"following administration of cadaveric human pituitary hormones (pre-1985), or following various transplants and other procedures (e.g. cadaveric dural grafting with products such as Lyodura)"
"T1: may show high signal in globus pallidus (uncommon) 25,30"
"T1 C+ (Gd): no abnormal enhancement"
"Nuclear medicine "
"especially if in the UK in the late-1980s to mid-1990s"
"diagnosed by standard neuropathological examinationAND/OR"
"immunocytochemicallyAND/OR"
"Western blot confirmed protease-resistant PrPAND/OR"
"neuropsychiatric disorderANDpositive RT-QuIC in cerebrospinal fluid (CSF) or other tissuesOR"
"rapidly progressive dementiaANDat least two out of the following four clinical features: myoclonus, visual or cerebellar signs, pyramidal/extrapyramidal signs, akinetic mutismANDa positive result on at least one of the following laboratory tests: typical EEG, positive 14-3-3 CSF assay with disease duration"
"at least two out of the four clinical features (as above)AND"
"definite or probable Creutzfeldt-Jakob disease plus definite or probable Creutzfeldt-Jakob disease in a first degree relativeAND/OR"
"CSF and/or olfactory mucosa real-time quaking-induced conversion (RT-QuIC) seeding assays: detects minute amounts of the disease-specific pathologic prion protein 12,13"
"Creutzfeldt-Jakob disease is a notifiable disease in most countries, including European Union countries, Australia, United Kingdom, USA, and Canada."
"cortical changes: rarely reported (e.g. AMPAR antibodies 34)"
"Western blot confirmed protease-resistant PrPAND/OR"
"Brownell-Oppenheimer variant: initial cerebellar ataxia"
"Heidenhain variant: initial visual symptoms such as impaired visual acuity, distortions of shapes and colours, and visual hallucinations"
"Stern-Garcin variant: initial extrapyramidal features"
"PrPSc type 1 (21 kDa) or type 2 (19 kDa)"
"Brownell-Oppenheimer variant: initially involves the cerebellum and sometimes basal ganglia, and may not be visible until atrophy develops 22,23"
"Heidenhain variant: initially involves the parieto-occipital cortex"
"Stern-Garcin variant: initially involves the basal ganglia (striatum) and thalamus"
"Sporadic Creutzfeldt-Jakob disease"
"Variant Creutzfeldt-Jakob disease"
"Clinical patterns have been linked to various molecular subtypes (e.g. Heidenhain variant linked to MM1 and MM2C 18, and Brownell-Oppenheimer variant linked to VV2 19,20)."
Expected headings
"Sporadic Creutzfeldt-Jakob disease"
"Variant Creutzfeldt-Jakob disease"
"Nuclear medicine "
"following administration of cadaveric human pituitary hormones (pre-1985), or following various transplants and other procedures (e.g. cadaveric dural grafting with products such as Lyodura)"
"Clinical patterns have been linked to various molecular subtypes (e.g. Heidenhain variant linked to MM1 and MM2C 18, and Brownell-Oppenheimer variant linked to VV2 19,20)."
"cortical changes: rarely reported (e.g. AMPAR antibodies 34)"
"rapidly progressive dementiaANDat least two out of the following four clinical features: myoclonus, visual or cerebellar signs, pyramidal/extrapyramidal signs, akinetic mutismANDa positive result on at least one of the following laboratory tests: typical EEG, positive 14-3-3 CSF assay with disease duration"
"rapidly progressive dementiaANDat least two out of the following four clinical features: myoclonus, visual or cerebellar signs, pyramidal/extrapyramidal signs, akinetic mutismANDa positive result on at least one of the following laboratory tests: typical EEG, positive 14-3-3 CSF assay with disease duration"
"Each variant of sporadic Creutzfeldt-Jakob disease results from the combination of codon 129 genotype (M or V) and PrPSc type (1, 2 or 1 + 2) 16,17."
"less common ("
"rapidly progressive dementiaANDat least two out of the following four clinical features: myoclonus, visual or cerebellar signs, pyramidal/extrapyramidal signs, akinetic mutismANDa positive result on at least one of the following laboratory tests: typical EEG, positive 14-3-3 CSF assay with disease duration"
"Sporadic Creutzfeldt-Jakob disease is characterised by rapidly progressive dementia and other features of neuropsychiatric decline resulting in death within a year of onset. Other common central features include myoclonus, visual hallucinations, cerebellar dysfunction (such as ataxia and nystagmus), pyramidal or extrapyramidal signs (such as spasticity, rigidity, dystonia, or bradykinesia), and eventually akinetic mutism 22. Peripheral nervous system involvement can be seen in 10% of cases 31."
"common (~80-90%): precuneus, cuneus, paracentral lobule, medial frontal gyrus, occipital gyri, angular/supramarginal gyrus, superior parietal lobule, inferior frontal gyrus"
"There is no curative treatment and the disease is invariably fatal with sporadic CJD having a median survival of 4-6 months, with lower life expectancies observed in older patients 9,26. The median survival tends to be longer in patients with familial CJD 33."