What is the degree of variability in formal training for pediatric orthopedic surgeons performing scoliosis surgery? / ¿Cuál es el grado de variabilidad en la educación formal de los cirujanos ortopédicos pediátricos que realizan intervenciones quirúrgicas de escoliosis?

Abstract: Introduction: although pediatric orthopedic surgeons worldwide perform scoliosis surgery, the training received is variable and poorly understood. By surveying the European Pediatric Orthopedic Society (EPOS) and the Sociedad Latinoamericana de Ortopedia (SLAOTI), we aim to characterize this variability. Material and methods: in 2021, we distributed an anonymous online questionnaire to EPOS and SLAOTI. Results: 43% EPOS and 22% SLAOTI perform scoliosis procedures (p < 0.05). 18% EPOS and 2% SLAOTI performed > 35 procedures annually (p < 0.05). 70% EPOS and 27% SLAOTI received formal training in spinal deformity surgery (p < 0.005). Conclusions: results show significant differences in training and performance of scoliosis procedures between societies.

Resumen: Introducción: aunque muchos cirujanos ortopédicos pediátricos alrededor del mundo realizan cirugías para la escoliosis, el entrenamiento es variable y poco conocido. A través de encuestar a la Sociedad Europea de Ortopedia Pediátrica (EPOS) y a la Sociedad Latinoamericana de Ortopedia (SLAOTI) queremos caracterizar esta variabilidad. Material y métodos: distribuimos un cuestionario anónimo en 2021 a los miembros de EPOS y SLAOTI. Resultados: realizan las cirugías de escoliosis 43% de EPOS, en comparación con 22% de SLAOTI (p < 0.05); 18% de EPOS realizó > 35 cirugías al año, en comparación con 2% de SLAOTI (p < 0.05); 70% de EPOS y 27% de SLAOTI recibieron capacitación formal en cirugía de deformidades de la columna (p < 0.005). Conclusiones: los resultados de este estudio subrayan las diferencias en el entrenamiento de las cirugías de escoliosis entre diferentes sociedades.

In vivo skin reactions from pulsed-type, bipolar, alternating current radiofrequency treatment using invasive noninsulated electrodes.

BACKGROUND: Bipolar, alternating current radiofrequency (RF) conduction using invasive noninsulated electrodes consecutively generates independent tissue coagulation around each electrode and then, the converged coagulation columns. METHODS: Two pulsed-type RF models at the on-time pulse width/pulse pack of 30 and 40 milliseconds were designed to amplify the early stage of RF-induced tissue reaction using hairless mouse skin in vivo. Then, structural and ultrastructural changes were evaluated in hairless mouse skin samples at baseline and immediately 1 day, 3 days, 7 days, and 14 days after treatment. RESULTS: Immediately after pulsed-RF treatment, a few chrysanthemum-like zones of electrothermal coagulation and hypereosinophilic collagen fibers were found in the dermis and dermo-subcutaneous fat junction. Histochemical staining for periodic acid-Schiff and immunohistochemical staining for type IV collagen revealed marked thickening of basement membranes. Transmission electron microscopy demonstrated that pulsed-RF treatment resulted in higher electron-dense and remarkably thicker lamina densa, as well as increases in anchoring fibrils, compared with untreated control specimens. Furthermore, CD31-positive blood vessels were smaller in size with a slit-like luminal appearance, without excessive damage to endothelial cells. CONCLUSION: Our data indicated that pulse-type, bipolar RF energy induces structural and ultrastructural changes in basement membranes and vascular components in hairless mouse skin.

Periorbital melasma: Hierarchical cluster analysis of clinical features in Asian patients.

