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A cyano-bridged coordination polymer, K{Co II 1.5 [Fe II (CN) 6 ]} {(K)Co-Fe}, reported as a highly active heterogeneous catalyst for water oxidation was solubilised by a conventional counter-cation exchange of K + with Me 4 N + ions to provide the homogeneous catalyst of (Me 4 N){Co II 1.5 [Fe II (CN) 6 ]} {(Me 4 N)Co-Fe}. (Me 4 N)Co-Fe exhibited enhanced catalytic activity for photocatalytic water oxidation using [Ru(2,2'-bipyridine) 3 ] 2+ and S 2 O 8 2- as a photosensitiser and a sacrificial electron acceptor, respectively, in terms of the initial reaction rate (1.26 μmol min -1 ), which is about twice that of (K)Co-Fe (0.61 μmol min -1 ). Powder X-ray diffraction, pair distribution function and electrospray ionization mass spectrometry measurements of (Me 4 N)Co-Fe manifested that small heptanuclear clusters of {Co 4 [Fe(CN) 6 ] 3 } 4- formed by depolymerisation are catalytically active species in solution., Competing Interests: There are no conflicts to declare., (This journal is © The Royal Society of Chemistry.)

Linkage isomers of homoleptic complexes, [Rh III (SCN) 6 ] 3- and [Rh III (NCS)(SCN) 5 ] 3- , formed in aqueous solution were successfully separated by employing methyltriphenylphosphonium (MePPh 3 + ) and 1-ethylquinolinium (EtQu + ) ions as countercations, respectively. The single-crystal X-ray analysis of (MePPh 3 ) 3 [Rh III (SCN) 6 ] ( 1 ) indicated that all of the SCN - ligands coordinate to the Rh III ion by S atoms with an octahedral symmetry, where the average bond length of Rh-S is 2.374(7) Å. On the other hand, the Rh III ion of (EtQu) 3 [Rh III ]·H 5 ]·H 2 O ( 2 ) is coordinated by five S atoms and one N atom of the SCN - ligands with a C 4 v symmetry. Structural trans influence was observed in the shorter bond length of Rh-S at the trans position of Rh-N. The Rh-S bond length is 2.3398(13) Å significantly shorter than those of 1 by ca. 0.04 Å, although DFT calculations based on the crystal structures indicated that the effective bond order of Rh-N is higher than those of Rh-S. Thermal stability examination by thermogravimetric and differential thermal analyses (TG/DTA) and IR spectroscopy indicated that the linkage isomerization of [Rh III (SCN) 6 ] 3- to [Rh III (NCS)(SCN) 5 ] 3- proceeded after melting around 174 °C. These results clearly indicate that [Rh III (NCS)(SCN) 5 ] 3- is thermodynamically more stable than [Rh III (SCN) 6 ] 3- in solid states, although further linkage isomerization hardly occurs.

Background and Purpose: Spinal cord lesions are observed in 40% of all central nervous system lesions in intravascular large B-cell lymphoma (IVLBCL). However, because IVLBCL is a very rare disease, its clinical features are not well defined, which may delay appropriate diagnosis and treatment, whilst the acute to subacute course of brain lesions in patients with IVLBCL is well established. Therefore, this study aimed to clarify the clinical features of spinal cord lesions in patients with IVLBCL., Methods: The medical records of patients with IVLBCL admitted to our hospital between 2010 and 2020 were searched. The inclusion criteria were preceding neurological symptoms without non-neurological symptoms and pathologically confirmed IVLBCL in various organs. Clinical features of spinal cord involvement in patients with IVLBCL were assessed and distinguished from those of brain involvement., Results: Sixteen consecutive patients with IVLBCL were divided into two groups: six patients with spinal involvement (spinal cord type) and 10 patients with brain involvement (brain type). In the spinal cord type, four patients had chronic progression and two had subacute progression. Acute progression (0% vs. 80.0%) and sudden onset (0% vs. 50.0%) occurred significantly less frequently in the spinal cord than in the brain. All spinal cord lesions involved the conus medullaris., Conclusions: Spinal cord involvement in IVLBCL has a predominantly chronic progressive course that is exclusive to brain involvement. Conus medullaris lesions are suggestive of IVLBCL and are useful for early and accurate diagnosis and treatment., (© 2023 European Academy of Neurology.)

