[Infantile acquired brain injury, a personal experience. Demands from the socio-familial point of view]. / Daño cerebral sobrevenido infantil, una experiencia personal. Reclamaciones desde el punto de vista sociofamiliar.

We report on the experience of a family in which the youngest child has acquired brain injury and the struggle undertaken by the family to improve the neurorehabilitation resources in the public health service. The article outlines the main demands, from the socio-familial point of view, as regards the improvement of neurological rehabilitation and the resources needed to deliver it.

TITLE: Daño cerebral sobrevenido infantil, una experiencia personal. Reclamaciones desde el punto de vista sociofamiliar.Se describe la experiencia de una familia en la que el hijo menor tiene daño cerebral sobrevenido y la lucha emprendida por la familia para mejorar los recursos neurorrehabilitadores de la sanidad publica. Se recogen las principales reclamaciones, desde el punto de vista sociofamiliar, en cuanto a la mejora en la atencion neurorrehabilitadora y los recursos necesarios.

Biomusic: a novel technology for revealing the personhood of people with profound multiple disabilities.

It is often difficult for family members and caregivers to interact with persons with profound multiple disabilities (PMD) because of the severely compromised communicative repertoire of this population. The resulting communication challenges may limit the ability of others to perceive personhood in individuals with PMD. This preliminary study investigated the effects of music generated in real time from physiological signals (biomusic) on caregiver perceptions of their interactions with persons with PMD. Caregivers (n = 10; parents and clinical staff) engaged in four, 10-min interactions with a person with PMD (n = 3; diagnoses = traumatic brain injury, pervasive developmental disorder, hypoxic brain injury), whose biomusic was projected throughout. Caregivers participated in two open-ended, semi-structured interviews to explore the effect of biomusic on these interactions. Most caregiver responses to biomusic were very positive, and many reported that biomusic caused an improvement in their interaction with and perceptions of the person with PMD. By providing audible evidence of the changing physiological state of persons with PMD, biomusic may enhance the perceived personhood of these individuals and enrich interactions with their family members and caregivers.

Hemispheric competence for auditory spatial representation.

Sound localization relies on the analysis of interaural time and intensity differences, as well as attenuation patterns by the outer ear. We investigated the relative contributions of interaural time and intensity difference cues to sound localization by testing 60 healthy subjects: 25 with focal left and 25 with focal right hemispheric brain damage. Group and single-case behavioural analyses, as well as anatomo-clinical correlations, confirmed that deficits were more frequent and much more severe after right than left hemispheric lesions and for the processing of interaural time than intensity difference cues. For spatial processing based on interaural time difference cues, different error types were evident in the individual data. Deficits in discriminating between neighbouring positions occurred in both hemispaces after focal right hemispheric brain damage, but were restricted to the contralesional hemispace after focal left hemispheric brain damage. Alloacusis (perceptual shifts across the midline) occurred only after focal right hemispheric brain damage and was associated with minor or severe deficits in position discrimination. During spatial processing based on interaural intensity cues, deficits were less severe in the right hemispheric brain damage than left hemispheric brain damage group and no alloacusis occurred. These results, matched to anatomical data, suggest the existence of a binaural sound localization system predominantly based on interaural time difference cues and primarily supported by the right hemisphere. More generally, our data suggest that two distinct mechanisms contribute to: (i) the precise computation of spatial coordinates allowing spatial comparison within the contralateral hemispace for the left hemisphere and the whole space for the right hemisphere; and (ii) the building up of global auditory spatial representations in right temporo-parietal cortices.

Crossmodal integration of object features: voxel-based correlations in brain-damaged patients.

How does the brain bind together the different sensory features of objects to form meaningful, multimodal object representations? Human functional imaging findings implicate the left posterior superior temporal sulcus/middle temporal gyrus (pSTS/MTG) in crossmodal integration, while animal ablation findings support a hierarchical object processing model in which outputs from each sensory stream are integrated in perirhinal cortex (PRc) of the anteromedial temporal lobe. To determine which neural regions are necessary for integrating audiovisual object features, and which regions are necessary for understanding the meaning of crossmodal objects, we administered crossmodal (audio-visual) and unimodal (auditory, visual) integration tasks to 16 brain-damaged patients. We correlated patients' behavioural performance with measures of neural integrity (signal intensity) of each voxel across the brains of each patient. The integrity of bilateral anteromedial and temporopolar regions, but not pSTS/MTG, was significantly correlated with poorer crossmodal compared with unimodal integration performance, and with meaningful aspects of crossmodal integration. Additional analyses confirmed the negative crossmodal integration findings in the pSTS/MTG: performance on a sentence-picture matching control task was significantly correlated with MTG/STG voxel signal intensities, suggesting that a truncated range of signals in this region could not have been responsible for the lack of a significant correlation between integrity and crossmodal integration performance, and individual analyses of three patients with lesions in pSTS/MTG but spared anteromedial temporal cortex revealed equivalent unimodal and crossmodal integration performance. These results extend findings from the non-human primate literature into the human domain by demonstrating that anteromedial temporal cortex is critically involved in crossmodal integration of object features. However, pSTS/MTG appears to play a supportive but non-essential role during crossmodal integration. Taken together, the present findings are consistent with a neurocognitive account of object representations which claims that anteromedial temporal lobe is critically involved in the formation and processing of complex, multimodal object representations.

