Fractionated stereotactic radiotherapy in patients with optic nerve sheath meningioma.

PURPOSE: To evaluate the effectiveness of fractionated stereotactic radiotherapy (SFRT) in the treatment of optic nerve sheath meningioma (ONSM). METHODS AND MATERIALS: Between 1993 and 2005, 109 patients (113 eyes) with primary (n = 37) or secondary (n = 76) ONSM were treated according to a prospective protocol with SFRT to a median dose of 54 Gy. All patients underwent radiographic, ophthalmologic, and endocrine analysis before and after SFRT. Radiographic response, visual control, and late side effects were endpoints of the analysis. RESULTS: Median time to last clinical, radiographic, and ophthalmologic follow up was 30.2 months (n = 113), 42.7 months (n = 108), and 53.7 months (n = 91), respectively. Regression of the tumor was observed in 5 eyes and progression in 4 eyes, whereas 104 remained stable. Visual acuity improved in 12, deteriorated in 11, and remained stable in 68 eyes. Mean visual field defects reduced from 33.6% (n = 90) to 17.8% (n = 56) in ipsilateral and from 10% (n = 94) to 6.7% (n = 62) in contralateral eyes. Ocular motility improved in 23, remained stable in 65, and deteriorated in 3 eyes. Radiographic tumor control was 100% at 3 years and 98% at 5 years. Visual acuity was preserved in 94.8% after 3 years and in 90.9% after 5 years. Endocrine function was normal in 90.8% after 3 years and in 81.3% after 5 years. CONCLUSIONS: SFRT represents a highly effective treatment for ONSM. Interdisciplinary counseling of the patients is recommended. Because of the high rate of preservation of visual acuity we consider SFRT the standard approach for the treatment of ONSM. Prolonged observation is warranted to more accurately assess late visual impairment. Moderate de-escalation of the radiation dose might improve the preservation of visual acuity and pituitary gland function.

Protracted hypofractionated radiotherapy for Graves' ophthalmopathy: a pilot study of clinical and radiologic response.

PURPOSE: To evaluate the clinical and radiologic response of patients with Graves' ophthalmopathy given low-dose orbital radiotherapy (RT) with a protracted fractionation. METHODS AND MATERIALS: Eighteen patients (36 orbits) received orbital RT with a total dose of 10 Gy, fractionated in 1 Gy once a week over 10 weeks. Of these, 9 patients received steroid therapy as well. Patients were evaluated clinically and radiologically at 6 months after treatment. Clinical response assessment was carried out using three criteria: by physical examination, by a modified clinical activity score, and by a verbal questionnaire considering the 10 most common signs and symptoms of the disease. Radiologic response was assessed by magnetic resonance imaging. RESULTS: Improvement in ocular pain, palpebral edema, visual acuity, and ocular motility was observed in all patients. Significant decrease in symptoms such as tearing (p < 0.001) diplopia (p = 0.008), conjunctival hyperemia (p = 0.002), and ocular grittiness (p = 0.031) also occurred. Magnetic resonance imaging showed decrease in ocular muscle thickness and in the intensity of the T2 sequence signal in the majority of patients. Treatments were well tolerated, and to date no complications from treatment have been observed. There was no statistical difference in clinical and radiologic response between patients receiving RT alone and those receiving RT plus steroid therapy. CONCLUSION: RT delivered in at a low dose and in a protracted scheme should be considered as a useful therapeutic option for patients with Graves' ophthalmopathy.

Eye position information on CT increases the identification of acute ischemic hypoattenuation.

