The Toolbox Barkhof, Frederik; Fox, Nick C.; Bastos-Leite, António J. ...
Neuroimaging in Dementia,
10/2010
Book Chapter
The focus of imaging in patients suspected of having dementia has shifted from an exclusionary to an inclusionary approach over the past decades. Exclusion of a (surgically) treatable cause of ...dementia (e.g. tumour or subdural haematoma) can be ascertained by using CT, but demonstration of positive disease markers (e.g. hippocampal atrophy for Alzheimer’s disease AD) becomes increasingly more relevant in an era where disease-specific pharmacological treatment is becoming available. It is in this regard – adding positive predictive value to the diagnosis in dementia – that MRI offers clear benefits over CT. Catheter angiography (DSA) is hardly ever indicated, except perhaps for suspicion of vasculitis that cannot be confirmed otherwise.
In addition to volume loss and signal change, structural MRI allows an assessment of ‘brain swelling’ – we use this here as an umbrella term to cover a range of conditions with apparent increases in ...volume (or pressure) of a part of the brain or a cerebrospinal fluid (CSF) compartment – the brain may not be literally ‘swollen’ in all of these.
Primary Grey Matter Loss Barkhof, Frederik; Fox, Nick C.; Bastos-Leite, António J. ...
Neuroimaging in Dementia,
10/2010
Book Chapter
Many dementias are characterised by grey matter (GM) abnormalities; these are usually, but not exclusively, atrophy. AD is the most prevalent and the prototypic cortical GM dementia. GM atrophy ...reflects a loss of neurons irrespective of the underlying protein defect (amyloid, tau, alpha-synuclein); atrophy may be generalised or focal, and the pattern of atrophy may be diagnostic in itself. The current chapter focuses on disorders principally affecting GM, encompassing, besides AD, the growing class of frontotemporal lobar degeneration syndromes, including FTD and PPA, CBD and PSP, as well as PD with dementia as in DLB, Huntington’s disease and many less common diseases. A special chapter is dedicated to the prion diseases that typically affect the more basal grey matter structures as well.
Description of the demographic, clinical and neuropathological features of 11 cases of Creutzfeldt-Jakob disease (CJD).
Review of the clinical and neuropathological features of patients with CJD ...diagnosed in hospitals in the North of Portugal between 1993 and 2002.
Eleven patients were identified, 4 females: mean age of onset of symptoms--64 years, mean duration of disease--8 months. All presented with a syndrome of progressive dementia with myoclonus, with four patients presenting with cerebellar signs. Neuropathological examination of brain at autopsy showed spongiosis and reactive gliosis associated with neuronal loss. In eight cases immunocytochemistry for prion protein (PrP) was carried out and was positive.
The group of patients described represents the heterogeneity of the clinical phenotypes possible in CJD. Neuropathological examination is still indispensable to make the definitive diagnosis of the disease.
INTRODUÇÃO E OBJETIVO: Descrição das características demográficas, clinicas e neuropatológicas de 11 doentes com doença de Creutzfeldt-Jakob (DCJ). MÉTODO: Revisão clínica e neuropatológica de ...doentes com DCJ diagnosticados entre 1993 e 2002 em hospitais do Norte de Portugal. RESULTADOS: Foram identificados 11 doentes (4 do sexo feminino; idade média de início dos sintomas, 64 anos; média de duração da doença, 8 meses). Todos apresentaram síndrome demencial progressiva associada a mioclonias, sendo a síndrome cerebelar a forma de apresentação inicial em quatro deles. O estudo neuropatológico revelou sempre espongiose e gliose reativa associada a perda neuronal. O estudo imunocitoquímico para proteína priônica (PrP) foi positivo nos oito casos em que foi executado. CONCLUSÃO: O grupo de doentes descritos constitui uma série clinica representativa da heterogeneidade de fenótipos possíveis da DCJ esporádica. O estudo neuropatológico é ainda indispensável para o diagnóstico definitivo da doença.
OBJECTIVE: Description of the demographic, clinical and neuropathological features of 11 cases of Creutzfeldt-Jakob disease (CJD). METHOD: Review of the clinical and neuropathological features of patients with CJD diagnosed in hospitals in the North of Portugal between 1993 and 2002. RESULTS: Eleven patients were identified, 4 females: mean age of onset of symptoms - 64 years, mean duration of disease - 8 months. All presented with a syndrome of progressive dementia with myoclonus, with four patients presenting with cerebellar signs. Neuropathological examination of brain at autopsy showed spongiosis and reactive gliosis associated with neuronal loss. In eight cases immunocytochemistry for prion protein (PrP) was carried out and was positive. CONCLUSION: The group of patients described represents the heterogeneity of the clinical phenotypes possible in CJD. Neuropathological examination is still indispensable to make the definitive diagnosis of the disease.
How to Use This Book? Barkhof, Frederik; Fox, Nick C.; Bastos-Leite, António J. ...
Neuroimaging in Dementia,
10/2010
Book Chapter
The focus of this book is on the practical use of neuroimaging in dementia in a clinical diagnostic setting. We felt that there are many publications that describe the imaging findings of a ...particular disease, but you first need to know what the diagnosis is in order to look up articles describing those findings. As well as offering a summary of the findings in the most relevant conditions causing cognitive decline we wished to provide a guide to interpreting a particular imaging finding. The organization of the book therefore takes as departure point the dominant imaging findings and incorporates the clinical features along the way. The topic of vascular dementia does not easily fit with this approach due to its heterogeneous appearance; however, it does form a natural bridge between primary white and grey matter disorders. The ‘route map’ aims to direct the reader towards additional tests (imaging and non-imaging) and clinical features in a practical way.
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