Cerebral Function Unit

Salford, United Kingdom

Cerebral Function Unit

Salford, United Kingdom
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Ghosh A.,Apollo Gleneagles Hospitals | Dutt A.,Apollo Gleneagles Hospitals | Bhargava P.,Apollo Gleneagles Hospitals | Snowden J.,Cerebral Function Unit
Journal of Neurology | Year: 2013

Environmental dependency (ED) behaviours, such as imitation behaviour (IB) and incidental utilization behaviour (UB), are considered pathognomonic of a frontal lesion and can play a unique role in diagnosing behavioural variant frontotemporal dementia (bvFTD). However, with only few focused observations of ED behaviour reported in earlier studies, their roles in the diagnosis of bvFTD have so far remained supportive. In this observational study, conducted in the cognitive clinic of a tertiary-care hospital, we explored the hypotheses that a focused and systematic search could uncover more ED behaviours in patients with bvFTD, and that the presence of ED behaviours such as incidental UB and IB should allow us to cleanly differentiate bvFTD from AD. Forty-one bvFTD patients and 75 probable AD patients, all diagnosed using accepted criteria, were seen by a neurologist and a neuropsychologist. Information regarding ED behaviour was obtained from the caregiver's history, observations for spontaneous behaviour and induction of the behaviour in the clinic. All ED behaviours were significantly more frequent in bvFTD compared with AD. UB (78 %; 66 % incidental) and IB (59 %) occurred exclusively in bvFTD. Multi-pronged and focused clinical assessment contributed to the high frequency of ED behaviours. Nearly two-thirds of bvFTD patients, but none with AD, showed three or more ED behaviours. We concluded that ED behaviours are more common in bvFTD than is currently recognized. UB, IB or three ED behaviours, if present, could clearly differentiate bvFTD from AD. A focused search should consistently uncover ED behaviours in bvFTD patients. © 2012 Springer-Verlag Berlin Heidelberg.


Snowden J.S.,Cerebral Function Unit | Snowden J.S.,University of Manchester | Kindell J.,The Meadows | Thompson J.C.,Cerebral Function Unit | And 5 more authors.
Cortex | Year: 2012

Primary progressive aphasia is clinically heterogeneous. We report a patient, alias Don, with a novel form of progressive aphasia, characterised by deep dyslexia and dysgraphia and dissociated access to phonological and orthographic word forms. The hallmarks of deep dyslexia and dysgraphia were present early in the course and persisted over time. Writing was initially poorer than reading, but this reversed over time. There was a lack of concordance between reading and writing errors. Don benefited from a semantic mediation strategy to learn letter sounds, involving associating letters with a country name (e.g., A = Afghanistan). Remarkably, he continued to be able to generate those phonologically complex country names when no longer able to name or sound letters. Don's performance is compatible with a traditional dual-route account of deep dyslexia and dysgraphia. The findings have potential practical implications for speech and language therapy in progressive aphasia. Moreover, they illustrate both the remarkable specificity yet clinical diversity in presentation of progressive aphasia. © 2012 Elsevier Srl.


Vardy E.R.L.C.,University of Manchester | Vardy E.R.L.C.,Cerebral Function Unit | Kellett K.A.B.,University of Leeds | Cocklin S.L.,University of Leeds | Hooper N.M.,University of Leeds
Neurodegenerative Diseases | Year: 2012

Background: Tissue non-specific alkaline phosphatase (TNAP) has been shown to promote the neurotoxicity of extracellular tau which contributes to the spread of pathology in Alzheimer's disease (AD). Objective: To investigate changes in TNAP activity in the hippocampus in both sporadic and familial AD, and to examine whether changes in neuronal TNAP are reflected systemically by looking at changes in plasma TNAP activity in AD. Methods: We measured the activity of TNAP in the hippocampus in sporadic AD, familial AD and appropriate age-matched controls, and in an ageing series (age: 25-88 years) of brains. In addition, we measured TNAP activity in plasma from 110 AD and 110 non-demented control participants. Results: TNAP activity was significantly increased in the hippocampus in sporadic (by 56%; p = 0.038) and familial AD (by 121%; p = 0.042) compared with the age-matched controls. However, there was no correlation of TNAP activity with age. Furthermore, plasma TNAP activity was increased in AD (by 13%; p = 0.018) and inversely correlated with cognitive function (r s = -0.211; p = 0.027). Conclusion: Together, these data indicate that TNAP is increased in both sporadic and familial AD but not in the aged brain, indicating that the increase is likely a consequence of AD-associated changes in the brain. The neuronal change in TNAP is reflected in an increase in plasma TNAP in AD and is inversely correlated with cognitive function. Copyright © 2011 S. Karger AG, Basel.


