv-FTD and 22 AD patients confirmed the high sensitivity (82.6 %) and specificity (81.8%) of the diagnostic equation in assigning fv-FTD and AD patients to the correct dementia group. Although both cognitive and behavioural differences exist between FTD and AD, previous studies have aimed at differentiating the two pathologies by considering the two aspects separately and discriminant analyses were focused only on neuropsychological or neuropsychiatric evaluations. The present results emphasise the importance of rating both cognitive and behavioural clinical features of the two syndromes as objectively as possible to improve differential diagnostic accuracy.
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PMID:Alzheimer's disease and frontal variant of frontotemporal dementia-- a very brief battery for cognitive and behavioural distinction. 1590 58

We report an 81-year-old patient with progressive dementia, disinhibition, and gait disturbance. He showed visuospacial disorientation, apathy, and gait disturbance at 76 years of age. When he was 77 years old, he was diagnosed Parkinson's disease and treated with the 1-dopa, the dopamine agonist, the amantadin, and the anti-cholinergic drug. These treatments didn't improve his motor disturbances. His motor disturbances, apathy, and abnormal behavior progressed gradually. He was admitted to the hospital at the age of 77. He was severely demented and akinetic. Sometime, violent behavior and hallucination were seen. The brain MRI showed frontotemporal lobe atrophy and severe leukoaraiosis of the frontal white matter. At 79 years of age, he became mute and bedridden. When he was 80 years old, large infarction occurred in his occipital lobe. He died due to renal failure and respiratory suppression at 81 years of age. His brain was examined pathologically. At the neurological CPC, the chief discussant arrived at the conclusion that his diagnosis was Binswanger's disease. Other possibilities discussed were FTD, CBD, and progressive subcortical gliosis. The post-mortem examination revealed diffuse white matter degeneration due to atherosclerotic change of the small artery, many lacunar infarctions, and severe infarction of the occipital lobe. These findings led the diagnosis of Binswanger's disease and cerebral infarction.
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PMID:[An 81-year-old man with personality change, dementia, and gait disturbance with diffuse leukoaraiosis of the cerebral white matter]. 1648 27

Behavioral manifestations may dominate the clinical picture of the frontal variant of frontotemporal dementia (fv-FTD) for a long time before the appearance of true cognitive deficits. On the other hand, a deficit in the episodic memory domain represents the main manifestation of Alzheimer's disease (AD). Many behavioral disorders have been described in the clinical course of both FTD and AD; however, apathy and personality changes characterize frontal dementias, while depression dominates in AD, at least in the earlier stages. Depending on the distribution of neural damage, different patterns of noncognitive manifestations may be expected in different subtypes of FTD. Recent research on the social cognition deficit in FTD has offered new insights into the relationship between cognition and behavior, suggesting that some aspects of the behavioral changes in dementia may be generated by impairment in this domain.
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PMID:Frontotemporal dementia to Alzheimer's disease. 1772 14

Frontotemporal lobar degeneration is a neurologic condition that manifests profound behavioral, personality, and language symptoms. These changes, often unique to each person, may include social misconduct, eating disturbances, apathy, emotional blunting, childlike behavior, habits and rituals, executive dysfunction, and marked difficulty with speech and language. These symptoms occur as the result of cell loss in discrete areas of the brain, the frontal and/or anterior temporal lobes. Little is known about effective management of the behavioral symptoms that result from this disease. The purpose of this paper is to describe a model that could be used in the assessment and potential management of frontotemporal lobar degeneration behavioral symptoms. Suggestions for interventions are given for some of the more common FTD behaviors. The impact of behavioral symptoms on the family caregiver is discussed along with strategies for supportive services and recommendations for future study.
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PMID:A model for management of behavioral symptoms in frontotemporal lobar degeneration. 1809 Apr 27

Mutations in the progranulin gene (GRN) are a major cause of frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions (FTLD-U) but the distinguishing clinical and anatomical features of this subgroup remain unclear. In a large UK cohort we found five different frameshift and premature termination mutations likely to be causative of FTLD in 25 affected family members. A previously described 4-bp insertion mutation in GRN exon 2 comprised the majority of cases in our cohort (20/25), with four novel mutations being identified in the other five affected members. Additional novel missense changes were discovered, of uncertain pathogenicity, but deletion of the entire gene was not detected. The patient collection was investigated by a single tertiary referral centre and is enriched for familial early onset FTLD with a high proportion of patients undergoing neuropsychological testing, MRI and eventual neuropathological diagnosis. Age at onset was variable, but four mutation carriers presented in their 40s and when analysed as a group, the mean age at onset of disease in GRN mutation carriers was later than tau gene (MAPT) mutation carriers and duration of disease was shorter when compared with both MAPT and FTLD-U without mutation. The most common clinical presentation seen in GRN mutation carriers was behavioural variant FTLD with apathy as the dominant feature. However, many patients had language output impairment that was either a progressive non-fluent aphasia or decreased speech output consistent with a dynamic aphasia. Neurological and neuropsychological examination also suggests that parietal lobe dysfunction is a characteristic feature of GRN mutation and differentiates this group from other patients with FTLD. MR imaging showed evidence of strikingly asymmetrical atrophy with the frontal, temporal and parietal lobes all affected. Both right- and left-sided predominant atrophy was seen even within the same family. As a group, the GRN carriers showed more asymmetry than in other FTLD groups. All pathologically investigated cases showed extensive type 3 TDP-43-positive pathology, including frequent neuronal cytoplasmic inclusions, dystrophic neurites in both grey and white matter and also neuronal intranuclear inclusions. Finally, we confirmed a modifying effect of APOE-E4 genotype on clinical phenotype with a later onset in the GRN carriers suggesting that this gene has distinct phenotypic effects in different neurodegenerative diseases.
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PMID:A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series. 1823 97

