Details der Publikation - Sporadic CJD in association with HIV

Sporadic CJD in association with HIV

Background Creutzfeldt-Jakob disease (CJD) is a rapidly progressive fatal neurodegenerative disorder. We report an unusual case of pathologically confirmed sporadic CJD developing in a HIV-positive patient but presenting with clinical and radiological features suggestive of variant CJD. Case presentation A 63-year-old man with chronic stable HIV developed progressive difficulties with decision-making, obsessive compulsive disorder and visual hallucinations over 3 months. CSF examination detected a weakly positive 14-3-3 protein, elevated S-100 protein, and siginificantly elevated total-Tau protein. Brain MRI revealed bilateral abnormal signal within the posterolateral thalami compatible with pulvinar sign. Further investigations revealed a negative tonsillar biospy and positive blood test consistent with variant CJD. However, prion protein genotyping detected MV heterozygosity at codon 129 and post-mortem histopathological examination was consistent with sporadic CJD. Conclusion Although MRI findings were suggestive of variant CJD, the short residence in the UK and MV heterozygosity are aytpical, and the histopathological examination was consistent with sporadic CJD. With only two cases of HIV and sporadic CJD reported so far, the association of CJD with HIV remains unclear..

Medienart:	E-Artikel

Erscheinungsjahr:	2018
Erschienen:	2018

Enthalten in:	Zur Gesamtaufnahme - volume:266
Enthalten in:	Journal of neurology - 266(2018), 1 vom: 16. Nov., Seite 253-257

Sprache:	Englisch

Beteiligte Personen:	van de Ven, Nikolien S. [VerfasserIn] Vera, Jaime [VerfasserIn] Jones, John R. [VerfasserIn] Vundavalli, Sriram [VerfasserIn] Ridha, Basil H. [VerfasserIn]

Links:	Volltext [lizenzpflichtig]

Themen:	Creutzfeldt–Jakob disease Human immunodeficiency virus

RVK:	RVK Klassifikation ELIB24

Anmerkungen:	© Springer-Verlag GmbH Germany, part of Springer Nature 2018

doi:	10.1007/s00415-018-9116-0

funding:
Förderinstitution / Projekttitel:

PPN (Katalog-ID):	OLC210548297X

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520			\|a Background Creutzfeldt-Jakob disease (CJD) is a rapidly progressive fatal neurodegenerative disorder. We report an unusual case of pathologically confirmed sporadic CJD developing in a HIV-positive patient but presenting with clinical and radiological features suggestive of variant CJD. Case presentation A 63-year-old man with chronic stable HIV developed progressive difficulties with decision-making, obsessive compulsive disorder and visual hallucinations over 3 months. CSF examination detected a weakly positive 14-3-3 protein, elevated S-100 protein, and siginificantly elevated total-Tau protein. Brain MRI revealed bilateral abnormal signal within the posterolateral thalami compatible with pulvinar sign. Further investigations revealed a negative tonsillar biospy and positive blood test consistent with variant CJD. However, prion protein genotyping detected MV heterozygosity at codon 129 and post-mortem histopathological examination was consistent with sporadic CJD. Conclusion Although MRI findings were suggestive of variant CJD, the short residence in the UK and MV heterozygosity are aytpical, and the histopathological examination was consistent with sporadic CJD. With only two cases of HIV and sporadic CJD reported so far, the association of CJD with HIV remains unclear.
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Sporadic CJD in association with HIV

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