EPS Mid-Career Award 2006. Understanding anterograde amnesia: disconnections and hidden lesions.

Three emerging strands of evidence are helping to resolve the causes of the anterograde amnesia associated with damage to the diencephalon. First, new anatomical studies have refined our understanding of the links between diencephalic and temporal brain regions associated with amnesia. These studies direct attention to the limited numbers of routes linking the two regions. Second, neuropsychological studies of patients with colloid cysts confirm the importance of at least one of these routes, the fornix, for episodic memory. By combining these anatomical and neuropsychological data strong evidence emerges for the view that damage to hippocampal-mammillary body-anterior thalamic interactions is sufficient to induce amnesia. A third development is the possibility that the retrosplenial cortex provides an integrating link in this functional system. Furthermore, recent evidence indicates that the retrosplenial cortex may suffer "covert" pathology (i.e., it is functionally lesioned) following damage to the anterior thalamic nuclei or hippocampus. This shared indirect "lesion" effect on the retrosplenial cortex not only broadens our concept of the neural basis of amnesia but may also help to explain the many similarities between temporal lobe and diencephalic amnesia.

Structure and function in acquired prosopagnosia: lessons from a series of 10 patients with brain damage.

Acquired prosopagnosia varies in both behavioural manifestations and the location and extent of underlying lesions. We studied 10 patients with adult-onset lesions on a battery of face-processing tests. Using signal detection methods, we found that discriminative power for the familiarity of famous faces was most reduced by bilateral occipitotemporal lesions that involved the fusiform gyri, and better preserved with unilateral right-sided lesions. Tests of perception of facial structural configuration showed severe deficits with lesions that included the right fusiform gyrus, whether unilateral or bilateral. This deficit was most consistent for eye configuration, with some patients performing normally for mouth configuration. Patients with anterior temporal lesions had better configuration perception, though at least one patient showed a more subtle failure to integrate configural data from different facial regions. Facial imagery, an index of facial memories, was severely impaired by bilateral lesions that included the right anterior temporal lobe and marginally impaired by fusiform lesions alone; unilateral right fusiform lesions tended to spare imagery for facial features. These findings suggest that (I) prosopagnosia is more severe with bilateral than unilateral lesions, indicating a minor contribution of the left hemisphere to face recognition, (2) perception of facial configuration critically involves the right fusiform gyrus and (3) access to facial memories is most disrupted by bilateral lesions that also include the right anterior temporal lobe. This supports assertions that more apperceptive variants of prosopagnosia are linked to fusiform damage, whereas more associative variants are linked to anterior temporal damage. Next, we found that behavioural indices of covert recognition correlated with measures of overt familiarity, consistent with theories that covert behaviour emerges from the output of damaged neural networks, rather than alternative pathways. Finally, to probe the face specificity of the prosopagnosic defect, we tested recognition of fruits and vegetables: While face specificity was not found in most of our patients, the data of one patient suggested that this may be possible with more focal lesions of the right fusiform gyrus.

An evaluation of the paediatric acquired brain injury community outreach programme (PABICOP).

PRIMARY OBJECTIVE: To examine the utility of a coordinated, family/community-focused programme (PABICOP) vs. a standard approach for improving outcomes for children with ABI and their families. RESEARCH DESIGN: Pre-test-post-test design, with comparison group and follow-up. METHODS AND PROCEDURES: Ninety-six children (64 children receiving PABICOP services and 32 children receiving standard care) participated in the study. Measures were completed at baseline and 3 and 12 months later. MAIN OUTCOMES AND RESULTS: Parents/caregivers with more than 10 contacts with PABICOP scored significantly higher on an ABI knowledge quiz than either parents/caregivers with 10 contacts or less or the comparison group at post-test and follow-up. Parents/caregivers with 10 contacts or less with PABICOP reported significantly greater improvements in children's school and total competence on the CBCL than either parents/caregivers with more than 10 contacts or the comparison group at post-test and follow-up. CONCLUSIONS: PABICOP may be more useful for enhancing knowledge of ABI for parents/caregivers and for integrating children into the community over a 1-year period than a standard approach. The amount of service received appears to influence outcomes.