Social functioning in FTD is profoundly affected, and forms the basis for the clinical diagnosis of the behavioural variant of the disease (bv-FTD). In particular, there are deficits in emotional processing, but the inter-relationship of such deficits to other aspects of social functioning remains unclear. We studied patients with bv-FTD (n = 14) and AD (n = 14), and compared their performance on a test of emotion recognition with their scores on two carer-based assessments: the Disability Assessment for Dementia (DAD) of activities in daily living (ADL), and the Cambridge Behavioural Inventory (CBI). The bv-FTD group had significantly greater impairments in ADLs, and had higher scores on the CBI, compared to the AD group. Despite a deficit in emotion recognition, particularly involving negative emotions, in the FTD group relative to AD and controls, performance on this task did not correlate with ADL ratings which instead, correlated highly with carer-rated apathy levels on the CBI. The study highlights the multifactorial nature of social dysfunction in FTD which is important in the management of these patients and in designing effective behavioural and therapeutic interventions. The relationship of emotional processing to other aspects of social cognition in FTD is reviewed.
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PMID:Emotion, social functioning and activities of daily living in frontotemporal dementia. 1917 32

Behavioral changes in patients with FTD can be interpreted by considering damage to the frontal lobes themselves and considering the interaction between the frontal lobes and other neural systems such as the posterior association cortices, the limbic system, and basal ganglia. Loss of insight and apathy primarily result from frontal lobes involvement. The latter is probably correlated with the severity of medial frontal-anterior cingulate involvement. Stimulus-bound behavior such as imitation behavior, utilization behavior and environmental dependency syndrome is caused by an imbalance between the activities of the frontal and parietal lobes. Frontal lobe damage, particularly damage to the medial frontal area, result in liberation of the parietal lobe activity, leaving the patient subject to any stimuli from the external environment. Disinhibition such as antisocial behavior is produced by an imbalance between the activities of the frontal and limbic lobes. Namely, loss of control of the frontal lobe, especially the orbitofrontal area, over the limbic system results in acts led by instinctive desires and uncontrolled by reason. Stereotypic behavior is due to an imbalance between the activities of the frontal cortex and basal ganglia. These behaviors range from simple stereotypies to complex repeated actions such as roaming, clock-watching or adherence to a strict daily timetable.
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PMID:[Symptomatology of fronto-temporal dementia]. 1919 45

Frontotemporal dementia is a disorder of paralimbic prefrontal-insular circuitry. The disorder is often sporadic but can be caused by genetic mutations in tau, progranulin, valosin, TDP-43 and CHMP2b. The major clinical manifestations of FTD include addictive behaviors, disinhibition, apathy, overeating and loss of sympathy and empathy for others. Treatment is currently focused around symptoms but disease-modifying therapies seem feasible.
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PMID:Early features in frontotemporal dementia. 1968 32

Our objectives were to assess the frequency of behavioural changes in patients with amyotrophic lateral sclerosis (ALS) and to compare the clinical profile of ALS patients with those with behavioural variant frontotemporal dementia (bvFTD). Ninety-two patients with ALS and their carers participated in a postal survey. ALS patients completed self-report measures of motor function and mood. Eighty-one carers of ALS patients and 25 carers of bvFTD patients completed the revised version of the Cambridge Behavioural Inventory (CBI-R). Results showed that reduced motivation was reported in more than 80% of the ALS cases, with almost 41% of them having moderate-severe apathy. Depression was present in 30% of ALS patients and did not contribute significantly to the presence of behavioural symptoms. Bulbar and limb onset ALS patients did not differ. Abnormal behaviour and stereotypical and motor behaviours were present to a moderate-severe degree in around 20%, and 11% reached the criteria for FTD. The rate of behavioural symptoms was significantly higher in the bvFTD group than ALS in all behavioural domains (p <0.001). In conclusion, apathy was the most prominent feature in ALS patients. A substantial proportion of ALS patients manifested behavioural changes of the type seen in FTD, with 11% fulfilling the criteria for FTD.
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PMID:How common are behavioural changes in amyotrophic lateral sclerosis? 2084 23


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