Find articles by Victor Pimentel. Clinical, genetic and pathological heterogeneity of frontotemporal dementia: Clear identification of a specific phenotype through prospective study would facilitate earlier identification and improved management of such patients. Frontotemporal degeneration, the next therapeutic frontier: Finally, important information can be gleaned from the routine neurologic consultation, and clinicians should consider this when faced with difficult questions of prognosis and referral for genetic testing. The bvFTD seems to equally affect both sexes, although some series have reported a male predominance. Below, we briefly review the cardinal clinical signs and symptoms of bvFTD in light of relevant cortical functional neuroanatomy.

In the group with changed BVFTD, cognitive and functional deterioration over time was seen and patients were likely to carry the C9orf72 expansion, whereas the group with unchanged BVFTD remained stable for several years. How common are behavioural changes in amyotrophic lateral sclerosis? Neuropsychological testing revealed significantly poorer scores than among controls across the board. Underdiagnosis of frontotemporal lobar degeneration in Brazil. Progranulin gene variability and plasma levels in bipolar disorder and schizophrenia.

Galimberti D, Scarpini E. Fears of contamination, compulsive checking and hand washing.

Examining the language and behavioural profile in FTD and ALS-FTD

Features were rated as present or absent based on information recorded from clinical interviews and detailed neuropsychological assessment. Patients’ mean age was The differential diagnosis of early bvFTD is far more challenging largely due to the syndromic overlap that exists between early bvFTD and late-onset psychiatric disease. In contrast, performance on executive tasks is impaired in patients with changed and unchanged disease status.

Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9ORF72 hexanucleotide expansion.

It is important to note that the signs and symptoms of bvFTD can all occur in a single patient at different time points in the disease course. Symmetric anteromedial temporal lobe atrophy.


The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry

Given that pathology-based pharmaceutical bgftd are on the horizon, the differentiation of FTLD from the pathological features of Alzheimer disease seems to be imperative. Finally, a continuum of neuropsychological and neuroimaging abnormalities are seen across bvffd and possible BVFTD and changed vs unchanged disease status, with the most severe changes seen in probable BVFTD.

These genes and their associated phenotypes will not be discussed further in this article. Whereas current diagnostic classification in psychiatry is based on identifying recognisable syndromes of observable behaviour, syndromic diagnoses in FTD are guided by knowledge of relevant functional neuroanatomy and neuropathology.

Demographics are shown in table 1. Frontal lobe dementia and motor neuron disease.

Research paper: Examining the language and behavioural profile in FTD and ALS-FTD

Carriers of C9ORF72 mutations are especially prone to psychiatric manifestations at disease onset, 396465 mainly presenting as psychotic, bipolar and compulsive disorders. Our results indicate that the likelihood of progression may be determined during a routine neurologic consultation by means of a detailed clinical interview and examination and a brief test of global cognition.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The utility of the Cambridge Behavioural Inventory in neurodegenerative disease. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account.

bvftd case study

The lower right-hand side column shows csae expected scores according to control-reference performance, 18 weight-adjusted for five levels of formal education.

Sudden personality change at age 25 followed by psychotic changes beginning at age Cognitive change in ALS: Cerebral metabolic patterns at early stages of frontotemporal dementia and semantic dementia.


The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry

Clinical presentation of prodromal frontotemporal dementia. Owing to the variety of psychiatric manifestations that occur in early bvFTD, often in the absence of cognitive impairment, distinguishing early bvFTD from some primary psychiatric disorders is challenging.

Provenance and peer review Not commissioned; externally peer reviewed. To describe the clinical profile at first evaluation of a sample of patients with bvFTD from a specialized outpatient neurological unit, with emphasis on the analysis of the delay between the onset of symptoms and diagnosis.

Sample of DSM-compatible diagnoses assigned to patients who were subsequently found to have possible, probable and definitive bvFTD. National Center for Biotechnology InformationU.

bvftd case study

The resulting gray matter, partial volume maps were aligned to the Montreal Neurological Institute standard space MNI using the nonlinear registration approach with Casd part of the FSL packagewhich uses a b-spline representation of the registration warp field.

Estimates of disease duration in FTD vary widely.

Executive dysfunction in ALS is not the whole story. These patients show mild deficits on tests previously found to be sensitive markers of BVFTD, such as inhibitory control and emotion processing. Case reports and small group studies involving casf with ALS and dementia have reported the etudy of characteristics associated with bvFTD: Clinical and psychometrical distinction of frontotemporal and Alzheimer frontotemporal dementias.

The patient was 55 years of age when she first came to our attention.