BACKGROUND AND PURPOSE: It is possible that identification of eye deviation may sensitize a scan reader to early brain hypodensity associated with an arterial occlusive process. Our aim was to investigate the value of observing eye deviation on blinded CT identification of early hypoattenuation following ischemic infarct. MATERIALS AND METHODS: Two staff and 2 fellow neuroradiologists reviewed 75 brain CT scans obtained within 3 hours of acute ischemia from subjects in the Interventional Management of Stroke Study. Films were reviewed 3 months apart, the first time with tape over the eyes on the images, the second with the eyes visible. Readers were asked if early hypoattenuation in the middle cerebral artery (MCA) distribution or if a hyperattenuated MCA was present. kappa statistics were calculated to determine agreement among the 4 readers and between each of the 2 readings by the same reader, not only for the original interpretation of the blinded study neuroradiologist but also for the Alberta Stroke Program Early CT Score (ASPECTS) for each subject assigned by an unblinded expert panel. A generalized estimating equations modeling approach was used to look at the overall effect of including eye information for agreement between interpretations. RESULTS: Eye information availability was associated with improved agreement for detection of early ischemic hypoattenuation not only among the 4 readers but also between the 4 readers and both the blinded study neuroradiologist (P = .02) and the unblinded expert ASPECTS panel. When comparing first and second readings for hypoattenuation, we also noted increased mean values for sensitivity (46.8% first, 56.5% second), specificity (78.2%, 80.2%), positive predictive value (72.0%, 80.7%), negative predictive value (55.5%, 61.0%), and percentage agreement (61.0%, 67.5%). CONCLUSION: Observation of CT eye deviation significantly improves reader identification of acute ischemic hypoattenuation.

Glucose utilization in the inferior cerebellar vermis and ocular myoclonus.

In a patient with symptomatic ocular myoclonus, the authors observed the regional cerebral metabolic rate of glucose use (rCMRGlu) before and after successful treatment with clonazepam. Even after the symptoms resolved, the rCMRGlu in the hypertrophic olive increased persistently, whereas that in the inferior cerebellar vermis contralateral to the hypertrophic olive decreased. The inferior cerebellar vermis, belonging to the vestibulocerebellar system, may be associated with the generation of symptomatic ocular myoclonus.

Impact of smoking on the response to treatment of thyroid associated ophthalmopathy.

BACKGROUND: In patients with Graves' disease, smoking considerably increases the incidence and severity of thyroid associated ophthalmopathy (TAO). The authors sought to determine if smoking also influences the course of TAO during treatment, and the efficacy of therapy. METHODS: 41 smokers and 19 non-smokers with moderate untreated TAO were included in this prospective study. All patients were treated with steroids and, 6 weeks after the beginning of drug therapy, with orbital irradiation. Follow up was performed 1.5, 4.5, 7.5, and 12 months after the beginning of the study. Proptosis, clinical activity score (CAS), and motility were evaluated. The extent of smoking was derived from the concentration of the haemoglobin adduct N-2-hydroxyethylvaline (HEV), a parameter of long term smoking. RESULTS: There was no difference in the clinical manifestations of TAO between smokers and non-smokers at the beginning of treatment. However, CAS decreased (p<0.05) and motility improved (p<0.02) significantly faster and to a greater extent in non-smokers than smokers. Inverse correlations between the CAS decrease and the HEV levels observed 4.5 and 7.5 months after the beginning of treatment and between the improvement of motility and the HEV levels after 1.5, 4.5, and 7.5 months indicated a dose dependence. Mean HEV levels did not vary much during the follow up period and were significantly different in smokers (mean 5.4 (SD 2.7) micro g/l) and non-smokers (mean 1.8 (1.3) micro g/l; p<0.01). CONCLUSION: Smoking influences the course of TAO during treatment in a dose dependent manner. The response to treatment is delayed and considerably poorer in smokers.

[Retrobulbar irradiation for Graves' ophthalmopathy -- long-term results]. / Retrobulbäre Bestrahlung bei endokriner Orbitopathie -- Erfahrungen im Langzeitverlauf.