Transparent and grain boundary-free substrates are essential to immobilize molecular photocatalysts for efficient photoirradiation reactions without unexpected light scattering and absorption by the substrates. Herein, membranes of coordination polymer glass immobilizing metalloporphyrins were examined as a heterogeneous photocatalyst for carbon dioxide (CO 2 ) reduction under visible-light irradiation. [Zn(HPO 4 )(H 2 PO 4 ) 2 ](ImH 2 ) 2 (Im = imidazolate) liquid containing iron(III) 5,10,15,20-tetraphenyl-21H,23H-porphine chloride (Fe(TPP)Cl, 0.1-0.5 w/w%) was cast on a borosilicate glass substrate, followed by cooling to room temperature, resulting in transparent and grain boundary-free membranes with the thicknesses of 3, 5, and 9 μm. The photocatalytic activity of the membranes was in proportion to the membrane thickness, indicating that Fe(TPP)Cl in the subsurface of membranes effectively absorbed light and contributed to the reactions. The membrane photocatalysts were intact during the photocatalytic reaction and showed no recrystallization or leaching of Fe(TPP)Cl.

A cyano-bridged heterometallic coordination polymer with partial deficiencies of CN - ligands, [Mn II (H 2 O) 8/3 ] 3/2 [Fe II (CN) 5 (NH 3 )], forms open metal sites both on Mn II and Fe II ions by liberation of monodentate ligands such as NH 3 and H 2 O. [Mn II (H 2 O) 8/3 ] 3/2 [Fe II (CN) 5 (NH 3 )] exhibits high catalytic activity and selectivity of benzene oxygenation to phenol in the presence of m -chloroperoxybenzoic acid as an oxidant. The postcatalytic spectroscopy of [Mn II (H 2 O) 8/3 ] 3/2 [Fe II (CN) 5 (NH 3 )] and catalysis comparison with a physical mixture of [Mn II (H 2 O) 3 ] 2 [Fe II (CN) 6 ] and [Fe(H 2 O) 3/2 ] 4/3 [Fe(CN) 6 ], which has open metal sites on both Mn II and Fe ions separately, indicated that the high activity resulted from high oxidation ability and phenol adsorption ability of Fe II and Mn II ions, respectively.

The structure of the melt state of one-dimensional (1D) coordination polymer crystal Cu(isopropylimidazolate) (melting temperature T m = 143 °C) was characterized by DSC, variable temperature PXRD, solid-state NMR (SSNMR), viscoelastic measurements, XAS, and DFT-AIMD calculations. These analyses suggested "coordination polymer-forming liquid" formation with preserved coordination bonds above T m . Variable chain configurations and moderate cohesive interaction in adjacent chains are the keys to the rarely observed polymer-forming liquid. The melt structure is reminiscent of the common 1D organic polymer melts such as entanglement or random coil structures., Competing Interests: There are no conflicts to declare., (This journal is © The Royal Society of Chemistry.)

The case is a 65 years old man. He noticed muscle weakness of lower limbs from 3 years ago. Dysesthesia was developed, He came in our hospital in X year. He was detected muscle weakness, sensory disturbance in distal lower limbs predominance and detected radicular significant swelling in spinal cord MRI and diagnosed it with chronic inflammatory demyelinating polyneuritis (CIDP). In the follow-up purpose of other diseases regularly, it was confirmed that nerve root was gradually swelling from around 7 years before the onset. The radicular swelling is one of the characteristics in CIDP, supports the diagnosis. This case was the valuable case that was able to chase image progress before the onset.

