Despite many advances in the characterization of the behavioral variant of frontotemporal dementia bvFTD , the diagnosis of this syndrome poses a significant challenge, while delays or diagnostic mistakes may impact the proper clinical management of these patients. Fear of contamination and compulsive hand washing. Further evidence for plasma progranulin as a biomarker in bipolar disorder. Main demographic and clinical features along with evaluation performance of the sample. The degenerative process targets preferentially cortical regions that involve the anterior cingulate, insular and orbitofrontal cortex, and anterior temporal pole, which are essential to value-guided decision-making, motivation, social cognition, emotion identification, emphatic concern, and appropriate personal conduct, with relative preservation of episodic memory. Spouses of patients with bvFTD often comment on their lack of interpersonal warmth, and diminished response to social cues. Thirty-one patients were consecutively selected after medical chart review.

The incidence of frontotemporal lobar degeneration in Rochester, Minnesota, through Dipeptide repeat protein pathology in C9ORF72 mutation cases: Sadness, decreased emotional responsiveness, decreased concentration. Progranulin GRN in two siblings of a Latino family and in other patients with schizophrenia. The majority of behavioural features were rated based on reports from carers.

Cruts M, Van Broeckhoven C. The deficit spans verbal and nonverbal components and perhaps testifies to the true nature of neurodegeneration in this group. Behavioural features A number of behavioural features, including apathy, reduction in insight and simple repetitive behaviours were common in both bvFTD and ALS-FTD table 2 and group comparisons were non-significant.

The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry

Back to top Article Information. Bvfd chose to include nine patients that only met possible bvFTD consensus criteria. Data given in Table 1 show that, at first evaluation in the unit, average performance of the participants was unequivocally impaired on all the brief cognitive screening tests, including the episodic memory test.


In mental health settings where it is difficult to order neuroimaging procedures, practitioners should make themselves aware of colleagues in psychiatry or neurology who are experts in dealing with neurodegenerative disorders and to whom patients can be referred for consultation.

Behavioral Variant FTD

Patients with ALS-FTD were more likely to show problems in use and understanding of grammar and in sentence comprehension. Her neurological examination was notable for stimulus-bound behaviours and lack of respect for social boundaries.

How many cases of possible BVFTD evolve to probable or definite disease, and where does the C9orf72 expansion fit into current diagnostic criteria?

Fronto-temporal dementia and motor neuron disease: This is a retrospective study which used clinical data from patients who had previously attended the clinic. Disinhibition A loss or lack of restraint based on social norms, leading to inappropriate behavior and impulsivity.

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

Requests for data sharing should be made in writing to the corresponding author. Loss of insight and functional neuroimaging in frontotemporal dementia. The online supplementary table presents some of the initial psychiatric manifestations in this patient cohort. We propose that largely due to the paucity of biomarkers for primary psychiatric disorders, and the limited use of FTLD-related biomarkers by psychiatrists at present, it is bvvftd difficult to separate patients with early bvFTD from those with primary psychiatric disorders based on clinical grounds.


bvftd case study

The patient was alive at time of case publication. The syndrome of cognitive impairment in amyotrophic lateral sclerosis: We then review several case reports that illustrate the syndromic overlap that exists between bvFTD and several primary psychiatric disorders. Patients’ clinical history suggested that the onset of disease was on average at In interpreting these findings, the greater frequency of behavioural change in bvFTD raises the possibility that the patients with bvFTD were simply more impaired overall.

Conflict of Interest Disclosures: The sample consisted of 31 patients, with a mean age of However, as this retrospective study recorded presence of impairment wtudy, a systematic prospective study is warranted to characterise severity and qualitative characteristics of executive disorder in ALS-FTD and bvFTD.

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bvftd case study

From genotype to phenotype: Bvtd findings at autopsy may result in refinement post-mortem to the initial clinical diagnosis. Macroscopically, FTLD results in varying degrees of focal atrophy of all the cortical regions that form part of the cortical-subcortical brain circuits discussed above. Voxel-based morphometry in patients with obsessive-compulsive behaviors in behavioral variant frontotemporal dementia.

Amyotroph Lateral Scler ; However, a minority of familial cases do not have a known gene defect, whereas the C9orf72 expansion is also present in a number of sporadic cases. Open in a separate window. Dement Geriatr Cogn Disord ;