BACKGROUND: Significance of retrobulbar irradiation in patients suffering form Graves' ophthalmopathy, though established since almost one century, is subject of scientific debate. The present study investigated the effect of retrobulbar irradiation using a standardized protocol focussing on long term results. PATIENTS AND METHODS: Between 1981 and 1997, 104 patients treated by retrobulbar irradiation (10 to 20 Gray) due to Graves' disease. Twenty-nine of these underwent irradiation as sole treatment (mean follow-up 57 months), while in the remaining 75, it was combined with a systemic steroid treatment (mean follow-up 40 months). Patients were evaluated regarding proptosis, intraocular pressure, lid signs, motility as well as subjective assessment of double vision and retrobulbar pain. RESULTS: While proptosis remained unchanged, lid signs, chemosis and intraocular pressure showed slight and statistically significant improvement. However, these findings were considered to be clinically insignificant. Retrobulbar pain was improved in 75 % of patients. 25 % of patients showed improved motility, 75 % remained stable, and in none of them was there a deterioration of ductions. Results proved stable even in long-term follow-up. 25 % per cent of patients underwent a surgical procedure one year after radiotherapy. CONCLUSIONS: In our series, we could not demonstrate an additional benefit of systemic steroids when combined with retrobulbar irradiation. Up to sixteen years after treatment, no treatment-related adverse reaction was seen. We found a remarkable improvement in ocular motility. This holds even more true in comparison to the natural course of the condition. Retrobulbar irradiation seems to shorten the duration of the disease, thus allowing earlier performance of eventual rehabilitative surgery.

The effect of retrobulbar irradiation on exophthalmos, ductions and soft tissue signs in Graves' ophthalmopathy: a retrospective analysis of 90 cases.

PURPOSE: Retrospective analysis of the effect of retrobulbar irradiation on exophthalmos, ductions and soft tissue signs in patients with Graves' ophthalmopathy. METHODS: We analysed the charts of 111 consecutive patients who were treated with retrobulbar irradiation according to standardised intake criteria between 1992 and 1997. After exclusion of patients who underwent other treatment (with steroids or orbital decompression) shortly before or within 6 months after irradiation, and on whom insufficient data were available, 90 patients were included. For these 90 patients, we analysed the exophthalmometry, ductions, soft tissue signs and visual acuity shortly before irradiation and after 3 and 6 months, respectively. RESULTS: In the whole group, the Hertel value was on average 22 mm (SD 2.9) both before irradiation and after 3 and 6 months of follow-up. Separate analysis of data on 25 patients with bilateral exophthalmos of more than 24 mm also revealed no change in exophthalmos at follow-up. In the whole group, both abduction and elevation had improved by about 1 degree (SD 6.6 degrees; p = 0.05) after 3 months. This improvement has little clinical significance. In a subgroup of 14 patients who showed more than 10 degrees of restricted eye motility in one or more directions, both abduction and elevation had increased by about 4 degrees (SD 10 degrees; p = 0.02) at 3 and 6 months follow-up. Soft tissue signs had improved at 6 months after irradiation. We found no change in visual acuity after irradiation. CONCLUSION: Retrobulbar irradiation in Graves' ophthalmopathy does not seem to reduce exophthalmos. It probably improves eye motility in patients with severe restrictions. The late improvement in soft tissue signs may either be a late effect of irradiation or be related to the natural history of the disease.

Radiotherapy for thyroid orbitopathy. Effects on extraocular muscle balance.

OBJECTIVE: To investigate the effects of radiotherapy on the restrictive ophthalmopathy and strabismus that occur with Graves' orbitopathy. DESIGN: A prospective study of patients with motility disturbances due to thyroid orbitopathy, followed by serial quantitative measurements of ocular restrictions and deviations, both before and at 1 and 6 months after treatment with high-energy radiotherapy. SETTING: Urban subspecialty private practice. PATIENTS: Thirty-three consecutive patients with restricted extraocular movements and diplopia secondary to thyroid orbitopathy who were treated with radiotherapy were seen during an 8-year period. MEASURES: Maximal horizontal and vertical ductions, plus ocular deviations in primary and reading positions of gaze, were quantitatively evaluated by one clinical investigator to ensure consistency. RESULTS: Statistically significant improvement in gaze restriction on supraduction and improvement of vertical and horizontal strabismus were demonstrated at follow-up visits after treatment. CONCLUSIONS: Although statistically significant improvement of motility disturbances were demonstrated after radiotherapy, there was less than desirable functional improvement of restrictive ophthalmopathy and strabismus; thus the usefulness of radiotherapy was limited when it was used alone to treat diplopia.