BACKGROUND: Studies have shown melasma lesions to be distributed across the face in centrofacial, malar, and mandibular patterns. Meanwhile, however, melasma lesions of the periorbital area have yet to be thoroughly described. METHODS: We analyzed normal and ultraviolet light-exposed photographs of patients with melasma. The periorbital melasma lesions were measured according to anatomical reference points and a hierarchical cluster analysis was performed. RESULTS: The periorbital melasma lesions showed clinical features of fine and homogenous melasma pigmentation, involving both the upper and lower eyelids that extended to other anatomical sites with a darker and coarser appearance. The hierarchical cluster analysis indicated that patients with periorbital melasma can be categorized into two clusters according to the surface anatomy of the face. Significant differences between cluster 1 and cluster 2 were found in lateral distance and inferolateral distance, but not in medial distance and superior distance. Comparing the two clusters, patients in cluster 2 were found to be significantly older and more commonly accompanied by melasma lesions of the temple and medial cheek. CONCLUSION: Our hierarchical cluster analysis of periorbital melasma lesions demonstrated that Asian patients with periorbital melasma can be categorized into two clusters according to the surface anatomy of the face.

The 7 × 1 Fermi Surface Reconstruction in a Two-dimensional f -electron Charge Density Wave System: PrTe3.

The electronic structure of a charge density wave (CDW) system PrTe3 and its modulated structure in the CDW phase have been investigated by employing ARPES, XAS, Pr 4 f RPES, and first-principles band structure calculation. Pr ions are found to be nearly trivalent, supporting the CDW instability in the metallic Te sheets through partial filling. Finite Pr 4 f spectral weight is observed near the Fermi level, suggesting the non-negligible Pr 4 f contribution to the CDW formation through the Pr 4 f -Te 5p hybridization. The two-fold symmetric features in the measured Fermi surface (FS) of PrTe3 are explained by the calculated FS for the assumed 7 × 1 CDW supercell formation in Te sheets. The shadow bands and the corresponding very weak FSs are observed, which originate from both the band folding due to the 3D interaction of Te sheets with neighboring Pr-Te layers and that due to the CDW-induced FS reconstruction. The straight vertical FSs are observed along kz, demonstrating the nearly 2D character for the near-EF states. The observed linear dichroism reveals the in-plane orbital character of the near-EF Te 5p states.

Electronic Structure of YbB_{6}: Is it a Topological Insulator or Not?

To finally resolve the controversial issue of whether or not the electronic structure of YbB_{6} is nontrivially topological, we have made a combined study using angle-resolved photoemission spectroscopy (ARPES) of the nonpolar (110) surface and density functional theory (DFT). The flat-band conditions of the (110) ARPES avoid the strong band bending effects of the polar (001) surface and definitively show that YbB_{6} has a topologically trivial B 2p-Yb 5d semiconductor band gap of ∼0.3 eV. Accurate determination of the low energy band topology in DFT requires the use of a modified Becke-Johnson exchange potential incorporating spin-orbit coupling and an on-site Yb 4f Coulomb interaction U as large as 7 eV. The DFT result, confirmed by a more precise GW band calculation, is similar to that of a small gap non-Kondo nontopological semiconductor. Additionally, the pressure-dependent electronic structure of YbB_{6} is investigated theoretically and found to transform into a p-d overlap semimetal with small Yb mixed valency.

Cross-sectional area of the ulnar nerve after decompression at the cubital tunnel.

UNLABELLED: A total of 48 patients undergoing surgical decompression of the ulnar nerve at the cubital tunnel between February 2010 and May 2013 were retrospectively studied to determine changes in the cross-sectional area of the nerve by the technique of neurosonography. The mean follow-up was 46 months. Post-operative follow-up examination of the cross-sectional area of the ulnar nerve showed a slight reduction in the mean value from 13.8 mm(2) (pre-operative) to 12.9 mm(2) (post-operative). Of the 48 patients, 36 showed a reduction in the cross-sectional area. No correlation was detected between the clinical and sonographic outcomes. Ultrasound seems to be of limited value in the post-operative assessment of patients with entrapment neuropathy of the ulnar nerve. LEVEL OF EVIDENCE: IV.

The effect of CD34+ cell-containing autologous platelet-rich plasma injection on pattern hair loss: a preliminary study.