The kinetic boundary condition (KBC) represents the evaporation or condensation of molecules at the vapor-liquid interface for molecular gas dynamics (MGD). When constructing the KBC, it is necessary to classify molecular motions into evaporation, condensation, and reflection in molecular-scale simulation methods. Recently, a method that involves setting the vapor boundary and liquid boundary has been used for classifying molecules. The position of the vapor boundary is related to the position where the KBC is applied in MGD analyses, whereas that of the liquid boundary has not been uniquely determined. Therefore, in this study, we conducted molecular dynamics simulations to discuss the position of the liquid boundary for the construction of KBCs. We obtained some variables that characterize molecular motions such as the positions that the molecules reached and the time they stayed in the vicinity of the interface. Based on the characteristics of the molecules found from these variables, we investigated the valid position of the liquid boundary. We also conducted an investigation on the relationship between the condensation coefficient and the molecular incident velocity from the vapor phase to the liquid phase. The dependence of the condensation coefficient on the incident velocity of molecules was confirmed, and the value of the condensation coefficient becomes small in the low-incident-velocity range. Furthermore, we found that the condensation coefficient in the non-equilibrium state shows almost the same value as that in the equilibrium state, although the corresponding velocity distribution functions of the incident velocity significantly differ from each other., Competing Interests: The authors have declared that no competing interests exist.

CN-deficient Prussian blue analogues (PBAs), [M N (H 2 O) x ] y [Fe II (CN) 5 (NH 3 )] (M N = Cu II , Co II , or Ga III ), were synthesized and examined as a new class of heterogeneous catalysts for hydrolytic decomposition of organophosphates often used as pesticides. The active species of the CN-deficient PBAs were mainly C-bound Fe II ions with only single open sites generated by liberation of the NH 3 ligand during the catalytic reactions. [Cu II (H 2 O) 8/3 ] 3/2 [Fe II (CN) 5 (NH 3 )] showed higher catalytic activity than [Co II (H 2 O) 8/3 ] 3/2 [Fe II (CN) 5 (NH 3 )] and [Ga III (H 2 O)][Fe II (CN) 5 (NH 3 )], although N-bound Cu II species has been reported as less active than Co II and Ga III species in conventional PBAs. IR measurements of a series of the CN-deficient PBAs after the catalytic reactions clarified that a part of the NH 3 ligands remained on [Co II (H 2 O) 8/3 ] 3/2 [Fe II (CN) 5 (NH 3 )] and that hydrogen phosphate formed as a product strongly adsorbed on the Fe II ions of [Ga III (H 2 O)][Fe II (CN) 5 (NH 3 )]. Hydrogen phosphate also adsorbed, but weakly, on the Fe II ions of [Cu II (H 2 O) 8/3 ] 3/2 [Fe II (CN) 5 (NH 3 )]. These results suggest that heterogeneous catalysis of the Fe II ions with single open sites were tuned by the M N ions through metal-metal interaction.

Postsynthetic installation of lanthanide cubanes into a metallosupramolecular framework via a single-crystal-to-single-crystal (SCSC) transformation is presented. Soaking single crystals of K 6 [Rh 4 Zn 4 O(l-cys) 12 ] (K 6 [1]; l-H 2 cys=l-cysteine) in a water/ethanol solution containing Ln(OAc) 3 (Ln 3+ =lanthanide ion) results in the exchange of K + by Ln 3+ with retention of the single crystallinity, producing Ln 2 [1] (2 Ln ) and Ln 0.33 [Ln 4 (OH) 4 (OAc) 3 (H 2 O) 7 ][1] (3 Ln ) for early and late lanthanides, respectively. While the Ln 3+ ions in 2 Ln exist as disordered aqua species, those in 3 Ln form ordered hydroxide-bridged cubane clusters that connect [1] 6- anions in a 3D metal-organic framework through coordination bonds with carboxylate groups. This study shows the utility of an anionic metallosupramolecular framework with carboxylate groups for the creation of a series of metal cubanes that have great potential for various applications, such as magnetic materials and heterogeneous catalysts., (© 2020 Wiley-VCH GmbH.)

