Summary: Near-infrared spectroscopy (NIRS) was used to monitor human visual cortical function during and after photic stimulation (PS) in five adult volunteers. Cerebral blood volume (CBV) increased on the occipital surface during PS, but NIRS parameters did not change on the frontal surface. The increase in CBV was caused by a rapid increase in oxyhemoglobin with but a small increase in deoxyhemoglobin, suggesting cerebral vascular dilata tion with decreased oxygen consumption. After PS A study of human subjects using positron emis sion tomography (PET) indicated that photic stim ulation (PS) raises blood flow and glucose consump tion in the visual cortex by about 50%, whereas oxygen consumption increases only 5% (Fox et al., 1988). Using proton magnetic resonance spectros copy, Prichard et al. (1991) demonstrated that PS causes an increase in brain lactate in the human visual cortex. However, the mechanism of PS ac tion on human visual cortical function is still not clear. Near-infrared spectroscopy (NIRS) is a non invasive tool for the continuous monitoring of changes in cerebral blood volume (CBV), oxyhemo globin (Hb02), deoxyhemoglobin (HbR) and redox state of cytochrome aa3 (Cyt). Voluntary hypervenReceived February 11, 1992; final revision received October 27, 1992; accepted October 27, 1992.Address correspondence and reprint requests to Dr. Toshinori Kato, Mental Retardation and Birth Defect Research Division, National Institute of Neuroscience, NCNP, 4-1-1 Ogawahigashi cho, Kodaira, Tokyo, Japan.Abbreviations used: CBV, cerebral blood volume; Cyt, cy tochrome aa3; DPF, differential path length factor; HbR, de oxyhemoglobin; Hb02, oxyhemoglobin; HbT, total hemoglobin (Hb02 + HbR); MRI, magnetic resonance imaging; NIRS, near infrared spectroscopy; PET, positron emission tomography; PC, photon count; PS, photic stimulation. 516stopped, CBV promptly decreased and then slightly in creased again. Cytochrome aa3 did not show any change during and after PS. These phenomena reappeared fol lowing repeated PS in all five subjects. These results may represent the first step in the development of NIRS im aging.

In response to the subduction of the young Shikoku Basin of the Philippine Sea Plate, arc magmas erupted in SW Japan throughout the late Cenozoic. Many magma types are present including ocean island basalt (OIB), shoshonite (SHO), arc-type alkali basalt (AB), typical subalkalic arc basalt (SAB), high-Mg andesite (HMA), and adakite (ADK). OIB erupted since the Japan Sea back-arc basin opened, whereas subsequent arc magmas accompanied subduction of the Shikoku Basin. However, there the origin of the magmas in relation to hot subduction is debated. Using new major and trace element and Sr-Nd-Pb-Hf isotope analyses of 324 lava samples from seven Quaternary volcanoes, we investigated the genetic conditions of the magma suites using a geochemical mass balance model, Arc Basalt Simulator version 4 (ABS4), that uses these data to solve for the parameters such as pressure/temperature of slab dehydration/melting and slab flux fraction, pressure, and temperature of mantle melting. The calculations suggest that those magmas originated from slab melts that induced flux melting of mantle peridotite. The suites differ mostly in the mass fraction of slab-melt flux, increasing from SHO through AB, SAB, HMA, to ADK. The pressure and temperature of mantle melting decreases in the same order. The suites differ secondarily in the ratio of altered oceanic crust to sediment in the source of the slab melt. The atypical suites associated with hot subduction result from unusually large mass fractions of slab melt and unusually cool mantle temperatures.

Early infantile epileptic encephalopathy with suppression-burst pattern (EIEE) is one of the most severe and earliest forms of epilepsy, often evolving into West syndrome; however, the pathogenesis of EIEE remains unclear. ARX is a crucial gene for the development of interneurons in the fetal brain, and a polyalanine expansion mutation of ARX causes mental retardation and seizures, including those of West syndrome, in males. We screened the ARX mutation and found a hemizygous, de novo, 33-bp duplication in exon 2, 298_330dupGCGGCA(GCG)9, in two of three unrelated male patients with EIEE. This mutation is thought to expand the original 16 alanine residues to 27 alanine residues (A110_A111insAAAAAAAAAAA) in the first polyalanine tract of the ARX protein. Although EIEE is mainly associated with brain malformations, ARX is the first gene found to be responsible for idiopathic EIEE. Our observation that EIEE had a longer expansion of the polyalanine tract than is seen in West syndrome is consistent with the findings of earlier onset and more-severe phenotypes in EIEE than in West syndrome.

The natural course of idiopathic pulmonary fibrosis (IPF) is variable. Predicting disease progression and survival in IPF is important for treatment. We previously demonstrated that serum periostin has the potential to be a prognostic biomarker for IPF. Our aim was to use monomeric periostin in a multicenter study to evaluate its efficacy in diagnosing IPF and predicting its progression. To do so, we developed a new periostin kit to detect only monomeric periostin. The subjects consisted of 60 IPF patients in a multicenter cohort study. We applied monomeric periostin, total periostin detected by a conventional kit, and the conventional biomarkers—KL-6, SP-D, and LDH—to diagnose IPF and to predict its short-term progression as estimated by short-term changes of %VC and % DL, CO. Moreover, we compared the fraction ratios of monomeric periostin to total periostin in IPF with those in other periostin-high diseases: atopic dermatitis, systemic scleroderma, and asthma. Monomeric periostin showed the greatest ability to identify IPF comparable with KL-6 and SP-D. Both monomeric and total periostin were well correlated with the decline of %VC and % DL, CO. Clustering of IPF patients into high and low periostin groups proved useful for predicting the short-term progression of IPF. Moreover, the relative ratio of monomeric periostin was higher in IPF than in other periostin-high diseases. Measuring monomeric periostin is useful for diagnosing IPF and predicting its short-term progression. Moreover, the ratio of monomeric periostin to total periostin is elevated in IPF compared to other periostin-high diseases.

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