Cases reported "Ataxia"

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1/10. Discrepancy between dysmetric centrifugal movements and normometric centripetal movements in psychogenic ataxia.

    A method to unravel an aberrant motor behaviour in psychogenic ataxia is reported. The kinematic features of fast reaching movements in the vertical plane are described in a patient presenting a psychogenic ataxia. The procedure compared centrifugal and centripetal movements. Path ratios were computed for each phase, as well as the ratios of the paths for centrifugal and centripetal directions. Trajectories of centrifugal phases were erratic but centripetal movements were very regular, whereas both centripetal and centrifugal movements were irregular in patients presenting an organic cerebellar syndrome. A similar incongruity between movements in opposite directions is also shown for a second patient exhibiting psychogenic ataxia. Discrepancies between the centrifugal phase and the centripetal phase of multi-joint reaching movements support the diagnosis of a psychogenic movement disorder.
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keywords = behaviour
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2/10. meningioma of the fourth ventricle presenting with intermittent behaviour disorders: a case report and review of the literature.

    Intraventricular meningiomas are rare, representing 0.5-5% of all intracranial meningiomas. They arise mostly within the lateral ventricles and more rarely in the third ventricle. Meningiomas of the fourth ventricle are exceptional. They are clearly defined as meningiomas arising from the choroid plexus and lying strictly within the fourth ventricle. We report a 76 year old male patient presenting with a 2-week history of headache and cognitive disorders with agitation and restlessness particularly exacerbated at night or when lying down. CT scan and MR imaging showed a contrast-enhancing lesion located purely within the whole fourth ventricle, with slight ventricular enlargement. At surgery, we totally removed a well-vascularised, greyish encapsulated mass attached to the choroid plexus. Pathological examination revealed a WHO grade I fibroblastic meningioma. We reviewed the literature concerning this unusual meningioma location.
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ranking = 4
keywords = behaviour
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3/10. Increasing functional communication through relaxation training and neuromuscular feedback.

    The following research examined the effects of behavioural relaxation training and biofeedback on ataxic tremor of an adult with acquired brain injury. The participant was taught relaxation techniques before biofeedback was introduced. Once he was proficient in relaxation, these skills were then used as a foundation for biofeedback training. Specific skills, facilitating the use of a letter board, were taught when the participant was able to relax the appropriate musculature to criterion. The results demonstrated that the participant learned how to significantly decrease the severity of tremor. As a result, he became more proficient at communicating via his letter board. Collateral effects were increased attempts at communication and fewer episodes of anger.
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ranking = 1
keywords = behaviour
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4/10. Behavioural changes due to pontine and extrapontine myelinolysis.

    OBJECTIVE: To report a case of central and extrapontine myelinolysis with unusual behavioural manifestations that developed after slow (not more than 0.5 mmol sodium per litre per hour) correction of hyponatraemia. CLINICAL FEATURES: A 51-year-old Caucasian woman with hyponatraemia caused by psychogenic polydipsia experienced a delayed onset of behavioural changes with some ataxia, but no pyramidal signs, after correction of the hyponatraemia. INTERVENTION AND OUTCOME: The hyponatraemia was corrected with a combination of hypertonic saline and fluid restriction at the currently recommended rate of not more than 0.5 mmol of sodium per litre per hour. CONCLUSION: Central and extrapontine myelinolysis may develop after slow correction of hyponatraemia. Behavioural manifestations may be the most prominent clinical feature.
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ranking = 2
keywords = behaviour
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5/10. Preserved prism adaptation in bilateral optic ataxia: strategic versus adaptive reaction to prisms.

    To date the anatomical substrate(s) of prism adaptation remain(s) particularly debated, with two main candidates emerging from the literature: the posterior parietal cortex (PPC) and the cerebellum. The functional processes involved in the acquisition of the adaptive aftereffects also remain largely unknown. The main result shown here is that a patient with a bilateral optic ataxia can adapt to an optical deviation, which allows us to make a step forward on these two issues. First, it demonstrates that the corresponding part of the PPC is not a necessary substrate for prism adaptation. Second, since this patient exhibits deficit for fast visuo-motor guidance, it provides direct evidence for a dissociation between on-line visuo-motor control and visuo-motor plasticity. Since the intermanual transfer rate of adaptation is larger in this patient than in control subjects, the PPC may still have an influence on adaptation under normal conditions. We propose a model of the relative contribution of the PPC and the cerebellum during prism exposure, associating these two structures with the two interacting behavioural components of prism adaptation described by previous psychophysical experiments: the strategic component would be linked to the PPC and the adaptive component to the cerebellum. In this model, the strategic component enters in conflict with the development and the generalisation of the adaptive aftereffects. This idea is compatible with the fact that a lesion of the PPC increases the transfer rate and the generalisation of the adaptation, as is also observed in unilateral neglect.
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keywords = behaviour
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6/10. Leucoencephalopathy with neuroaxonal spheroids (LENAS) presenting as the cerebellar subtype of multiple system atrophy.