Psychoneuroimmunology of stroke.

There is now considerable evidence from both experimental and clinical studies that immune and inflammatory processes can contribute to the onset of stroke and the neurologic and psychologic outcomes. Several specific therapeutic targets have been identified that may significantly improve the devastating impact of stroke.

Keeping time: effects of focal frontal lesions.

This study examined the performance of 32 normal subjects and 39 patients with focal lesions of the frontal lobes on two simple timing tasks-responding in time with a tone that regularly repeated at a rate of once every 1.5s, and then maintaining the same regular response rhythm without any external stimulus. The hypothesis was that lesions to the right prefrontal cortex would disrupt timing performance. The two main findings were (1) an abnormally high variability in the timing performance (both self-timed and tone-timed) of patients with lesions to the right lateral frontal lobe, particularly involving Brodmann area 45 and subjacent regions of the basal ganglia; (2) an increase in the variability of timing performance as the task continued in patients with lesions to the superior medial regions of the frontal lobe. These findings indicate that the right lateral frontal lobe is crucially involved in the ongoing control of timed behavior, either because of its role in generating time intervals or in monitoring the passage of these intervals. In contrast, the superior medial regions of the frontal lobe are necessary to maintain consistent timing performance over prolonged periods of time.

Assessing a microswitch-based stimulation procedure for eye-blinking responses in a young woman with profound multiple disabilities.

We applied a new microswitch-based stimulation procedure for eye-blinking responses with a young woman with profound multiple disabilities, and compared effects of this procedure on the eye-blinking responses and smiling with the effects of a caregiver-based stimulation condition. Analysis showed that the microswitch-based stimulation procedure, with stimulation occurring contingent on eye-blinking responses, increased the frequencies of these responses significantly above the levels recorded in baseline and caregiver-based stimulation conditions. No changes in smiling frequencies occurred. Implications of the findings in terms of alertness, learning, and quality of life are discussed.

Thalassemia and learning: Neurocognitive functioning in children.

As more effective management and even cure of thalassemia become possible, attention is beginning to be directed to the potential neurologic and resulting neurocognitive effects of this illness on adults and children. Recent studies indicate that for adults with beta-thalassemia major and intermedia, and for children with sickle beta-thalassemia (Sbeta-thalassemia), there is a substantial risk for silent brain infarcts that may be associated with neurocognitive impairment similar to that reported for children with sickle cell anemia. Here the available literature in this area is reviewed and the limited outcomes are compared with those available from large, multicenter longitudinal studies of sickle cell anemia. On the basis of these comparisons, it is recommend that children with thalassemia be screened for specific neuropsychological impairments and that they be provided early intervention and special education access as available under the Individuals with Disabilities Education Act (IDEA) or the 504 Regulations of the Rehabilitation Act of 1973.

Acute barium nitrate intoxication treated by hemodialysis.

A 22-year-old male was admitted to hospital with diarrhea and vomiting, cardiac arrhythmias, severe hypokalemia and gradual onset of muscular weakness. A potassium infusion was started, but for several hours serum potassium remained low. Evidence of toxic ingestion was initially lacking. When it became clear -- after a considerable delay -- that the patient had ingested barium nitrate, hemodialysis was started. This resulted in rapid clinical improvement with correction of hypokalemia and restored muscular function. Intoxication with barium causes hypokalemia, arrhythmias, muscular weakness and paralysis, often requiring respiratory support. This patient presented with symptoms typical of severe barium intoxication, non-responsive to potassium supplementation. There are few published reports on the use of hemodialysis in barium poisoning. This case confirms the possible benefit of hemodialysis in severe cases, where potassium supplementation alone is insufficient.

The impact of orbital prefrontal cortex damage on emotional activation to unanticipated and anticipated acoustic startle stimuli.

Damage to the orbital prefrontal cortex has been implicated in selectively diminishing electrodermal autonomic nervous system responses to anticipated punishing stimuli (e.g., losing money; Bechara, Damasio, & Damasio, 2000), but not to unanticipated punishing stimuli (e.g., loud noises; Damasio, Tranel, & Damasio, 1990). We extended this research by examining the effects of orbitofrontal damage on emotional responses to unanticipated and anticipated acoustic startles and collecting a more extensive set of physiological measures, emotional facial behavior, and self-reported emotional experience. Consistent with previous research, patients showed intact physiology to an unanticipated startle but failed to show appropriate anticipatory cardiovascular responses (patients' heart rates decreased, controls' increased). In addition, patients displayed more surprise facial behavior and reported marginally more fear than did controls in response to the unanticipated startle. Thus, orbitofrontal damage may compromise the ability to anticipate physiologically the onset of aversive stimuli, despite intact or enhanced emotional responses when such stimuli occur unexpectedly.

