A steroid-sensitive solitary intracranial plasmacytoma.
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Biomedical subjects
Publications and source records attributed to J S Kennerdell.
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The nonspecific orbital inflammatory syndromes are a peculiar group of inflammations that may occur in acute or subacute forms and can become chronic. They may be diffuse or primarily localized to a specific tissue of the orbit. The nonspecific inflammations that are targeted toward specific tissues are myositis, dacryoadenitis, perineuritis, and periscleritis. Each of these syndromes has definite signs, symptoms, ultrasonic and radiologic findings. Therefore, this group of nonspecific inflammations should be more clearly classified for purposes of better understanding and better management. All of these inflammatory syndromes in the acute form respond well to high doses of oral corticosteroids tapered gradually over a period of months, but may be reoccurrent or become chronic. The subacute form responds less well. Occasionally, patients require radiation to stop the inflammation in the subacute or chronic state, but these patients are often left with a functional deficit. The cause, although presumed to be an immune disorder involving the orbital tissues, remains unknown.
Schwannomas (neurilemomas) are benign tumors that arise from Schwann cells in the peripheral nervous system. The most commonly involved nerves that cause neuro-ophthalmic manifestations are cranial nerves V and VIII. In this series of three women, schwannomas presented as intraconal masses that mimicked a cavernous hemangioma, a superior orbital mass transgressing the superior orbital fissure, and an expansive frontal lobe mass with clinical symptoms and signs of increased intracranial pressure. Although all three complained of visual blurring, none of our patients presented with Vth or VIIIth cranial nerve dysfunction. Histopathologic studies demonstrated well-circumscribed, encapsulated spindle-cell lesions with classic Antoni A and B patterns. Histopathologic examination is essential to confirm the diagnosis of a schwannoma that may be otherwise clinically confusing. Direct optic nerve compression, globe indentation with induced hyperopia, or increased intracranial pressure with optic nerve compromise may be responsible for visual symptoms. A multidisciplinary approach is often required because of the size and location of schwannomas.
Acute orbital cellulitis is most commonly caused by sinusitis. This complication is based on the anatomy of the sinuses, orbit, and their venous connections. A series of 134 patients is reviewed to demonstrate the etiology and clinical course of orbital cellulitis. Antibiotic therapy alone does not prevent permanent complications. Surgical intervention is found necessary in all age groups and is required in 20% of the study population.
OBJECTIVE: To review the current role of measurement of serum eye muscle antibodies in thyroid-associated ophthalmopathy (TAO). METHODS: We conducted laboratory studies to determine the prevalences of serum autoantibodies reactive with eye muscle antigens in patients with active and inactive TAO, Graves' hyperthyroidism, and Hashimoto's thyroiditis as well as in normal subjects. RESULTS: The two antigens most often recognized in immunoblotting with crude human or porcine eye muscle membranes by serum autoantibodies in patients with TAO are eye muscle membrane proteins of 55 and 64 kd. One 64-kd eye muscle protein has recently been cloned by screening a human eye muscle expression library with two different antibody probes and identified from a computer gene bank search as the calcium-binding protein calsequestrin. A fragment of a 220-kd eye muscle protein, called G2s, has also been cloned by screening the eye muscle library with affinity-purified antibodies reactive with a 55-kd eye muscle membrane protein. The prevalences of autoantibodies reactive with these two antigens in our study groups were as follows. Antibodies against calsequestrin were detected in 38% of patients with TAO for <1 year, in 17% of those with TAO for >3 years, in 17% of patients with Graves' hyperthyroidism without ophthalmopathy, in 12% of patients with Hashimoto's thyroiditis without ophthalmopathy, and in 21% of normal subjects. Antibodies reactive with the 64-kd protein were demonstrated in 62% of patients with recent-onset active TAO, in 33% with eye disease for >3 years, in 39% of patients with Graves' hyperthyroidism without ophthalmopathy, in 25% of patients with Hashimoto's thyroiditis, and in 16% of normal control subjects. Antibodies reactive with G2s fusion protein were detected in 67% of patients with recent-onset active TAO, in 46% of patients with Graves' hyperthyroidism, and in 20% of normal subjects. Antibodies reactive with the parent protein, of which G2s is a fragment, may be markers of early eye muscle swelling and inflammation, whereas those reactive with the 64-kd protein and, less often, calsequestrin are associated with established eye disease. CONCLUSION: Measurement of serum eye muscle antibodies is recommended as an aid to the early diagnosis of ophthalmopathy in predisposed patients and first-degree relatives of patients with TAO as well as to monitor active or progressive eye disease.
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Presentation is made of a case of orbital aspergillosis, a rare condition that may clinically mimic nonspecific orbital inflammatory disease. It can be a subacute or chronic disease that develops in otherwise healthy individuals. Fine needle aspiration biopsy, which has been useful in the diagnosis of a variety of orbital diseases, provided the initial diagnosis in this case.
In the course of performing approximately 100 orbital aspirates, glandular epithelial inclusions were seen in three specimens from three patients. These inclusions, now recognized as benign and normal structures, initially led to false-positive cytologic results in two of the three cases. On the basis of reconstructing the clinical events, they appear to have been derived from the minor lacrimal glands in two cases and from the major lacrimal glands in the third. The potential hazard of regarding these glandular inclusions as indicating metastatic neoplasm is emphasized.
In order to determine whether components of the interleukin-2 receptor (IL-2R) and lymphoid cells are present in extra ocular and periocular tissues from patients with chronic, stable thyroid-associated ophthalmopathy (TAO) we studied 16 specimens of extra ocular muscle and periorbital connective tissue from 14 patients with chronic, stable, TAO using an immunohistochemical assay and a panel of murine monoclonal antibodies reactive with IL-2R alpha and beta components and lymphoid cell surface markers. As controls we studied orbital tissues from 11 patients undergoing surgery for unrelated orbital disorders. All extra ocular muscle specimens from patients with TAO exhibited IL-2R beta expression primarily on the perimysium and endomysium surrounding the ocular muscle fasciculi and fibers of which nine specimens stained intensely. The Natural Killer (NK) cell marker CD57 was the most common cell surface antigen detected, in seven of nine specimens, whose localization often corresponded to that of IL-2R beta distribution. No IL-2R alpha expression was detected in any specimen. Seven of the 11 control specimens were positive for IL-2R beta but in a less intense fashion than in TAO specimens while no CD57 staining was detected. T cell, B cell, and cells of granulocyte and monocyte lineage were only occasionally found in both TAO and control specimens. The aberrant expression of IL-2R beta and CD57 which may be representative of NK cell presence in extra ocular muscle tissues from patients with stable, chronic TAO may play a role in the pathogenesis of the ophthalmopathy.(ABSTRACT TRUNCATED AT 250 WORDS)
Fine needle aspiration (FNA) cytology of the orbit was used to diagnose an unusual case of multiple myeloma with secondary amyloid deposition in a 27-year-old man who presented with bilateral keratoconjunctivitis and left-sided ptosis. The FNA smears revealed immature plasma cells, extracellular eosinophilic material (amyloid) and numerous multinucleate giant cells. Subsequent histologic studies of excised tissue confirmed the cytologic diagnosis of plasmacytoma with secondary amyloidosis. Amyloid was further confirmed by electron microscopy. The patient was subsequently diagnosed as having multiple myeloma. The cytologic features of amyloidosis and plasmacytoma are presented. The differential diagnosis and the diagnostic utility of FNA cytology in the evaluation of these orbital lesions are also discussed. This apparently was the first case of orbital amyloidosis diagnosed by FNA cytology.