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[A syndrome of visuo-verbal and visuo-gestural disconnexion. Optic aphasia and optic apraxia (author's transl)].

A 71 year-old female patient was investigated during several months after a double left hemispheric lesion of vascular origin. The larger lesion was temporo-occipital, the second one parietal. The main neurological disorder was a right homonymous hemianopia. Clinical and neuroradiological data confirmed the integrity of the right hemisphere. There was neither a global deterioration of intellectual capacities nor aphasia. In particular, visual agnosia was excluded by a series of matching and functional classification tests. Nevertheless the objects and their pictures, which the patient could recognize, could not be named. The lack of words was limited to the visual modality. No apraxia neither ideomotor nor ideational was present on verbal commands, but the evocation of movements for the use of objects was deficient if the stimulation was visual. This disorder is considered in the frame of the visual-gestural disconnection and thus represents what we propose to call visual apraxia. Further we discuss the disorders of constructive apraxia which are characterized by the poor reproduction of models when executed by the right hand whereas the reproduction by the left hand is incorrect.

Aged↗

Histomorphometric analysis of optic nerve changes in experimental glaucoma.

PURPOSE: To assess relative changes in different tissue components of optic nerve and their relationship to nerve fiber loss in the experimental monkey model of glaucoma. METHODS: Chronic intraocular pressure (IOP) elevation was induced by laser trabeculoplasty in the right eye of eight monkeys (Macaca fascicularis). Both experimental right optic nerves and control left optic nerves were studied. Histomorphometric analysis was performed on optic nerve cross-sections using bright field microscopy with camera lucida. Cross-sectional areas of optic nerve tissue components were estimated by point counting. Nerve fiber density was estimated by unbiased random sampling. Nerve fiber number was calculated by multiplying nerve fiber density with neuroglial area. RESULTS: Varying degrees of nerve fiber loss were seen in eight optic nerves with chronic IOP elevation. More than 50% nerve fiber loss was noted in four of eight experimental optic nerves. In these severely affected optic nerves, total optic nerve area was significantly decreased compared with control optic nerves. Among the optic nerve tissue components, only the ratio of myelinated fiber area to total optic nerve area was significantly decreased. The ratio of extraaxonal area to total optic nerve area was significantly increased, whereas the ratio of interfascicular septal area to total optic nerve area did not change significantly. For all optic nerves, differences in nerve fiber count between control and experimental optic nerves showed the strongest correlation with differences in myelinated fiber area, followed by differences in extraaxonal area and total optic nerve area. CONCLUSION: This histomorphometric study suggests the validity of the experimental monkey model of glaucoma in studying changes occurring in the nonaxonal optic nerve tissue components in human glaucomatous optic neuropathy. Glial scar tissue area was significantly increased in optic nerves with severe glaucomatous damage. Although a decrease in total optic nerve area was observed, among the optic nerve tissue components only myelinated nerve fiber area decreased significantly. Myelinated nerve fiber area also showed the strongest association with nerve fiber loss in experimental glaucoma.

Animals↗

Fibre organization of the monkey's optic tract: II. Noncongruent representation of the two half-retinae.

The representations of the two half-retinae were examined in the monkey's optic tract. Intravitreal injections of tritiated amino acids were made to reveal the distributions of the crossed and uncrossed populations of optic axons, while localized implants of horseradish peroxidase (HRP) were made into different regions of the optic tract in order to examine the distributions and morphological types of retrogradely labelled cells at corresponding loci in the two half-retinae. Crossed and uncrossed optic axons are intermingled throughout most of the optic tract, but uncrossed axons are very sparse or absent along both the deep and superficial extremes of the tract. Implants of HRP into the deeper regions of the tract demonstrate that the crossed and uncrossed optic axons of the P beta retinal ganglion cells are slightly out of binocular registration, with the uncrossed map being shifted to a slightly superficial location relative to the crossed map. The optic axons for the remaining cell classes, revealed by implants of HRP into the superficial portion of the tract, are much more conspicuously out of binocular registration (in particular, the P alpha optic axons); but in their cases, the uncrossed optic axons are shifted to deeper locations relative to the crossed optic axons. Further evidence that these optic axon classes are markedly out of binocular registration comes from the two optic tracts of a bilaterally destriated monkey, in which most of the P beta optic axons have undergone a transneuronal retrograde degeneration. Following a uni-ocular injection of tritiated amino acids, the distributions of the remaining crossed and uncrossed axonal labelling occupied different positions within the tract rather than being intermingled, with the uncrossed optic axons situated deep to the majority of crossed optic axons. These results demonstrate that the optic chiasm does not combine binocularly corresponding optic axons of similar type. They also demonstrate that noncongruent field defects should be a common consequence of damage to the optic tract in humans. If the fibre order in the mammalian optic tract arises as a consequence of the sequence of axonal addition during development, then differences in the relative times of genesis for nasal and temporal members of any cell class, and/or differences in the relative pathlengths between the eye and two optic tracts, may produce the fibre ordering described herein.

Animals↗

Three-dimensional imaging of xenograft tumors using optical computed and emission tomography.

