Reply to letter regarding the article entitled: ‘Rapidly progressive sporadic Creutzfeldt-Jakob disease: isolated Heidenhain variant or a combination with PRES?’

Dear Editor,

We thank Onder[1] for his valuable comments regarding our article[2] on the manifestation of probable Creutzfeldt-Jakob Disease (CJD) with Posterior Reversible Encephalopathy Syndrome (PRES).

First, we report a case of a patient with a pretty clear neurodegenerative course of the disease. Regarding the diagnosis of CJD, we believe that it is very likely because of clinical, imaging, electroencephalographic and cerebrospinal fluid findings - rapid-onset cognitive impairment with ataxia and myoclonus; diffusion-weighted images (DWI) revealing bilateral hyperintensities in parietal areas, cingulate gyrus, and basal ganglia; periodic sharp wave complex and positive 14-3-3 in cerebrospinal fluid[3].

Second, we deeply agree with the author that the persistence of the early neuroimaging findings after the acute-onset visual agnosia would support the diagnosis of CJD, as shown in the figures of our article and described in Figure 1 original legend[2].

Then, we recognize that temporal evolution due to limitations of space could be more precise. Still, we believe that it is clear that neurodegeneration is not associated with PRES, but with CJD in this case, given that the cognitive dysfunction, ataxia, and myoclonus were present before acute-onset of visual loss as it can be seen in the article[3],[4].

Moreover, the association of DCJ and PRES has been previously described in the literature[5], and the main focus of our article is a discussion of acute-onset visual loss is a manifestation of CJD Heidenhain variant - recognized by visual loss and occipital cortex hyperintensities in T2-FLAIR imaging - or a PRES manifestation associated with CJD[5],[6].

Conflict of interest:

There is no conflict of interest to declare.

Authors’ contributions:

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

• References

• 1 Onder H. Letter regarding the article entitled: ‘Rapidly progressive sporadic Creutzfeldt Jakob disease: isolated Heidenhain variant or a combination with PRES? Arq Neuropsiquiatr. 2021;79(9):854. https://doi.org/10.1590/0004-282X-ANP-2021-0206
• 2 Fraiman PHA, Teixeira CM, de Oliveira JCD, et al. Rapidly progressive sporadic Creutzfeldt-Jakob disease: isolated Heidenhain variant or a combination with PRES? Arq Neuropsiquiatr. 2021;79(4):352-3. https://doi.org/10.1590/0004-282X-ANP-2020-0428.
• 3 Uttley L, Carroll C, Wong R, et al. Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. Lancet Infect Dis. 2020;20(1):e2-e10. https://doi.org/10.1016/S1473-3099(19)30615-2.
• 4 Kastrup O, Gerwig M, Frings M, Diener H-C. Posterior reversible encephalopathy syndrome (PRES): electroencephalographic findings and seizure patterns. J Neurol. 2012;259(7):1383-9. https://doi.org/10.1007/s00415-011-6362-9
• 5 Bittar J, Joshi P, Genova J, et al. Creutzfeldt-Jakob Disease Presenting as Posterior Reversible Encephalopathy Syndrome. Cureus. 2020;12(3):e7211. https://doi.org/10.7759/cureus.7211
• 6 Mendonça LFP de, Saffi PMNR, Martini LLL, et al. Heidenhain variant of Creutzfeldt-Jakob disease in Brazil: A case report. Geriatr Gerontol Aging. 2020; 14:71-5. https://doi.org/10.5327/Z2447-212320191900063.

Publication History

Received: 12 August 2021

Accepted: 13 August 2021

Article published online:
10 July 2023