    Leucoencephalopathy with neuroaxonal spheroids (LENAS) is a rare disease of cerebral and cerebellar white matter. LENAS usually presents as a disorder of cognition and behaviour, or with gait dysfunction and ataxia. This report describes a patient who had a 14 year course of progressive neurological decline consistent with a clinical diagnosis of probable multiple system atrophy, with prominent cerebellar dysfunction and dysautonomia. Formal autonomic laboratory testing was consistent with global autonomic dysfunction of central origin. However, magnetic resonance imaging showed extensive white matter signal abnormalities, in addition to moderate cerebral and cerebellar atrophy. On postmortem microscopic examination, there were numerous axonal spheroids throughout the white matter of both regions. This case of LENAS presented unique clinical characteristics, and typical pathological findings.
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keywords = behaviour
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7/10. The space of senses: impaired crossmodal interactions in a patient with Balint syndrome after bilateral parietal damage.

    Balint syndrome after bilateral parietal damage involves a severe disturbance of space representation including impaired oculomotor behaviour, optic ataxia, and simultanagnosia. Binding of object features into a unique spatial representation can also be impaired. We report a patient with bilateral parietal lesions and Balint syndrome, showing severe spatial deficits in several visual tasks predominantly affecting the left hemispace. In particular, we tested whether a loss of spatial representation would affect crossmodal interactions between simultaneous visual and tactile events occurring at the same versus different locations. A tactile discrimination task, where spatially congruent or incongruent visual cues were delivered near the patient's hands, was used. Following stimulation of the left hand in the left side of space, we observed visuo-tactile interactions that were not modulated by spatially congruent conditions. In contrast, performance following stimulation of the right hand in the right side of space was affected in a spatially selective manner--facilitated for congruent stimuli and slowed for incongruent stimuli. To dissociate effects on somatotopic and spatiotopic coordinates, we crossed the patient's hands during unimodal tactile discriminations. Tactile performance of the left hand improved when it was positioned in the right hemispace, whereas placing the right hand in left space produced no significant changes, suggesting that left-sided tactile inputs are coded with respect to a combination of limb- and trunk-centred coordinates. These data converge with recent findings in animals and healthy humans to indicate a critical role of the posterior parietal cortex in multimodal spatial integration, and in the fusion of different coordinates into a unified representation of space.
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ranking = 1
keywords = behaviour
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8/10. Absence status: a report of two cases.

    Two known epileptics presented with abnormal behaviour. One was inattentive, aggressive and ataxic. The other carried out quite complex, though purposeless activity. EEG's revealed that the patients' behavioural abnormalities were due to uncontrolled seizures. Absence Status is an uncommon condition and its various manifestations can lead to delays in its recognition and treatment. It should be considered as a possible diagnosis in any patient who presents with recurring episodes of confusion.
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ranking = 2
keywords = behaviour
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9/10. Duplication of the 15q11-13 region in a patient with autism, epilepsy and ataxia.

    Various developmental abnormalities can give rise to the clinical syndrome of autism, and some are due to chromosomal anomalies. One syndrome has been identified in which behavioural disorder is associated with the clinical features of epilepsy and ataxia, and with the chromosomal anomaly of an extra marker chromosome containing a duplication of 15q11-13. The authors report a boy with autism, epilepsy, ataxia and an interstitial duplication of 15q, in whom molecular analysis reveals duplication of the GABRA5 and GABRB3 genes on the maternally derived chromosome.
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ranking = 1
keywords = behaviour
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10/10. A study of motor performance and motor learning in episodic ataxia.

    Episodic ataxias are rare disorders in which periodic episodes of ataxia are separated by normal or near normal motor behaviour. They probably arise from dysfunctional membrane ion channels in the cerebellum. A patient with episodic ataxia EA-2 performed three motor tasks, before, during and after an ataxic episode. In all three tasks there were significant performance deficits during the ataxic episode. Two of the tasks also assessed motor adaptation (prism adaptation) or motor learning (ideogram drawing). In neither task was there significant disruption of motor adaptation or learning. These results suggest that the cerebellum may have separate roles in learning and in performance of visually guided movements, and that the dysfunction in this patient affected only his motor performance.
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ranking = 1
keywords = behaviour
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