Tolosa Hunt syndrome: a rare syndrome

Tolosa Hunt Syndrome (THS) is rare syndrome with an estimated annual incidence of 1 case per million per year. THS is painful ophthalmoplegia caused by nonspecific inflammation of cavernous sinus or superior orbital fissure. It is often unilateral with severe headache and ophthalmoplegia involving third, fourth, fifth and sixth cranial nerves. We present a case admitted in our hospital that came with complaint of severe unilateral headache with loss of vision of left eye. He did not have any other significant history except that he was complaining of partial sensory loss over left upper part of face. Ophthalmologist reference was taken to rule out any other cause involving optic disc and funduscopy which was normal. Further investigations were done which ruled out all possible causes. Patient was started on steroids and on MRI scan and clinical presentation, patient was diagnosed as Tolosa- Hunt Syndrome. Thus we report a rare case of THS which showed gradual recovery with corticosteroids

Scleroderma: a case report

Scleroderma is systemic multi organ autoimmune disorder characterized by hardening of skin. Also known as systemic sclerosis. Estimated annual incidences of approximately 19 cases per million persons. The limited skin disease has a 10-year survival rate of 71%, whereas those with diffuse skin disease have a 10-year survival rate of just 21%. Risk is higher in women than men and peak in individuals aged 30-50 years. It has no definitive treatment. It may be limited or diffuse depending upon manifestations of symptoms or signs affecting internal organs especially lungs, heart, or kidney. We report a case of scleroderma with pulmonary hypertension and interstitial lung disease in our hospital who presented with tightening of skin, joint pain, dysphagia, and breathlessness. On examination skin appeared dark, shiny, and tight, with loss of hair, paraesthesia and digital ulceration. Patient also has history of Raynaud's phenomenon. On investigation, Scl-70 and ANA (antinuclear antibodies) by enzyme immunoassay came positive. HRCT thorax was suggestive of interstitial fibrosis and PFT revealed moderate restriction. On 2D echocardiography, mild pulmonary hypertension was present while barium swallow showed motility disorder involving oesophagus. On view of extensive systemic involvement like skin, respiratory system, gastrointestinal system and heart, we would like to present this rare disorder

Diagnostic Performance of the PalmScan VF2000 Virtual Reality Visual Field Analyzer for Identification and Classification of Glaucoma

Purpose: To evaluate the diagnostic test properties of the Palm Scan VF2000® Virtual Reality Visual Field Analyzer for diagnosis and classification of the severity of glaucoma. Methods: This study was a prospective cross-sectional analysis of 166 eyes from 97 participants. All of them were examined by the Humphrey® Field Analyzer (used as the gold standard) and the Palm Scan VF 2000® Virtual Reality Visual Field Analyzer on the same day by the same examiner. We estimated the kappa statistic (including 95% confidence interval [CI]) as a measure of agreement between these two methods. The diagnostic test properties were assessed using sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). Results: The sensitivity, specificity, PPV, and NPV for the Virtual Reality Visual Field Analyzer for the classification of individuals as glaucoma/non-glaucoma was 100%. The general agreement for the classification of glaucoma between these two instruments was 0.63 (95% CI: 0.56–0.78). The agreement for mild glaucoma was 0.76 (95% CI: 0.61–0.92), for moderate glaucoma was 0.37 (0.14–0.60), and for severe glaucoma was 0.70 (95% CI: 0.55–0.85). About 28% of moderate glaucoma cases were misclassified as mild and 17% were misclassified as severe by the virtual reality visual field analyzer. Furthermore, 20% of severe cases were misclassified as moderate by this instrument. Conclusion: The instrument is 100% sensitive and specific in detection of glaucoma. However, among patients with glaucoma, there is a relatively high proportion of misclassification of severity of glaucoma. Thus, although useful for screening of glaucoma, it cannot replace the Humphrey® Field Analyzer for the clinical management in its current form

Individual and Combined Effects of Diabetes and Glaucoma on Total Macular Thickness and Ganglion Cell Complex Thickness: A Cross-sectional Analysis