BACKGROUND: Mobilized CD34+ cells in peripheral blood have angiogenic potential, which is an important factor in active hair growth. In addition, activated autologous platelet-rich plasma (PRP) has been reported to induce the proliferation of dermal papilla cells. OBJECTIVES: To investigate the clinical efficacy of interfollicular injection of CD34+ cell-containing PRP preparation for pattern hair loss. PATIENTS AND METHODS: CD34+ cell-containing PRP preparation was injected on the scalps of 13 patients with pattern hair loss, and 13 patients were treated with interfollicular placental extract injection as a control. The numbers of platelets in PRP were microscopically counted and CD34+ cells were evaluated with flow cytometry. RESULTS: Three months after the first treatment, the patients presented clinical improvement in the mean number of hairs, 20.5 ± 17.0% (P < 0.0001), mean hair thickness, 31.3 ± 30.1% (P < 0.0001), and mean two-point score, 84.4 ± 51.7% (P < 0.0001) compared with baseline values. At 6 months, the patients presented clinical improvement in mean hair count, 29.2 ± 17.8% (P < 0.0001), mean hair thickness, 46.4 ± 37.5% (P < 0.0001), and mean two-point score, 121.3 ± 66.8% (P < 0.0001) compared with baseline. The MIXED procedure revealed that CD34+ cell-containing PRP treatment presented a higher degree of improvement than placental extract treatment in hair thickness (P = 0.027) and overall clinical improvement (P = 0.023). CONCLUSION: Our data suggest that the interfollicular injection of autologous CD34+ cell-containing PRP preparation has a positive therapeutic effect on male and female pattern hair loss without remarkable major side-effects.

White matter damage is related to ataxia severity in SCA3.

Spinocerebellar ataxia type 3 (SCA3) is the most frequent inherited cerebellar ataxia in Europe, the US and Japan, leading to disability and death through motor complications. Although the affected protein ataxin-3 is found ubiquitously in the brain, grey matter atrophy is predominant in the cerebellum and the brainstem. White matter pathology is generally less severe and thought to occur in the brainstem, spinal cord, and cerebellar white matter. Here, we investigated both grey and white matter pathology in a group of 12 SCA3 patients and matched controls. We used voxel-based morphometry for analysis of tissue loss, and tract-based spatial statistics (TBSS) on diffusion magnetic resonance imaging to investigate microstructural pathology. We analysed correlations between microstructural properties of the brain and ataxia severity, as measured by the Scale for the Assessment and Rating of Ataxia (SARA) score. SCA3 patients exhibited significant loss of both grey and white matter in the cerebellar hemispheres, brainstem including pons and in lateral thalamus. On between-group analysis, TBSS detected widespread microstructural white matter pathology in the cerebellum, brainstem, and bilaterally in thalamus and the cerebral hemispheres. Furthermore, fractional anisotropy in a white matter network comprising frontal, thalamic, brainstem and left cerebellar white matter strongly and negatively correlated with SARA ataxia scores. Tractography identified the thalamic white matter thus implicated as belonging to ventrolateral thalamus. Disruption of white matter integrity in patients suffering from SCA3 is more widespread than previously thought. Moreover, our data provide evidence that microstructural white matter changes in SCA3 are strongly related to the clinical severity of ataxia symptoms.

A stable fixed-dose combination tablet of pseudoephedrine and KOB extracts for the extended release.

Allergic rhinitis (AR) is characterized by inflammation of the nasal mucosa with hypersensitivity resulting from seasonal or perennial responses to specific environmental allergens and by symptoms like nasal rubbing, sneezing, rhinorrhea, lacrimation, nasal congestion and obstruction, and less frequently cough. KOB extracts, which is a polyherbal medicine consisting of 5 different herbs (Atractylodes macrocephala, Astragalus membranaceus, Saposhnikovia divaricata, Ostericum koreanum and Scutellaria baicalensis) had commonly been used for the treatment of various allergic diseases showed an anti-allergic effect by modulating mast cell-mediated allergic responses in allergic rhinitis, recently. On the other hand, pseudoephedrine is a sympathomimetic amine commonly used to relieve congestion in patients with allergic rhinitis and common colds. Considering the KOB's therapeutic mechanism, the combination with pseudoephedrine would be suitable for allergic rhinitis. This study is to obtain an effective extended release formulation using pseudoephedrine and KOB extracts to reduce side effects of drug due to repeated dosing and improve the compliance of patients for treatment of rhinitis and nasal decongestion. So, the fixed-dose combination tablet of pseudoephedrine and KOB extracts was prepared by direct compression and characterized by drug content, flowing characteristics and dissolution test. The drug content of baicalin of KOB extracts was within the range of 95-105% except for T1 formulation. The hardness and friability values of all formulations ranged from 9 to 13 kp and less than 1%, respectively. Taken together, T4 or T8 could be a stable fixed-dose combination tablet for extended release of pseudoephedrine and KOB extracts for nasal rhinitis.

