Ocular neuromyotonia secondary to a cavernous sinus meningioma.
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Acetazolamide↗
Biomedical subjects
Alain Vighetto
Publications and source records attributed to Alain Vighetto.
Saccadic lateropulsion in Wallenberg syndrome: a window to access cerebellar control of saccades?
Saccadic lateropulsion is characterized by an undershoot of contralaterally directed saccades, an overshoot of ipsilaterally directed saccades and an ipsilateral deviation of vertical saccades. In Wallenberg syndrome, it is thought to result from altered signals in the olivo-cerebellar pathway to the oculomotor cerebellar network. In the current study we aimed to determine whether saccadic lateropulsion results from a cerebellar impairment of motor related signals or visuo-spatial related signals. We studied the trajectory, the accuracy, the direction and the amplitude of a variety of vertical and oblique saccades produced by five patients and nine control subjects. Some results are consistent with previous data suggesting altered motor related signals. Indeed, the horizontal error of contralesional saccades in patients increased with the desired horizontal saccade size. Furthermore, the initial directional error measured during the saccadic acceleration phase was smaller than the global directional error, suggesting that the eye trajectory curved progressively. However, some other results suggest that the processes that specify the horizontal spatial goal of the saccades might be impaired in the patients. Indeed, the horizontal error of ipsilesional saccades in patients did not change significantly with the desired horizontal saccade size. In addition, when comparing saccades with similar intended direction, it was found that the directional error was inversely related to the vertical saccade amplitude. Thus we conclude that the cerebellum might be involved both in controlling the motor execution of saccades and in determining the visuo-spatial information about their goal.
Aged↗
Experiencing and detecting happiness in humans: the role of the supplementary motor area.
Understanding emotions in others may involve neural structures implicated in both perception and action.1 Laughter is a socially important behavior and its neural correlates are poorly understood. Depth electrode electrophysiological techniques offer a rare opportunity to both record and stimulate neural structures involved in emotion processing. (2,3) This kind of direct electrophysiological investigation in humans is particularly determinant because laughter processing cannot be studied in animals.
Adult↗
[Emotional facial expression recognition impairment in Parkinson disease].
INTRODUCTION: some behavioral disturbances observed in Parkinson's disease (PD) could be related to impaired recognition of various social messages particularly emotional facial expressions. METHOD: facial expression recognition was assessed using morphed faces (five emotions: happiness, fear, anger, disgust, neutral), and compared to gender recognition and general cognitive assessment in 12 patients with Parkinson's disease and 14 controls subjects. RESULTS: facial expression recognition was impaired among patients, whereas gender recognitions, visuo-perceptive capacities and total efficiency were preserved. Post hoc analyses disclosed a deficit for fear and disgust recognition compared to control subjects. CONCLUSION: the impairment of emotional facial expression recognition in PD appears independent of other cognitive deficits. This impairment may be related to the dopaminergic depletion in basal ganglia and limbic brain regions. They could take a part in psycho-behavioral disorders and particularly in communication disorders observed in Parkinson's disease patients.
Aged↗
[Vertigo (including treatment)].
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Diagnosis, Differential↗
Impairment of gaze-centered updating of reach targets in bilateral parietal-occipital damaged patients.
Recent studies have suggested that internal updating of visuospatial targets in humans occurs in gaze-centered coordinates and takes place in the parietal and extrastriate cortices. We explored how information for reaching is updated in two patients with bilateral lesions in these areas. Subjects performed two visuomotor tasks: (i) a fixation reaching task, which began with the appearance of one of five fixation positions (varying eye positions) followed by a central reaching target. Subjects reached to the target while fixating on the presented fixation position (relative to gaze the target was always presented in the periphery); and (ii) a saccade reaching task, in which subjects foveated on the central reaching target, then made a saccade to the presented fixation position before reaching to the central target. In both tasks, subjects reached to targets after a 500 or 5000 ms delay. Gaze-centered updating predicts similarities in reaching errors between fixation and saccade trials. Control subjects showed evidence for gaze-centered updating during both 500 and 5000 ms delay conditions. In contrast, patient AT, who had extensive occipital-parietal damage, only showed signs of gaze-centered representation after 5 s. Patient IG, with a more focal lesion in the parietal cortices, showed partial updating in gaze-centered coordinates when reaching with the small memory delay but recovered a complete gaze-centered representation after the longer delay. This suggests that patients with bilateral occipital-parietal lesions may rely on non-gaze-centered frames to store immediate target locations in reaching space but, given enough time, this information may be rerouted to access other gaze-centered motor cortical mechanisms.
Adult↗
Beware of neuro-syphilis.
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Diagnosis, Differential↗
Anti-GAD antibodies and periodic alternating nystagmus.
