Interest of dual-task-related gait changes in idiopathic normal pressure hydrocephalus.

BACKGROUND: Gait disorders in patients with idiopathic normal pressure hydrocephalus (iNPH) share similar characteristics found in pathologies presenting with higher-level gait disorders that have been specifically associated with gait changes during walking while simultaneously performing an attention-demanding task (i.e. dual tasking). The current study assessed the effect of cerebrospinal fluid (CSF) tapping on quantitative gait modification during single and dual tasking in patients with a suspicion of iNPH. METHODS: Of 53 patients suspected of iNPH, 18 have been included in this study. Gait analysis during single- and dual-task condition (walking and backward counting) before and after tapping of 40 ml CSF has been performed. RESULTS: Gait speed (P < 0.01) and stride length (P < 0.05) were significantly improved during dual-task conditions after CSF tapping compared to the gait performance before spinal tapping, without such improvement for gait parameters during single-tasking. CONCLUSION: Dual-tasking condition better reveals gait improvement after CSF tapping than single-tasking in patients suspected of iNPH.

Psychiatric comorbidity in patients evaluated for chronic epilepsy: a differential role of the right hemisphere?

INTRODUCTION: Psychiatric disorders are known to occur frequently in chronic epilepsy. The aim of this study is to investigate the prevalence of psychiatric comorbidity and its relationship to regional cerebral dysfunction in patients admitted to a tertiary epilepsy center for epilepsy surgery. METHODS: 217 patients were investigated. A presurgical workup was performed and allowed precise localization of the epileptogenic focus in 156 patients. Sixty-one patients had multifocal or generalized discharges. After 1-3 psychiatric interviews, a psychiatric diagnosis was made (DSM-IV classification). RESULTS: Psychiatric comorbidity was found in 85 patients (39%), more often in those with right or bilateral hemispheric dysfunction (74%, p = 0.04) with no difference between temporal or extratemporal foci location frequency. Additionally, patients with psychiatric disorders were less likely to undergo epilepsy surgery compared to 'epilepsy-only' patients (p = 0.003), despite similar good outcome in patients with and without psychiatric comorbidity. CONCLUSIONS: Right-sided or bilateral foci seem to represent a risk factor for psychiatric comorbidity in epilepsy, although we did not find any particular association between a psychiatric syndrome and focus localization. Recognition and treatment of psychiatric comorbidity is of major importance since its presence may interfere with patient's decision making for epilepsy surgery treatment.

"Gourmand syndrome" in a child with pharmacoresistant epilepsy.

We report the case of a 10-year-old boy with pharmacoresistant epilepsy, symptomatic of a right temporoparietal hemorrhagic lesion, who displayed an eating passion as described for the gourmand syndrome (GS) in adults and discuss the role of epilepsy in GS. This patient presented with a significant change in his eating habits (abnormal preoccupation with the preparation and eating of fine-quality food) concordant with the onset of his seizure disorder, without any previous history of eating disorders or psychiatric illness. This observation corroborates the important role of the right cerebral hemisphere in disturbed eating habits, including the relatively benign GS, and, possibly rarely, in less benign eating disorders such as anorexia and obesity.

Complete sparing of high-contrast color input to motion perception in cortical color blindness.

It is widely held that color and motion are processed by separate parallel pathways in the visual system, but this view is difficult to reconcile with the fact that motion can be detected in equiluminant stimuli that are defined by color alone. To examine the relationship between color and motion, we tested three patients who had lost their color vision following cortical damage (central achromatopsia). Despite their profound loss in the subjective experience of color and their inability to detect the motion of faint colors, all three subjects showed surprisingly strong responses to high-contrast, moving color stimuli--equal in all respects to the performance of subjects with normal color vision. The pathway from opponent-color detectors in the retina to the motion analysis areas must therefore be independent of the damaged color centers in the occipitotemporal area. It is probably also independent of the motion analysis area MT/V5, because the contribution of color to motion detection in these patients is much stronger than the color response of monkey area MT.

Innate and learned components of human visual preference.