SVCT-2 in breast cancer acts as an indicator for L-ascorbate treatment.

L-ascorbate (L-ascorbic acid, vitamin C) clearly has an inhibitory effect on cancer cells. However, the mechanism underlying differential sensitivity of cancer cells from same tissue to L-ascorbate is yet to be clarified. Here, we demonstrate that L-ascorbate has a selective killing effect, which is influenced by sodium-dependent vitamin C transporter 2 (SVCT-2) in human breast cancer cells. Treatment of human breast cancer cells with L-ascorbate differentially induced cell death, dependent on the SVCT-2 protein level. Moreover, knockdown of endogenous SVCT-2 via RNA interference in breast cancer cells expressing high levels of the protein induced resistance to L-ascorbate treatment, whereas transfection with SVCT-2 expression plasmids led to enhanced L-ascorbate chemosensitivity. Surprisingly, tumor regression by L-ascorbate administration in mice bearing tumor cell xenograft also corresponded to the SVCT-2 protein level. Interestingly, SVCT-2 expression was absent or weak in normal tissues, but strongly detected in tumor samples obtained from breast cancer patients. In addition, enhanced chemosensitivity to L-ascorbate occurred as a result of caspase-independent autophagy, which was mediated by beclin-1 and LC3 II. In addition, treatment with N-acetyl-L-cysteine, a reactive oxygen species (ROS) scavenger, suppressed the induction of beclin-1 and LC3 II, implying that the differential SVCT-2 protein-dependent L-ascorbate uptake was attributable to intracellular ROS induced by L-ascorbate, subsequently leading to autophagy. These results suggest that functional SVCT-2 sensitizes breast cancer cells to autophagic damage by increasing the L-ascorbate concentration and intracellular ROS production and furthermore, SVCT-2 in breast cancer may act as an indicator for commencing L-ascorbate treatment.

Inventory of Non-Ataxia Signs (INAS): validation of a new clinical assessment instrument.

Although ataxia is by definition the prominent symptom of ataxia disorders, there are various neurological signs that may accompany ataxia in affected patients. Reliable and quantitative assessment of these signs is important because they contribute to disability, but may also interfere with ataxia. Therefore we devised the Inventory of Non-Ataxia Signs (INAS), a list of neurological signs that allows determining the presence and severity of non-ataxia signs in a standardized way. INAS underwent a rigorous validation procedure that involved a trial of 140 patients with spinocerebellar ataxia (SCA) for testing of inter-rater reliability and another trial of 28 SCA patients to assess short-term intra-rater reliability. In addition, data of the ongoing EUROSCA natural history study were used to determine the reproducibility, responsiveness and validity of INAS. Inter-rater reliability and short-term test-retest reliability was high, both for the total count and for most of the items. However, measures of responsiveness, such as the smallest detectable change and the clinically important change were not satisfactory. In addition, INAS did not differentiate between subjects that were subjectively stable and those that worsened in the 2-year observation period. In summary, INAS and INAS count showed good reproducibility, but unsatisfactory responsiveness. The present analysis and published data from the EUROSCA natural history study suggest that INAS is a valid measure of extracerebellar involvement in progressive ataxia disorders. As such, it is useful as a supplement to the measures of ataxia, but not as a primary outcome measure in future interventional trials.

The natural history of spinocerebellar ataxia type 1, 2, 3, and 6: a 2-year follow-up study.

