Writing Impairments in Japanese Patients with Mild Cognitive Impairment and with Mild Alzheimer's Disease.

BACKGROUND/AIMS: We investigated writing abilities in patients with the amnestic type of mild cognitive impairment (aMCI) and mild Alzheimer's disease (AD). To examine the earliest changes in writing function, we used writing tests for both words and sentences with different types of Japanese characters (Hiragana, Katakana, and Kanji). METHODS: A total of 25 aMCI patients, 38 AD patients, and 22 healthy controls performed writing to dictation for Kana and Kanji words, copied Kanji words, and wrote in response to a picture story task. Analysis of variance was used to test the subject group effects on the scores in the above writing tasks. RESULTS: For the written Kanji words, the mild AD group performed worse than the aMCI group and the controls, but there was no difference between the aMCI group and the controls. For the picture story writing task, the mild AD and aMCI groups performed worse than the controls, but the difference between the AD and the aMCI groups was not significant. CONCLUSIONS: The mild AD group showed defects in writing Kanji characters, and the aMCI group showed impairments in narrative writing. Our study suggests that narrative writing, which demands complex integration of multiple cognitive functions, can be used to detect the subtle writing deficits in aMCI patients.

[Pure word deafness due to a localization-related seizure: a case study].

We describe a 31-year-old, right-handed man who exhibited pure word deafness (PWD) due to a localization-related seizure. The patient was suddenly unable to comprehend spoken words, whereas he had no difficulty in speaking, comprehending written language, or discriminating non-verbal sounds. Diffusion-weighted imaging and fluid-attenuated inversion recovery on brain magnetic resonance imaging revealed hyperintense lesions in the left superior temporal gyrus (STG) and left superior marginal gyrus (SMG). Furthermore, brain single-photon emission computed tomography showed that these lesions were hyperperfused. An electroencephalogram (EEG) showed multiple spike-and-slow-wave complexes spreading to the left temporal regions. Immediately after administering midazolam intravenously, the patient was able to comprehend spoken words, and the epileptiform discharges in the left temporal regions disappeared. These findings indicate that he suffered from PWD resulting from a dysfunction of the left hemisphere alone. Furthermore, they suggest that the left STG and left SMG play an important role in the recognition of spoken words.

[A case of myelitis with anti-aquaporin 4 antibody concomitant with immune thrombocytopenic purpura].

We report a 44-year-old woman who had anti-aquaporin 4 (AQP4) antibody-positive myelitis and immune thrombocytopenic purpura (ITP). She was admitted to our hospital with paraparesis, dysesthesia below the Th8 dermatome level on her right side and lower extremities, constipation and urinary retention. Magnetic resonance imaging revealed a longitudinally extending lesion at the level of Th4-Th10. Her serum sample was positive for anti-AQP4 antibody. Corticosteroid therapy was initiated, and her symptoms were largely ameliorated. Furthermore, concurrently with the myelitis, her platelet count dropped (99 × 10(9)/l). A diagnosis of ITP was made with positive serum platelet-associated IgG (PA-IgG) and negative work-up for blood malignancies by bone marrow aspiration. Since a causal relationship between Helicobacter pylori (H. pylori) and ITP is suggested by several studies, she was also examined and diagnosed with H. pylori-positive ITP. After the bacteria eradication therapy, her platelet count and PA-IgG returned to normal range. Furthermore, the anti-AQP4 antibody titer declined and her symptoms were almost resolved. We considered that H. pylori might influence progression of the myelitis as well as induction and development of ITP.

Neural substrates for writing impairments in Japanese patients with mild Alzheimer's disease: a SPECT study.

Language is fairly well preserved in most patients with mild Alzheimer's disease, but writing ability seems to be impaired even in the early stages of the disease. To investigate the neural bases of writing impairments in Alzheimer's disease (AD), we examined the correlation between writing ability and regional cerebral blood flow (rCBF) in 52 Japanese patients with mild AD compared to 22 controls, using single photon emission computed tomography (SPECT). We found that, compared with control subjects, Kana writing to dictation and copying Kanji words were preserved in AD patients, but writing to dictating Kanji words was impaired. We classified the errors in the Kanji dictation task into four types to investigate the correlation between rCBF and the error type, as follows: non-response errors, phonologically plausible errors, non-phonologically plausible errors, and peripheral errors. Non-response errors, which indicated difficulty with retrieving Kanji graphic images, were the most frequent. When controlled for confounding factors, the number of non-response errors negatively correlated with rCBF in the left inferior parietal lobule, the posterior middle and inferior temporal gyri, and the posterior middle frontal gyrus. Thus, the impaired recall of Kanji in early Alzheimer's disease is related to dysfunctional cortical activity, which appears to be predominant in the left frontal, parietal, and temporal regions.

MRI findings from a case of fulminating adult-onset measles encephalitis.

We report a rare case of fulminating adult-onset measles encephalitis. A 34-year-old man developed a comatose state after measles eruptions and ultimately akinetic mutism. Titers of anti-measles IgM antibodies were elevated in both serum and cerebrospinal fluid. Brain magnetic resonance imaging (MRI) 3 months after onset revealed widespread hyperintense lesions in the periventricular white matter and marginal hyperintense lesions in the brainstem on fluid-attenuated inversion recovery and diffusion-weighted images. The marginal lesions in the brainstem are similar to subpial demyelinating lesions seen in postinfectious encephalomyelitis. This case of encephalitis may be related to an autoimmune-mediated process triggered by measles infection.

Familial inclusion body myositis: a report on two Japanese sisters.

Familial occurrence of inclusion body myositis is extremely rare, and only a few cases in Western countries have been reported. In these reports, a strong association of this disease with DR3 (DRB1*0301/0302) and the efficacy of immunosuppressants suggested that an immune pathomechanism is involved in the disease. We, for the first time, report two Japanese sisters who suffered myopathy clinicopathologically similar to inclusion body myositis. One sister received corticosteroid and azathioprine and the therapy relieved dysphagia. Both of our patients had DR15(2)/4 (DRB1*1502/0405), suggesting a distinct genetic association with the disease in the Japanese population.