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Biomedical subjects

K G Noble

Publications and source records attributed to K G Noble.

At least 37 records · Page 2Linked to original sources

Progressive cone dystrophy.

Psychophysical, reflectometric, and electrophysiologic studies were done on four members of a dominant pedigree with progressive cone dystrophy. The two youngest individuals were asymptomatic at the initial examination, and none of the subjects complained of problems associated with night vision. Nevertheless, absent or grossly reduced cone-mediated electroretinographic (ERG) responses showed the widespread loss of cone function, and moderate elevations (less than 1 log unit) in absolute threshold together with reductions in rhodopsin levels in the mid-peripheral retina provided evidence of impairment of the rod system. The progressive nature of the disease was apparent from the case histories and the changes in visual performance that occurred on re-test after a 5-year interval. Moreover, the results of increment threshold measurements at several retinal loci suggested that peripheral cones may be affected earlier and more severely than those in the central retina.

Adolescent↗

Progressive cone dystrophy.

Psychophysical, reflectometric, and electrophysiological studies were performed on four members of a dominant pedigree with progressive cone dystrophy. The two youngest individuals were asymptomatic at the initial examination, and none of the subjects complained of problems associated with night vision. Absent or grossly reduced cone-mediated ERG responses revealed the widespread loss of cone function. Moderate elevations (1 log unit) in absolute threshold together with reductions in rhodopsin levels in the midperipheral retina provided evidence of a mild impairment of the rod system also, although not to the degree seen in a cone-rod dystrophy. The progressive nature of the disease was apparent from the case histories and the changes in visual performance that occurred on re-test after a 5-year interval. Likewise, the results of incremental threshold measurements at several retinal loci suggested that peripheral cones may be affected earlier and more severely than those in the central retina.

Adolescent↗

Visual evoked potential (VEP) delays in central serous choroidopathy.

The authors examined a series of ten consecutive patients with unilateral, idiopathic central serous choroidopathy. Visual acuities ranged from 20/20 to 20/70 during the active stage. VEPs were recorded to square sizes of 14, 28, and 56 min of arc. Overall, 90% of the patients had statistically significant VEP delays from the affected eye, while only 30% had statistically significant reductions in amplitude during the active stage. Six of the ten patients were reevaluated after the condition fully resolved. In all six, the VEP latency returned to normal. Although the mechanism of these VEP delays is not clear, their presence has been well documented. Therefore, a VEP delay in isolation of other tests should not be used in the differential diagnosis of macular vs optic nerve disease. One should specifically rule out macular disease in any patient with a delayed VEP before presuming the presence of a visual pathway dysfunction.

Adult↗

Retinal vascular remodelling in radiation retinopathy.

A 34-year-old female developed bilateral radiation retinopathy three months after external beam radiotherapy for pituitary adenoma. The clinical symptoms, fundus abnormalities, and microvascular pathology noted on fluorescein angiography resolved spontaneously over a few months. The most dramatic aspect was the progressive improvement and partial reperfusion of the retinal capillary bed.

Adult↗

Pigmented paravenous chorioretinal atrophy.

Six patients (three men and three women, ranging in age from 19 to 65 years) with pigmented paravenous chorioretinal atrophy, a rare disorder of unknown origin, were studied for three- to 21-year periods. The diagnosis was made on the basis of the distinctive fundus appearance of bilateral, symmetrical bone corpuscular pigment accumulation exclusively along the distribution of the retinal veins. Although the fundus abnormalities can be mild or severe, retinal function tests indicated that this is a geographic and not a generalized disorder. Central visual acuities were normal. Follow-up studies showed no evidence of ophthalmoscopic or functional deterioration in five of the patients. In the sixth (Patient 6, the most severely affected), there was a mild progression of the fundus abnormality during the 21-year follow-up period.

Adult↗

Vitelliform macular dystrophy of late onset.

A family with vitelliform macular dystrophy is presented in which the proband became symptomatic at age 51 rather than during the more typical first or second decade. Elderly patients with vitelliform macular dystrophy may have clinical findings resembling age-related degenerative choroidopathy of the macula.

Adult↗