Abstract
IntroductionSlowly progressive cognitive decline is the most frequent initial manifestation in MM2-cortical-type sporadic Creutzfeldt-Jakob disease. Agraphia has never been noted in patients with this type of sporadic Creutzfeldt-Jakob disease, however, we report the case of a Japanese patient with sporadic Creutzfeldt-Jakob disease in whom agraphia of Kanji was an initial cardinal symptom.Case presentationA 59-year-old right-handed Japanese woman complained of agraphia of Kanji (Chinese characters) as an initial symptom. A neurological examination revealed mild word-finding difficulty, constructive disturbance, hyperreflexia in her jaw and lower limbs, and bilateral extensor plantar reflexes. An examination of her cerebrospinal fluid revealed increased levels of 14-3-3 and total tau proteins, and abnormal conformation of the proteinase K-resistant prion protein. Diffusion-weighted magnetic resonance imaging showed diffuse hyperintensity in bilateral cerebral cortices. Single-photon emission computed tomography scans revealed hypoperfusion in the left temporal lobe, bilateral parietal and occipital lobes. An analysis of the prion protein gene demonstrated no mutation with homozygous for methionine at the codon 129. We diagnosed our patient with sporadic Creutzfeldt-Jakob disease. Although a histological examination was not performed, it was assumed that our patient could be the MM2-cortical type according to the clinical findings and the elevated levels of 14-3-3 protein in her cerebrospinal fluid. The left posterior inferior temporal area, which was affected in our patient as a hypoperfusion area, is associated with selecting and recalling Kanji characters.ConclusionsFocal signs as an early symptom and hypoperfusion areas in sporadic Creutzfeldt-Jakob disease are critical to recognize initial brain lesions damaged by the proteinase K-resistant prion protein accumulation.
Highlights
Progressive cognitive decline is the most frequent initial manifestation in MM2-cortical-type sporadic Creutzfeldt-Jakob disease
Focal signs as an early symptom and hypoperfusion areas in sporadic Creutzfeldt-Jakob disease are critical to recognize initial brain lesions damaged by the proteinase K-resistant prion protein accumulation
Creutzfeldt-Jakob disease (CJD), a degenerative neurological disorder caused by prions, is neuropathologically characterized by the accumulation of the proteinase Kresistant prion protein (PrPres), which leads to spongiform changes in tissues of the central nervous system
Summary
We report a first case of sCJD with agraphia of Kanji as an initial and cardinal symptom. It is assumed that this patient could be categorized as MM2-cortical type according to the clinical presentation. Focal signs as an early symptom and functional imaging in early-stage sCJD are critical to Consent Written informed consent was obtained from the patient and the patient’s of kin for publication of this case report with any accompanying images. Competing interests The authors declare that they have no competing interests. Authors’ contributions KN collected the clinical data and drafted the manuscript. KS, MS, and IN were involved in critically revising the manuscript for important intellectual content. MY is the supervising consultant and gave the final authorization for publication of the manuscript. All authors read and approved the final manuscript
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