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15 Creutzfeldt-Jakob Disease and Other Prion Disorders

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Abstract:

Creutzfeldt-Jakob disease (CJD) is a rare, progressive, fatal, degenerative disease of the central nervous system (CNS) with the pathological features of multifocal neuronal loss, gliosis, and parenchymal spongy change. A further pathological feature seen occasionally in sporadic CJD (sCJD), more commonly in scrapie and kuru, and invariably in some inherited forms of prion disease and vCJD is the presence of microscopic plaques of amyloidlike material. Abnormalities in the eponymic forms of inherited CJD, fatal familial insomnia (FFI) and GSS , include sleep disturbance and dysautonomia for FFI and ataxia and dementia for GSS. Iatrogenic forms that have sufficient numbers of cases to review include patients who have received cadaveric growth hormone or cadaveric dura mater. A consolidated table of clinical features found across the several forms of human prion disease is presented and provides a basis for the descriptions of the specific types of prion disorders. However, across all types of prion disorders, subacute dementia, ataxia, and psychiatric disorders are the triad of most common clinical features. Familial CJD , FFI, and GSS will be considered next, followed by transmission associated with ingestion of contaminated proteins which includes cannibalism and consumption of bovine spongiform encephalopathy (BSE)-contaminated beef. Although the risk of transmissibility in most health care settings is negligible, comprehensive institutionwide evaluation for risk and implementation of infection control guidelines for the human prion disorders are necessary.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.1
FIGURE 15.1

Coronal slice through fixed brain from a case of CJD. There is only minimal cerebral atrophy with ventricular dilation and prominent sulci.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.2
FIGURE 15.2

Low-power view of cerebral cortex in CJD showing mild patchy spongiform change, mainly in layers 2 and 3. Hematoxylin and eosin stain.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.3
FIGURE 15.3

Low -power view of cerebral cortex showing more extensive spongiform change. PTAH stain.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.4
FIGURE 15.4

Low-power view of cerebral cortex from a healthy control (a) and a case of C JD (b). In CJD, neurons are lost and the cortical organization is haphazard. Small nuclei are those of glial cells. Arrows in (a) and (b) indicate the pial surface. Nissl stain.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.5
FIGURE 15.5

Reactive gliosis in the putamen in a case of CJD. Cajal's stain.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.6
FIGURE 15.6

Cerebellar cortex containing kuru plaques in the molecular layer (above). Thioflavine T stain photographed under fluorescent light.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.7
FIGURE 15.7

“Florid” plaque in vCJD surrounded by spongiform vacuoles.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.8
FIGURE 15.8

PrP immunostained deposits (arrows) in cortex in sCJD.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.9
FIGURE 15.9

PrP immunostained plaques in cerebellum in inherited ataxic form of CJD.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.10
FIGURE 15.10

PrP immunostaining of vCJD cerebellar cortex.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.11
FIGURE 15.11

Peridendritic pattern of immunostaining for PrP in the basal ganglia from a case of vCJD.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.12
FIGURE 15.12

Electron micrograph of cerebral cortex in CJD. Vacuolar distension of a postsynaptic neuritic process is seen. Courtesy of I. Janota.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.13
FIGURE 15.13

EEG in sCJD: a 72-year-old woman with a history of progressive dementia and myoclonus. EEG shows periodic complexes predominantly over the left hemisphere. Courtesy of Huned Patwa, Yale University School of Medicine.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.14
FIGURE 15.14

Axial FLAIR MRI of sCJD with graded hyperintensity more marked in the anterior putamen relative to the posterior half of the nucleus. N o significant pulvinar or dorsal thalamic hyperintensity. There is also some cortical hyperintensity in the right insula. Courtesy of David Summers, U.K. National CJD Surveillance Unit, Edinburgh, Scotland.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Image of FIGURE 15.15
FIGURE 15.15

Axial FLAIR MRI of vCJD demonstrating symmetrical hyperintensity of the pulvinar nuclei of the thalamus relative to the caudate head and other gray matter. Courtesy of David Summers, U.K. National CJD Surveillance Unit, Edinburgh, Scotland.

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
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Tables

Generic image for table
TABLE 15.1

Spongiform encephalopathies of humans and animals a

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
Generic image for table
TABLE 15.2

Initial clinical features found across the spectrum of human prion disorders

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
Generic image for table
TABLE 15.3

Risk factors for human prion disease

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
Generic image for table
TABLE 15.4

Diagnoses in which CJD has been suspected a

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
Generic image for table
TABLE 15.5

Laboratory investigations for human prion disease

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
Generic image for table
TABLE 15.6

Diagnostic criteria for probable sCJD a

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
Generic image for table
TABLE 15.7

Types of human prion disease by type of transmission

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
Generic image for table
TABLE 15.8

Case definition for vCJD a

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15
Generic image for table
TABLE 15.9

Iatrogenic cases of C JD to mid-2000 a

Citation: Booss J, Esiri M. 2003. 15 Creutzfeldt-Jakob Disease and Other Prion Disorders, p 253-270. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch15

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