Dementia
Jane Higgs, Gill Wakley, Ruth Chambers, Clare Gerada in Demonstrating your Clinical Competence in Depression, Dementia, Alcoholism, Palliative Care and Osteoporosis, 2018
There remain around 10% of people with dementia arising from other conditions — about 5% for fronto-temporal dementia including Pick’s disease and another 5% for other dementias including acquired immunodeficiency syndrome (AIDS). Pick’s disease affects the front of the brain, leading to loss of judgement and loss of inhibitions.Dementia occurs in the majority of cases of Huntington’s disease. This is a hereditary brain disorder causing the loss of cells in the basal ganglia. This cell damage affects cognitive ability (thinking, judgement, memory), movement and emotional control. The symptoms appear gradually, usually in midlife, between the ages of 30 and 50 years.Some people with AIDS develop dementia in the later stages of the illness. The AIDS virus itself may attack certain brain cells, and people with AIDS may develop viral infections of the brain because of their weakened immune system.The symptoms of Creutzfeldt-Jakob disease (CJD) are similar to those of Alzheimer’s disease. An electroencephalogram (EEG) and analysis of the cerebrospinal fluid distinguishes the two conditions.
Clinical Theory and Skills EMIs
Michael Reilly, Bangaru Raju in Extended Matching Items for the MRCPsych Part 1, 2018
Dementia in Creutzfeldt-Jakob disease.Dementia in HIV disease.Dementia in Huntington’s disease.Dementia in general paralysis of the insane.Dementia in Parkinson’s disease.Multi-infarct dementia.Pick’s disease.Post-encephalitic parkinsonism.Pseudo-bulbar palsy.
Dementia in Movement Disorders
W. R. Wayne Martin in Functional Imaging in Movement Disorders, 2019
Any patient developing prominent behavioral symptoms or intellectual decline may at first be considered to have Alzheimer’s disease or Pick’s disease. Yet both of these disorders cause distinctive patterns of cerebral glucose hypometabolism. PET may therefore be of diagnostic utility in demented patients whose motor abnormalities have been overlooked. Alzheimer’s disease causes a decline in glucose throughout the cerebral cortex, in the putamen, thalamus, and caudate nucleus, but most prominently in the temporoparietal association areas (Plate 9*).25,66,68 This pattern makes it clearly distinguishable from PSP.69 Pick’s disease also shows distinctive changes by PET. In this disorder, hypometabolism is seen predominantly in the frontal cortex.70,71 Thus, among the movement disorders associated with dementia, confusion would only occur between Parkinson’s disease and Alzheimer’s disease or between Pick’s disease and PSP if PET alone were relied upon for diagnosis. In the case of Pick’s disease, glucose metabolism in subcortical structures has not yet been described, and therefore further experience may permit PSP and Pick’s disease to be distinguished by PET.
Posterior cortical atrophy: clinical, neuroimaging, and neuropathological features
Published in Expert Review of Neurotherapeutics, 2023
John Best, Marianne Chapleau, Gil D. Rabinovici
The purpose of this review is to provide readers with an overview of Posterior Cortical Atrophy syndrome, including clinical, imaging, pathological, and genetic features, and treatments. Posterior cortical atrophy (PCA) is a term first introduced by Frank Benson and colleagues in 1988 [1]. Benson’s original case series described five patients with early and prominent visual dysfunction, including alexia and visual agnosia. The patients all developed progressive visuospatial and navigational dysfunction (environmental agnosia) with preserved visual acuity. Eventually, most parietal lobe functions were affected, though memory was notably spared until late disease stages. Atrophy of posterior association cortices was noted on computed tomography (CT) or magnetic resonance imaging (MRI). Benson hypothesized that this newly described dementia syndrome represented an atypical presentation of Alzheimer’s disease, Pick’s disease, or possibly a novel clinicopathological entity.
Mixed neuropathology in frontotemporal lobar degeneration
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2020
Catherine Pennington, Luca Marini, Elizabeth Coulthard, Seth Love
Tauopathies are frequently seen as co-existent or incidental pathologies in patients with other forms of neurodegeneration (23,24) but the incidence of mixed pathology in those with a primary tauopathy is understudied. An autopsy series of 33 people with neuropathologically diagnosed CBD found coexisting Alzheimer’s spectrum pathology in 3 cases (25). An archival review of 66 cases of Pick’s disease identified proteinopathy consistent with low or intermediate level probability of AD in 3 cases, and occasional alpha-synuclein immunopositivity (26). In our cohort, most of those with FTLD-tau had no other neuropathology present. Why mixed neuropathology is less frequent in FTLD-tau than FTLD-TDP-43 is unclear; this discrepancy was not explained by age at death, or by an excess of any one particular neuropathology with FTLD-TDP.
Glycogen synthase kinase 3 (GSK-3) inhibitors: a patent update (2016–2019)
Published in Expert Opinion on Therapeutic Patents, 2020
Carlos Roca, Nuria E. Campillo
Thanks to the capacity of the GSK-3 inhibitors to improve hematopoiesis and neural stem cell self-renewal and differentiation capability, Wuxi Hanquiang Pharmaceutical Tech Co LTD has claimed the use of GSK3 inhibitors in preparing a drug to treat Niemann-Pick disease type C (NPC) [57]. NPC disease is a progressive and life limiting autosomal recessive disorder caused by mutations in either the NPC1 or NPC2 gene. NPC is a rare genetic disease whose clinical spectrum ranges from a fatal antenatal disorder to an adult-onset chronic neurodegenerative disease [58]. Wuxi Hanquiang Pharmaceutical Tech Co LTD identified an abnormal increase of GSK3 activity associated with NPC. They showed that GSK3 inhibitors have a positive effect in treating Niemann-Pick disease type C.
Related Knowledge Centers
- Frontotemporal Lobar Degeneration
- Progressive NONfluent Aphasia
- Semantic Dementia
- Dementia
- Frontal Lobe
- Temporal Lobe
- Neuron
- Von Economo Neuron
- Primary Progressive Aphasia
- Corticobasal Syndrome