Primary brain myxoma, an unusual tumor of meningeal origin: case report.

OBJECTIVE AND IMPORTANCE: Primary myxoma of the central nervous system is an extremely rare tumor arising from cells of primitive mesenchymal origin. Only two cases of primary intracranial myxoma have been described previously. We report a patient with a primary myxoma originating from the right frontoparietal convexity dura, which we studied in detail with diagnostic imaging and pathological analysis. CLINICAL PRESENTATION: A female adolescent presented to the emergency department with a 3-day history of mild headache, abdominal pain, and intermittent left-sided focal motor seizures. Neurological examination was remarkable for left leg hyperreflexia and difficulty with tandem gait. Cranial computed tomography and magnetic resonance imaging demonstrated an inhomogeneously enhancing mass in the right frontoparietal region. INTERVENTION: A right frontoparietal craniotomy was performed. During surgery, a tumor appearing similar to a typical convexity meningioma was completely removed along with the dural attachment. CONCLUSION: The patient had an uneventful recovery and returned to normal activity. Primary intracranial myxoma should be distinguished from other meningeal tumors and metastatic cardiac myxoma by appropriate pathological analysis and cardiac evaluation. A circumscribed myxoma completely excised with adequate dural margin carries a good prognosis for surgical cure.

A survey of moyamoya disease in Hawaii.

Moyamoya disease (MMD) is a rare entity in the US with a few retrospective series and sporadic cases reported in the US and North America. Although it is known that MMD exists in all races, there is a predilection for people of Asian origin. Because of the relatively high percentage of Asians living in Hawaii, it was hypothesized that the estimated prevalence of MMD would be higher in Hawaii than the remaining US. All practicing neurologists, neurosurgeons, neuroradiologists and major hospitals in Hawaii were surveyed for MMD patients treated during the past 10 years. Medical records and angiograms (when available) were reviewed and the diagnostic guidelines for MMD from the Ministry of Health and Welfare of Japan applied. There were 53 records reviewed in 42 patients; 21 fulfilled the criteria for definite Moyamoya disease which were the focus of this study. In Hawaii, the prevalence of MMD was higher in patients of Japanese descent compared to Caucasians (P = 0.012) and higher than in the remaining US (P < 0.001). Non-Japanese Asians and Pacific Islanders had a higher incidence of MMD than Caucasians that was not statistically significant. There was no difference in MMD among Japanese living in Hawaii or Japan. Males had an equal percentage of hemorrhage and infarcts; females tended to have a higher incidence of ischemic events rather than hemorrhage. Age and sex distribution of our series were similar to larger reported MMD studies. Our results suggest that: (1) Moyamoya disease in Hawaii has a higher incidence and prevalence than the rest of the US, largely due to the larger percentage of Asians, particularly Japanese, living in Hawaii; and (2) genetic rather than environmental factors may explain the increased MMD in Hawaii.

Conjunctival cilia entrapment: an unrecognized cause of ocular irritation.

Conjunctival entrapment of cilia is form of ocular irritation that to our knowledge has not yet been described. Scarring predisposes the conjunctiva to this phenomenon by creating blind recesses in which loose cilia can become entrapped. Once caught, the rigid cilia can act as a mechanical irritant or act as a nidus for microbial growth. We present herein three patients with entrapped cilia and discuss their pathophysiology and management. The symptoms in all three patients resolved with conjunctivoplasty and removal of the cilia.

Infectious crystalline keratopathy due to Streptococcus pneumoniae. Possible association with serotype.

BACKGROUND: Infectious crystalline keratopathy is a distinctive clinical entity characterized by bacterial replication within the corena without inflammation. The authors report on a patient with infectious crystalline keratopathy due to Streptococcus pneumoniae serotype 11F. They used this isolate to study the contribution of the pneumococcal polysaccharide capsule to the pathogenesis of the infectious crystalline keratopathy. METHODS: Aliquots containing 10(6) colony-forming units of pneumococci serotype 11F, serogroup 9 or 15, were inoculated into New Zealand white rabbit corneas. The corneas were examined at 24, 48, and 72 hours. Representative corneas were excised at 24 hours and processed for histopathologic analysis. RESULTS: Pauci-inflammatory crystalline lesions developed in all corneas inoculated with the serotype 11F ocular isolate by 24 hours. Suppurative keratitis developed in control corneas inoculated with serogroup 9 or 15 pneumococci. The nonocular 11F isolates produced lesions with some features compatible with infectious crystalline keratopathy. CONCLUSION: Different pneumococcal serotypes vary in their ability to produce infectious crystalline keratopathy. Because serotype differences reflect differences in the antigenic polysaccharides of the bacterial capsule, this study suggests that properties of the pneumococcal capsule may influence the initial development of infectious crystalline keratopathy.

