Biomedical subjects
Publications and source records attributed to S Lessell.
Carotid artery dissection frequently causes transient ipsilateral visual impairment. We present 2 cases of permanent ocular vaso-occlusion sequelae consequent to dissection of the ipsilateral internal carotid, definite in one and probable in the other. In both, the ocular strokes led to the recognition of the underlying carotid vasculopathy.
We examined ten patients with nonarteritic anterior ischemic optic neuropathy in whom the visual deficit progressed over a number of weeks or who suffered a recurrence in the same eye. The visual deficits progressed over a month or more in five patients, four patients suffered a recurrence in the same eye after a long interval, and one patient had bilateral progression and a recurrence.
A 69-year-old hyperopic man developed acute night blindness and hallucinations of shimmering lights three years after resection of a cutaneous malignant melanoma. There were no metastatic ocular lesions and he had received no medications. His electroretinogram showed abnormalities comparable to those of patients with congenital stationary night blindness with myopia. Metastatic melanoma was recognized several months later. His electroretinographic responses were also identical to those ascribed to vincristine therapy in a previously described patient with malignant melanoma. Our findings showed that acquired night blindness, apparently resulting from interruption of intraretinal rod signal transmission, can be a paraneoplastic effect of a malignant melanoma.
Ophthalmic and neurologic involvement in chronic lymphocytic leukemia is uncommon, and if it does occur, it is usually only late in the course of the disease. We report three cases in which progressive visual loss from optic nerve infiltration was an early clinical manifestation of chronic lymphocytic leukemia. Progressive optic atrophy with loss of acuity and visual field occurred in all cases, preceded in one patient by transient visual obscurations and disc edema. Surface marker studies of cerebrospinal fluid lymphocytes were useful in differentiating leukemic optic nerve infiltration from other causes of optic nerve damage. Optic nerve irradiation gave considerable clinical improvement in all three cases.
We prospectively studied 60 white patients living in New England who presented with uncomplicated optic neuritis (ON) to determine the risk of developing multiple sclerosis (MS). The diagnoses of ON and MS were made solely on the basis of clinical criteria. Patients were followed for a mean of 14.9 years. Life table analysis indicated that 74% of the women and 34% of the men will have developed MS 15 years after their attack of ON. The risk of developing MS was 3.4 times greater for women than for men. Onset of ON between the ages of 21 and 40 years may have had a modest effect in increasing the risk of developing MS. Recurrence of ON did not appear to affect the risk.
Two case histories, each spanning more than 20 years, demonstrate the complexity and persistence of modern neurosyphilis. Both patients present uncommon manifestations of neurosyphilis that may be easily overlooked today: superior orbital fissure syndrome, posterior fossa gumma, and bilateral deafness. Computed tomographic scans were performed in both patients but were diagnostically nonspecific, and syphilis serology testing in both serum and cerebrospinal fluid was repeatedly misleading. Numerous mistaken diagnoses were put forward until tissue was finally available for histologic examination. Perhaps the most disturbing aspects of these two cases are the persistent failure of specific treponemal serologic testing to indicate the diagnosis, and the inability of repeated "adequate" and/or supramaximal doses of penicillin to prevent continued neurologic deterioration. We believe that chronic neurosyphilis may demand a reevaluation of current diagnostic and therapeutic practice.
We examined a 32-year-old, previously healthy man who developed episodic bilateral visual impairment and confusion. Coincident hyperammonemia led to the diagnosis of ornithine transcarbamoylase deficiency, which was established by enzymatic analysis of a liver biopsy specimen. The available data were insufficient to determine if the metabolic derangement impaired vision at the level of the optic nerves or at the cerebral level.
We examined two patients who awoke with profound bilateral visual loss after operations under general anesthesia. Their fundi, initially normal, later showed bilateral optic atrophy. Neither patient showed other neurologic deficits, although one demonstrated radiologic evidence of a small cerebral infarction in the deep white matter. These patients probably suffered intraoperative infarction of the retrobulbar segments of both optic nerves, producing posterior ischemic optic neuropathy. Profound systemic hypotension may have been a contributing factor in one patient, the use of the pump-oxygenator in the other, and anemia in both.
Explore the source record for details and available documents.
A steroid-sensitive meningo-encephalopathy developed in a healthy young woman 5 1/2 years after the occurrence of acute posterior multifocal placoid pigment epitheliopathy (APMPPE). The association of APMPPE with systemic and neurologic inflammatory disorders has been well documented, but to the knowledge of the authors, this is the first report of an APMPPE patient in which there was delayed onset of the meningo-encephalopathy. The association with dermal vasculitis, nephropathy, meningo-encephalitis, cerebral vasculitis, and thyroiditis suggests that APMPPE may be one manifestation of a diffuse systemic inflammatory condition.
