Neuro-ophthalmology in the recent European literature. Part I. The visual system.
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Biomedical subjects
Publications and source records attributed to A B Safran.
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A 9-year-old boy with cyanotic congenital heart disease demonstrated congenital horizontal gaze paralysis and dysplasia of the left external and middle ear. Another patient associating congenital horizontal gaze paralysis and left hemifacial atrophy including ear dysplasia had previously been reported. The coincidence of such rare dysgenetic phenomena as congenital horizontal gaze paralysis and ear dysplasia in 2 patients seems to be significant. Since patients with congenital horizontal gaze paralysis often replace lateral gaze by convergence spasms, gaze paralysis sometimes remains undiagnosed. Therefore, when ocular motor disturbance is associated with ear dysplasia, the possibility of congenital horizontal gaze paralysis should be considered.
A unique case of sustained, rhythmical contractions of both orbicularis oculi induced by voluntary eye closure is described. It was observed in a 25-year-old man who had suffered a severe head trauma. The lid phenomenon was synchronous with macro square-wave jerks. Sustained blepharoclonus upon eye closure is likely to be related to dysfunction in the cerebellar system.
Two patients with generalized arteriosclerotic changes presented with oval-shaped pupils in both eyes. Sectorial palsy of the iris sphincters, essentially in the lower temporal segments, was demonstrated. Paralyzed sectors did not react to 2% pilocarpine. There was no dilator weakness, and the iris did not exhibit atrophy or synechia. We consider the sectorial palsy of the sphincters in these patients to be due to circulatory insufficiency of the iris. We reviewed previously published cases of diffuse or multifocal circulatory insufficiency with ischemic iris changes, and analyzed the accompanying photographs. We found that temporal segments of the iris are predominantly involved in these patients. Predisposition to this sectorial palsy, especially in the pupillary temporal region, might be explained by several anatomical particularities of the vascular network in the iris. Bitemporal palsy of the pupillary sphincters may be a sign of generalized circulatory insufficiency.
A case is reported of cholesterol crystal embolus in the retina. Up until now, no in vivo demonstration in the form of a printed photograph seems to have been published, showing changes in cholesterol embolus reflection, when the light and gaze incidence is modified.
Electro-oculographic recordings of horizontal eye movements in two patients with dorso-mesencephalic lesions are analysed. Tracings shows essentially: (A) dysconjugate glissadic dysmetria, with adduction overshoot and abduction undershoot, and (B) instability of ocular fixation. Fixation instability consists of bursts of 2 or 3 bilateral to-and-fro eye movements, of 4 degree or less amplitude. During a burst there is no evident latency between successive ocular movements. Bursts often appear following horizontal refixation saccades, and are then repeated once or twice with regular intervals. In one patient, fixation instability phases are more often elicited by saccades to the right than to the left. The typical occurrence of fixation instability after the fast phase of optokinetic nystagmus is described. The alteration of ocular fixation described in our patients belongs to the general group of "saccadic intrusions" as defined by Daroff et al. (1977). Resemblance with "lightning eye movements" (Atkin and Bender, 1964) and "opposed adducting saccades" in the Sylvian aqueduct syndrome (Ochs et al., 1979) is discussed.
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We studied two patients with eyelid nystagmus induced by ocular convergence. The first patient, a 29-year-old man, had the signs of a cerebellar syndrome after sustaining severe craniocerebral injuries. The second patient, a 12-year-old girl, had a tumor of the anterior vermis cerebelli. After treatment with corticosteroids (Patient 1) and surgery (Patient 2), the eyelid nystagmus gradually disappeared. An alteration in the cerebellar system is the most likely cause of convergence-evoked nystagmus.
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