Two types of direction-changing positional nystagmus with neutral points.

OBJECTIVES: We encountered patients who had static direction-changing positional nystagmus (DCPN) canceled at about 20-30° yaw head rotation from the supine position. This nystagmus was also canceled when the head was rotated 180° from this position. We termed these head positions neutral points. The positional nystagmus observed (except at the neutral points) was thought to occur due to a "heavy cupula" or "light cupula". The purpose of this study was to examine DCPN with neutral points as well as the pathomechanism of this condition. METHODS: Retrospective case review of patients attending two hospitals. Sixteen patients who exhibited DCPN with neutral points were examined using an infrared camera (installed in goggles). Using this system, the vestibulo-ocular reflex (VOR) was recorded, and VOR gain was obtained. Vestibular function and the affected side were determined. In addition, the angle between the supine position and neutral point was measured in each patient. We also examined other positional nystagmus occurring at other times. RESULTS: In the heavy cupula type group, we noted positional nystagmus for which repositioning maneuvers were successful, whereas, in the light cupula type group, repositioning maneuvers were not effective. The angle between supine position and neutral point was 26.5 ± 11.6°. CONCLUSIONS: Heavy cupula type may occur as a result of otoconia while light cupula type may be due to the specific gravity of the endolymph. The VOR gain and side of the benign paroxysmal positional vertigo (BPPV) observed suggested that the affected side was that to which the neutral point was deviated.

[Successfully treated case of hypopharyngeal and cervical esophageal squamous cell carcinoma with laryngeal preservation].

The patient suffering from getting something lodged was admitted to our hospital in October 2008. He was diagnosed as hypopharyngeal cancer (cT2N1M0, cStage III) and cervical esophageal cancer (cT2N1M0, cStage III). Firstly he was administered 5-FU, DXR and CDDP as induction chemotherapy. The response evaluation was PR according to RECIST criteria. After the induction chemotherapy, he was treated with chemoradiotherapy (64.8 Gy/54 fr, concurrent with weekly DOC 10 mg/m2). Since cervical lymph node metastases were still remaining with complete response of the primary sites, we performed a neck lymph node dissection as salvage surgery in July 2009. There has been no evidence of recurrence after the salvage surgery.

Bioluminescence imaging of vascular endothelial growth factor promoter activity in murine mammary tumorigenesis.

Vascular endothelial growth factor (VEGF) is a major inducer of angiogenesis. We generated a transgenic reporter mouse, VEGF-GL, in which an enhanced green fluorescent protein-luciferase fusion protein is expressed under the control of a human VEGF-A promoter. The VEGF-GL mouse exhibited intense bioluminescence throughout the body at 1 week of age. The signals rapidly declined to a relatively low level as the mice grew. The adult VEGF-GL mouse showed restricted bioluminescence to the areas undergoing wound healing. In contrast, the VEGF-GL mice, which were crossed with mouse mammary tumor virus-polyoma virus middle T antigen transgenic mammary tumor mice, exhibited prominent bioluminescence in the tumors, correlating with VEGF transcription. Tumor bioluminescence was observed in the bigenic mice as early as 8 weeks, before tumors were palpable, and the signals increased with tumor growth. In conclusion, the VEGF-GL mouse permits longitudinal and quantitative assessment of VEGF promoter activity in vivo. The model should facilitate understanding of the molecular controls and pathways that regulate VEGF transcription in vivo.

Low-dose oral methotrexate for the management of childhood Cogan's syndrome: a case report.

Cogan's syndrome is a rare inflammatory disease characterized by nonsyphilitic ocular interstitial keratitis associated with hearing loss and vestibular impairment. Although systemic corticosteroids are usually used as the standard therapy, hearing ability in most cases gradually deteriorates. We, herein, describe a patient with childhood Cogan's syndrome who was treated with low-dose oral methotrexate, which successfully helped to taper the doses of the systemic corticosteroids. The serum levels of the complements were good markers for the disease activity in this patient.

A mutant receptor tyrosine phosphatase, CD148, causes defects in vascular development.

Vascularization defects in genetic recombinant mice have defined critical roles for a number of specific receptor tyrosine kinases. Here we evaluated whether an endothelium-expressed receptor tyrosine phosphatase, CD148 (DEP-1/PTPeta), participates in developmental vascularization. A mutant allele, CD148(DeltaCyGFP), was constructed to eliminate CD148 phosphatase activity by in-frame replacement of cytoplasmic sequences with enhanced green fluorescent protein sequences. Homozygous mutant mice died at midgestation, before embryonic day 11.5 (E11.5), with vascularization failure marked by growth retardation and disorganized vascular structures. Structural abnormalities were observed as early as E8.25 in the yolk sac, prior to the appearance of intraembryonic defects. Homozygous mutant mice displayed enlarged vessels comprised of endothelial cells expressing markers of early differentiation, including VEGFR2 (Flk1), Tal1/SCL, CD31, ephrin-B2, and Tie2, with notable lack of endoglin expression. Increased endothelial cell numbers and mitotic activity indices were demonstrated. At E9.5, homozygous mutant embryos showed homogeneously enlarged primitive vessels defective in vascular remodeling and branching, with impaired pericyte investment adjacent to endothelial structures, in similarity to endoglin-deficient embryos. Developing cardiac tissues showed expanded endocardial projections accompanied by defective endocardial cushion formation. These findings implicate a member of the receptor tyrosine phosphatase family, CD148, in developmental vascular organization and provide evidence that it regulates endothelial proliferation and endothelium-pericyte interactions.