The silent sinus syndrome. A cause of spontaneous enophthalmos.

PURPOSE: Spontaneous enophthalmos and hypoglobus, in the absence of other symptoms and unrelated to trauma or surgery, may be alarming to both physician and patient. The authors describe the clinicopathologic features of a benign syndrome ("silent sinus syndrome") with this constellation of features and discuss the possible pathophysiology. METHODS: A multicenter retrospective search for similar clinical cases was performed. All clinical records, computed tomographs, and pathology reports for each case were reviewed at one center. A literature search for similar cases also was conducted. RESULTS: Nineteen cases of a new syndrome are presented. This syndrome affects individuals at approximately the fourth decade of life (average age, 36 years; range, 29-46 years); is characterized by bone resorption and remodeling of the orbital floor due to otherwise asymptomatic maxillary sinus disease; is associated with ipsilateral maxillary sinus hypoplasia; and is not fully explained by any previously described, classic cystic lesion of the maxillary antrum. CONCLUSION: Enophthalmos and hypoglobus unassociated with prior trauma, surgery, or other symptoms may represent "silent sinus syndrome," which is ipsilateral maxillary sinus hypoplasia and orbital floor resorption.

Combination drug testing of Mycobacterium chelonae.

PURPOSE: Medical therapy of Mycobacterium chelonae keratitis is difficult because there are so few effective antimicrobial agents and single agent therapy frequently fails clinically. To identify more effective medical treatment regimens, the in vitro antimicrobial efficacy of amikacin, the most frequently used single agent, was investigated in combination with four antibiotics previously reported to have activity against M. chelonae: erythromycin, imipenem, ciprofloxacin, and vancomycin. METHODS: The drug combinations were tested by the checkerboard method against seven corneal isolates of M. chelonae. RESULTS: The combination of amikacin with erythromycin or vancomycin consistently led to synergistic or additive effect, however the minimum inhibitory concentrations for vancomycin were very high. The combination of amikacin with imipenem or ciprofloxacin led to results ranging from antagonism to additive effects. CONCLUSIONS: Of the antibiotics tested, erythromycin showed the most activity against M. chelonae in combination with amikacin. In vitro combination drug testing of M. chelonae by the checkerboard method should be further evaluated for clinical relevance in microbial keratitis.

Infectious crystalline keratopathy. The role of bacterial exopolysaccharide.

OBJECTIVES: Infectious crystalline keratopathy is a unique pauci-inflammatory infection of the cornea most commonly due to viridans type streptococci. We investigated the hypothesis that production of exopolysaccharide by streptococci, a property that can be induced by growth conditions, may contribute to the pathogenesis of infectious crystalline keratopathy by suppressing the ocular immune response. METHODS: Streptococcus sanguis type II was grown under two conditions, conventionally in brain-heart infusion broth and in 5% sucrose-supplemented brain-heart infusion broth, to promote exopolysaccharide formation. Rabbit corneas were inoculated by passage of 9-0 silk sutures soaked in bacterial suspensions. RESULTS: Arborizing, sharply demarcated pauci-inflammatory lesions were noted in 71% of rabbit corneas inoculated with S sanguis type II grown in sucrose-supplemented media and in 25% of control corneas (P = .05). Suppurative lesions developed in the remaining corneas. Histologic evaluation of infectious crystalline keratopathy lesions revealed characteristic features. CONCLUSION: Increased exopolysaccharide formation by S sanguis type II is associated with production of infectious corneal lesions that resemble those of infectious crystalline keratopathy.

The solubility of antibiotic and corticosteroid combinations.

