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.
The nervous system and the eye
C. Simon Herrington in Muir's Textbook of Pathology, 2020
Creutzfeldt–Jakob disease is due to an unconventional transmissible agent that is very resistant to normal disinfecting procedures such as standard autoclaving. It appears to have no nucleic acid but to be composed only of protein, hence the term prion. The disease process is characterized by the conversion of a normal cellular protein (PrPc) into an abnormal isoform (PrPsc) by a change in conformation. The PrPsc that accumulates in affected tissue is derived from the host PrP gene and can be detected by immunohistochemistry. The relationship between transmissibility and genetic factors is not yet entirely clear. However, there are thought to be three ways in which PrPsc can form: first, by a point mutation in the PrP gene rendering the protein more likely to misfold to form PrPsc and initiate the disease – this occurs in the familial form of CJD. Second, the presence of PrPsc induces the conversion of PrPc into more PrPsc – this occurs when the disorder is transmitted. Third, sporadic CJD occurs, presumably, when PrPsc is formed from PrPc as a chance event in the aged brain. Homozygosity at codon 129, which codes for either methionine or valine, of the host PrP gene appears to represent a genetic susceptibility factor.
Infection Prevention: 2020 Review and Update for Neurodiagnostic Technologists
Published in The Neurodiagnostic Journal, 2020
Anna M. Bonner, Petra Davidson
One rare disorder frequently requiring successive EEG studies is Creutzfeldt-Jakob Disease. Creutzfeldt-Jakob Disease (CJD) is neither bacterial nor viral; it is a rare, but transmissible spongiform encephalopathy that is prion-based (prion: derived from “protein” and “infectious”) and known to be fatal. CJD affects approximately one person per million annually worldwide, 350 cases of which occur in the US (NIH 2019). There are three modes by which CJD is transmitted: Sporadic CJD is the most common form, accounting for more than 80% of cases, and as the name implies, this form of CJD appears without known cause or risk factors for the disease.Hereditary CJD accounts for approximately 10–15% of cases in the US and transmission is genetically based.Acquired CJD accounts for fewer than 1% of all cases (approximately 250 patients worldwide) and occurs when the disease is transmitted by exposure to the brain or nervous system tissue, such as during a medical procedure or surgery (NIH 2019; CDC 2019a).
Proceedings of the 44th Annual Upper Midwest Neuro-Ophthalmology Group Meeting
Published in Neuro-Ophthalmology, 2023
Negar Moheb, Adam Baim, Collin McClelland, John. J. Chen
Khawla Abusamra, MBBS, University of Kentucky, presented a case of a 75-year-old woman, who was evaluated for progressive bilateral vision loss, intermittent binocular horizontal diplopia, and encephalopathy. Her visual symptoms continued despite bilateral cataract surgery. She underwent an unrevealing full stroke work up. Over the next 8 weeks she developed prosopagnosia, visual agnosia, unsteady gait, numbness, generalised weakness, dysphagia, visual hallucinations, abnormal body postures, myoclonic jerks and she eventually became mute. Serological and CSF studies were notable for an elevated CSF protein, but otherwise she had a negative infectious and autoimmune work up. Repeat brain MRI revealed diffuse T2 hyperintensities and restricted diffusion with cortical ribboning throughout the cerebral cortex and parts of the deep grey nuclei, which were evident on the initial MRI in retrospect. She was diagnosed with Creutzfeldt-Jakob disease (CJD) and passed away within 2 weeks of diagnosis. Real-time quaking-induced conversion testing was indeterminate and was felt to be the result of a traumatic lumbar puncture. This case showed the typical clinical features and MRI findings of the Heidenhain variant of CJD with visual cortical and parietal predominant cortical ribboning pattern. CJD should be in the differential diagnosis for patients with rapidly progressive vision loss, cognitive decline, and other cortical signs.
Atypical and early symptoms of sporadic Creutzfeldt – Jakob disease: case series and review of the literature
Published in International Journal of Neuroscience, 2021
Grammatiki Katsikaki, Ioannis E. Dagklis, Petros Angelopoulos, Dimitrios Ntantos, Angeliki Prevezianou, Sevasti Bostantjopoulou
Spongiform encephalopathies are a group of rare and lethal neurodegenerative diseases of the Central Nervous System, caused by accumulation of a misfolded pathologic prion protein (PrPSc) [1–3]. Creutzfeldt-Jakob disease (CJD) is the most common prion disorder and is classified as sporadic (sCJD), genetic/familial or acquired, which can be further subdivided into variant, Kuru disease and iatrogenic [4]. Approximately 85–90% of CJD cases are sporadic and affect 1–1.5 out of 1 million people per year [4]. Pathogenesis of sCJD is not well established and possible mechanisms include spontaneous prion protein transformation or somatic gene mutation [2,4]. Sporadic CJD usually presents in the seventh decade and the mean illness duration is about 5 months [5–7].
Related Knowledge Centers
- Blood Transfusion
- Central Nervous System Disease
- Coma
- Heredity
- Prion
- Protein Folding
- Dementia
- Neurodegenerative Disease
- Dominance
- Variant Creutzfeldt–Jakob Disease