The photosynthetic reaction center (RC) converts light energy into electrochemical energy. The RC of heliobacteria (hRC) is a primitive homodimeric RC containing 58 bacteriochlorophylls and 2 chlorophyll a s. The chlorophyll serves as the primary electron acceptor (Chl a -A 0 ) responsible for light harvesting and charge separation. The single-molecule spectroscopy of Chl a -A 0 can be used to investigate heterogeneities of the RC photochemical function, though the low fluorescence quantum yield (0.1%) makes it difficult. Here, we show the fluorescence excitation spectroscopy of individual Chl a -A 0 s in single hRCs at 6 K. The fluorescence quantum yield and absorption cross section of Chl a -A 0 increase 2- and 4-fold, respectively, compared to those at room temperature. The two Chl a -A 0 s in single hRCs are identified as two distinct peaks in the fluorescence excitation spectrum, exhibiting different excitation polarization dependences. The spectral changes caused by photobleaching indicate the energy transfer across subunits in the hRC.

A series of thiocyanato-bridged heterometallic coordination polymers with a 3D reticular network have been synthesised by the reaction of [Pt IV (SCN) 6 ] 2- with M II ions to form {M II [Pt IV (SCN) 6 ]} n and {[M II (CH 3 OH) 2 ][Pt IV (SCN) 6 ]} n (M II = Mn II , Fe II , Co II , Ni II or Cu II ) in water and methanol, respectively. Single-crystal X-ray analyses revealed the absence of open metal sites in {M II [Pt IV (SCN) 6 ]} n s and the formation of potential open metal sites at the M II ions of {[M II (CH 3 OH) 2 ][Pt IV (SCN) 6 ]} n s by the coordination of methanol. One of the two coordinating methanol molecules in {[Co II (CH 3 OH) 2 ][Pt IV (SCN) 6 ]} n was replaced with pyridine to stabilise the open metal sites, because the methanol molecules are too labile to maintain open metal sites in water. The heterogeneous catalysis of coordination polymers with and without open metal sites was examined for organophosphate hydrolysis and photocatalytic water oxidation to clarify the requisites for heterogeneous catalysts.

We prepared a pair with a visible-absorbing donor dye and a near-infrared fluorescing acceptor dye. The donor and the acceptor were covalently linked close enough for Förster resonance energy transfer to occur. Under cryogenic conditions at 1.7 K, we observed the fluorescence excitation spectra of the individual pairs in a water matrix. We tested one rhodamine, two Bodipy, and one carbopyronine derivatives as the donor. Among these donors, Bodipy derivatives show the narrowest spectral width of the individuals with respect to the ensemble width. Thus, Bodipy dyes were favorable as the donor for the spectral selection of individual pairs. At 1.7 K, from the several Bodipy-acceptor pairs in the diffraction-limited volume, an individual pair was selected by the fluorescence excitation spectrum of the donor. The spectrally selected pair was localized using the near-infrared fluorescence of the acceptor.

Crystalline porous materials have been investigated for development of important applications in molecular storage, separations, and catalysis. The potential of protein crystals is increasing as they become better understood. Protein crystals have been regarded as porous materials because they present highly ordered 3D arrangements of protein molecules with high porosity and wide range of pore sizes. However, it remains difficult to functionalize protein crystals in living cells. Here, we report that polyhedra, a natural crystalline protein assembly of polyhedrin monomer (PhM) produced in insect cells infected by cypovirus, can be engineered to extend porous networks by deleting selected amino acid residues located on the intermolecular contact region of PhM. The adsorption rates and quantities of fluorescent dyes stored within the mutant crystals are increased relative to those of the wild-type polyhedra crystal (WTPhC) under both in vitro and in vivo conditions. These results provide a strategy for designing self-assembled protein materials with applications in molecular recognition and storage of exogenous substances in living cell as well as an entry point for development of bioorthogonal chemistry and in vivo crystal structure analysis.

Photoactivatable CO releasing protein crystals were developed by immobilization of Mn carbonyl complexes in polyhedral crystals, which are spontaneously formed in insect cells. The photoactivatable CO release from the engineered protein crystals activates nuclear factor kappa B (NF-κB) upon stimulation by visible light irradiation with suppression of cytotoxicity of the Mn complex.