The physical basis and preliminary applications of optical computed tomography (optical-CT) and optical emission computed tomography (optical-ECT) are introduced, as new techniques with potential to provide unique 3D information on a variety of aspects of tumor structure and function. A particular focus here is imaging tumor micro-vasculature, and the spatial distribution of viable tumor cells, although the techniques have the potential for much wider application. The principle attractiveness of optical-CT and optical-ECT are that high resolution (<20 microm) and high contrast co-registered 3D images of structure and function can be acquired for relatively large intact samples. The unique combination of high contrast and resolution offers advantages over micro-CT and micro-MRI, and the lack of requirement for sectioning offers advantages over confocal microscopy, conventional microscopy, and histological sectioning techniques. Optical-CT/ECT are implemented using in-house custom apparatus and a commercial dissecting microscope capable of both transmission and fluorescence imaging. Basic studies to characterize imaging performance are presented. Negligible geometrical distortion and accurate reconstruction of relative attenuation coefficients was observed. Optical-CT and optical-ECT are investigated here by application to high resolution imaging of HCT116 xenograft tumors, about 1 cc in dimension, which were transfected with constitutive red fluorescent protein (RFP). Tumor microvasculature was stained in vivo by tail vein injection of either passive absorbing dyes or active fluorescent markers (FITC conjugated lectin). Prior to imaging, the tumors were removed (ex vivo) and optically cleared in a key process to make the samples amenable to light transmission. The cleared tumors were imaged in three modes (i) optical-CT to image the 3D distribution of microvasculature as indicated by absorbing dye, (ii) optical-ECT using the FITC excitation and emission filter set, to determine microvasculature as indicated by lectin-endothelial binding, and (iii) optical-ECT using the DSRed2 filter set to determine the 3D distribution of viable tumor as indicated by RFP emission. A clear correlation was observed between the independent vasculature imaging modes (i) and (ii) and postimaging histological sections, providing substantial validation of the optical-CT and optical-ECT techniques. Strong correlation was also observed between the RFP imaging of mode iii, and modes i and ii, supporting the intuitive conclusion that well-perfused regions contain significant viable tumor. In summary, optical-CT and optical-ECT, when combined with new optical clearing techniques, represent powerful new imaging modalities with potential for providing unique information on the structure and function of tumors.

Animals↗

Significant improvement of visual functions after removal of an intracranial giant optic nerve glioma revealing exophytic growth: case report.

OBJECTIVE AND IMPORTANCE: Intracranial giant optic nerve gliomas, usually presumed as optic chiasmatic gliomas, are much less common. The architectural tumor form of optic nerve glioma without neurofibromatosis type 1 is usually the expansile-intraneural pattern. The exophytic optic nerve gliomas without neurofibromatosis type 1 are relatively uncommon. Surgical decompression for intracranial optic gliomas frequently leads to clinical improvement, but obvious improvement of vision is rare. We report a case that demonstrated significant recovery of visual function after removal of the intracranial giant optic nerve glioma, revealing exophytic growth. CLINICAL PRESENTATION: A 13-year-old boy presented with visual impairment in both eyes. Magnetic resonance images (MRI) disclosed a 6 cm diameter mass in the suprasellar area. On heavily T2-reversed MRIs, it was obvious that the intracranial portion of right optic nerve was enlarged, and optic tracts were shifted to the left by the tumor. The relationship of the tumor to the chiasma could not be affirmed on MRIs. INTERVENTION: A right frontotemporal craniotomy for decompression of the optic apparatus was performed. After the majority of the tumor was resected, it became clear that the tumor originated in the right optic nerve. The tumor exophytically grew and dislocated the optic chiasma and optic tracts. Significant improvement of visual functions began from the first week after surgery and continued gradually thereafter. The histological diagnosis was pilocytic astrocytoma. A follow-up MRI taken 4 years after surgery showed no regrowth of the residual tumor. CONCLUSION: Giant exophytic gliomas without neurofibromatosis type 1 may arise from the intracranial portion of an isolated optic nerve. Direct visualization of optic component by heavily T2-reversed MRI could more precisely delineate the relationship of the intracranial optic nerve glioma to the optic apparatus. Surgery may be indicated in giant exophytic intracranial optic nerve gliomas and preoperative postulated optic chiasmatic gliomas. Microsurgical resection can induce postoperative visual improvement without regrowth of the residual tumor.

Adolescent↗

The aberrant retino-retinal projection during optic nerve regeneration in the frog. III. Effects of crushing both nerves.

Previous reports from this laboratory have shown that a substantial number of optic axons are misrouted after optic nerve regeneration in the adult frog, Rana pipiens. Regenerating axons from a crushed optic nerve are distributed throughout the opposite nerve. In this study, we report the effect of crushing both optic nerves (double crush) on the pattern and degree of axonal misrouting. In 28 frogs both optic nerves were crushed at the same time (simultaneous double crush) and animals survived for varying periods before the right eye was injected with 3H-proline and the brain processed for autoradiography 24 hours later. In every frog with postoperative survivals longer than 2 weeks, labeled axons from the right eye were found in the left optic nerve. However, when compared to the amount of label seen in frogs in which only the right optic nerve was crushed (single crush) there was substantially less label within the left nerve of frogs after crushing both nerves. Label was also found only at the edge of the left nerve in material from double crush frogs, unlike that found after single crush. In four of six frogs where the left nerve was crushed 1 week after the right nerve (delayed double crush), the proximal end of the left nerve was completely filled with label, but more distally, label was found only along the edge of this nerve. Although fewer optic axons were labeled in the opposite optic nerve of double crush frogs, label did extend to the optic disc of that eye. However, label was not apparent in the ganglion cell fiber layer of the opposite eye. Instead, it was confined to the edge of the optic disc in a space apparently associated with papilledema resulting from crushing the optic nerve of that eye. In six frogs the retina of the left eye was removed at the same time the right optic nerve was crushed. Labeled axons of the right eye filled the left optic nerve to the retina-less shell of the left eye. Thus, these data show that the amount and distribution of axonal misrouting into the opposite optic nerve during optic nerve regeneration is affected by intact or regenerating optic axons from the other eye.

Animals↗