Agraphia of Kanji (Chinese characters): an early symptom of sporadic Creutzfeldt-Jakob disease in a Japanese patient: a case report

Journal of Medical Case Reports, Aug 2014

Introduction Slowly 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 presentation A 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. Conclusions 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.

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Agraphia of Kanji (Chinese characters): an early symptom of sporadic Creutzfeldt-Jakob disease in a Japanese patient: a case report

Journal of Medical Case Reports Agraphia of Kanji (Chinese characters): an early symptom of sporadic Creutzfeldt-Jakob disease in a Japanese patient: a case report Keiko Nakamura 0 Kenji Sakai 0 Miharu Samuraki 0 Ichiro Nozaki Masako Notoya Masahito Yamada 0 0 Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences , 13-1 Takara-machi, Kanazawa, Ishikawa 9208640 , Japan Introduction: Slowly 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 presentation: A 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. Conclusions: 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. Agraphia; Creutzfeldt-Jakob disease; Kana (Japanese syllabary); Kanji (Chinese characters); Magnetic resonance imaging - Introduction 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. CJD is classified according to its causes: sporadic CJD (sCJD), the idiopathic form; familial CJD, caused by inherited mutations in the prion protein (PrP) gene; and acquired CJD, related to previous infectious episodes [1]. Sporadic CJD is classified into six types based on the genotype at polymorphic codon 129 of the PrP gene and the physicochemical properties of the pathologic PrPres: MM1, MM2, MV1, MV2, VV1, and VV2 [2]. MM2-type sCJD comprises of two pathological phenotypes: cortical and thalamic forms. MM2-cortical-type sCJD is the most common subtype as an atypical sCJD form in Japan [3]. Although slowly progressive cognitive decline is the most frequent initial manifestation in this subtype, aphasia, ataxia, psychiatric symptoms, and visual disturbance are also described [1,4,5]. However, agraphia has never been noted in patients with MM2-corticaltype sCJD. The Japanese language has two writing systems, that is, Kanji (Chinese characters) and Kana (Japanese syllabary), which are different from those of Western languages. Kanji are the structurally complex morphograms introduced from China, often having several phonetic readings, while Kana are the relatively simple syllabograms having unambiguous phonetic readings [6]. Japanese sentences consist of various combinations of Kanji and Kana. The major lexical morphemes of Japanese words are written in Kanji, and conjugated endings of verbs, adjectives, and functional words are written in Kana. Both these systems are associated with distinct regions of the brain [7]. We report a Japanese patient with sCJD in whom agraphia of Kanji was an initial cardinal symptom. This patient was presumed to be MM2-cortical-type sCJD according to the clinical presentation. Case presentation A 59-year-old right-handed Japanese woman had difficulty in writing Kanji. She could neither recognize forms of the Kanji characters clearly nor write them. One month later, she developed progressive cognitive impairment; however, her social behavior remained appropriate. A neurological examination performed two months after the disease onset revealed mild word-finding difficulty and constructive disturbance such as copying simple diagrams. Hyperreflexia was present in her jaw and l (...truncated)


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Keiko Nakamura, Kenji Sakai, Miharu Samuraki, Ichiro Nozaki, Masako Notoya, Masahito Yamada. Agraphia of Kanji (Chinese characters): an early symptom of sporadic Creutzfeldt-Jakob disease in a Japanese patient: a case report, Journal of Medical Case Reports, 2014, pp. 269, 8, DOI: 10.1186/1752-1947-8-269