Protein crystals generally are stable solid protein assemblies. Certain protein crystals are suitable for use as nanovessels for immobilizing metal complexes. Here we report the preparation of ruthenium carbonyl-incorporated cross-linked hen egg white lysozyme crystals (Ru·CL-HEWL). Ru·CL-HEWL retains a Ru carbonyl moiety that can release CO, although a composite of Ru carbonyl-HEWL dissolved in buffer solution (Ru·HEWL) does not release CO. We found that treatment of cells with Ru·CL-HEWL significantly increased nuclear factor kappa B (NF-κB) activity as a cellular response to CO. These results demonstrate that Ru·CL-HEWL has potential for use as an artificial extracellular scaffold suitable for transport and release of a gas molecule.

Natural materials such as rock, ore, and clay, containing natural radioactive nuclides are widely used as industrial raw materials in Japan. If these are high concentrations, the workers who handle the material can be unknowingly exposed to radiation at a high level. In this study, about 80 nonmetallic natural materials frequently used as industrial raw materials in Japan were comprehensively collected from several industrial companies, and the activity concentrations of (238)U series, (232)Th series and (40)K in the materials was determined by ICP-MS (inductively-coupled plasma mass spectrometer) and gamma ray spectrum analyses. Effective doses to workers handling them were estimated by using methods for dose estimation given in the RP 122. We found the activity concentrations to be lower than the critical values defined by regulatory requirements as described in the IAEA Safety Guide. The maximum estimated effective dose to workers handling these materials was 0.16 mSv y(-1), which was lower than the reference level (1-20 mSv y(-1)) for existing situation given in the ICRP Publ.103., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Porous protein crystals, which are protein assemblies in the solid state, have been engineered to form catalytic vessels by the incorporation of organometallic complexes. Ruthenium complexes in cross-linked porous hen egg white lysozyme (HEWL) crystals catalyzed the enantioselective hydrogen-transfer reduction of acetophenone derivatives. The crystals accelerated the catalytic reaction and gave different enantiomers based on the crystal form (tetragonal or orthorhombic). This method represents a new approach for the construction of bioinorganic catalysts from protein crystals., (© 2014 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.)

The design of artificial metalloenzymes has become an important topic in biological chemistry and inorganic chemistry due to the potential applications of artificial metalloenzymes in nanoscience and biotechnology. One of the general methods used to produce artificially metalloenzymes involves the encapsulation of non-natural metal cofactors within protein scaffolds. This method has been used in the construction of small artificial metalloproteins with high activity and selectivity. However, the important roles of protein assemblies have not yet been systematically investigated in this field, even though natural enzymatic systems employ protein assemblies as molecular scaffolds for elaborate enzymatic reactions. In recent years, the above-mentioned general strategy has been applied to functionalize protein assemblies such as protein cages and protein crystals. These assembled structures form confined interior environments, which can be used to accommodate metal complex catalysts and to prepare metal nanoparticles. The development of artificial metalloenzymes with hierarchically-assembled proteins would enable us to provide powerful tools for industrial and biological applications. In this Focus Review, we discuss the most significant recent research in this field as well as future directions., (Copyright © 2013 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.)

The authors measured the ambient dose rate and activity concentration of natural radionuclides in raw materials, products, and aerosols on worksites, as well as the (222)Rn and (220)Rn concentrations in an unshaped refractory, a shaped refractory, and an electrocast refractory plant processing zirconium ore in Japan. Estimations were made of the effective doses to plant workers. The activity concentration of the (238)U series in raw materials and products in the refractory plants was higher than the critical values (10 Bq g(-1) for (40)K and 1 Bq g(-1) for all other radionuclides of natural origin) specified in the International Atomic Energy Agency Safety Guide. The ambient dose rate in the raw material warehouse of the electrocast refractory plant was 0.75 μSv h(-1), which was the highest among all the worksites at all the refractory plants studied. The activity concentrations of aerosols in the product-output site of the unshaped refractory plant was 0.0015 Bq m for U and 0.00078 Bq m(-3) for (232)Th, which were the highest of all the worksites for all refractory plants. The indoor (222)Rn and (220)Rn concentrations in all worksites of all the refractory plants were almost the same levels as those in everyday indoor places in Japan. The maximum value of the effective dose to workers was 430 μSv y(-1), which was lower than the intervention exemption level (1,000 μSv y(-1)) specified in ICRP Publication 82.

