Central visual, acoustic, and motor pathway involvement in a Charcot-Marie-Tooth family with an Asn205Ser mutation in the connexin 32 gene.
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BACKGROUND: X linked dominant Charcot-Marie-Tooth disease (CMT1X) is an inherited motor and sensory neuropathy that mainly affects the peripheral nervous system. CMT1X is associated with mutations in the gap junction protein connexin 32 (Cx32). Cx32 is expressed in Schwann cells and oligodendrocytes in the peripheral (PNS) and in the (CNS) respectively. METHODS: A CMT1X family with a Cx32 mutation was examined clinically and electrophysiologically to determine whether PNS, or CNS, or both pathways were affected. RESULTS: In a CMT1X family a novel mutation (Asn205Ser) was found in the fourth transmembrane domain of Cx32. The patients showed typical clinical and electrophysiological abnormalities in the PNS, but in addition visual, acoustic, and motor pathways of the CNS were affected subclinically. This was indicated by pathological changes in visually evoked potentials (VEPs), brainstem auditory evoked potentials (BAEPs), and central motor evoked potentials (CMEPs). CONCLUSIONS: These findings underscore the necessity of a careful analysis of CNS pathways in patients with CMT and Cx32 mutations. Abnormal electrophysiological findings in CNS pathway examinations should raise the suspicion of CMTX and a search for gene mutations towards Cx32 should be considered. (+info)
Action of the brain stem saccade generator during horizontal gaze shifts. I. Discharge patterns of omnidirectional pause neurons.
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Omnidirectional pause neurons (OPNs) pause for the duration of a saccade in all directions because they are part of the neural mechanism that controls saccade duration. In the natural situation, however, large saccades are accompanied by head movements to produce rapid gaze shifts. To determine whether OPNs are part of the mechanism that controls the whole gaze shift rather than the eye saccade alone, we monitored the activity of 44 OPNs that paused for rightward and leftward gaze shifts but otherwise discharged at relatively constant average rates. Pause duration was well correlated with the duration of either eye or gaze movement but poorly correlated with the duration of head movement. The time of pause onset was aligned tightly with the onset of either eye or gaze movement but only loosely aligned with the onset of head movement. These data suggest that the OPN pause does not encode the duration of head movement. Further, the end of the OPN pause was often better aligned with the end of the eye movement than with the end of the gaze movement for individual gaze shifts. For most gaze shifts, the eye component ended with an immediate counterrotation owing to the vestibuloocular reflex (VOR), and gaze ended at variable times thereafter. In those gaze shifts where eye counterrotation was delayed, the end of the pause also was delayed. Taken together, these data suggest that the end of the pause influences the onset of eye counterrotation, not the end of the gaze shift. We suggest that OPN neurons act to control only that portion of the gaze movement that is commanded by the eye burst generator. This command is expressed by driving the saccadic eye movement directly and also by suppressing VOR eye counterrotation. Because gaze end is less well correlated with pause end and often occurs well after counterrotation onset, we conclude that elements of the burst generator typically are not active till gaze end, and that gaze end is determined by another mechanism independent of the OPNs. (+info)
Functionally independent components of the late positive event-related potential during visual spatial attention.
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Human event-related potentials (ERPs) were recorded from 10 subjects presented with visual target and nontarget stimuli at five screen locations and responding to targets presented at one of the locations. The late positive response complexes of 25-75 ERP average waveforms from the two task conditions were simultaneously analyzed with Independent Component Analysis, a new computational method for blindly separating linearly mixed signals. Three spatially fixed, temporally independent, behaviorally relevant, and physiologically plausible components were identified without reference to peaks in single-channel waveforms. A novel frontoparietal component (P3f) began at approximately 140 msec and peaked, in faster responders, at the onset of the motor command. The scalp distribution of P3f appeared consistent with brain regions activated during spatial orienting in functional imaging experiments. A longer-latency large component (P3b), positive over parietal cortex, was followed by a postmotor potential (Pmp) component that peaked 200 msec after the button press and reversed polarity near the central sulcus. A fourth component associated with a left frontocentral nontarget positivity (Pnt) was evoked primarily by target-like distractors presented in the attended location. When no distractors were presented, responses of five faster-responding subjects contained largest P3f and smallest Pmp components; when distractors were included, a Pmp component appeared only in responses of the five slower-responding subjects. Direct relationships between component amplitudes, latencies, and behavioral responses, plus similarities between component scalp distributions and regional activations reported in functional brain imaging experiments suggest that P3f, Pmp, and Pnt measure the time course and strength of functionally distinct brain processes. (+info)
Frontal brain potentials during recognition are modulated by requirements to retrieve perceptual detail.
