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Query: UMLS:C0028738 (nystagmus)
 7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

26 of both blind or partially blind children were subjected to equilibriometric tests. By way of a vestibular-ocular test they were given the Rotatory-Intensity-Damping test. During the tests the per and postrotatory nystagmus reactions were electronystagmographicly registrated. For the vestibular-spinal tests the Unterberger's stepping test with photographic registration of the body-sway in the Cranio-Corpo-Gram was used. In most of the children you could identify a definite per and postrotatory reaction in spite of considerable spontaneous reactions. The strong eyelid movements of these patients, the intensive spontaneous deviations and diminished corneo-retinal potential made precise judgement of the electronystagmogram difficult. Comparison between the blind and partially blind children enabled the differences to be objectified. Blind-born children have a higher spontaneous activity, slightly diminished per and postrotatory nystagmus reactions and a diminished bodyequilibrium. These results can be explained on one side by habituation and on the other side by efferent structures, which have an inhibitory effect on the vestibular input.
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PMID:[Equilibriometric findings in partially blind and blind children (author's transl)]. 30 Jun 24

A survey of 40 individuals registered with the Canadian National Institute for the Blind (CNIB) as blind from congenital nystagmus revealed that an abnormal single gene was responsible for the disorder in 33 patients. Fifteen of these were due to autosomal recessive conditions while X-linked disorders accounted for another 15 patients. In 3 cases the pedigrees were consistent with both autosomal recessive or X-linked inheritance. A clearly defined environmental origin was present in 1 case while specific genetic or environmental factors were not detected in the remaining six patients. The albinism, achromatopsia and Leber's congenital amaurosis groups of disorders were those most frequently detected.
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PMID:Congenital nystagmus--genetic and environmental causes. 30 14

Neuropsychologic findings during recovery from cortical blindness are described in four right-handed patients (two female, two male) aged 53 to 70 years. The lesions were due to occlusion of both posterior cerebral arteries (Case 1) and vascular spasm following subarachnoidal hemorrhage (Case 3) or angiography (Cases 2 and 4). Complete blindness lasted for 3 months until death in Case 1, 4 weeks in case 2, and 1 to 2 days in Cases 3 and 4. Confusional states and visual hallucinations were pronounced in three cases. Anosognosia (Anton's syndrome) was most pronounced in a patient with occlusion of both posterior cerebral arteries and less obvious in the remaining patients. During the recovery phase, symptoms of right hemisphere involvement were prominent with left-sided hemianopsia and diminished optokinetic nystagmus to the left, prosopagnosia in two cases, and dysmorphopsia with altered physiognomic recognition in one case. Transient disturbances of color recognition occurred in three patients. The observations are discussed with reference to clinical and neuropathologic findings of the literature.
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PMID:[Prosopagnosia and disturbance of color recognition during recovery from cortical blindness]. 30 40

The familial occurrence of retinal dysplasia in five affected male children suggested X-chromosome-linked recessive inheritance. The clinical features were childhood onset, severe visual impairment, head posture, nystagmus, and strabismus. The ophthalmoscopic findings varied in shape and extension; they ranged from retinal folds to dysplastic tissue covering the posterior pole or gliosis with tumor-like protrusion in the vitreous. The marked variability of the retinal findings was paralleled by the visual acuity, which ranged from some vision to blindness. Electroretinograms coordinated well with ophthalmoscopic observations. Of the five mothers, who are the presumed heterozygous carriers, two showed retinal changes.
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PMID:Primary retinal dysplasia transmitted as X-chromosome-linked recessive disorder. 56 35

The authors report 9 cases of cortical blindness (7 of vascular origin, 2 due to septic embolism). These were characterised by blindness, loss of the blink reflex in response to threat, and of optokinetic nystagmus, conservation of the pupil reflexes, a confusional state with memory disturbances and anosognosia. The accompanying E.E.G. changes were basically impoverishment of occipital alpha activity, with theta and delta waves mainly in posterior regions on one or both sides; blocking to eye opening was absent, and following of the occiptal background rhythm in response to photic stimulation was rare and partial. One patient developed repetitive slow waves in the left occiptal region, another developed bi-occipital spikes and a third developed bi-occipital paroxymal activity, more predominant on the right. These abnormalities resemble those seen during extraterritorial circulatory insufficiency or air emboli.
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PMID:[Clinical and E.E.G. study of 9 cases of cortical blindness (author's transl)]. 59 53

