Mercado-Flores M, Taboada-Barajas J, González-Patiño MA, Flores-Rivera JJ

Language: Spanish
References: 18
Page: 292-298
PDF size: 986.73 Kb.

ABSTRACT

Creutzfeldt-Jakob disease is a rare neurodegenerative disorder, with progressive and invariably fatal evolution. It is characterized by rapidly progressive dementia and neurological signs. There are several types and the sporadic form is the most common; its cause is not yet known. The diagnosis is based on clinical signs, electroencephalogram results, and detection of 14-3-3 protein in spinal fluid. Magnetic resonance, although it is not part of diagnostic studies, has high sensitivity, especially with fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted sequences, because there is a correlation with findings and the stage of the disease. The combination of positron emission tomography and simple tomography is not well established in this condition, but hypometabolism could be detected in the cortical regions and gray nuclei. We present the case of a patient, age 57, who was admitted with incoordination for fine work and subsequently cognitive deterioration characterized by rapidly progressive dementia. The first resonance showed discrete hyperintensities in diffusion sequence; the combination of simple and positron emission tomographies evidenced generalized brainstem and cerebellar hypometabolisms, of caudate nuclei, putamen, and thalami. The electroencephalogram was abnormal due to generalized dysfunction. Subsequently, resonance recorded hyperintensities of the brainstem and caudate nuclei and the electroencephalogram a periodic generalized triphasic and biphasic pattern; spinal fluid with TAU and 14-3-3 positive proteins. The patient developed akinetic mutism and we were able to confirm and correlate the clinical evolutionary stages and differential diagnoses with laboratory studies and image methods.

REFERENCES

1. Ironside JW. (2009). La enfermedad de Creuzfeldt-Jakob. World Federation of Hemophilia, Tratamiento para la hemofilia. No. 49: 1-15.

2. DeArmond SJ, Kretzschmar HA, Prusiner SB. (2002). Prion diseases. In: Graham DI, Lantos PL, eds. Greenfield’s neuropathology, 7th ed. London, England: Arnold :273–323.

3. National Prion Disease Pathology Surveillance Center, 2009 (internet). Disponible en: http://www.cjdsurveillance.com/index.html.

4. Fundacion CJD Mexico.org (internet). Guadalajara, Jalisco, México, actualización 2010. Disponible en: http://www.fundacioncjd.com/.html.

5. Collie D, Summers D, Sellar R, Ironside J, Cooper S, Zeidler M, Knight R, Robert G. Will (2003). Diagnosing Variant Creutzfeldt-Jakob Disease with the Pulvinar Sign: MR Imaging Findings in 86 Neuropathologically Confirmed Cases AJNR Am J Neuroradiol 24:1560–1569.

6. World Health Organization WHO, Human transmissible spongiform encephalopathies. Weekly Epidemiological Record, 1998. 73: p. 361-5.

7. Brown P, Cathala F, Castaigne P, Gajdusek DC. (1986). Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Annals of Neurology;20:597–602.

8. Begué C, Piccardo P, Taratuto A. (2008). Encefalopatías espongiformes transmisibles: enfermedad de Creutzfeldt-Jakob. Neuroinfecciones en el adulto y el niño. Montevideo, Rev. Méd. Urug. vol.28 no.1 Editorial Arena,. p. 203-11.

9. Gertz, H.J., H. Henkes, and J. Cervos-Navarro, (1988). Creutzfeldt-Jakob disease: correlation of MRI and neuropathologic findings. Neurology 38(9): p. 1481-2.

10. Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann, (2009). Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 132:2659–2668.

11. Schaefer PW, Grant PE, Gonzalez RG. (2000). Diffusionweighted MR imaging of the brain. Radiology 217:331–345

12. Ryutarou Ukisu, Tamio Kushihashi, (2005).Serial Diffusion-Weighted MRI of Creutzfeldt-Jakob Disease AJR:184.

13. Young GS, Geschwind MD, Fischbein NJ, et al. Diffusionweighted and fluidattenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. Am J Neuroradiol 2005;26:1551–62

14. HJ Tschampa K. Kallenberg HA. Kretzschmar B. Meissner. Pattern of Cortical Changes in Sporadic Creutzfeldt-Jakob Disease. American Journal of Neuroradiology 2007;28:1114–18.

15. K. Kallenberg. Creutzfeldt-Jakob Disease: Comparative Analysis of MR Imaging Sequences. Am J Neuroradiol 2006;27:1459–62.

16. Zeidler M, Sellar RJ, Collie DA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 2000;355:1412–18.

17. Tschampa HJ, Murtz P, Flacke S, et al. Thalamic involvement in sporadic Creutzfeldt-Jakob disease: a diffusion-weighted MR imaging study. Am J Neuroradiol 2003;24:908–15.

18. WJ Zhang. Premortem Diagnosis of Sporadic Creutzfeldt- Jakob Disease Aided by Positron-Emission Tomography Imaging. Am J Neuroradiol 2011;32:E18.