Biomedical subjects
Publications and source records attributed to G J Morgan.
Two patients with Cogan's syndrome had acute bilateral hearing loss and vestibular dysfunction. Interstitial keratitis was discovered by slit-lamp examination. Both cases responded to corticosteroid treatment. Immediate recognition of this syndrome and the institution of high-dose corticosteroid therapy should lead to resolution of the hearing loss in patients with Cogan's syndrome.
Duchenne muscular dystrophy (DMD) is an X-linked recessive genetic disorder for which the biochemical defect is as yet unknown. Recently, two cloned segments of human X-chromosome DNA have been described which detect structural alterations within or near the genetic locus responsible for the disorder. Both of these cloned segments were described as tightly linked to the locus and were capable of detecting deletions in the DNA of boys affected with DMD. In an attempt to determine more precisely the occurrence of these deletions within a large population of DMD patients and the accuracy of one of the segments, DXS164 (pERT87), in determining the inheritance of the DMD X chromosome, the subclones 1, 8 and 15 were made available to many investigators throughout the world. Here we describe the combined results of more than 20 research laboratories with respect to the occurrence of deletions at the DXS164 locus in DNA samples isolated from patients with DMD and Becker muscular dystrophy (BMD). The results indicate that the DXS164 locus apparently recombines with DMD 5% of the time, but is probably located between independent sites of mutation which yield DMD. The breakpoints of some deletions are delineated within the DXS164 locus, and it is evident that the deletions at the DMD locus are frequent and extremely large.
A central auditory test battery including dichotic digits, competing sentences, frequency patterns, and the staggered spondaic word test was administered to a patient diagnosed as having vasculitis. Test batteries were conducted before prednisone treatment and 2 months later. The central tests were not only helpful in indicating probable intracranial right hemisphere involvement, but seemed to be sensitive to the patient's improvement over this 2-month period. This case demonstrated a potentially valuable application of central auditory assessment.
The use of biological agents in inflammatory conditions is rapidly increasing. TNFalpha blocking treatments have changed the course of rheumatoid arthritis, Crohn's disease, juvenile rheumatoid arthritis and psoriatic arthritis. Open label studies with TNFalpha inhibitors in other inflammatory conditions such as adult Still's disease, uveitis, Wegener's granulomatosis, Behçet's disease, scleroderma, Sjögren's syndrome, sarcoidosis, pyoderma gangrenosum, polymyositis/dermatomyositis have shown promising results. However, whether anti-TNFalpha therapy can be safely and efficaciously applied to these other inflammatory disorders requires further controlled studies.
During a five-year period, surgical release of the carpal tunnel was performed on 150 patients with median nerve compression. Seventeen (11%) had rheumatoid arthritis. Among this subgroup, six patients had anomalous muscles in the wrist and proximal palm. An additional patient with psoriatic arthritis demonstrated a comparable abnormality. No other patients in this series had similar findings. This statistically highly significant occurrence is probably related to additive effects of the two disorders in producing median nerve compression.
Two cases of suspected septic arthritis of the sacroiliac joint with normal radiographs and nuclide scans were investigated by computed tomography. Bone and soft tissue abnormalities consistent with septic arthritis were found. Diagnosis was confirmed by needle aspiration of the sacroiliac joint. Computed tomography may be useful in the early diagnosis of the sacroiliac joint when plain radiographs and nuclide scans are normal.
A 29-year-old woman had surgery for a level IV malignant melanoma of the right calf. After a disease-free interval of almost 4 years, she presented with right knee arthritis that proved to be due to recurrent melanoma in the femoral condyle. Metastatic carcinomatous arthropathy should be considered in the differential diagnosis of monoarthritis.