Behavioral-variant frontotemporal dementia: Distinct phenotypes with unique functional profiles

Objective: To identify distinct behavioral phenotypes of behavioral variant frontotemporal dementia (bvFTD), and elucidate differences in functional, neuroimaging, and progression to residential care placement. Methods: Eighty-eight patients with bvFTD were included in a cluster analysis applying levels of disinhibition and apathy (Cambridge Behavioural Inventory-Revised) to identify phenotypic subgroups. Between-group (Kruskal-Wallis; Mann-Whitney U) functional differences (Disability Assessment for Dementia), and time to residential care placement (survival analyses) were examined. Cortical thickness differences (whole brain MRI) were analyzed in bvFTD patients versus healthy controls (n=30) and between phenotypic subgroups. Results: Four phenotypic subgroups were identified: “Primary severe apathy” (n=26), “Severe apathy and disinhibition” (n=26), “Mild apathy and disinhibition” (n=27), “Primary severe disinhibition” (n= 9). Severely apathetic phenotypes were more functionally impaired and had more extensive brain atrophy than those with mild apathy or severe disinhibition alone. Further imaging analyses indicated that the right middle temporal region is critical for the development of disinhibition, an association that remains with disease progression and in the context of severe apathy. Finally, no difference in time to residential care admission was found between phenotypes. Conclusions: This study reveals that different clinical behavioral phenotypes of bvFTD have differing profiles of functional decline and distinct patterns of associated cortical changes. These findings emphasize the importance of apathy in functional impairment, highlight the role of the right temporal region in disinhibition and suggest that disability may be a sensitive outcome measure for treatments targeting reduction of apathy. These phenotypes could also support understanding of prognosis and clinical management

Calling for help: a responsive evaluation of Child Health Line

The project examined the responsiveness of the telenursing service provided by the Child Health Line (hereinafter referred to as CHL). It aimed to provide an account of population usage of the service, the call request types and the response of the service to the calls. In so doing, the project extends the current body of knowledge pertaining to the provision of parenting support through telenursing. Approximately 900 calls to the CHL were audio-recorded over the December 2005-2006 Christmas-New Year period. A protocol was developed to code characteristics of the call, the interactional features between the caller and nurse call-taker, and the extent to which there was (a) agreement on problem definition and the plan of action and (b) interactional alignment between nurse and caller. A quantitative analysis examined the frequencies of the main topics covered in calls to the CHL and any statistical associations between types of calls, length of calls and nurse-caller alignment. In addition, a detailed qualitative analysis was conducted on a subset of calls dealing with the nurse management of calls seeking medical advice and information. Key findings include: • Overall, 74% of the calls discussed parenting and child development issues, 48% discussed health/medical issues, and 16% were information-seeking calls. • More specifically: o 21% discussed health/medical and parenting and child development issues. o 3% discussed parenting and information-seeking issues. o 5% discussed health/medical, parenting/development and information issues. o 18% exclusively focussed on health and medical issues and therefore were outside the remit of the intended scope of the CHL. These calls caused interactional dilemmas for the nurse call-takers as they simultaneously dealt with parental expectations for help and the CHL guidelines indicating that offering medical advice was outside the remit of the service. • Most frequent reasons for calling were to discuss sleep, feeding, normative infant physical functions and parenting advice. • The average length of calls to the CHL was 7 minutes. • Longer calls were more likely to involve nurse call-takers giving advice on more than one topic, the caller displaying strong emotions, the caller not specifically providing the reason for the call, and the caller discussing parenting and developmental issues. • Shorter calls were characterised by the nurse suggesting that the child receive immediate medical attention, the nurse emphasising the importance or urgency of the plan of action, the caller referring to or requesting confirmation of a diagnosis, and caller and nurse call-taker discussion of health and medical issues. • The majority of calls, 92%, achieved parent-nurse alignment by the conclusion of the call. However, 8% did not. • The 8% of calls that were not aligned require further quantitative and qualitative investigation of the interactional features. The findings are pertinent in the current context where Child Health Line now resides within 13HEALTH. These findings indicate: 1. A high demand for parenting advice. 2. Nurse call-takers have a high level of competency in dealing with calls about parenting and normal child development, which is the remit of the CHL. 3. Nurse call-takers and callers achieve a high degree of alignment when both parties agree on a course of action. 4. There is scope for developing professional practice in calls that present difficulties in terms of call content, interactional behaviour and call closure. Recommendations of the project: 1. There are numerous opportunities for further research on interactional aspects of calls to the CHL, such as further investigations of the interactional features and the association of the features to alignment and nonalignment. The rich and detailed insights into the patterns of nurse-parent interactions were afforded by the audio-recording and analysis of calls to the CHL. 2. The regular recording of calls would serve as a way of increasing understanding of the type and nature of calls received, and provide a valuable training resource. Recording and analysing calls to CHL provides insight into the operation of the service, including evidence about the effectiveness of triaging calls. 3. Training in both recognising and dealing with problem calls may be beneficial. For example, calls where the caller showed strong emotion, appeared stressed, frustrated or troubled were less likely to be rated as aligned calls. In calls where the callers described being ‘at their wits end’, or responded to each proposed suggestion with ‘I’ve tried that’, the callers were fairly resistant to advice-giving. 4. Training could focus on strategies for managing calls relating to parenting support and advice, and parental well-being. The project found that these calls were more likely to be rated as being nonaligned. 5. With the implementation of 13HEALTH, future research could compare nurse-parent interaction following the implementation of triaging. Of the calls, 21% had both medical and parenting topics discussed and 5.3% discussed medical, parenting and information topics. Added to this, in 12% of calls, there was ambiguity between the caller and nurse call-taker as to whether the problem was medical or behavioural

Cognitive and behavioral symptoms in ALSFTD: detection, differentiation, and progression

Brief screening tools that detect and differentiate patients with amyotrophic lateral sclerosis and frontotemporal dementia (ALSFTD) from those more subtle cognitive or behavioral symptoms (ALS plus) and motor symptoms only (ALS pure) is pertinent in a clinical setting. The utility of 2 validated and data-driven tests (Mini-Addenbrooke’s Cognitive Examination [M-ACE] and Motor Neuron Disease Behavioral Scale [MiND-B]) was investigated in 70 ALS patients (24 ALSFTD, 19 ALS plus, and 27 ALS pure). More than 90% of patients with ALSFTD scored at or below the cutoff on the M-ACE, whereas this was seen in only about 20% of ALS patients without dementia. The MiND-B differentiated between ALS pure and ALS plus diagnostic categories. Rasch modeling of M-ACE and MiND-B items revealed early cognitive (fluency, memory recall) and behavioral (apathy) symptoms in ALSFTD. The combined use of the M-ACE and MiND-B detects patients with ALSFTD, differentiates along the ALS continuum, and offers insight into the progression of nonmotor symptomatology in ALSFTD