This report is a retrospective study of 33 patients seen over a 16-year period in whom a diagnosis of Leber's congenital amaurosis was made. The findings of an autosomal recessive heredity in 33%, connatal blindness (visual acuity less than 20/200) in 95%m nystagmus in 75%, and a markedly abnormal electroretinogram in 100% is in agreement with the findings of previously published large series. The difficulty in making the correct diagnosis initially was related to the wide variety of fundus findings and a high association (30%) of central nervous system disease. In the differential diagnosis of connatal blindness, only Leber's congenital amaurosis exhibits an absent or markedly diminished response on electroretinogram. The histopathologic findings in a 6-month-old infant with this disorder are compared with those of previously published reports.
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PMID:Leber's congenital amaurosis. A retrospective study of 33 cases and a histopathological study of one case. 65 18

Clinical signs of toxicosis, neurologic lesions, and increased tissue residues of methylmercury (MM)were produced in 9 cats by oral administration of 1.29 and 0.86 mg of Hg/kg of body weight/day as methylmercuric hydroxide. Clinical signs, which began after 15 days of exposure, included anorexia, ataxia, hypermetria, proprioceptive impairment, blindness, vertical nystagmus, and grand mal convulsions. Significant lesions occurred in cerebrum, brainstem, and cerebellum and correlated well with clinical signs. Microscopic central nervous system lesions consisted of neuronal degeneration, necrosis and loss of neurons, swollen axons, demyelination, loss of nerve cell processes, vacuolation of neuropil, gliosis, neuronophagia, perivascular cuffs, endothelial hypertrophy and hyperplasia, leptomeningitis, and infrequent vascular necrosis. Overall distribution of central nervous system lesions was unrelated to daily dose, but more advanced lesions were produced by the smaller daily dose. Mean tissue residues of MM were generally directly related to daily dose, and the average distribution among tissues was constant, with highest concentrations in liver, followed by kidney, spleen, muscle, and brain. In utero exposure of kittens to MM, revealed transplacental accumulation.
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PMID:Pathology of subacute methylmercurialism in cats. 83 68

Eight cases of obstructive hydrocephalus manifesting palsy of upward gaze and other features of the Sylvian aqueduct syndrome are reported. During the crisis of intracranial hypertension, all of them developed upward gaze palsy and variable abnormalities of the convergence mechanism such as paralysis, spasm, and convergence nystagmus. The frequent apparent blindness was probably related to gaze paralysis, since visual evoked responses were present. All these ocular abnormalities disappeared after shunting. Periaqueductal dysfunction on the basis of raised intracranial pressure is postulated as the possible mechanism for the above ocular manifestations. The 'setting sun' sign is frequently seen in infants and children with hydrocephalus and has been considered in the past to result from displacement of eyeballs by pressure from the orbital roof plate. Our observations would suggest periaqueductal dysfunction rather than the mechanical displacement as the possible mechanism for this sign.
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PMID:Sylvian aqueduct syndrome as a sign of acute obstructive hydrocephalus in children. 115 9

During the period 1970-73, 1,046 children under 20 years of age were registered with the Canadian National Institute for the Blind. The three most common registration diagnoses were Cataract (13%), Optic Atrophy (12%) and Nystagmus (10%); Retrolental Fibroplasia was responsible for a smaller proportion (6%). Twenty per cent of the registration diagnoses were non-specific and included "Nystagmus", "Site or Type not Established", "Affection of Visual Centre" and "Amblyopia". Without a specific diagnosis one could not decide whether the blindness was due to genetic or environmental causes. It is recommended that the C.N.I.B. establish a procedure by which children with a non-specific registration diagnosis can be referred for further investigation.
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PMID:Causes of blindness in children. 1046 cases registered with the Canadian National Institute for the Blind 1970-1973. 119 67

The authors investigated 7 children with developmental anomalies of the optic disc. In all cases a typical pattern of hypoplasia was observed. In all patients was found a very marked impairment of visual acuity or even blindness of the affected eye with most frequently squint and nystagmus. Besides ophthalmological investigation, neurological examination were carried out in these children and the Wassermann reaction was determined, together with tests for toxoplasmosis and radiograms of the optic foramina.
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PMID:[Congenital hypoplasia of the optic nerve (author's transl)]. 119 50


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