AIM: to investigate psychophysics and electrophysiology in disease states with altered retinal function. METHODS: determination of colour and luminance contrast sensitivity functions using computer controlled VDUs: recording of flash and pattern ERGs to short and long flashes of coloured lights under differing conditions of light adaptation: recording of pattern ERG. RESULTS: In melanoma-associated retinopathy (MAR) low spatial frequency temporal flicker loss occurs for achromatic Gaussians, but colour Gaussians are seen normally. Low spatial frequency luminance contrast sensitivity and motion losses are severe while red-green gratings are seen normally. In Cuban Tropical Amblyopia, achromatic luminance contrast sensitivity may be normal, in the presence of considerable losses of colour vision, to spatial frequencies as high as 32 c/degree. There are supernormal cone ERGs. CONCLUSIONS: these 2 conditions represent highly selective loss of 'M' and 'P' pathways respectively. Almost the entire 'bell shaped curve' normally represents M activity. In CTA, there may be a selective loss of the receptive field surrounds of P ganglion cells.

Color Perception↗

PubMedDate not supplied

Congenital duplication of the thumb.

Duplication or polydactyly of the thumb is among the most common congenital deformities that involve the hand. The purpose of this presentation is to review the Mayo Clinic experience with thumb duplication and to present preferred operative procedures to achieve the best clinical outcome and esthetic appearance for the hand. We propose that a combination or "best of parts", utilizing the components of both duplicates with retention of the ulnar duplicate as the base for reconstruction will provide the most functional clinical result. A clinical grading system and analysis of results from our personal experience during two different time frames provides the scientific support for our conclusions that combination procedures are preferred to simple excisions or to central joining of parts as in the Bilhaut-Cloquet procedure.

Child↗

PubMedDate not supplied

Pseudo oculomotor palsy as the presenting sign of linear scleroderma.

A case of linear scleroderma presenting as a pseudo oculomotor palsy is reviewed. The patient's facial skin abnormalities suggested the underlying etiology. This case illustrates a previously unreported finding in linear scleroderma, mydriasis without atrophy, reminding the clinician to look at the skin in patients with unexplained unilateral pupillary disturbances.

Biopsy↗

PubMedDate not supplied

Expression of folylpolyglutamyl synthetase predicts poor response to methotrexate therapy in patients with rheumatoid arthritis.

OBJECTIVE: The enzyme folylpolyglutamyl synthetase (FPGS) is involved in the resistance to methotrexate in tumor cell lines. The aim of the present study was to determine the impact of FPGS mRNA expression on resistance to methotrexate therapy in patients with rheumatoid arthritis (RA). METHODS: We determined the expression of FPGS mRNA using the Reverse Transcriptase Polymerase Chain Reaction (RT-PCR) in 141 patients with RA. All patients received methotrexate therapy. The primary outcome measures were disease activity as determined by a disease activity score (DAS) and response to therapy. RESULTS: Seventy-eight of 141 patients (55%) showed expression of FPGS mRNA. FPGS mRNA expression was not associated with age, sex, disease duration, white blood cell count, erythrocyte sedimentation rate, C-reactive protein (CRP), number of swollen joints, number of painful joints, and combined therapy with other disease-modifying antirheumatic drugs (DMARDs) or additional corticosteroids. The response rate to methotrexate therapy was 44% for the total study population. Patients without FPGS mRNA expression showed a significantly higher response rate than patients with FPGS mRNA expression (57% versus 33%; p = 0.005). Multivariate logistic regression analysis revealed that female sex (p = 0.009) and FPGS mRNA expression (p = 0.004) were independent predictive factors for failure to achieve a response to methotrexate therapy. CONCLUSION: FPGS mRNA expression is an independent predictive factor associated with poor response to methotrexate therapy in RA patients.

Adult↗

PubMedDate not supplied

Synovial extracellular matrix II. Specific incorporation of immunoglobulin into the cell-free matrix of pannus.

To determine whether immune complex-like material is incorporated into the extracellular matrix (ECM) of proliferated RA synovium, cell-free matrices were isolated from pannus removed at joint replacement surgery, and were subjected to differential extraction. When the IgG and albumin concentrations in the ECM extracts were compared to those in simultaneously obtained synovial fluids, the IgG was found to be enriched 8.8-fold. Approximately 95% of the IgG was extractable with 6M Guanidine-HCl and 8 M Urea-B-ME. Further extraction with collagenase and low-pH buffers did not result in any additional recovery of IgG. Matrix-associated IgG demonstrated a restricted mobility on IEF with a pI of 4.8. The extracellular matrix of RA pannus is enriched in an acidic IgG species. Incorporation of IgG appears to be secondary to non-covalent interactions and may represent an additional reservoir of immune complex material in the rheumatoid joint.

Albumins↗