OBJECTIVE: To obtain quantitative data on the progression of the most common spinocerebellar ataxias (SCAs) and identify factors that influence their progression, we initiated the EUROSCA natural history study, a multicentric longitudinal cohort study of 526 patients with SCA1, SCA2, SCA3, or SCA6. We report the results of the 1- and 2-year follow-up visits. METHODS: As the primary outcome measure we used the Scale for the Assessment and Rating of Ataxia (SARA, 0-40), and as a secondary measure the Inventory of Non-Ataxia Symptoms (INAS, 0-16) count. RESULTS: The annual increase of the SARA score was greatest in SCA1 (2.18 ± 0.17, mean ± SE) followed by SCA3 (1.61 ± 0.12) and SCA2 (1.40 ± 0.11). SARA progression in SCA6 was slowest and nonlinear (first year: 0.35 ± 0.34, second year: 1.44 ± 0.34). Analysis of the INAS count yielded similar results. Larger expanded repeats and earlier age at onset were associated with faster SARA progression in SCA1 and SCA2. In SCA1, repeat length of the expanded allele had a similar effect on INAS progression. In SCA3, SARA progression was influenced by the disease duration at inclusion, and INAS progression was faster in females. CONCLUSIONS: Our study gives a comprehensive quantitative account of disease progression in SCA1, SCA2, SCA3, and SCA6 and identifies factors that specifically affect disease progression.

[Hirayama disease in Germany: case reports and review of the literature]. / Hirayama-Syndrom in Deutschland : Fallberichte und Literaturübersicht.

Hirayama disease is a juvenile benign distal upper limb muscular atrophy rarely observed in Europe, usually monomelic involving C7-Th1 innervated muscles. It is characterized by insidious onset and a self-limited course within a few years. The pathogenesis of this mostly sporadic disease is not fully clarified. Cervical flexion myelopathy with mechanical ischemic damage of spinal motoneurons is the best established pathogenetic hypothesis, but neurodegenerative and autoimmune causes are also debated. Typically, young men of Asian origin are affected. Here we describe three German Caucasian patients with Hirayama disease and provide an up-to-date review of the literature.

Pharmacokinetics and bioequivalence of 20 mg omeprazole capsule in 24 healthy Korean male volunteers.

OBJECTIVE: A randomized, two-way, crossover bioequivalence study in 24 healthy Korean male volunteers was conducted to compare bioequivalence of two brands of 20 mg omeprazole capsules, Hutex omeprazole (Hutex Pharm Co. Korea) as a test and Yuhan Losec (Yuhan Co. Ltd., Korea) as a reference drug. VOLUNTEERS AND METHODS: Subjects were administered single dosage of 1 capsule of 20 mg of each formulation with 240 ml of water after 10 hs overnight fasting on 2 treatment days separated by one-week washout period. After dosing, serial blood sampling was held during 9 hs. Plasma was analyzed for omeprazole by a validated HPLC method with ultraviolet detection in the range of 10 approximately 1,000 ng/ml with the lowest limit of quantification of 10 ng/ml. RESULTS: Several pharmacokinetic (PK) parameters were determined from the plasma samples, and data from reference and test formulations in the plasma were represented such as AUC0-t (1,223.3 vs 1,284.3 ng x h/ml), [formula in text](1,311.1 vs 1,410.0 ng x h/ml), Cmax (598.7 vs 598.1 ng/ml), tmax (1.9 vs 1.9 h), t1/2 (1.3 vs 1.4 h) and Ke (0.67 vs 0.67 h-1), respectively. AUC0-t, [formula in text] and Cmax were tested for bioequivalence after log-transformation of plasma data. PK parameters with 90% confidence interval (CI) of test/reference ratio based on ANOVA analysis were 0.961 approximately 1.135 for AUC0-t, 0.968 approximately 1.144 for [formula in text] and 0.951 approximately 1.117 for Cmax. CONCLUSIONS: PK parameters with 90% CI were within the bioequivalence range of 80 - 125% of FDA statistical limit. Therefore, both omeprazole formulations were bioequivalent during fasting state in these healthy Korean male volunteers.