BACKGROUND: Recent claims in neuroscience and evolutionary biology suggest that the aesthetic sense reflects preferences for image signals whose characteristics best fit innate brain mechanisms of visual recognition. RESULTS: This hypothesis was tested by behaviourally measuring, for a set of initially unfamiliar images, the effects of category learning on preference judgements by humans, and by relating the observed data to computationally reconstructed internal representations of categorical concepts. Category learning induced complex shifts in preference behaviour. Two distinct factors - complexity and bilateral symmetry - could be identified from the data as determinants of preference judgements. The effect of the complexity factor varied with object knowledge acquired through category learning. In contrast, the impact of the symmetry factor proved to be unaffected by learning experience. Computer simulations suggested that the preference for pattern complexity relies on active (top-down) mechanisms of visual recognition, whereas the preference for pattern symmetry depends on automatic (bottom-up) mechanisms. CONCLUSIONS: Human visual preferences are not fully determined by (objective) structural regularities of image stimuli but also depend on their learned (subjective) interpretation. These two aspects are reflected in distinct complementary factors underlying preference judgements, and may be related to complementary modes of visual processing in the brain.

Human colour discrimination based on a non-parvocellular pathway.

BACKGROUND: Traditionally, colour information is assumed to be carried by neural channels in the parvocellular pathway and to be encoded in an opponent manner, while other, non-parvocellular, spectrally non-opponent channels are thought to play no part in colour vision. But is the parvocellular pathway the only way that colours can be discriminated in human vision? We studied two patients with cerebral achromatopsia, who lack conscious colour perception but are nevertheless able to make use of colour information. In particular, we investigated whether, in these patients, colour discrimination is mediated by the parvocellular pathway. RESULTS: The achromatopsic patients carried out a forced-choice colour- and luminance-discrimination task, and showed clear evidence of unconscious colour processing, consistent with previous studies. We added different types of luminance noise to see when this unconscious colour information could be masked. The results of the colour-discrimination-with-noise and the brightness-non-additivity experiments showed a double-dissociation between patients. This indicates that, in one patient, unconscious colour discrimination may be subserved by a spectrally non-opponent mechanism, which does not have the characteristics of the parvocellular pathway and which is responsive to fast flicker. Spectral sensitivity, contrast sensitivity and motion perception experiments confirmed that this patient lacks a working opponent parvocellular system. The second achromatopsic patient showed evidence of a residual parvocellular system. CONCLUSIONS: Our results show that chromatic discrimination need not be mediated by neural mechanisms, the parvocellular system in particular, normally assumed to subserve conscious colour perception. Such discrimination may be mediated by a neural subsystem which responds to fast flicker, is spectrally non-opponent, and supports normal motion perception.

Distinct mechanisms of form-from-motion perception in human extrastriate cortex.

The exquisite sensitivity of the human visual system to form-from-motion (FfM) cues is well documented. However, identifying the neural correlates of this sensitivity has proven difficult, particularly determining the respective contributions of different motion areas in extrastriate visual cortex. Here we measured visual FfM perception and more elementary visual motion (VM) perception in a group of 32 patients suffering from acute posterior brain damage, and performed MRI-based lesion analysis. Our results suggest that severe FfM perception deficits without an associated deficit of VM perception are due to damage to ventral occipito-temporal cortex (VOT), whereas associated deficits of FfM and VM perception are due to damage either in proximity to area MT+/V5 or an area including lateral occipital complex (LOC) and VOT. These data suggest the existence of at least three functionally and anatomically distinct regions in human visual cortex that process FfM signals.

Favourable outcome of progressive multifocal leucoencephalopathy in two patients with dermatomyositis.

Progressive multifocal leucoencephalopathy (PML), a demyelinating disease caused by the JC virus (JCV), occurs in immunosuppressed patients and carries a poor prognosis. A favourable outcome is reported in two patients with PML and dermatomyositis. Immunosuppressive drugs were stopped in patient 1 but could only be partially tapered in patient 2. The JCV-specific CD8+ T cell response was strong in patient 1 and weak in patient 2. Both were treated with cytosine-arabinoside, and patient 2 was also treated with mirtazapine, a 5HT2A receptor antagonist. Combination of these drugs might be helpful to treat HIV-negative patients with PML.

[Status epilepticus and neuronal degeneration: of circuitries and vicious circles]. / Etat de mal épiteptique et neurodégénérescence: entre circuits et cercles vicieux.

