A family is described with hypoplasia of the anterior iris stroma, no angle anomalies, and no glaucoma. The pedigree is suggestive of an autosomal dominant mode of inheritance. This is compared to the main peripheral malformations of the anterior chamber cleavage syndrome and other disease entities with similar iris changes. The pedigree represents a point on the continuum of mesoectodermal dysgenesis or anterior chamber cleavage syndrome.
This is a typical case of Hallermann-Streiff-Francois Syndrome in which the glaucomatous complication may be caused by developmental malformations in the anterior segment of the eye. Bilateral trabeculectomy succeeded in controlling the tension after a follow-up of two years. Glaucomatous complication seems to be frequent and as important as the other described cardinal symptoms. The histologic examination of the skin confirmed the atrophic changes that were observed clinically.
Among the total of 537 patients treated for retinal detachment 27 (5.02%) were under the age of 20. In 44.44 percent of the cases, there was a history of trauma. In 58.33% of the fellow eyes in this group of patients, retinal pathology was found. This pathology is considered to be a predisposing factor and trauma itself is only a trigger in causing retinal detachment.
Three cases of opsoclonus in normal neonates are reported. There was no evidence of neuroblastoma, encephalitis, or infantile polymyoclonia. It appears that opsoclonus may occur as a transient phenomenon in healthy infants.
A patient with a characteristic picture of rubella eye disease is presented. Its interest lies in the associated finding of asymmetric nystagmus and ocular flutter. The presence of flutter would indicate cerebellar or cerebellar pathway disease, a previously unassociated finding.
Intermittent exotropia is classified according to the AC/A ratio. Convergence amplitude measured on a light includes accommodative convergence. Amplitude measured while maintaining clear, single, binocular vision on 20/30 print utilizes only true fusional convergence.
A spike and wave discharge of photic-evoked myoclonic epilepsy was investigated employing square-wave pulses of a stimulator having mechanical shutter, and the following conclusions were reached. The amplitude of the spike and wave complex of photogenic epilepsy depends on the intensity of photic stimulation when this is investigated in the range from 2.5 X 10(3) ergs/cm2 sec to its 4.31 log units high. A spike prior to the slow wave appears as the stimulus intensity increases. The implicit time is shortened as the intensity increases. Prolongation of the stimulus duration makes the ascending phase of the slow wave steep, and an off-response appeared when stimuli of 100 msec or longer were applied. Clinically, a biphasic myoclonic convulsion of limbs is clearly observed in on- and off-phases when stimuli of 300 msec or longer are applied. The responses become prominent when frequency increases from 0.5 to 3.0/sec.
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