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To assess the role of prefrontal cortex in retrieval and address the controversy about whether prefrontal retrieval operations are engaged only following successful retrieval, we recorded event-related brain potentials during two recognition tests with differing demands on retrieval effort. Both tests included object drawings that were (1) identical to those studied, (2) the same but with altered aspect ratios, and (3) previously unseen. Instructions were to respond "old" only if drawings were not modified (specific test) or regardless of modifications (general test). Frontal potentials were enhanced during the specific relative to the general test for all three types of drawings. We conclude that these potentials reflected differential engagement of strategic retrieval, that this function relied on left prefrontal cortex, and that it was not contingent on successful retrieval. (+info)
Mid-peripheral pattern electrical retinal responses in normals, glaucoma suspects, and glaucoma patients.
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AIMS: Reliance on intraocular pressure, optic nerve cupping changes, nerve fibre layer integrity, and visual field changes may delay treatment of glaucoma since irreversible changes may have already occurred at the time of diagnosis. Abnormal pattern electrical retinal responses (PERR or PERG) have been demonstrated in patients with ocular hypertension (no visual field changes) and glaucoma when visual stimulation was presented to the central field. Since glaucomatous visual field changes tend to occur first in the mid-periphery, the use of PERR outside of the central field may offer an earlier indication of glaucomatous involvement. METHODS: Glaucoma suspects and glaucoma patients were derived from a university practice. Normal subjects were recruited from non-patient volunteers. Alternating bar gratings were presented in the supranasal, supratemporal, infratemporal, and infranasal visual field. Six spatial frequencies, from 0.25 to 6.0 cycles per degree, were used for normal volunteers; three spatial frequencies, from 0.38 to 1.5 cycles per degree, were presented to suspects and glaucoma patients. Time of onset of the first negative (N35) and first positive peak (P50) and the amplitude consisting of the absolute difference between the first negative peak and first positive peak (P50 amplitude) are reported. Age corrected values were determined for normals, suspects, and glaucoma patients for each spatial frequency and for each quadrant in the visual field. RESULTS: Mean P50 amplitudes from normal subjects showed spatial tuning in all quadrants with reduced low frequency attenuation. Normals demonstrated a small decline in amplitude with age. Glaucoma patients demonstrated an age corrected reduction in amplitude and early implicit times. Glaucoma suspects had values between those of normal and glaucoma subjects. P50 amplitudes were weakly correlated with increasing cup to disc diameter ratio. A glaucoma patient with asymmetric visual field loss demonstrated significant diminution of the PERR bilaterally. CONCLUSION: The PERR, using mid-peripheral stimulation, may be a sensitive tool for the early detection of glaucoma. Further refinements can speed clinical data acquisition and enhance signal to noise ratio. (+info)
Temporal analysis of the chromatic flash VEP--separate colour and luminance contrast components.