In Japan, many dwellings have decorative wall coverings made from granite, andesite, tuff, gabbro, and marble. However, information regarding activity concentrations and radon (Rn) and thoron (Rn) exhalation rates for such rocks is very scarce. Therefore, samples of the granite, andesite, tuff, and marble that are used as wall coverings in Japan were collected from mining companies, and their activity concentrations and Rn and Rn exhalation rates were measured. Dose estimations for inhabitants living in houses built with these materials were also carried out. The activity concentration of natural radionuclides in all the materials was lower than the critical values described by the International Atomic Energy Agency (IAEA) (10,000 Bq kg for K and 1,000 Bq kg for all other radionuclides of natural origin). The maximum values of Rn and Rn mass exhalation rates for the granite samples were 0.12 and 430 mBq kg s, and those for the area exhalation rates were 1.8 and 6300 mBq m s, respectively; these values are higher than those for other samples. The maximum value of effective doses to inhabitants was 0.68 mSv y, which is lower than the intervention exemption level (1 mSv y) given in the International Commission on Radiological Protection (ICRP) Publication 82.

We report a patient of a 20-year-old woman of Takayasu's arteritis and hypertensive encephalopathy. The symptoms started with headache and vomiting following status epilepticus. On arrival at the emergency room in our hospital, fever was apparent and cerebrospinal fluid examination revealed pleocytosis. After the admission, the patient presented with hypertension, decreased right brachial pulse and the difference between bilateral brachial arterial blood pressures on examination. There had been no history of arterial hypertension. The MR angiography revealed stenoses of the bilateral cervical, especially right cervical, right middle cerebral and left renal arteries. Brain MRI showed transient hyperintense lesions of the left fronto-parieto-occipital cortices and subcortical white matter in FLAIR and diffusion weighted images. These alterations suggested the presence of reversible vasogenic edema induced by hypertensive encephalopathy. We need to be aware of young patients with convulsion, especially young women, who has arterial hypertension as well as the difference with blood pressures between extremities.

Purpose: This study was designed to investigate the changes in stomatognathic function through orthognathic treatment in patients with mandibular prognathism., Patients and Methods: Thirty-six patients with mandibular prognathism were tested and compared with 30 healthy controls with normal occlusion. For each subject, the occlusal contact area and occlusal force were measured during maximum voluntary clenching (MVC). Activities of the masseter and temporalis muscles were recorded during MVC and voluntary gum chewing. Jaw movement was analyzed during chewing on the left and right sides. For the analyses, 2 parameters, asymmetry index (AI) and error index (EI), were established to further investigate the nature of masticatory function. AI was used to evaluate the bilateral balance of masticatory muscle activity, and EI indicates the rate of abnormal jaw movement pattern., Results: In patients with mandibular prognathism, the occlusal contact area and maximum bite force decreased before surgery, and increased after surgery. The masseter and temporal muscle activities also decreased before surgery, but showed no substantial increase even after surgery. The occlusal and muscle efficiency exhibited significantly smaller values in the patient group than in the controls, irrespective of treatment stages. The AI decreased after surgery. The EI decreased significantly after surgery, but was still significantly greater in the patient group than in the controls., Conclusions: It is suggested that masticatory muscles in the patients with mandibular prognathism may adapt to the new environment achieved with surgically corrected dentofacial structure, although the activities remain at lower levels as compared with the controls.

The purpose of the present study was to investigate the influence of an activator appliance and a spring active appliance on masticatory muscle activity by means of electromyography (EMG). Twelve adult males with good general health were recruited as subjects. Three functional appliances were used in each subject for long-period EMG recording during daytime and sleep and short-time EMG recording during voluntary biting. Following findings were obtained. (1) The activity of all muscles was greater during biting than during daytime and sleep, (2) the muscle activities tended to increase in the digastric muscle and to decrease in the temporal muscle with activators under all conditions, and (3) the temporalis-masseter ratios became lower with the biting use of appliances. Therefore, this study suggests that functional appliances should be used during sleep and during the day in combination with voluntary biting to achieve adaptation and development of the masticatory muscles.