Snowden J.S.,Cerebral Function Unit | Snowden J.S.,University of Manchester | Thompson J.C.,Cerebral Function Unit | Thompson J.C.,University of Manchester | And 2 more authors.
Behavioural Neurology | Year: 2012

It is generally accepted that the anterior temporal lobes support knowledge of famous people. The specific roles of the right and left temporal lobe remain a subject of debate, with some studies suggesting differential roles based on modality (visual versus verbal information) and others category (person knowledge versus general semantics). The present study re-examined performance of semantic dementia patients with predominantly right and predominantly left temporal lobe atrophy on famous face, famous name and general semantic tasks, with the specific aim of testing the hypothesis that the right temporal lobe has a privileged role for person knowledge and the left temporal lobe for general semantic knowledge. Comparisons of performance rankings across tasks showed no evidence to support this hypothesis. By contrast, there was robust evidence from naming, identification and familiarity measures for modality effects: right-sided atrophy being associated with relatively greater impairment for faces and visual tasks and left-sided atrophy for names and verbal tasks. A double dissociation in test scores in two patients reinforced these findings. The data present a challenge for the influential 'semantic hub' model, which views the anterior temporal lobes as an area of convergence in which semantic information is represented in amodal form. © 2012-IOS Press and the authors. All rights reserved.


Kobylecki C.,Cerebral Function Unit | Kobylecki C.,University of Manchester | Jones M.,Cerebral Function Unit | Jones M.,University of Manchester | And 12 more authors.
Journal of Neurology | Year: 2015

Cognitive impairment is common in patients with the neurodegenerative tauopathy progressive supranuclear palsy (PSP). Although a pattern of ‘subcortical’ cognitive impairment is considered prototypical in PSP, pathological and clinical observations suggest an overlap with frontotemporal dementia (FTD). Our objective was to evaluate behavioural and cognitive symptoms in a retrospective study of patients with PSP syndrome (PSPS) and their relationship to features seen in behavioural variant FTD. We reviewed the records of 62 patients (29 male, 33 female, median age 65.5 years) evaluated at a tertiary cognitive clinic who met NINDS–SPSP criteria for probable or possible PSP, and collected clinical details of their presenting history, cognitive and behavioural features. We also evaluated the proportion of patients fulfilling FTD Consensus criteria. Cognitive and behavioural symptoms were a predominant presenting feature in 58 % of patients evaluated. Cognitive slowing, executive impairments, and inefficient memory recall, consistent with ‘subcortical’ impairment, were identified in the majority of patients. Twenty patients (32 %) fulfilled cognitive and behavioural criteria for possible FTD at initial assessment, whereas behavioural changes not meeting formal diagnostic criteria were present in a greater proportion of the patients. Our findings support the existence of a spectrum of cognitive–behavioural features in PSPS, with significant clinical overlap with behavioural variant FTD. Cognitive and behavioural profiling should be an integral part of the assessment of patients with PSPS. © 2015, Springer-Verlag Berlin Heidelberg.


Snowden J.S.,Cerebral Function Unit | Snowden J.S.,University of Manchester | Thompson J.C.,Cerebral Function Unit | Thompson J.C.,University of Manchester | And 9 more authors.
Brain | Year: 2011