Ocular neuromyotonia is a paroxysmal monocular deviation that results from spasm of eye muscles secondary to spontaneous discharges from third, fourth, or sixth nerve axons. We observed this rare disorder in four patients who had been treated with radiation for tumors in the region of the sella turcica and cavernous sinus. Based on these cases and four others identified in the literature it would appear that radiation predisposes to a cranial neuropathy in which ocular neuromyotonia may be the major manifestation. Radiation appears to be the most common cause of ocular neuromyotonia.
We examined three patients with classic findings of familial dysautonomia (Riley-Day syndrome) whose visual impairment was associated with optic atrophy. The presence of an optic atrophy in familial dysautonomia is indicative of central nervous system involvement, at least in these cases. Each of these patients was first noted to have visual impairment after the first decade. The late onset of optic atrophy may partly explain its apparent rarity. Since the life span of patients with familial dysautonomia is increasing, optic atrophy may be more commonly recognized in the future.
Altitudinal visual field defects were discovered in four young women on routine eye or general physical examinations. This led to extensive investigations because three of them were falsely presumed to have acquired disorders of the central nervous system. In each case the defect appeared to result from congenital hypoplasia of the optic nerve associated with maternal diabetes mellitus. Congenital optic nerve hypoplasia should be included in the differential diagnosis of visual field defects even when they are discovered in adults. Hypoplasia consequent to maternal diabetes mellitus is apt to be associated with excellent visual acuity and is usually an isolated defect. The female offspring of diabetic mothers may be at greater risk than the male offspring for the development of this anomaly.
Contrary to statements in the literature, youth does not seem to confer protection from visual loss in pseudotumor cerebri. We examined five children and adolescents who suffered permanent loss of visual acuity or field in association with this disorder. All of them had impairment at the time of diagnosis, and two of the five developed further visual failure while under observation. Children and adolescents with pseudotumor should be kept under the same close ophthalmic surveillance as has been advocated for adults with this disease.
Questionnaires were completed by 1083 nonesotropic control subjects and 170 patients with nonparalytic esotropia to determine handedness. The subjects, who had been drawn from patients attending ophthalmic clinics and private practices, were classified as right-handed, left-handed, or ambidextrous based on their answers to five questions about hand preference. Analysis of the results indicated that the handedness of patients with esotropia differed significantly from that of nonesotropic controls. The difference resulted primarily from an excess of non-right-handers among those with esotropia. Non-right-handedness is probably a marker of anomalous cerebral dominance and the disproportion of left-handed and ambidextrous subjects with esotropia may indicate that some persons with esotropia have anomalous brain architecture. In such cases, the structural anomalies might be the cause of the strabismus.
The nosology of the Laurence-Moon and Bardet-Biedl syndromes has been controversial. Presented is a patient with polydactyly, retinopathy, ataxia, low-average intellectual function, and obesity. These features constitute a composite of both syndromes and reflect the clinical heterogeneity that may be seen. Accordingly, the authors suggest the use of the term "Laurence-Moon-Bardet-Biedl phenotype" until these syndromes can be defined in some other manner. The neuroradiologic studies document atrophy of the cerebellum that accounts for the ataxia. Electroretinograms (ERG) demonstrate the decline in retinal function over a 16-year interval and the delayed cone ERG b-wave implicit time with normal cone amplitudes to 30 Hz white flicker that can exist in the early stage of this disorder.
After years of episodic monocular visual loss, two migraineurs suffered sudden, persisting loss of vision from retinal vascular occlusion. One was a 34-year-old woman with systemic lupus erythematosus who showed abnormalities of the cilioretinal arterial and retinal venous circulations. The other was a 62-year-old man with hypertension and arteriosclerosis who had a central retinal vein occlusion. Persisting monocular visual loss is a rare consequence of migraine. Our cases suggest a role for venous lesions. Occlusion of retinal vessels in some migraineurs may result from the synergistic effect of another vascular disorder with the migraine.
A 40-year-old woman developed profound bilateral loss of vision from chronic atrophic papilledema. There were striking optociliary shunt vessels on the right optic disc. Within 1 month after removal of a massive intracranial meningioma, the shunt vessels had regressed markedly. This regression was evident on fluorescein angiograms. We attribute their resolution to the normalization of intracranial pressure.