A 41-year-old man presented with a large mass bulging over the suprazygomatic temporal region. Neuroradiological examination showed that the huge extra-axial mass with osteolytic character originated from the upper surface of the petrous bone. Preoperative obliteration of the feeding arteries with super-selective intravascular embolization was helpful for the total removal of the tumor. Histological examination revealed that the tumor consisted of massive fibrohistiocytic proliferation with numerous heavily hemosiderin-laden macrophages and numerous multinucleated giant cells. The most probable diagnosis was giant cell reparative granuloma. Therefore, no postoperative irradiation or other adjuvant therapy was given.

A 20-year-old Japanese woman was diagnosed as suffering from adult type A neuronal ceroid lipofuscinosis (NCL) by rectal biopsy in the first year of manifestation. Her sister was in good health, and her parents were non-consanguineous. She had graduated from a public high school and then went to a typist school, when she developed action myoclonus and dystonia. On admission, she was of short stature and her clinical features included high arched palate, cataracta, and accentuated deep tendon reflexes. Her IQ was 50. Visual failure was not observed. Brain MRI showed no abnormalities. Together with myoclonus and the abnormalities in EEG which included poly spike and wave complex, progressive myoclonus epilepsy was considered as differential diagnoses. Ultrastructurally, lipopigments of granular matrix and curvilinear profile were found in Schwann cells in rectal biopsy. Adult NCL, known as Kufs' disease, is classified into two clinical types; progressive myoclonus epilepsy (type A) and dementia with motor disturbance (type B). Adult NCL is very rare in Japan, and this is the first report of adult NCL type A in Japan.

A 44-year-old Japanese man was diagnosed as having late adult-onset dentatorubral-pallidoluysian atrophy (DRPLA), whose CAG repeats in the DRPLA gene were 60 and 15. He developed gait disturbance, limb ataxia, pyramidal tract signs, dementia, and psychiatric symptoms including character changes within a few years of the above diagnosis. His T 2-weighted brain MRI showed symmetric high-signal lesions in the cerebral white matter and brain stem, in addition to cerebellar, brain stem, and cerebral cortical atrophy. Since the results of RI cisternography indicated that he manifested the clinical features of normal pressure hydrocephalus (NPH), V-P shunt operation was done. In a week after the operation, his gait disturbance, pyramidal tract signs, dementia and psychiatric symptoms were remarkably improved. White matter lesions have been thought to be concomitant with late adult-onset DRPLA patients, but some of these patients may have characteristics of NPH pathophysiology.

Using specimens from the posterior-superior quadrant of the human tympanic membrane, meatal skin, retroauricular skin and middle ear cholesteatoma, epidermal cell proliferation was studied by cultures in FC43 emulsion containing bromodeoxyuridine (BrdU), and cell death was detected by in situ labeling of nuclear DNA fragmentation (TUNEL staining). The culture of specimens with BrdU revealed labeling in the basal cell layer and/or the layer immediately above it. The counts of BrdU-labeled cells both at and beside the malleus handle and at the annulus were significantly higher than those in the tympanic membrane, meatal skin, retroauricular skin and cholesteatoma, indicating the existence of epidermal proliferation centers in the annulus and malleus handle. TUNEL-positive cells were observed in the uppermost layer of the epidermis, and counts of dying cells did not show any significant differences among specimens from the different areas. From these observations, we conclude that addition of newly proliferated cells at the proliferation center and uniform cell death cause epidermal cell migration over the tympanic membrane and ear canal. In addition, no proliferation center was seen in the epidermis of cholesteatoma, suggesting a disturbance of epidermal cell migration. Furthermore, BrdU-labeling at the margin of persistently perforated tympanic membranes from patients with chronic otitis media revealed that, at the perforation margin, the counts of BrdU-labeled cells were not higher than those of the normal tympanic membrane. In addition, a few BrdU-labeled cells were observed in the lamina propria and mucosal cell layer, indicating that persistent perforation of the tympanic membrane results from the failure of proliferating cells to increase at the margin of the perforation.