Accuracy of clinical diagnosis of dementia is increasingly important for therapeutic and scientific investigations. In this study, we examine diagnostic accuracy in a consecutive series of 228 patients referred to a specialist early-onset dementia clinic, whose brains were subsequently examined at post-mortem. Diagnosis was based on structured history, neurological examination and neuropsychological assessment, with emphasis on qualitative as well as quantitative aspects of performance. Neuroimaging provided support for but did not alter the clinical diagnosis. We set out the principles that guided diagnosis: (i) time course of illness; (ii) weighting of physical, behavioural and cognitive symptoms and signs; (iii) 'anterior' versus 'posterior' hemisphere character of cognitive change; and (iv) specificity of deficit, paying attention to the differentiation between syndromes of frontotemporal lobar degeneration and focal forms of Alzheimer's disease. Forty-two per cent of the patients had clinical diagnoses of one of the syndromes of frontotemporal lobar degeneration, the high proportion reflecting the research interests of the group. Forty-six per cent were diagnosed with Alzheimer's disease and the remaining patients, dementia with Lewy bodies, Creutzfeldt-Jakob disease, vascular or unclassified dementia. Frontotemporal lobar degeneration was identified with 100 sensitivity and 97 specificity and Alzheimer's disease with 97 sensitivity and 100 specificity. Patients with other pathologies were accurately identified on clinical grounds. Examination of subsyndromes of frontotemporal lobar degeneration showed a relatively predictable relationship between clinical diagnosis and pathological subtype. Whereas the behavioural disorder of frontotemporal dementia was associated with tau, transactive response DNA binding protein 43 and fused-in-sarcoma pathology, cases of frontotemporal dementia with motoneuron disease, semantic dementia and, with one exception, progressive non-fluent aphasia were associated with transactive response DNA binding protein 43 pathology, distinguished by ubiquitin subtyping (types B, C and A, respectively). Clinical diagnoses of progressive apraxia, corticobasal degeneration and progressive supranuclear palsy were, with one exception, associated with Pick, corticobasal and progressive supranuclear palsy subtypes of tau pathology, respectively. Unanticipated findings included Alzheimer pathology in two patients presenting with the behavioural syndrome of frontotemporal dementia and corticobasal pathology in four others with clinical frontotemporal dementia. Notwithstanding such anomalies, which serve as a reminder that there is not an absolute concordance between clinical phenotype and underlying pathology, the findings show that dementias can be distinguished in life with a high level of accuracy. Moreover, careful clinical phenotyping allows prediction of histopathological subtype of frontotemporal lobar degeneration. The principles guiding diagnosis provide the foundation for future prospective studies. © 2011 The Author.


Vardy E.,Newcastle Upon Tyne Hospitals | Vardy E.,Northumbria University | Holt R.,Pinderfields Hospital | Gerhard A.,University of Manchester | And 7 more authors.
International Journal of Geriatric Psychiatry | Year: 2014

Background Delirium is common and is associated with an increased risk of dementia. However, it is not clear whether delirium confers increased risk of any particular type of dementia. We performed a retrospective study of Alzheimer's disease (AD) and Dementia with Lewy bodies (DLB) to ascertain whether a suspected episode of preceding delirium was more common prior to diagnosis in either type of dementia. Methods The study was carried out in a tertiary referral unit for the diagnosis of dementia. Clinic letters from the first presentation to the unit of 85 cases with DLB and 95 cases of AD were reviewed for documentation of any previous episodes of suspected delirium. Results In this study, 25% of DLB cases had at least one reported episode of suspected delirium as compared to 7% of AD cases (p = 0.001). For the DLB cases who had a prior suspected delirium, 23% had more than one episode compared with 14% of the AD group. The median time between most recent suspected episode of delirium and diagnosis of dementia in both groups was less than a year Conclusions A greater proportion of those presenting and diagnosed with DLB had a documentation of a suspected delirium than those diagnosed with AD. Delirium may lead to a higher risk of DLB as opposed to other forms of dementia, or delirium may, at least in some cases, represent the early stages of DLB. These data suggest that a diagnosis of DLB should be specifically considered in those presenting with a delirium. Copyright © 2013 John Wiley & Sons, Ltd.