Purpose: Presence of diabetes in glaucoma patients may influence findings while documenting the progression of glaucoma. We conducted the study to compare individual and combined effects of diabetes and glaucoma on macular thickness and ganglion cell complex thickness. Methods: The present study is a cross-sectional analysis of 172 eyes of 114 individuals. The groups were categorized according to the following conditions: glaucoma, diabetes mellitus, both glaucoma and diabetes (‘both’ group), and none of these conditions (‘none’ group). Patients with diabetes did not have diabetic retinopathy (DR). We compared retinal nerve fiber layer (RNFL) thickness, ganglion cell complex (GCC) thickness, foveal loss of volume (FLV), and global loss of volume (GLV) among the groups. We used random effects multivariate analysis to adjust for potential confounders. Results: The mean (SD) age of these individuals was 60.7 (10.1) years. The total average RNFL and GCC were significantly lower in the glaucoma group (RNFL: –36.27, 95% confidence intervals [CI]: –42.79 to –29.74; P < 0.05, and GCC: –26.24, 95% CI: –31.49 to –20.98; P < 0.05) and the ‘both’ group (RNFL: –24.74, 95% CI: –32.84 to –16.63; P < 0.05, and GCC: –17.92, 95% CI: –24.58 to –11.26; P < 0.05) as compared with the ‘none’ group. There were no significant differences in the average RNFL values and total average GCC between the diabetes group and the ‘none’ group. The values of FLV and GLV were significantly higher in the ‘glaucoma’ group and the ‘both’ group as compared with the ‘none’ group. The foveal values were not significantly different across these four groups. Among the glaucoma cases, 25% were mild, 30% were moderate, and 45% were severe; there was no significant difference in the proportion of severity of glaucoma between the ‘glaucoma only’ and ‘both’ groups (P = 0.32). After adjusting for severity and type of glaucoma, there were no statistically significant differences in the values of average RNFL (6.6, 95% CI: –1.9 to 15.2; P = 0.13), total average GCC (3.6, -95% CI: –2.4 to 9.6; P = 0.24), and GLV (–3.9, 95% CI: –9.5 to 1.6; P = 0.16) in the ‘both group’ as compared with the glaucoma only group. Conclusion: We found that diabetes with no DR did not significantly affect the retinal parameters in patients with glaucoma. Thus, it is less likely that thickness of these parameters will be overestimated in patients with glaucoma who have concurrent diabetes without retinopathy

Duration of post-operative hypocortisolism predicts sustained remission after pituitary surgery for Cushing’s disease

Purpose: Transsphenoidal surgery (TSS) is the primary treatment modality for Cushing’s disease (CD). However, the predictors of post-operative remission and recurrence remain debatable. Thus, we studied the post-operative remission and long-term recurrence rates, as well as their respective predictive factors. Methods: A retrospective analysis of case records of 230 CD patients who underwent primary microscopic TSS at our tertiary care referral centre between 1987 and 2015 was undertaken. Demographic features, pre- and post-operative hormonal values, MRI findings, histopathological features and follow-up data were recorded. Remission and recurrence rates as well as their respective predictive factors were studied. Results: Overall, the post-operative remission rate was 65.6% (early remission 46%; delayed remission 19.6%), while the recurrence rate was 41% at mean follow-up of 74 ± 61.1 months (12–270 months). Significantly higher early remission rates were observed in patients with microadenoma vs macroadenoma (51.7% vs 30.6%, P = 0.005) and those with unequivocal vs equivocal MRI for microadenoma (55.8% vs 38.5%, P = 0.007). Patients with invasive macroadenoma had poorer (4.5% vs 45%, P = 0.001) remission rates. Recurrence rates were higher in patients with delayed remission than those with early remission (61.5% vs 30.8%, P = 0.001). Duration of post-operative hypocortisolemia ≥13 months predicted sustained remission with 100% specificity and 46.4% sensitivity. Recurrence could be detected significantly earlier (27.7 vs 69.2 months, P < 0.001) in patients with available serial follow-up biochemistry as compared to those with infrequent follow-up after remission. Conclusion: In our study, remission and recurrence rates were similar to that of reported literature, but proportion of delayed remission was relatively higher. Negative/equivocal MRI findings and presence of macroadenoma, especially those with cavernous sinus invasion were predictors of poor remission rates. In addition to early remission, longer duration of post-operative hypocortisolism is an important predictor of sustained remission. Regular biochemical surveillance may help in identifying recurrence early