Download PDF
CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2021; 79(04): 352-353
DOI: 10.1590/0004-282X-ANP-2020-0428
Images in Neurology

Rapidly progressive sporadic Creutzfeldt-Jakob disease: isolated Heidenhain variant or a combination with PRES?

Doença de Creutzfeldt-Jakob esporádica rapidamente progressiva: variante Heidenhain isolada ou PRES combinada?
Pedro Henrique Almeida FRAIMAN 
1   Universidade Federal do Rio Grande do Norte, Hospital Universitário Onofre Lopes, Divisão de Neurologia, Natal RN, Brazil.
,
Carolina Militão TEIXEIRA 
1   Universidade Federal do Rio Grande do Norte, Hospital Universitário Onofre Lopes, Divisão de Neurologia, Natal RN, Brazil.
,
Juliano César Dantas DE OLIVEIRA 
2   Casa de Saúde São Lucas, Divisão de Radiologia, Natal RN, Brazil.
,
Thadeu Alexandre Paulino DE SOUSA 
2   Casa de Saúde São Lucas, Divisão de Radiologia, Natal RN, Brazil.
,
Manuel MOREIRA-NETO 
3   Universidade Federal do Rio Grande do Norte, Hospital Universitário Onofre Lopes, Divisão de Radiologia, Natal RN, Brazil.
,
Clecio de Oliveira GODEIRO-JUNIOR 
1   Universidade Federal do Rio Grande do Norte, Hospital Universitário Onofre Lopes, Divisão de Neurologia, Natal RN, Brazil.
› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

A 70-year-old man presented with rapidly progressive cognitive impairment with ataxia and myoclonus. Visual agnosia was noticed after blood pressure oscillations, during immunoglobulin therapy for suspected autoimmune encephalitis. Brain magnetic resonanbce imaging scans before and after visual agnosia are presented in [Figure 1]. Electroencephalogram disclosed periodic sharp wave complex, and cerebrospinal fluid was positive for 14-3-3 protein. The final diagnosis was probable Creutzfeldt-Jakob disease (CJD) associated with posterior reversible encephalopathy syndrome (PRES). Blood pressure and immunoglobulin therapy may explain PRES[1]. It seems that some types of CJD prion proteins could induce the activation of microglia and dysfunction of vasoconstrictors upregulation, leading to vasospasm, ischemia and PRES[1],[2]

Zoom Image
Figure 1 Magnetic resonance imaging features of sporadic Creutzfeld-Jakob disease and posterior reversible encephalopathy syndrome. (A, B, C and D) Initial diffusion-weighted images revealing bilateral hyperintensities in parietal areas, mostly on the right, and hyperintensities in cingulate gyrus. The same findings apply albeit to a lesser extent in the right basal ganglia (caudate and putamen, white arrows). (E, F, G and H) T2WI and T2-fluid-attenuated inversion-recovery reveals new cortico-subcortical hyperintensities in parietal and occipital areas and cerebellum, compatible with vasogenic edema, highly suggestive of PRES (thick white arrows). Previously described alterations remain the same. T1W1 does not reveal contrast enhancement.

Authors’ contributions:

PF: analysis and interpretation, writing and drafting the manuscript; CMT: data acquisition, analysis and interpretation, writing the manuscript; JCDO: data acquisition, analysis and interpretation; TAPS: data acquisition, analysis and interpretation; MMN: critical revision of the manuscript for intellectual content; CGJ: critical revision of the manuscript for intellectual content.




Publication History

Received: 04 September 2020

Accepted: 19 September 2020

Article published online:
01 June 2023

© 2021. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Thieme Revinter Publicações Ltda.
Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil

 

  • References

  • 1 Dirzius E, Balnyte R, Steibliene V, Gleizniene R, Gudinaviciene I, Radziunas A, et al. Sporadic Creutzfeldt-Jakob disease with unusual initial presentation as posterior reversible encephalopathy syndrome: a case report. BMC Neurol. 2016 Nov;16(1):234. https://doi.org/10.1186/s12883-016-0751-8
  • 2 Freitas CS, Pinheiro MGM, Fonte EJD, Hazin AN, Smid J, Barbosa BJAP. Posterior cortical ribboning in the Heidenhain variant of Creutzfeldt-Jakob Disease. Arq Neuro-Psiquiatr. 2020 Apr;78(4):241. https://doi.org/10.1590/0004-282x20190176