The use of collagen shields soaked in various combinations of medications has been advocated to enhance drug delivery to the cornea. Recently, severe corneal toxicity associated with aggregate formation in mixtures of gentamicin and methylprednisolone prompted our study of the effect of drug concentration, pH, and temperature on the solubility of several antibiotic and corticosteroid formulations commonly used to treat ocular disease. Selected combinations of cefazolin, vancomycin, tobramycin, gentamicin, methylprednisolone, and dexamethasone were evaluated. Mixtures of tobramycin and vancomycin produced no precipitates, but many spheroid aggregates were seen when methylprednisolone and gentamicin were combined. Although the effects of precipitate formation on drug bioavailability and toxicity have not been fully determined, until such information is available, the use of combinations of drugs that remain in solution during administration is recommended.

Visual results and complications of phacoemulsification with intraocular lens implantation performed by ophthalmology residents.

The authors retrospectively analyzed the visual outcome and incidence of complications of 181 consecutive cases of phacoemulsification performed by eight third-year ophthalmology residents. A posterior chamber intraocular lens was implanted in 179 of these eyes. Vitreous loss occurred in 5.5% of cases. The overall incidence of posterior capsule rupture was 9.9%; of the 18 posterior capsular ruptures, 72% were detected during cortical removal and 22% during nuclear emulsification. Postoperative follow-up for 177 eyes was at least 2 months, and 70% were followed for 6 months or longer. Final best corrected visual acuity of 20/40 or better was obtained in 92.6% of eyes. These results are comparable with previous reports of residents' experience in performing planned extracapsular cataract extraction. With appropriate training and faculty supervision, residents learning to perform phacoemulsification and intraocular lens implantation can achieve acceptable results.

The effects of fibronectin on the adherence of bacteria to corneal epithelium.

The aim of our experiments was to determine whether treatment with topical fibronectin led to increased adherence of Staphylococcus aureus or Pseudomonas aeruginosa to rabbit corneas with epithelial defects. No significant effect of fibronectin was demonstrated. For all strains of S. aureus tested, the number of recoverable organisms was decreased at 24 h compared to 1 h. None of the rabbits developed infectious keratitis.

Ocular disease associated with Epstein-Barr virus infection.

Epstein-Barr virus (EBV) is a ubiquitous DNA virus of the herpesvirus genus with a high prevalence rate for antibody (about 90%) in the adult population. It is the most common causative agent of infectious mononucleosis syndrome. During recent years an increasing number of ocular disease entities have been reported to be linked to EBV infection. These entities include oculoglandular syndrome, conjunctivitis, dry eye, keratitis, uveitis, choroiditis, retinitis, papillitis and ophthalmoplegia. While EBV-specific serologic tests can now document recent and past primary infection with EBV and also identify patients manifesting atypical immunologic reactions to EBV, the lack of an animal model, the absence of clear-cut response to therapy and the paucity of documentation by culture render the pathogenesis uncertain or the association questionable in many of these cases.

Bacterial keratitis after radial keratotomy.

The authors identified nine patients with culture-proven keratitis after radial keratotomy (RK). Three patients became infected in the immediate post-operative period, and six patients had delayed-onset keratitis. Gram-negative rods were the predominant pathogens in late-onset keratitis (4 of 6 infections). Gram-positive cocci were implicated in all three postoperative infections. The inferior corneal quadrants were involved in seven of nine patients. Two episodes of late-onset keratitis were associated with contact lens wear. Five of these patients had transverse incisions or greater than eight radial cuts. All six patients regained visual acuity of 20/40 or better. Two additional cases of early-onset and six cases of late-onset keratitis with many features similar to these cases have been reported previously.

The use of fluorescein-conjugated lectins for visualizing atypical mycobacteria.

We investigated the feasibility of using fluorescein-conjugated lectins for visualizing and differentiating two species of atypical mycobacteria. Pure cultures of Mycobacterium fortuitum and Mycobacterium chelonei were established, as was an experimental model of infectious keratitis involving these two organisms. Samples from the pure cultures and corneal scrapings were placed on glass slides, fixed, and incubated with one of a panel of 22 fluorescein-conjugated lectins. The slides were examined using an epifluorescence microscope. Fluorescein-conjugated concanavalin A brightly stained both species of atypical mycobacteria, in both the pure culture and experimental keratitis samples. Several additional fluorescein-conjugated lectins (wheat germ agglutinin, succinylated wheat germ agglutinin, Phaseolus vulgaris erythroagglutinin, and Psophocarpus tetragonolobus agglutinin) brightly stained M chelonei, but only moderately stained M fortuitum. These staining patterns are consistent with the known carbohydrate compositions of the cell walls of atypical mycobacteria and suggest that fluorescein-conjugated lectins may be useful for the visualization of these organisms in corneal infections.

