Oculocutaneous manifestations of thyroid disease.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to R C Sergott.
Explore the source record for details and available documents.
We injected guinea pig optic nerves with serum from patients with MS or acute optic neuritis (ON), or normal subjects. Serum from 12 of 17 MS patients, 3 of 3 patients with ON, and 5 of 11 normal age- and sex-matched controls produced myelin vesiculation and demyelination 24 hours after injection. Nerves injected with demyelinating serum contained oligodendrocytes with pyknotic nuclei and edematous, rarefied cytoplasm. Nerves injected with serum that did not cause demyelination did not have these oligodendrocyte changes. Serum from normal subjects or patients with MS may induce in vivo demyelination in mammalian CNS.
Acquired sixth-nerve palsies are relatively rare in younger adults. We reexamined 49 patients, aged from 15 to 50 years, with isolated sixth-nerve palsies who were seen between 1972 and 1982 at the Wills Eye Hospital in Philadelphia. In this group, the following etiologies were encountered: vasculopathy (14 patients [29%]), tumors (eight patients [16%]), multiple sclerosis (six patients [12%]), presumed inflammation (four patients [8%]), trauma (three patients [6%]), postlumbar puncture (two patients [4%]), and orbital amyloidosis (one patient [2%]). Eleven patients (22%) had no determined cause of their sixth-nerve palsy. The implications for the clinical management of isolated sixth-nerve palsies in younger adults are discussed.
Explore the source record for details and available documents.
Antiserum to the myelin lipid galactocerebroside (GalC) causes rapidly progressive focal demyelination when injected into guinea pig optic nerves. The capacity of anti-GalC to induce central nervous system demyelination is complement-dependent, and demyelinating activity is present in the immunoglobulin fraction of anti-GalC serum. Demyelination appears to result from primary damage to oligodendrocytes.
The etiology of anterior ischemic optic neuropathy (AION), when not associated with giant cell arteritis, is usually unknown. Clinical, pathologic, and experimental studies have not determined a cause. The optic disc appearance in both the involved and normal fellow eye was studied in 51 patients with acute nonarteritic AION. The number of discs (both involved and fellow) without a physiologic cup was significantly greater than would be expected from normal population studies. The etiology of nonarteritic AION may be related to the anatomic configuration of the optic nerve.
Two patients with presumed ocular histoplasmosis syndrome developed optic disc edema. In both cases the edema resolved without residual impairment of visual function. Although peripapillary changes in ocular histoplasmosis are common, optic disc edema is rare. The pathogenesis of the edema is unknown.
Two patients presented with simultaneous oculomotor nerve palsies clinically associated with diabetes mellitus. Although unilateral cranial nerve palsies frequently occur in diabetes mellitus, this report is the first documented occurrence of simultaneous, concurrent diabetic (vasculopathic) oculomotor nerve palsies. The differential diagnosis of bilateral ophthalmoplegia is discussed in detail.
Explore the source record for details and available documents.
Of 196 patients with anterior ischemic optic neuropathy, 169 had the nonarteritic form and 27 had the arteritic type. Visual acuities were 20/40 or better in 83 of 184 eyes with nonarteritic anterior ischemic optic neuropathy but only eight of 45 eyes with the arteritic type. We found systemic disease associations for hypertension and diabetes mellitus only for patients with nonarteritic anterior ischemic optic neuropathy who were between 45 and 64 years of age. After a mean follow-up period of five years, 92 nonarteritic patients showed no changes in the first affected eye; there was eventual involvement of the second eye in 20 patients.
The cases of three patients who developed combined central retinal artery/vein obstruction secondary to retrobulbar anesthesia are presented. In one case the obstruction was presumably present immediately after surgery, while in the other two it was observed to evolve over a period of several days. A dilated optic nerve sheath was demonstrated by contact B-scan ultrasonography in two cases and by computerized tomography in the third case, suggesting intravaginal sheath hemorrhage as a common underlying pathophysiologic process. Partial return of vision was evident in the latter case following orbitotomy with optic nerve sheath decompression.