The relationship between status epilepticus (SE) and neuronal degeneration is quite complex and difficult to investigate. Animal studies suggest that SE induces a progressive neuronal death; however, clinical observations on humans are less uniform: generalized SE may precede the development of hippocampal sclerosis, but other forms (such as simple-partial or complex-partial) seem less ominous. On the other side, SE can be encountered in many patients with neurodegenerative disorders, particularly those with severe forms. It is thus likely that SE represents a sign, rather than an aetiology, of clinical gravity. Therefore, while it is ancillary to treat rapidly any SE form, the therapeutic approach, especially the use of aggressive treatments, should be tailored according to the underlying medical conditions.

Blinking and schizotypal thinking.

Spontaneous eye blink rate (SBR) is thought to be a biological marker for cerebral dopamine (DA) activity. Accordingly, positive psychotic symptoms have been found to be associated with an increased SBR and negative psychotic symptoms with a decreased SBR. However, modulations of the DA system in patient populations also result from prior neuroleptic treatment. Here, we tested the possible relationship between SBR and positive and negative schizotypal thought. To test the direct influence of DA on SBR in general and as a function of schizotypy, half of a sample of 40 healthy men received levodopa and the other half placebo in a double-blind procedure. SBR did not differ between substance groups suggesting that a pharmacologically induced DA increase in healthy individuals does not generally increase SBR. However, in the levodopa group, increasing SBR correlated with increasing negative schizotypy scores, while no relationship was found between SBR and (1) negative schizotypy in the placebo group, or (2) positive schizotypy in either substance group. We conjecture that a pre-existing hypodopaminergic state in high negative schizotypy scorers, made these individuals susceptible to an increased DA concentration, as it has been observed in Parkinson's disease. Furthermore, the absence of any relationship in the placebo group might suggest that variations in DA concentration as a function of schizotypy are too subtle to influence SBR. Finally, the lack of any association of SBR with positive schizotypy might indicate that SBR and positive schizotypy are mediated by functionally distinct neural circuits.

Hemispheric control of speech expression in aphasia. A mouth asymmetry study.

Clinical observation of aphasic patients often shows a dissociation between impaired propositional speech and preserved automatic speech. The question of differing hemispheric control between these speech tasks was examined by measuring asymmetry in facial muscle activity. In spite of right-sided facial palsy, greater opening of the right side of the mouth was seen for spontaneous speech, repetition, and word list generation, suggesting that the damaged left hemisphere still controlled motor output in these propositional tasks. However, greater opening of the left side of the mouth for serial speech and singing indicated at least a relatively greater role of the right hemisphere in these "automatic" tasks.

Preserved stenography reading in a patient with pure alexia. A model for dissociated reading processes.

A proficient stenographer who had had cerebral metastases suffered from pure alexia for normal print but could still read stenography with ease. It is suggested that especially the visuospatial properties of stenography made possible "alternative" reading, most likely via the right hemisphere.

Four legs. Illusory reduplication of the lower limbs after bilateral parietal lobe damage.

OBJECTIVE: To report an unusual disorder of body schema and its neurologic and neuropsychological correlates. DESIGN AND METHODS: We describe a patient with a reduplicative phantom illusion of her lower limbs. Motor and sensory functions, as well as mental representation of body and space, were studied during the reduplication experience until its resolution. SETTING: Clinical neurology department in a primary care hospital. PATIENT: A 64-year-old, left-handed woman who experienced the uncontrollable and distressing feeling of having 4 legs, without delusional belief, after surgical removal of a right-predominant parasagittal parietal meningioma. This phenomenon spontaneously resolved after 2 weeks. INTERVENTION: None. MAIN OUTCOME MEASURES: Clinical neurologic examinations and standardized neuropsychological tests, with emphasis on tests assessing orientation to body parts, right-left discrimination, and mental orientation in space. RESULTS: The patient had severe weakness and proprioceptive sensory loss in both lower limbs. She had no disturbances of body schema knowledge but a striking impairment in tasks requiring mental orientation in space, particularly for right-left laterality discrimination. Resolution of the reduplication experience correlated with improvement in the affected spatial abilities, while motor, sensory, and other cognitive functioning did not significantly change. CONCLUSION: This patient's reduplicative phantom illusion might be related to the combination of the severe somatosensory loss with an underlying impaired mental representation of relative positions in space.

Dissociation of color from object in amnesia.

A patient with persistent amnesia after bilateral medial temporal and left inferotemporo-occipital infarction could not match colors to objects in verbal, visual, or visuoverbal tasks. A severe naming disorder for objects was present but the patient could name colors and point to colors whose name was given. The matching disorder appeared to be a deficit in the semantic classification of objects rather than a problem in identifying colors.