Thompson J.C.,Cerebral Function Unit | Thompson J.C.,University of Manchester | Harris J.,Cerebral Function Unit | Harris J.,University of Manchester | And 10 more authors.
Journal of Neuropsychiatry and Clinical Neurosciences | Year: 2012

A group of 111 patients with Huntington's disease (HD) underwent a minimum of three annual neuropsychiatric assessments, using the Problem Behaviors Assessment for Huntington's Disease (PBA-HD). Longitudinal prevalence of neuropsychiatric symptoms was notably higher than baseline prevalence, suggesting that previous studies may have underestimated the extent of this clinical problem. Moreover, apathy, irritability, and depression were each associated with distinct longitudinal profiles. Apathy progressed over time and across disease stages. Irritability also increased significantly, but only in early stages of HD. Depression did not increase significantly at any stage of disease. The neuropsychiatric syndrome of apathy appears to be intrinsic to the evolution and progression of HD. © 2012 American Psychiatric Association.


Shi J.,Beijing University of Chinese Medicine | Wei M.,Beijing University of Chinese Medicine | Tian J.,Beijing University of Chinese Medicine | Snowden J.,Cerebral Function Unit | And 10 more authors.
BMC Psychiatry | Year: 2014

Background: Decline in verbal episodic memory is a core feature of amnestic mild cognitive impairment (aMCI). The delayed story recall (DSR) test from the Adult Memory and Information Processing Battery (AMIPB) discriminates MCI from normal aging and predicts its conversion to Alzheimer's dementia. However, there is no study that validates the Chinese version of the DSR and reports cut-off scores in the Chinese population.Methods: A total of 631 subjects were screened in the memory clinics of Dongzhimen Hospital, Beijing University of Chinese Medicine, China. 249 were considered to have normal cognition (NC), 134 met diagnostic criteria for MCI according to the MCI Working Group of the European Consortium on Alzheimer's Disease, and 97 met criteria for probable Alzheimer's disease (AD) according to the NINCDS/ADRDA criteria, 14 exhibited vascular dementia (VaD), and 50 had a diagnosis of another type of dementia. Receiver operating characteristic (ROC) curve analyses were used to calculate the story recall cutoff score for detecting MCI and AD. Normative data in the NC group were obtained as a function of age and education.Results: In this Chinese sample, the normative mean DSR score was 28.10 ± 8.54 in the 50-64 year-old group, 26.22 ± 8.38 in the 65-74 year-old group, and 24.42 ± 8.38 in the 75-85 year-old group. DSR performance was influenced by age and education. The DSR test had high sensitivity (0.899) and specificity (0.799) in the detection of MCI from NC using a cut-off score of 15.5. When the cutoff score was 10.5, the DSR test obtained optimal sensitivity (0.980) and specificity (0.938) in the discrimination of AD from NC. Cutoff scores and diagnostic values were calculated stratified by age and education.Conclusions: The Chinese version of the DSR can be used as a screening tool to detect MCI and AD with high sensitivity and specificity, and it could be used to identify people at high risk of cognitive impairment. © 2014 Shi et al.; licensee BioMed Central Ltd.


PubMed | Cerebral Function Unit
Type: Journal Article | Journal: Journal of neurology | Year: 2015

Cognitive impairment is common in patients with the neurodegenerative tauopathy progressive supranuclear palsy (PSP). Although a pattern of subcortical cognitive impairment is considered prototypical in PSP, pathological and clinical observations suggest an overlap with frontotemporal dementia (FTD). Our objective was to evaluate behavioural and cognitive symptoms in a retrospective study of patients with PSP syndrome (PSPS) and their relationship to features seen in behavioural variant FTD. We reviewed the records of 62 patients (29 male, 33 female, median age 65.5 years) evaluated at a tertiary cognitive clinic who met NINDS-SPSP criteria for probable or possible PSP, and collected clinical details of their presenting history, cognitive and behavioural features. We also evaluated the proportion of patients fulfilling FTD Consensus criteria. Cognitive and behavioural symptoms were a predominant presenting feature in 58% of patients evaluated. Cognitive slowing, executive impairments, and inefficient memory recall, consistent with subcortical impairment, were identified in the majority of patients. Twenty patients (32%) fulfilled cognitive and behavioural criteria for possible FTD at initial assessment, whereas behavioural changes not meeting formal diagnostic criteria were present in a greater proportion of the patients. Our findings support the existence of a spectrum of cognitive-behavioural features in PSPS, with significant clinical overlap with behavioural variant FTD. Cognitive and behavioural profiling should be an integral part of the assessment of patients with PSPS.

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