Dear Editor,
We thank Onder11. 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
https://doi.org/10.1590/0004-282X-ANP-20...
for his valuable comments regarding our article22. 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.
https://doi.org/10.1590/0004-282X-ANP-20...
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 fluid33. 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.
https://doi.org/10.1016/S1473-3099(19)30...
.
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 legend22. 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.
https://doi.org/10.1590/0004-282X-ANP-20...
.
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 article33. 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.
https://doi.org/10.1016/S1473-3099(19)30...
,44. 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
https://doi.org/10.1007/s00415-011-6362-...
.
Moreover, the association of DCJ and PRES has been previously described in the literature55. 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
https://doi.org/10.7759/cureus.7211...
, 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 CJD55. 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
https://doi.org/10.7759/cureus.7211...
,66. 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.
https://doi.org/10.5327/Z2447-2123201919...
.
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
» 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
» 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
» 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
» 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
» 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
» https://doi.org/10.5327/Z2447-212320191900063
Publication Dates
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Publication in this collection
18 Oct 2021 -
Date of issue
Sept 2021
History
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Received
12 Aug 2021 -
Accepted
13 Aug 2021