BACKGROUND: Autoantibodies directed against glutamic acid decarboxylase (GAD-Ab) have recently been described in a few patients with progressive cerebellar ataxia, suggesting an autoimmune physiopathologic mechanism. OBJECTIVE: To determine the exact role of GAD-Ab and gamma-aminobutyric acid (GABA)-ergic neurotransmission in the pathogenesis of cerebellar ataxia. DESIGN: Case report. SETTING: University neurological hospital. PATIENT: We report the case of a patient with subacute cerebellar ataxia associated with GAD-Ab showing periodic alternating nystagmus (PAN). INTERVENTION: Baclofen, a GABAergic medication, was given to the patient. MAIN OUTCOME MEASURES: Eye movement recording of spontaneous nystagmus and postrotatory vestibular responses. RESULTS: Baclofen was effective in suppressing PAN and improving postrotatory vestibular responses but not for improving cerebellar ataxia. CONCLUSION: The presence of PAN and the response to baclofen provide a unique opportunity to suggest a direct role of GAD-Ab in cerebellar dysfunction in this patient.
Aged↗
Potential role of anti-GAD antibodies in abnormal eye movements.
Glutamic acid decarboxylase (GAD) catalyzes the conversion of glutamic acid to gamma-aminobutyric acid (GABA). Autoantibodies directed against GAD (antiGAD-Ab) have been described in patients with insulin-dependent diabetes mellitus, stiff-man syndrome, and in a few patients with progressive cerebellar ataxia. The presence of these autoantibodies suggests an autoimmune pathophysiological mechanism for the neurological manifestations in these disorders. However, the exact role of antiGAD-Ab and GABAergic neurotransmission in the pathogenesis of the neurological manifestations, particularly in progressive cerebellar ataxia, is not fully understood. The cases of two patients with subacute cerebellar ataxia associated with antiGAD-Ab presenting with abnormal eye movements are reported. One patient presented a periodic alternating nystagmus (PAN), whereas the other presented a downbeat nystagmus (DBN) and slow vertical saccades. The potential role of antiGAD-Ab and the resultant GABAergic neurotransmission deficit in oculomotor manifestations is discussed.
Aged↗
Automatic avoidance of obstacles is a dorsal stream function: evidence from optic ataxia.
When we reach out to pick something up, our arm is directed to the target by visuomotor networks in the cortical dorsal stream. However, our reach trajectories are influenced also by nontarget objects, which might be construed as potential obstacles. We tested two patients with bilateral dorsal-stream (parietal lesions, both of whom were impaired at pointing to visual stimuli (optic ataxia). We asked them to reach between two cylinders, which varied in location from trial to trial. We found that the patients' reaches remained invariant with changes in obstacle location. In a control task when they were asked to point midway between the two objects, however, their responses shifted in an orderly fashion. We conclude that the dorsal stream provides the visual guidance we automatically build into our movements to avoid potential obstacles, as well as that required to ensure arrival at the target.
Adult↗
Early amygdala reaction to fear spreading in occipital, temporal, and frontal cortex: a depth electrode ERP study in human.
The amygdala involvement in fear processing has been reported in behavioral, electrophysiological, and functional imaging studies. However, the literature does not provide precise data on the temporal course of facial emotional processing. Intracranial event-related potentials to facial expressions were recorded in epileptic patients implanted with depth electrodes during a presurgical evaluation. Specific potentials to fear beginning 200 ms poststimulus were observed in amygdala, both individually in two patients and in a ten patient population study. These potentials occurred 100 ms earlier than potentials to disgust recorded in insula in a previous study. Potentials to fear were confined in amygdala during a first transient period and then, during a second period of sustained activity, spread to occipito-temporal, anterior temporal, and orbitofrontal cortex in two patients. This study clarifies the temporal course of the involvement of these structures known to be part of a neural network recruited to process emotional information.
Amygdala↗
Pulse-synchronous eye oscillations revealing bone superior canal dehiscence.
Superior canal dehiscence syndrome is a newly recognized syndrome characterized by vertigo and nystagmus induced by sound (Tullio phenomenon) or changes of middle ear (Hennebert sign) or intracranial pressure. We report on a patient with bilateral superior canal dehiscence syndrome who presented with unusual manifestations including pulse-synchronous vertical pendular nystagmus and Valsalva-induced, up and counterclockwise-beating jerk nystagmus. These unusual symptoms may be a clue to a better understanding of the pathophysiology of superior canal dehiscence syndrome. Abnormal communication between the inner ears and the intracranial space may explain the vertical pendular and pulse-synchronous nystagmus, modulated by increased intracranial pressure.
Adult↗
Optic ataxia revisited: visually guided action versus immediate visuomotor control.
Optic ataxia and visual agnosia have been proposed to constitute a double dissociation which provides the main argument for the assimilation of the anatomical distinction between a dorsal and a ventral visual stream to the functional distinction between perception and action. In the present review, we argue that insufficient evidence has been collected to argue for this double dissociation. Several criteria are reviewed: (1) exploration of the visuomotor behavior in central versus peripheral vision has not been matched for the two types of patients; (2) the temporal constraints of visual processes that are impaired in the two neurological conditions appear to play a crucial role in the apparent dissociation; (3) the necessary reductionism of experimental conditions used to study action has led to an overconsideration of optic ataxia as a global deficit for action. Altogether optic ataxia appears to result from a specific impairment of immediate visuomotor control rather than of visually guided action as a whole. These results are discussed in the light of recent research on optic ataxia and on motor control, and directions for future research are proposed.
