Optico-chiasmatic distortions in pituitary adenomas: correlation between postoperative morphological reversal and follow-up functional visual recovery.

PURPOSE: To objectively correlate distortions of optic apparatus morphology in patients with visual outcomes in patients with pituitary adenomas (PAs) undergoing trans-sphenoidal surgery. METHODS: In this retrospective analysis, visual acuity (VA), visual field (VF) and total visual (TV) function was objectively scored in patients selected from an institutional cohort of PAs The following imaging measures of optico-chiasmatic morphology were recorded preoperatively, and at 3 months after surgery: chiasm area (CA), mid-chiasm height (CH), optic nerve-canal bending angle (BA), and optic nerve kinking angle (ONKA). Receiver operator characteristic (ROC) analysis was performed to establish optimal thresholds for achieving a 'normal' TV score of 200 at 1-year follow-up. RESULTS: Seventy-one eyes were individually studied. VA, VF and TV scores significantly improved after surgery, both at the 3-month and at the 1-year follow-up visits (p<0.001). Cranio-caudal tumor dimension was significantly associated with VA, VF and TV scores, both pre- and post-operatively. There were significant changes in CH (p<0.001), BA (p<0.001) and ONKA (p<0.001) at 3 months after surgery, but not in CA (p=0.061). Baseline VA, VF and TVS scores were positively correlated with preoperative CH and ONKA, while VF and TV scores also demonstrated significant negative correlations with preoperative BA. VF scores at 1-year follow-up were significantly correlated with preoperative and change in BA values, as well as with preoperative and change in ONKA values. ROC analysis revealed that only the preoperative ONKA was found to have acceptable discrimination (AUC>0.7) for predicting 'normal' TV score. Chiasm sag was noted in 45.8% of patients at one year follow-up, but was not associated with delayed visual deterioration in any case. CONCLUSIONS: Anatomic realignment of the optic apparatus 3 months following trans-sphenoidal surgery predicts VF scores, but not VA or TV scores at 1 year follow-up. Patients with preoperative ONKA values of more than 139.3° have a 76% chance of achieving normal TV scores one year after surgery. Postoperative chiasm sag appears to be clinically irrelevant at short term follow-up.

Primary intracranial pediatric ganglioneuroblastoma-report of two cases and review of an unusual masquerader.

Ganglioneuroblastoma (GNB) is a rare neurogenic tumor with a predilection for extracranial sites. Its primary intracranial occurrence is anecdotal, with less than ten cases reported in pediatric literature. We report two cases of this unusual entity. The first was a 1-year-old boy who presented with a progressive scalp swelling that radiologically appeared to be a mesenchymal tumor. The tumor was resected completely, and its histopathological examination was suggestive of a pigmented GNB. The clinico-radiological presentation and melanin pigmentation of the tumor were unique features of the case. The second case was a 7-year-old girl who presented with left hemiparesis and raised intracranial pressure due to a large right parietal intraparenchymal tumor that histologically proved to be a GNB. The child succumbed to disease progression a month after undergoing near-total resection of the tumor. Analysis of our cases in the light of a literature review reveals that pediatric intracranial GNBs have diverse clinico-radiological features and can easily be confused for commoner pathologies in both intra- and extra-axial locations.

Moving Beyond Morphometrics and Alignment: Prospective Longitudinal Study on Cognition, Quality of Life, and Diffusion Metrics in Congenital Craniovertebral Junction Anomalies.

