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Biomedical subjects

B R Younge

Publications and source records attributed to B R Younge.

At least 37 records · Page 2Linked to original sources
PubMed1990

Late juvenile-onset Krabbe's disease.

Krabbe's disease is an autosomal recessive leukodystrophy characterized by a lack of galactocerebroside beta-galactosidase activity. In contrast to the classic early infantile-onset form of Krabbe's disease, less recognized, late-onset variants exist. The authors present a case of late juvenile-onset Krabbe's disease, including the associated magnetic resonance imaging (MRI) findings. Most patients with late-onset Krabbe's disease present with visual loss due to optic atrophy. Associated gait abnormalities and parental consanguinity should increase the clinician's suspicion that a child may have late-onset Krabbe's disease. Because of the prolonged survival in late-onset Krabbe's disease, the recent development of bone marrow transplantation for these patient makes diagnosis of this disorder particularly important.

Adolescent↗
PubMed1989

The significance of retinal emboli.

This review contrasts two of the more common forms of retinal emboli, i.e., cholesterol emboli and calcific emboli. These two forms vary in ophthalmoscopic appearance and in regard to significance.

Calcium↗
PubMed1987

Optic neuropathy and amiodarone therapy.

Optic neuropathy has been diagnosed in several amiodarone-treated patients, including the 13 patients described in this report. The clinical severity of this drug-related optic neuropathy is milder than that characteristically described in anterior ischemic optic neuropathy. The incidence of occurrence was significantly higher than that found in an age-matched general population sample. Whether this result was due solely to amiodarone therapy, to the underlying poor health of these patients, or to a combination of these two factors is uncertain. The findings in this study prompt us to recommend that all patients who receive amiodarone undergo complete ophthalmologic examinations, including careful evaluation of the ocular fundus regularly during such therapy. Appearance of optic neuropathy is probably a relative indication for discontinuing the use of the drug, in the hopes of avoiding bilateral involvement or perhaps recovering vision. The risks of complications of amiodarone treatment must be weighed against the benefit of therapy in patients whose lives are threatened by cardiac arrhythmias. On the basis of this study, the benefits of treatment seem to outweigh the small risk of optic neuropathy. No randomized study has been undertaken to determine the true incidence of complications associated with this medication and at this time could not be justified.

Aged↗
PubMed1986

Cogan's syndrome: 18 cases and a review of the literature.

In this article, we review 78 cases of Cogan's syndrome--18 from our institution and 60 from the English medical literature--and describe the clinical, laboratory, radiographic, and pathologic findings associated with this disorder. Patients with Cogan's syndrome should be examined by an ophthalmologist, otolaryngologist, and internist because, in addition to the eye and audiovestibular involvement, systemic manifestations are common. An elevated erythrocyte sedimentation rate, anemia, leukocytosis, and thrombocytosis are common but nonspecific laboratory abnormalities. Serious outcomes include deafness and, less frequently, vasculitis, aortic insufficiency, blindness, and death. Glucocorticoids seem to be effective therapy for active Cogan's syndrome. Aortic valve replacement and vascular bypass grafting are indicated in selected cases. The roles of cytotoxic-immunosuppressive agents and cochlear implantation remain to be clarified.

Adolescent↗
PubMed1985

Cogan's syndrome: audiovestibular involvement and prognosis in 18 patients.

The experience at the Mayo Clinic with 18 patients who had Cogan's syndrome is reviewed. Typically, the illness began with systemic symptoms. The ocular and audiovestibular symptoms included ocular discomfort and redness, photophobia, and fluctuating sensorineural deafness with imbalance. Each patient had interstitial keratitis; the ocular symptoms periodically recurred but responded to therapy. Thirteen patients who had fluctuating bilateral sensorineural deafness subsequently suffered total bilateral deafness. Two patients who are unilaterally deaf have good residual hearing. Two other patients regained hearing within normal limits after treatment with corticosteroids. No hearing symptoms were observed in one patient who had severe vertigo and whose condition is stable. Seventeen patients had vertigo: 11 have no labyrinthine function, 1 had absent responses on vestibular testing initially but currently demonstrates good responses, and the 5 others have remained stable with diminished vestibular function. If Cogan's syndrome is diagnosed early and treatment with corticosteroids is initiated promptly, hearing can be stabilized; otherwise, the prognosis for auditory function is poor.

Adolescent↗
PubMed1983

Ophthalmic manifestations of acoustic neurinoma.

Acoustic neurinomas comprise 8% of all primary intracranial neoplasms. The authors reviewed 100 cases of pathologically confirmed acoustic neurinomas and compared tumor size, based on observations at operation, with findings on history and physical examination. Of the 100 patients, 36 had a decreased corneal reflex, 36 had nystagmus, and 8 had papilledema. Ninety-two percent of the patients with nystagmus had brainstem compression from tumor, and unilateral nystagmus when present was predominantly on gaze toward the side of the tumor. A positive correlation was found between tumor size and the presence of signs and symptoms. Tumors causing nystagmus were at least 2 cm in greatest dimension; those causing a diminished corneal reflex were at least 2.5 cm; and those causing subjective symptoms and papilledema were at least 4.0 and 4.5 cm, respectively.

Adolescent↗
PubMed1982

Ophthalmodynamometry and ocular pneumoplethysmography for detection of carotid occlusive disease.

