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We herein report a sporadic Creutzfeldt-Jakob disease (sCJD) patient followed from the presymptomatic phase to death. A 67-year-old woman had abnormal hyperintense cortical lesions on diffusion-weighted magnetic resonance imaging (MRI) one year before the onset. The levels of 14-3-3 protein and total tau protein, and findings from a real-time quaking-induced conversion test were normal at first but became abnormal after disease onset. Although there are four reports of presymptomatic sCJD identified by MRI, this is the first case report in which all three biomarkers had been assessed before and after the disease onset. MRI might be the most sensitive modality for detecting presymptomatic sCJD patients.

Japanese people born before World War II learned Japanese kana (Japanese syllabograms) writing in a style that is not currently used. These individuals had to learn the current style of kana orthography after the war. An 85-year-old man was taken to our hospital by his family who were surprised by his diary. It was written with kanji (Japanese ideograms) and katakana using the prewar style. A neuropsychological examination revealed impaired recall of hiragana. Neuroimaging studies revealed atrophy of the left fronto-parietal lobe and hypoperfusion of the left frontal lobe. His allographic agraphia might have resulted from the disturbance of the current style of kana orthography.

We herein describe a Japanese family with distal hereditary motor neuropathy carrying a K141Q mutation of small heat shock protein HSPB1. Two patients among them had late onset disease (older than 50 years). The muscles of the distal legs were weak and atrophic. Sensory and autonomic dysfunction were not seen. Even eight years after onset, one patient could still walk without support. A nerve conduction study revealed axonal degeneration of the motor nerves of the legs. A heterozygous K141Q mutation was detected in the affected patients. The late onset and mild clinical phenotype might reflect the mild biochemical alteration of HSP27 induced by the K141Q mutation.

Background The guidelines recommend combination therapy for patients who are unable to achieve target BP with monotherapy; some fixed dose therapies including an angiotensin II receptor blocker (ARB) and diuretics are available in Japan. However, to date there have been few reports on this long-term treatment and the patient profiles suited for this combination remain ambiguous. Method The Aichi Research on Combination therapy for Hypertension Study was a multicenter, open-label, prospective observational study that investigated the efficacy and safety of 1-year treatment with the losartan-hydrochlorothiazide (HCTZ) combination tablet in patients with hypertension uncontrolled by either ARB monotherapy or combination therapy with a calcium channel blocker (CCB). An ARB was switched to a losartan-HCTZ tablet after a pre-observation period. Results A total of 614 of 648 patients were evaluable (mean age, 66.3 years; 52.8% men; mean baseline blood pressure, BP, 157.7/87.9 mmHg). The BP had decreased significantly to 138.0/78.2 mmHg by month 3 (p

A 76-year-old woman presented with catatonia, refusal to eat due to delusion, and visual hallucination. Single photon emission computed tomography showed remarkable occipital hypoperfusion and frontal hyperperfusion. (123)I metaiodobenzyl guanidine myocardial scintigraphy revealed decreased uptake. She was diagnosed as probable dementia with Lewy bodies (DLB). Intravenous or oral L-dopa had no effect on catatonia. Amitriptyline and lorazepam improved catatonia and visual hallucination. Cerebral blood flow of the frontal and occipital lobes seemed to be normalized. Occipital hypoperfusion is one of the features of DLB. Although the mechanism of perfusion abnormality in DLB remains to be clarified, our case suggested that it might be reversible.

A 75-year-old woman was admitted because of pain and numbness in the extremities and trunk. She subsequently suffered from lymphadenopathy and spiky fever. The immunohistochemical analysis of the biopsied lymph nodes and sural nerve and electrophysiological examination supported a diagnosis of angioimmunoblastic lymphadenopathy-like T cell lymphoma with polyneuropathy. The infiltrating lymphoma cells of the sural nerve and lymph node shared the same phenotype (CD45RO, CD3, CD30 positive). An increased expression of HLA-DR antigen was observed in endothelial and Schwann cells. Chemotherapy with CHOP-Bleomycin markedly relieved her pain. These findings suggest that a direct lymphocytic infiltration in the nerve may be associated with neuropathy in this case.