BACKGROUND AND OBJECTIVES: Chiari type I malformation (CMI) has been implicated to cause cognitive impairment. Unusual for a craniovertebral junction (CVJ) anomaly, this phenomenon is postulated to be related to microstructural changes across the brain. However, this has not been confirmed in a longitudinal study, in bony CVJ anomalies, or in the context of quality of life (QOL). This study aimed to analyze cognition, QOL, and diffusion tensor imaging (DTI) metrics before and after surgery for congenital CVJ anomalies and evaluate their correlations with conventional clinico-radiological factors. METHODS: Fifty-seven patients with congenital CVJ anomalies-CMI, atlantoaxial dislocation (AAD), and basilar invagination (BI)-were evaluated in this prospective longitudinal study over 3 years with a neuropsychological battery, QOL and functional scores, and DTI metrics. A total of 43 patients (23 with CMI; 20 with AAD or BI) with 1-year postsurgical data were included in the final analysis. RESULTS: Cognitive dysfunction in one or more domains was noted in 15 (65%) of the patients with CMI and 16 (80%) of the patients with AAD/BI. Memory and visual motor perception were the most-affected domains in both the cohorts. At the 1-year follow-up, the proportion of patients demonstrating an overall cognitive dysfunction did not change significantly (P > .05). All patients demonstrated significant postoperative improvement in functional and QOL scores at follow-up. DTI metrics improved in many regions postoperatively, but did not correlate with functional or cognitive changes (P > .05). CONCLUSION: Cognition is affected in a majority of patients with CMI, AAD, or BI. These patients experience good functional and QOL outcomes after surgery but most of them continue to exhibit cognitive impairment. DTI metrics improve after surgery, but do not correlate with cognitive changes. These findings underline the multidimensional nature of the pathology of these anomalies that complicate achieving the concept of a "successful" surgery.

Cervical Flexor-Extensor Muscle Disparity in Monomelic Amyotrophy (Hirayama Disease): Evidence from a Comprehensive Morphometric Evaluation of Subaxial Paraspinal Musculature.

Background Monomelic amyotrophy (Hirayama disease) has been established to have accompanied biomechanical abnormalities such as flexion hypermobility and sagittal imbalance. Paraspinal muscles, the major contributor to cervical biomechanics, have, however, not been comprehensively evaluated in the disease. The objective of this study was to compare the morphology of the subaxial cervical paraspinal musculature in patients with and without Hirayama disease. Materials and Methods A retrospective case-control study of 64 patients with Hirayama disease and 64 age- and sex-matched controls was performed . Cross-sectional areas (CSAs) of the superficial and deep flexors and extensors from C3 to C7 were measured on T2-weighted magnetic resonance imaging sequences. Student's t -test was used to compare differences between the paraspinal muscle CSAs in the study and control groups. Results Compared with controls, patients with Hirayama disease were found to have larger flexors and smaller extensors at all levels. The overall subaxial muscle area values for superficial flexors and deep flexors were significantly larger ( p < 0.0001) in patients, while the corresponding superficial extensor and deep extensor area values were significantly smaller than in controls ( p = 0.01 and < 0.0001, respectively). The patient group demonstrated stronger subaxial deep flexor-deep extensor, superficial flexor-superficial extensor, and total flexor-total extensor ratios ( p < 0.0001). Conclusion Patients with Hirayama disease have morphometric alterations at all levels of their subaxial cervical paraspinal musculature. These patients have abnormally large flexors and small extensors compared with controls. This flexor-extensor muscle disparity could be utilized as a potentially modifiable factor in the management of the disease.

Knowledge, attitudes, and poultry-handling practices of poultry workers in relation to avian influenza in India.

UNLABELLED: Avian influenza (AI) is a viral disease of domestic and wild birds. The recent pandemics caused by highly pathogenic AIA (H5N1) in domestic poultry is currently rated phase 3 by the World Health Organization on the pandemicalert scale. MATERIALS AND METHODS: A pretested and semistructured survey instrument was administered to both live bird market and poultry farm workers in two most populous cities in Karnataka in South India to collect data on demographics, knowledge, attitude, and practices among them. RESULTS: The mean age was similar among both population groups (31.5 years). There was a higher level of biosecurity practices adopted in poultry farms compared with those adopted in live bird market. Knowledge regarding AI was acceptable but poorly correlated with actual biosecurity practices. DISCUSSION: Live bird market and poultry farm workers have been identified as the weakest link in the prevention and control of the spread of AI in the two most populous cities studied in Karnataka. CONCLUSION: Risk reduction models of behavior change targeting these groups are important toward the control and prevention of AI spread.