The accuracy of ophthalmodynamometry and ocular pneumoplethysmography for predicting pressure-significant lesions of the internal carotid system was compared in a series of 119 consecutive patients (210 arteries) having cerebral angiography. Overall accuracy (92.5%) for ocular pneumoplethysmography was significantly better than that for ophthalmodynamometry (76%). The difference was most apparent in predicting abnormality (85% for ocular pneumoplethysmography and 49% for ophthalmodynamometry), particularly for bilateral lesions (80% for ocular pneumoplethysmography and 37% for ophthalmodynamometry). There were few false-positive results with either test.

Arterial Occlusive Diseases↗
PubMed1982

Glaucoma visual field analysis by computed profile of nerve fiber function in optic disc sectors.

Automated perimetry has decreased the subjective aspects of data collection, but analysis has remained largely subjective. A microcomputer permits more objective analysis by regrouping and averaging data points in the threshold static visual field according to their retinotopic projection onto the optic disc rather than according to their eccentricity from the point of fixation. The applications of the technique include (1) following glaucomatous visual field loss, (2) differentiating glaucomatous from other forms of visual loss, and (3) studying the effects of aging on the visual field. Six formulas for data analysis are described, and their relative usefulness discussed. Percent loss or gain seemed to convey the most diagnostic information to the clinician. Percent of expected sensitivity was less than the decimal visual acuity when the diagnosis was glaucoma but greater when the diagnosis was cataract. In some cases this analytic method should provide information that could favorably affect patient management.

Adolescent↗
PubMed1982

The neurosurgical management of optic glioma. Results in 104 patients.

A series of 104 patients with surgically verified optic nerve gliomas is presented. Anterior lesions primarily affecting the optic nerve were present in 42 patients, who have a long-term survival rate of 85.5%. Posterior lesions primarily affecting the optic chiasm were present in 62 patients, most of whom underwent radiation therapy postoperatively, and they have a long-term survival rate of 50%. Unequivocal signs of neurofibromatosis were present in 14%. On the basis of these findings and a review of the literature, recommendations are made for the surgical management of these tumors.

Adolescent↗
PubMed1982

Optic glioma. Long-term follow-up of 85 histopathologically verified cases.

Eighty-five cases of histologically verified gliomas of the optic nerve or chiasm were studied retrospectively. Patient survival was analyzed according to location of tumor, treatment received, presence of neurofibromatosis, and degree of histologic anaplasia. Thirty-three patients had optic nerve tumors, and 52 had tumors involving the chiasm. Follow-up ranged from 6 days to 43.7 years. Of the 33 patients with optic nerve tumors, 28 (85%) survived a mean duration of 17 years, and survival was significantly associated with completeness of surgical excision. Twenty-three of the 52 patients (44%) with chiasmal tumors survived a mean duration of 19 years. Survival among patients with chiasmal tumors was unrelated to therapy received and was affected adversely by concomitant involvement of adjacent brain structures. The presence of neurofibromatosis conferred a protective benefit to patients with chiasmal tumors. No other significant factors could be statistically identified to influence the prognosis of patients with optic gliomas.

Adolescent↗
PubMed1981

Paralysis of cranial nerves III, IV, and VI. Cause and prognosis in 1,000 cases.

An unselected series of 1,000 cases of paralysis of cranial nerves III, IV, and VI was retrospectively analyzed regarding ultimate recovery and final causal diagnosis. The frequency of involvement of the third, fourth, and sixth cranial nerves was relatively unchanged from earlier similar reports. The number of patients (263) whose cranial nerve paralysis was initially of undetermined cause was surprisingly high despite the availability of computerized tomographic scanning. Subsequently, the cause for the paralysis was diagnosed in only ten of the 127 patients who could be traced. About half (51%) of the patients with no known cause for paralysis underwent spontaneous remission. Forty-eight percent of all patients recovered. Cranial nerve impairment due to vascular disease (diabetes mellitus, atherosclerosis, or hypertension) was temporary in 71% of the patients, regardless of the cranial nerve affected. Patients with palsies caused by aneurysm, trauma, and neoplasm was predictably less likely to recover.

Abducens Nerve↗
PubMed1981

Visual prognosis in carotid-cavernous fistula.

The initial signs and symptoms and subsequent course of 74 patients with carotid-cavernous fistula were evaluated in relation to the prognosis for final visual acuity. Two thirds of the patients underwent carotid artery surgery because of the fistula. Of this surgical group, 12 (28%) had further visual loss and 5 (10%) had cerebral ischemia. Elevated intraocular pressure tended to be more frequent in patients with proptosis and conjunctival injection, but elevated pressure did not seems to be a major cause of visual loss. The visual prognosis was more favorable in this series than in previous reports.

Arteriovenous Fistula↗
PubMed1981

Tobacco-alcohol amblyopia.

A series of 52 patients with scotomas attributed to tobacco smoking, alcohol consumption, and nutritional deficits--alone and in combination--was reviewed. Whether use of tobacco alone can produce a scotoma has been a controversial point; our series suggests that it can. Central and cecocentral scotomas did occur in association with smoking alone; there seems to be an association between cecocentral scotoma and cigar smoking especially. Central scotomas were seen more often in patients who consumed alcohol. Recovery from either type of scotoma was observed in 3 months when a therapeutic program of abstinence and B vitamins was followed. Although the number of patients we see with these scotomas has decreased, clinicians are urged to be aware of this disorder.

Aged↗