Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a hereditary cerebral small vessel disease caused by mutations in NOTCH3, is characterized by recurrent stroke without vascular risk factors, mood disturbances, and dementia. MRI imaging shows cerebral white matter (WM) hyperintensity, particularly in the external capsule and temporal pole. Missense mutations related to a cysteine residue in the 34 EGFr on the NOTCH3 extracellular domain (N3ECD) are a typical mutation of CADASIL. On the other hand, atypical mutations including cysteine sparing mutation, null mutation, homozygous mutation, and other associate genes are also reported. From the viewpoint of gain of function apart from Notch signaling or loss of function of Notch signaling, we review the research article about CADASIL and summarized the pathogenesis of small vessel, stroke, and dementia in this disease.

We propose the new criteria of high sensitivity, which will help physicians to assess the need for genetic testing.

Objective: The aim of this investigation was to determine the factors influencing acute intracerebral hemorrhage severity on admission and clinical outcomes at discharge. Methods: Sixty acute stroke hospitals throughout Japan participated in the Japan Standard Stroke Registry Study (JSSRS), documenting the in-hospital course of 16,630 consecutive patients with acute stroke from January 2001 to March 2004. We identified 2,840 adult patients from the JSSRS who had intracerebral hemorrhage. Results: Intracerebral hemorrhage severity on admission was strongly related to age, previous stroke history, and hemorrhage size in a monotone fashion [χ²(9) = 374.5, p < 0.0001]. Drinking history was also predictive of intracerebral hemorrhage severity on admission, but the association was not monotone. Interestingly, intracerebral hemorrhage severity on admission was increased in nondrinking and heavy drinking compared to mild drinking (p < 0.05). Unsuccessful outcome (modified Rankin scale score = 3–6) was related to age, previous stroke history, hemorrhage size, and intracerebral hemorrhage severity on admission [χ²(9) = 830.4, p < 0.0001]. Mortality was related to hemorrhage size, intraventricular hemorrhage, intracerebral hemorrhage severity on admission, and surgical operation [χ²(7) = 540.4, p < 0.0001]. Conclusion: We could find four varied factors associated with intracerebral hemorrhage severity and its outcomes. Interestingly, intracerebral hemorrhage severity tended to be greater in nondrinking and heavy drinking than mild drinking. Additionally, surgical operation decreased intracerebral hemorrhage mortality.

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Dust events have been observed in Japan with high frequency since 2000. On the other hand, the frequency of dust storms is said to have decreased in the desert regions of China since about the middle of the 1970s. This study simulates dust storms and transportation of mineral dust aerosols in the east Asia region from 1981 to 2001 using an aerosol transport model, Spectral Radiation‐Transport Model for Aerosol Species (SPRINTARS), implemented in the Center for Climate System Research/National Institute for Environmental Studies atmospheric global circulation model, in order to investigate the main factors that control a dust event and its long‐term variation. The model was forced to simulate a real atmospheric condition by a nudging technique using European Centre for Medium‐Range Weather Forecasts reanalysis data on wind velocities, temperature, specific humidity, soil wetness, and snow depth. From a comparison between the long‐term change in the dust emission and model parameters, it is found that the wind speed near the surface level had a significant influence on the dust emission, and snow is also an important factor in the early spring dust emission. The simulated results suggested that dust emissions from northeast China have a great impact on dust mass concentration in downwind regions, such as the cities of northeastern China, Korea, and Japan. When the frequency of dust events was high in Japan, a low‐pressure system tended to develop over the northeast China region that caused strong winds. From 2000 to 2001 the simulated dust emission flux decreased in the Taklimakan desert and the northwestern part of China, while it increased in the Gobi desert and the northeastern part of China. Consequently, dust particles seem to be transported more from the latter region by prevailing westerlies in the springtime to downwind areas as actually observed. In spite of the similarity, however, there is still a large disagreement between observed and simulated dust frequencies and concentrations. A more realistic land surface and uplift mechanism of dust particles should be modeled to improve the model simulation. Desertification of the northeastern China region may be another reason for this disagreement.