Human leukocyte antigens (HLA) in patients with Graves' disease were investigated according to the ophthalmic classification system of the American Thyroid Association. All prior HLA studies of Graves' disease have disregarded the patients' specific ophthalmic manifestations. Examination of 18 A and 34 B loci antigens disclosed an increased frequency (corrected P = 0.002, relative risk = 13.1) of HLA-BW35 in patients improving with oral corticosteroids who have severe extraocular muscle and orbital inflammation (class 4-5) when compared to a geographically and racially matched control population. No statistically significant associations were found when patients without ophthalmic manifestations or with other categories of Graves' ophthalmopathy were compared to controls. The association of severe Graves' ophthalmopathy with HLA-BW35 may provide immunologic evidence to explain both the unpredictable association of the orbital and thyroid disturbances of Graves' disease as well as the unpredictable response of the orbital inflammation to oral corticosteroids.
Outlined is a coherent differential diagnostic approach to the neuro-ophthalmic syndrome of the "red orbit" that is accompanied by visual loss or diplopia that too frequently is misconstrued as intracranial pathology.
Three patients with multiple sclerosis (women 20, 24, and 30 years old) had demyelinative plaques that produced homonymous hemianopsia. These lesions were identified by computed tomography which showed the plaques as hypodense areas. All patients with homonymous hemianopsia should undergo computed tomography; if a hypodense or enhancing lesion is identified, the diagnosis of multiple sclerosis must be considered.
Supervoltage radiotherapy was used in 21 orbits of 19 patients with idiopathic inflammatory orbital pseudotumor. Seventeen orbits (15 patients) were initially treated with systemic corticosteroids, but recurrence of orbital inflammation during dosage tapering was the most frequent indication for radiotherapy. Fifteen orbits (14 patients) responded favorably, as judged by reduced proptosis, decreased lid edema and conjunctival injection, improved ocular motility, and increased visual acuity. Six orbits (five patients) did not improve with radiotherapy. Patients who were successfully treated with radiotherapy have been free of recurrence for a mean follow-up period of 25.05 months; these patients have not required further corticosteroid treatment or additional radiotherapy. Low-dose (1,000 to 2,000 rad) supervoltage radiotherapy seems to have a definite role in the management of idiopathic orbital pseudotumor in the following instances: (1) when corticosteroids fail or systemic complications are unacceptable (2) when signs and symptoms recur during decreasing corticosteroid dosage, and (3) when systemic corticosteroids are medically contraindicated.
Graves' ophthalmopathy is frequently a diagnostic and therapeutic dilemma for practicing ophthalmologists. We have reviewed the clinical immunology of this disorder with regard to clinical manifestations, humoral immunity, and cellular immunity. Special emphasis with applicable clinical and laboratory data is given to the hypothesis that Graves' thyrotoxicosis and ophthalmopathy may be frequently associated diseases rather than disorders caused by a common pathogenetic mechanism. Dysthyroidism may be the substrate for the development of the ophthalmopathy, but the two disorders probably are caused by different immunopathogenetic mechanisms.
Patients with severe Graves' ophthalmopathy may or may not improve with systemic corticosteroids. In an attempt to find immunologic distinctions to correspond with this clinical phenomenon, we have evaluated various parameters in corticosteroid-responsive and corticosteroid-resistant individuals. Percentages and absolute numbers of thymus-derived active and total rosette-forming cells (A-RFC and T-RFC) underwent statistically significant (p < 0.001) increases during successful prednisone therapy in 17 patients . B lymphocytes and complement receptor mononuclear cells did not significantly change with steroid therapy. In the second group, five patients therapeutically resistant to corticosteroids presented with elevated A-RFC and normal T-RFC. When treated with oral prednisone, these patients' A-RFC decreased (p < 0.001), and the T-RFC were unchanged. Corticosteroids increased the lymphoblast transformation response to phytohemagglutinin (PHA) of a steroid-responsive patient, whereas steroids decreased the PHA lymphoblast transformation response of a corticosteroid-resistant patient. A disease activity index to correlate the clinical and immunologic data has been devised. The findings may allow the clinician to predict which patients with Graves' ophthalmopathy are likely to benefit corticosteroid therapy and which patients may be managed better by other methods.
Pars planitis has rarely been noted to occur in more than one member of a family. We report the occurrence of this disorder in more than one member of two unrelated families. The affected members of one of these families included a 37-year-old woman and three of her six children. The affected members of the other family were two teenaged brothers. No specific cause for the familial clustering of pars planitis has been identified in the affected members of these families.