Spinocerebellar ataxia types 1, 2, 3, and 6: disease severity and nonataxia symptoms.

OBJECTIVE: To identify factors that determine disease severity and clinical phenotype of the most common spinocerebellar ataxias (SCAs), we studied 526 patients with SCA1, SCA2, SCA3. or SCA6. METHODS: To measure the severity of ataxia we used the Scale for the Assessment and Rating of Ataxia (SARA). In addition, nonataxia symptoms were assessed with the Inventory of Non-Ataxia Symptoms (INAS). The INAS count denotes the number of nonataxia symptoms in each patient. RESULTS: An analysis of covariance with SARA score as dependent variable and repeat lengths of the expanded and normal allele, age at onset, and disease duration as independent variables led to multivariate models that explained 60.4% of the SARA score variance in SCA1, 45.4% in SCA2, 46.8% in SCA3, and 33.7% in SCA6. In SCA1, SCA2, and SCA3, SARA was mainly determined by repeat length of the expanded allele, age at onset, and disease duration. The only factors determining the SARA score in SCA6 were age at onset and disease duration. The INAS count was 5.0 +/- 2.3 in SCA1, 4.6 +/- 2.2 in SCA2, 5.2 +/- 2.5 in SCA3, and 2.0 +/- 1.7 in SCA6. In SCA1, SCA2, and SCA3, SARA score and disease duration were the strongest predictors of the INAS count. In SCA6, only age at onset and disease duration had an effect on the INAS count. CONCLUSIONS: Our study suggests that spinocerebellar ataxia (SCA) 1, SCA2, and SCA3 share a number of common biologic properties, whereas SCA6 is distinct in that its phenotype is more determined by age than by disease-related factors.

SCA Functional Index: a useful compound performance measure for spinocerebellar ataxia.

OBJECTIVE: To evaluate the usefulness of functional measures in patients with spinocerebellar ataxia (SCA). METHODS: We assessed three functional measures-8 m walking time (8MW), 9-hole peg test (9HPT), and PATA repetition rate-in 412 patients with autosomal dominant SCA (genotypes 1, 2, 3, and 6) in a multicenter trial. RESULTS: While PATA rate was normally distributed (mean/median 21.7/20.5 per 10 s), the performance times for 8MW (mean/median 10.8/7.5 s) or 9HPT (mean/median 47.2/35.0 s in dominant, 52.2/37.9 s in nondominant hand) were markedly skewed. Possible learning effects were small and likely clinically irrelevant. A composite functional index (SCAFI) was formed after appropriate transformation of subtest results. The Z-scores of each subtest correlated well with the Scale for the Assessment and Rating of Ataxia (SARA), the Unified Huntington's disease Rating Scale functional assessment, and disease duration. Correlations for SCAFI with each of these parameters were stronger (Pearson r = -0.441 to -0.869) than for each subtest alone. Furthermore, SCAFI showed a linear decline over the whole range of disease severity, while 9HPT and 8MW had floor effects with respect to SARA. Analysis of possible confounders showed no effect of genotype or study site and only minor effects of age for 8MW. CONCLUSION: The proposed functional measures and their composite SCAFI have favorable properties to assess patients with spinocerebellar ataxia.

Stages of sleep pathology in spinocerebellar ataxia type 2 (SCA2).

