Chiasmal Disorders Research Articles

Cognitive Visual Disturbances in Chiasmal Disorders: A Review on Exceptional Visual Disabilities as Based on 30 Years Clinical Experience

Purpose: To direct attention to a generally ignored cognitive element in chiasmal symptomatology, with the aim of reducing the diagnostic delay in such cases, where complaints are typically vague. A minority of chiasmal patients thus present with cognitive ignorance or mis-interpretation in visual space, probably caused by conductive loss of information as usually required for higher brain centres to induce relevant eye movements. Methodology: Personal experience from more than 30 years’ neuro-ophthalmological service is overviewed, primarily based on observation of abnormal behaviour during routine clinical testing for visual acuity (eventually summing up to 64 patients). Neuro-psychological testing and extended imaging (including functional positron emission tomography imaging as a pilot study) were carried out in part of the series. Major findings: A main feature has been lateralizing behaviour during unilateral visual acuity testing, showing a lateral ignorance on the board. Many also presented with inability to read print and a few had bizarre misinterpretations in visual space. Conclusions: The cognitive aspects have gained little attention in the literature. As a guide to early diagnostic attention, however, acknowledging aberrant behaviour in the eye clinic may b

Imaging of Optic Neuropathy and Chiasmal Syndromes.

Optic neuropathy involves loss of visual acuity, color vision, and visual field defect with a swollen, pale, anomalous, or normal optic disc seen on fundoscopy. Chiasmal disorders classically present with gradual onset of vision loss, bitemporal hemianopsia, and occasionally, endocrinopathy if the pituitary gland and/or hypothalamus are the causes or are involved. Advance in neuroimaging, especially magnetic resonance (MR) imaging, can reveal pathologic conditions previously detected only clinically. Some entities have imaging characteristics, leading to appropriate treatment without requiring tissue biopsies. Imaging also provides disease surveillance and posttreatment assessment, with computed tomography and MR imaging being complementary to each other.

Functional PET Scan in Four Patients with Higher Order Neglect-Like Cognitive Dysfunction Associated to Chiasm Related Pathology

Cognitive disturbances with neglect-like features have been reported occasionally in patients with chiasmal disorders, so far however with no obvious substrate by conventional brain imaging. Thus, there were no right hemisphere lesions that could explain the lateralised visual inattention as observed in particular during monocular visual acuity testing. On this background, we further examined four adult patients who consented to functional 18F-fluoro-deoxyglucose (FDG) positron emission tomography (PET) scan. In three there were no significant findings. The fourth patient, a 26-year-old male with cognitive defects after surgery for craniopharyngioma, will be discussed in more detail. His PET scan demonstrated a widespread reduction of regional metabolic activity in left hemisphere primary visual cortex and higher order visual areas, despite absence of explanatory pathological signal changes on MRI. As present in only one out of four patients, however, the findings do not allow specific pathogenetic mechanisms to be suggested, nor generally to substantiate involvement of higher cerebral circuits. Obviously, even developed imaging has its limits, and in the very theory the visual dysfunctions observed might still depend on higher brain centres’ faulty adaptation to loss of pre-geniculate visual information.

Humphrey Matrix Perimetry in Optic Nerve and Chiasmal Disorders: Comparison with Humphrey SITA Standard 24-2

To compare the Humphrey Matrix 24-2 perimetry (Matrix; Carl Zeiss Meditec, Inc., Dublin, CA) with the standard automated perimetry Humphrey Visual Field Analyzer using SITA (Swedish Interactive Threshold Algorithm) program 24-2 (SAP; Carl Zeiss Meditec, Inc.) in neuro-ophthalmic disorders affecting the optic nerve and chiasm. Matrix and SAP were performed on 93 patients with neuro-ophthalmic disorders affecting the optic nerve and optic chiasm. Three readers compared the total and pattern deviation probability plots and judged the similarity and the extent of the visual field defects. The sensitivity and specificity of both perimeters were calculated. Concordance was good in 61%, fair in 30%, and poor in 9% of the total deviation plots. For the pattern deviation, concordance was good in 52%, fair in 34%, and poor in 14%. The extent of field loss was equal in 50%, 23% more extensive with Matrix, and 27% more extensive with SAP for total deviation plots. For the pattern deviation, the extent was equal in 47%, 20% more extensive with Matrix and 33% more extensive with SAP. The sensitivity for detecting defects was 84% (SAP) and 77% (Matrix) for total deviation and 80% (SAP) and 79% (Matrix) for pattern deviation (no significant difference, P > 0.05). The specificity was 84% (SAP) and 86% (Matrix) for total deviation and 68% (SAP) and 74% (Matrix) for pattern deviation (no significant difference, P > 0.05). The new Humphrey Matrix 24-2 testing strategy provides a visual field testing method for optic nerve and chiasmal disorders that has fair to good concordance with the Humphrey SITA Standard 24-2 program. Both tests have similar sensitivity and specificity.

Chiasmal syndromes.

This review of the more recent literature and testing strategies in patients with chiasmal syndromes focuses on the clinical evaluation and management of these patients. Visual loss is often the initial manifestation of disorders involving the optic chiasm. Hemianopic defects and preferential involvement of the temporal visual field are the earliest and most common visual deficits. Progression of the lesion may cause compression of adjacent structures, including the optic nerves and cavernous sinuses, and may result in more profound visual loss, ocular motor deficits, and hypopituitarism. Although a number of modalities have been used to assess visual function in patients with chiasmal disorders, perimetry remains the most effective means of detecting and following the visual deficit. Most chiasmal syndromes can be categorized as intrinsic (thickening of the chiasm itself) or extrinsic (compression of the chiasm from an adjacent structure). Magnetic resonance imaging is the best mode of neuroimaging for most chiasmal lesions and may also provide clues to the etiology of an isolated chiasmal syndrome.