Agnosia↗
Human lateral geniculate nucleus and visual cortex respond to screen flicker.
The first electrophysiological study of the human lateral geniculate nucleus (LGN), optic radiation, striate, and extrastriate visual areas is presented in the context of presurgical evaluation of three epileptic patients (Patients 1, 2, and 3). Visual-evoked potentials to pattern reversal and face presentation were recorded with depth intracranial electrodes implanted stereotactically. For Patient 1, electrode anatomical registration, structural magnetic resonance imaging, and electrophysiological responses confirmed the location of two contacts in the geniculate body and one in the optic radiation. The first responses peaked approximately 40 milliseconds in the LGN in Patient 1 and 60 milliseconds in the V1/V2 complex in Patients 2 and 3. Moreover, steady state visual-evoked potentials evoked by the unperceived but commonly experienced video-screen flicker were recorded in the LGN, optic radiation, and V1/V2 visual areas. This study provides topographic and temporal propagation characteristics of steady state visual-evoked potentials along human visual pathways. We discuss the possible relationship between the oscillating signal recorded in subcortical and cortical areas and the electroencephalogram abnormalities observed in patients suffering from photosensitive epilepsy, particularly video-game epilepsy. The consequences of high temporal frequency visual stimuli delivered by ubiquitous video screens on epilepsy, headaches, and eyestrain must be considered.
Adult↗
An attention modulated response to disgust in human ventral anterior insula.
The human brain is expert in analyzing rapidly and precisely facial features, especially emotional expressions representing a powerful communication vector. The involvement of insula in disgust recognition has been reported in behavioral and functional imaging studies. However, we do not know whether specific insular fields are involved in disgust processing nor what the processing time course is. Using depth electrodes implanted during presurgical evaluation of patients with drug-refractory temporal lobe epilepsy, we recorded intracerebral event-related potentials to human facial emotional expressions, that is, fear, disgust, happiness, surprise, and neutral expression. We studied evoked responses in 13 patients with insular contacts to specify the insular fields involved in disgust processing and assess the timing of their activation. We showed that specific potentials to disgust beginning 300 milliseconds after stimulus onset and lasting 200 milliseconds were evoked in the ventral anterior insula in four patients. The occurrence and latency of event-related potentials to disgust in the ventral anterior insula were affected by selective attention. The analysis of spatial and temporal characteristics of insular responses to disgust facial expression lead us to underline the crucial role of ventral anterior insula in the categorization of facial emotional expressions, particularly the disgust.
Attention↗
Measuring unconscious actions in action-blindsight: exploring the kinematics of pointing movements to targets in the blind field of two patients with cortical hemianopia.
We tested two patients with posterior cerebral lesions on two pointing tasks. In the first task, the patients pointed to targets presented on a touch screen monitor and pointing accuracy was recorded. One patient (JR) demonstrated good localisation of targets presented to her blind field while the other patient (YP) did not. Movement kinematics were measured in the second task to compare the kinematics of movements made to sighted field targets with those made to blind field targets. For this version of the task both patients demonstrated above chance localisation of blind field targets although the slope of the relationship between the end of pointing movements and the target locations was significantly steeper for JR than for YP. Furthermore, JR showed a kinematic profile for movements made to blind field targets that mirrored the profile of kinematics to sighted field targets. That is, both peak velocity and time to peak velocity increased with increasing target eccentricity for movements made to blind and sighted field targets alike. Although patient YP now showed more reliable spatial localisation on this pointing task when compared with the touch screen task, his kinematics for movements made to targets in his blind field were quite different from those made to targets in his sighted field. Based on the patients' CT scans, we suggest that the superior performance of patient JR is a consequence of greater sparing of her parietal cortex in the damaged hemisphere.
Adult↗
Leigh-like encephalopathy complicating Leber's hereditary optic neuropathy.
Leber's hereditary optic neuropathy is a mitochondrial disease caused by point mutations in mitochondrial DNA. It usually presents as severe bilateral visual loss in young adults. We report on a neurological disorder resembling Leigh syndrome, which complicated Leber's hereditary optic neuropathy in three unrelated male patients harboring mitochondrial DNA mutations at nucleotide positions 3460, 14459, and 14484, respectively. This Leigh-like encephalopathy appears to be associated with a much more severe outcome than isolated Leber's hereditary optic neuropathy.
Adult↗
Saccadic lateropulsion and upbeat nystagmus: disorders of caudal medulla.
A patient developed a primary position upbeat nystagmus and a left saccadic lateropulsion. Magnetic resonance imaging demonstrated a probable cavernoma at right caudal paramedian medullary level. Anatomical correlations are discussed. Saccadic lateropulsion is attributed to olivocerebellar pathway impairment but usually is described in more rostral medullar lesions. Our case would still support this hypothesis because the lesion could have involved the olivocerebellar pathway at its very caudal level. Upbeat nystagmus could be attributed to impairment of the nucleus intercalatus and/or cell groups of the paramedian tract.
Adult↗