Polychromatic corneal and conjunctival crystals secondary to clofazimine therapy in a leper.

A 67-year-old man had a diagnosis of dapsone-resistant lepromatous leprosy. He received clofazimine (Lamprene) at a dosage of 100 mg twice daily. After 3 years of therapy, results of slit-lamp examination disclosed myriad polychromatic crystals diffusely involving the cornea and perilimbal conjunctiva of both eyes. Thick sections (1 micron) from a conjunctival biopsy showed numerous rectangular-to-rhomboidal crystals within stromal fibroblasts and macrophages. By electron microscopy, these cells contained elongated, membrane-bound, cleft-like spaces that corresponded to the sites where crystals had been present previously. Additionally, complex lipid inclusions were observed in mesenchymal cells as well as in endothelial cells and pericytes of blood vessels. The ocular side effects of clofazimine therapy are reviewed. Clofazimine-induced keratopathy should be included in the differential diagnosis of patients with polychromatic crystalline deposits in the corneas. To the best of the authors' knowledge, this complication of clofazimine therapy has not been described previously.

The effects of freezing and antibiotics on the viability of Acanthamoeba cysts.

The effects of cryotherapy and antibiotics (paromomycin, neomycin, or propamidine isethionate) on the viability of Acanthamoeba polyphaga and Acanthamoeba castellani cysts were studied in vitro. Either cryotherapy or exposure to antibiotic led to a decrease in the number of viable A castellani detected; A polyphaga showed variable response to the antibiotics tested. The combination of cryotherapy and antibiotic therapy was more cysticidal than either modality alone and eliminated detectable viable organisms in five of six experiments. Of the antibiotic solutions tested, paromomycin (15 mg/mL) was the most effective.

[Epidural hematoma without intradural lesions--clinical study of 120 cases].

One hundred twenty cases of epidural hematoma without intradural lesion treated between 1975 and 1983 to which CT scan was available, were presented. Mortality rate was 1.7% and morbidity rate was 6.7%. In all cases of epidural hematoma including with and without intradural lesion in the same term (164 cases), mortality and morbidity rate were 7.3 and 10.4% respectively. Ninety five cases were male and 25 cases were female. Causes of injury were falls from a height (42 cases, 35%), traffic accident (41 cases, 34%), and falls at ground level (24 cases, 20%). The site of hematoma in 120 cases of 128 hematoma were frontal (17 cases, 13%), temporoparietal (74 cases, 58%), parasagittal (4 cases, 3%), middle fossa (8 cases, 6%), occipital (16 cases, 13%) and posterior fossa (9 cases, 7%). Fractures were shown in 109 cases out of 120 cases on skull X-P and depressed fractures were 12 cases. Those which showed no fracture on skull X-P, revealed slow growth of hematoma and delayed onset of clinical signs and were mainly children. Seven operated cases of epidural hematoma does not show fractures on skull X-P nor initial unconscious. Clinical course of disturbance of consciousness were divided in five types as follows; I. no unconsciousness (22 cases, 18%), II. latent interval (17 cases, 14%), III. primary but no secondary unconsciousness (29 cases, 24%), IV. lucid interval (22 cases, 18%), V. unconscious throughout (16 cases, 14%) and unknown (14 cases, 12%). Cases which Glasgow Coma Scale Score were over 7 showed good recovery in Glasgow Outcome Scale.(ABSTRACT TRUNCATED AT 250 WORDS)

Corneal subepithelial infiltrates associated with systemic Epstein-Barr viral infection.

Corneal subepithelial infiltration resembling adenoviral keratitis developed in two patients with serologic evidence of systemic Epstein-Barr viral (EBV) infection and nondetectable antibody levels for adenovirus (ADV). These cases suggest that EBV is capable of mimicking more commonly recognized corneal infectious disease entities.