Loss of topographic familiarity. An environmental agnosia.

Sixteen patients manifested the syndrome of loss of environmental familiarity. The syndrome is characterized by an inability to recognize familiar surroundings in spite of relatively intact verbal memory, cognition, and perception. In addition to the loss of environmental familiarity, other clinical disturbances, including central achromatopsia, prosopagnosia, palinopsia, visual hallucinations, dressing disturbances, or impaired revisualization, were present in several cases. Radiologic studies revealed that all patients had right medial temporo-occipital lesions; three had additional left-sided lesions. Clinical observations suggest that the syndrome is a class-specific agnosia similar to prosopagnosia.

Corticosteroid-responsive postmalaria encephalopathy characterized by motor aphasia, myoclonus, and postural tremor.

OBJECTIVES: To study the clinical spectrum of an acute severe encephalopathy occurring in 2 patients after recovery from falciparum malaria infection and to compare it with the reported clinical features of the postmalaria neurological syndrome. DESIGN: Case report. SETTING: Tertiary care hospital. PATIENTS: Two patients presented with acute onset of fluctuating motor aphasia, severe generalized myoclonus, and postural tremor. Additional signs were cerebellar ataxia, and in 1 patient, generalized epileptic seizures. Magnetic resonance imaging of the brain revealed patchy white matter lesions in 1 patient. Clinically, the patients' conditions continued to worsen until corticosteroids were introduced, the use of which induced a rapid, albeit incomplete, recovery. CONCLUSIONS: We describe a new, severe variant of the still poorly defined postmalaria neurological syndrome. We propose a preliminary classification of this syndrome, according to its clinical characteristics, as follows: a mild or localized form, characterized by isolated cerebellar ataxia or postural tremor; a diffuse, but relatively mild encephalopathic form, characterized by acute confusion or epileptic seizures; and a severe, corticosteroid-responsive encephalopathy that is characterized by motor aphasia, generalized myoclonus, postural tremor, and cerebellar ataxia.

Lupus erythematosus and Miller-Fisher syndrome.

OBJECTIVE: To compare the clinical course of an unusual case of Miller-Fisher syndrome in systemic lupus erythematosus with therapeutic interventions, in particular with plasma exchanges. DESIGN: The clinical state and laboratory and electrophysiologic parameters were controlled for over a year and related to therapeutic attempts with immunoglobulins, steroids, and plasma exchanges. SETTING: Medical intensive care unit of a university hospital. PATIENT: A 17-year-old black female student with known systemic lupus erythematosus who developed ataxia, are flexia, and ophthalmoplegia (Miller-Fisher syndrome) and later became tetraplegic and required full mechanical ventilatory support. RESULTS: High-dose immunoglobulin treatment combined with corticosteroid pulse therapy was not beneficial. However, plasma exchange (performed five times over a period of 4 months) was followed by a striking clinical improvement within hours after each plasma exchange. CONCLUSIONS: Plasma exchange appears to remove a yet unknown agent producing a distal motor nerve conduction block and is efficacious in severe neuropathy associated with Miller-Fisher syndrome in lupus erythematosus.

"Gourmand syndrome": eating passion associated with right anterior lesions.

We present a new benign eating disorder associated with lesions involving parts of the right anterior cerebral hemisphere. This "gourmand" syndrome describes a preoccupation with food and a preference for fine eating. Two exemplary case reports illustrate this new syndrome. Analysis of the clinical and anatomical data of 36 patients who displayed this behavior revealed, in 34, a strong association with lesion location in the right anterior part of the brain involving cortical areas, basal ganglia, or limbic structures. Our finding provides further evidence of a correlation between right hemispheric damage, eating, and other impulse control disorders. We conjecture that the serotonergic system subserves different functions in the left and right hemisphere.

Persistent cognitive impairment in climbers after repeated exposure to extreme altitude.

We performed neuropsychological testing in eight world class climbers who had reached summits higher than 8,500 meters without supplementary oxygen. Five had mildly impaired concentration, short-term memory, and ability to shift concepts and control errors. There were no defects in perception or other cognitive activities. The pattern of impairment suggests malfunctioning of bifronto-temporo-limbic structures. Repeated extreme-altitude exposure can cause mild but persistent cognitive impairment.