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Temporal analysis of the chromatic flash visual evoked potential (VEP) was studied in human subjects with normal and anomalous colour vision using a deterministic pseudo-random binary stimulus (VERIS). Five experiments were carried out on four normal subjects investigating heterochromatic red-green exchange and single colour/achromatic (either red/grey or green/grey) exchange over a wide range of luminance ratios for the two stimuli, the effects of lowered mean luminance on the chromatic VEP and the effects of colour desaturation at constant mean luminance and constant luminance contrast. Finally, the performance of three dichromats, a protanope and two deuteranopes, on heterochromatic exchange VEP and on colour desaturation were investigated. In contrast to the chromatic electroretinogram, which shows great symmetry with respect to luminance ratio on opposite sides of the isoluminant point, the chromatic VEP demonstrated a distinct asymmetry when the colours exchanged included red. On the red side of isoluminance (red more luminant than green), a wave with longer latency and altered waveform became dominant. The effects of green stimulation were indistinguishable from those of achromatic stimulation at the same luminance contrast over the whole range of chromatic contrast and for all levels of desaturation studied. Desaturation of red with constant luminance contrast (desaturated red/grey stimulation) resulted in a systematic alteration in the evoked waveform. Subtraction of the achromatic first- and second-order responses from responses recorded in the red desaturation series resulted in remarkably uniform waveforms, with peak amplitudes growing linearly with saturation. The absence of interaction between achromatic and coloured components for all (including the most intense colour) stimulus parameters used suggests that the generators of these components are separate. Recordings from the dichromats showed that the contrast response minimum shifted from the point of photopic isoluminance to the point of zero cone contrast (at the silent substitution point) for the remaining cone type. The waveforms recorded with a series of luminance ratios were much simpler than those recorded from trichromats and symmetrical with respect to their isoluminant points. Despite the indication of the presence of L cones of apparently normal spectral sensitivity in the deuteranopes (on the basis of flicker photometry), there was no evidence for a red-sensitive component in the desaturation or heterochromatic stimulation series. The results are discussed in terms of the possibility of separate generation of chromatic and achromatic contributions to the VEP. (+info)
Amodal completion in texture visual evoked potentials.
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Amodal completion refers to the phenomenological finding of perceiving partly occluded objects as continuing uninterrupted behind an occluder. The outlying problem is how the visual system processes such non-local stimuli because the known processes of early vision are spatially restricted operations which segregate local differences in the visual image, and little is known about their interactions in producing the segmentation of the image into functionally coherent, or global, objects. We recorded human visual evoked potentials (VEPs) to texture stimuli and addressed local/non-local relationships in comparing a condition in which local edges were present, due to texture segregation, with a condition in which, in addition to local edges, textures appeared to continue as surfaces behind gray stripes due to non-local amodal completion. Subtraction of offset from onset responses showed: (1) a difference component due to texture segregation characterized by a negativity with onset at about 95 ms and lasting up to about 280 ms; (2) a further negativity, specifically elicited by amodal completion, with onset at about 142 ms, peaking at 175 ms, and lasting up to about 188 ms. Therefore, amodal completion occurs at an early processing stage of image analysis and the difference component in VEPs can be related to figure-ground perception. (+info)
Effects of bicarbonate ion on chick retinal pigment epithelium: membrane potentials and light-evoked responses.
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The purpose of this study was to determine how changes in [HCO3-] alter the electrical properties of the retinal pigment epithelium (RPE). Experiments were conducted on the isolated chick retina-RPE-choroid preparation. The chamber holding the preparation allowed independent perfusion of the retinal and the choroidal surfaces. The light-evoked trans-tissue potential (TTP), the trans-epithelial potential (TEP), the trans-retinal potentials, and the intracellularly-recorded apical and basal membrane potentials were studied. Increasing the [HCO3-]0 in the choroidal bath from 25 to 40 mEq/1 led to an increase in the TTP and TEP. The same change in the retinal bath decreased the TTP because of a biphasic change of the RPE membrane potentials. There was also an increase in the amplitudes of the TEP, the c-wave and the slow PIII. The light-evoked subretinal K+ decrease was greater which is consistent with an increase in the photoreceptor light response. These observations indicated that the decrease of TTP resulted from a basal membrane hyperpolarization followed by an apical membrane depolarization induced by an increase in retinal [HCO3-]0. The relationship of these potential changes to the human bicarbonate responses is discussed. (+info)