BACKGROUND: Autosomal dominant spinocerebellar ataxia type 2 (SCA2) bears clinical and neuropathologic similarities to sporadic multisystem atrophy (MSA) or Parkinson disease, in which sleep pathology is well documented. However, those clinical entities have a marked variability of the reported sleep disturbances, and their etiology is heterogeneous. In contrast, the study of SCA2 provides an opportunity to examine a molecularly homogeneous patient group, in which disease stages can be defined not only based on disease duration and ataxia scores, but also with regard to modulatory effects of mutation size. OBJECTIVE: To examine the presence and progression of sleep pathology in SCA2. METHODS: We analyzed eight patients with disease durations of 3 to 31 years, all with medium size SCA2 expansions (CAG 38 to 49), using clinical scores, sleep interviews, and video-polysomnography (VPSG) recordings. RESULTS: Almost all patients reported good subjective sleep quality and negated incidents of REM behavior disorder (RBD). At early disease stages, however, REM without atonia in four patients' VPSG suggested subclinical RBD. This was accompanied by a consistent reduction of REM density. In three patients at later SCA2 stages, REM sleep was undetectable, whereas slow wave sleep (SWS) was markedly increased at the cost of light sleep. Periodic leg movements, apnea, or hypopnea were not prominent. CONCLUSIONS: Progressive loss of dream recall in spinocerebellar ataxia type 2 was found and correlated with stages of REM more than non-REM pathology in video-polysomnography. These stages correspond to the progressive atrophy from the pons, nigrostriatal projection, and locus ceruleus to the thalamus.

Scale for the assessment and rating of ataxia: development of a new clinical scale.

OBJECTIVE: To develop a reliable and valid clinical scale measuring the severity of ataxia. METHODS: The authors devised the Scale for the Assessment and Rating of Ataxia (SARA) and tested it in two trials of 167 and 119 patients with spinocerebellar ataxia. RESULTS: The mean time to administer SARA in patients was 14.2 +/- 7.5 minutes (range 5 to 40). Interrater reliability was high, with an intraclass coefficient (ICC) of 0.98. Test-retest reliability was high with an ICC of 0.90. Internal consistency was high as indicated by Cronbach's alpha of 0.94. Factorial analysis revealed that the rating results were determined by a single factor. SARA ratings showed a linear relation to global assessments using a visual analogue scale, suggesting linearity of the scale (p < 0.0001, r(2) = 0.98). SARA score increased with the disease stage (p < 0.001) and was closely correlated with the Barthel Index (r = -0.80, p < 0.001) and part IV (functional assessment) of the Unified Huntington's Disease Rating Scale (UHDRS-IV) (r = -0.89, p < 0.0001), whereas it had only a weak correlation with disease duration (r = 0.34, p < 0.0002). CONCLUSIONS: The Scale for the Assessment and Rating of Ataxia is a reliable and valid measure of ataxia, making it an appropriate primary outcome measure for clinical trials.

Spatial chemical inhomogeneity and local electronic structure of Mn-doped Ge ferromagnetic semiconductors.

We have investigated the chemical distributions and the local electronic structure of potential diluted magnetic semiconductor Ge0.94Mn0.06 single crystals using scanning photoelectron microscopy (SPEM), x-ray absorption spectroscopy (XAS), and photoemission spectroscopy (PES). The SPEM image shows the stripe-shaped microstructures, which arise from the chemical phase separation between the Mn-rich and Mn-depleted phases. The Mn 2p XAS shows that the Mn ions in the Mn-rich region are in the divalent high-spin Mn2+ states but that they do not form metallic Mn clusters. The Mn 3d PES spectrum exhibits a peak centered at approximately 4 eV below E(F) and the negligible spectral weight near E(F). This study suggests that the observed ferromagnetism in Ge1-xMnx arises from the phase-separated Mn-rich phase.

Electronic structures of RTe2 (R = La,Ce): a clue to the pressure-induced superconductivity in CeTe1.82.

Electronic structures of RTe2 (R=La,Ce) have been investigated by using the local spin density approximation (LSDA) and the LSDA + U (U: on-site Coulomb interaction) band methods. Both LaTe2 and CeTe2 show the very similar Fermi surface nesting features along the [100] direction, which drive the charge-density wave (CDW) instability in the Te(1) sheets. The contribution near E(F) from Ce 4f states is negligible in agreement with the measured ARPES spectra. In the semimetallic CDW-distorted RTe2, both Te vacancy and pressure induce the charge transfer from Te(1) 5p to R 5d states, producing the enhanced density of states at E(F). We suggest that these increased self-doped Te(1) 5p hole carriers are responsible for the pressure-induced superconductivity in nonstoichiometric CeTe1.82.