The use of a skill-based activity in therapeutic induction.

This paper describes a hypnotherapeutic intervention for a brain damaged 36-year-old male who has suffered from asthma since infancy and seizure disorder from the age of eight. In early sessions it was discovered that conventional "passive-relaxation" induction techniques seemed to exacerbate certain disturbing somatic experiences, which he refers to as scary feelings. It was found that his performance of a previously learned skilled activity (the playing of the computer game Tetris) permitted the experience of a highly focused but relaxed state that was conducive to therapeutic interaction. This approach to induction bears similarity to "active-alert" procedures but may be more importantly related to Mihaly Csikszentmihalyi's principle of flow, in that it involves engagement in a subjectively meaningful, skill-based activity.

The neurodevelopmental price of survival in children with malignant brain tumours.

Increasing survival rates in malignant brain tumors treatment have directed attention to the side effects of long-term disease control. Nevertheless, although the treatment protocols are continuously remodelled, the quality of life of children surviving for a long time is still poor. The most severe sequelae are neurocognitive disorders, which are associated with neurobehavioural alterations. The last are partly derived directly from the lesion localisation and treatments, but are often reinforced by academic and social failure. The deleterious effect of radiotherapy (CRT) is very well documented and confirmed in all the studies. The radiation dose delivered according to the age has reduced, but not fully eliminated, the negative influence on mental functioning. Also the CRT hyperfractionation has reduced, but not cancelled, this cognitive negative impact. Intrathecal methotrexate per se is responsible for a severe cognitive impairment, which can be even more severe in association to CRT. Some surgical approaches have been responsible for postoperative behavioural disturbances. Serial neuropsychological and behavioural evaluations, which should also include the survivors' own perception of their quality of life, are badly needed. The results of these evaluations should be covariate with several factors (age, type of surgery, lesion site, hydrocephalus, complementary therapies) in an attempt to define interdisciplinary treatment protocols to maximise survival while minimising cognitive/behavioural deficits.

Paroxetine versus citalopram treatment of pathological crying after brain injury.

Selective serotonin reuptake inhibitors (SSRIs) are the treatment of choice for pathological crying after brain injury, independent of accompanying depression. In a series of 26 consecutive patients with acquired brain damage and episodes of involuntary crying, the efficacy and tolerability of paroxetine and citalopram were compared. The severity of pathological crying or laughing was rated based on clinical interviews with symptom provocation. The first 13 patients were treated with paroxetine and another 13 patients received citalopram in single daily doses of 10 to 40 mg. Rapid onset (within 1-3 days) and highly significant (p < 0.001) improvements of emotionalism were observed after both paroxetine and citalopram. There were no efficacy differences, despite the longer symptom duration in the citalopram group. The only adverse effect after paroxetine was nausea, which was reversible, in two patients, and nausea with vomiting in another two patients, who were switched to citalopram. Citalopram was tolerated without adverse effects.

Association learning in the acute confusional state.

The usefulness of cognitive rehabilitative treatment in the acute stages after brain injury seems questionable because patients in severe acute confusional state early after coma clinically seem unable to learn and store new information. Therefore, the capability of patients in acute confusional state to learn and retain associative information was assessed. On two occasions pairs of simple nouns were presented to six patients in severe acute confusional state. Stimuli were presented repeatedly either in written form only or with additional pictorial representations. Immediate and 20 minutes delayed recall was measured. Patients in acute confusional state were able to learn progressively more word pairs across several presentations. They retained some information over an interval of 20 minutes. In addition, they learned and remembered pictorially supported associations better than pure verbal associations. Patients in severe acute confusional state may retain some explicit information and may profit from an imagery mnemonic aid. These results were not expected on the basis of clinical findings alone and they have potential implications for the care of patients in acute confusional state.

Somatosensory stimulation improves imagery disorders in neglect.

A group of 9 right brain damaged patients with unilateral neglect performed a set of tasks involving mental imagery with and without a transcutaneous electrical neural stimulation (TENS) to both sides of the neck. Results showed that TENS on the left side of the neck produced significant improvements of performances on the left side of mental representations of objects (drawing, shape comparison) as well as on left side of mental images of space (description of squares). The results suggest that the modification induced by TENS may affect the imagery systems involved in objects as well as in space representation.

Ginseng improves strategic learning by normal and brain-damaged rats.

Adult rats were prepared with either sham or medial prefrontal cortex lesions and administered, beginning on the third post-operative day, either, 0, 40, or 80 mg kg-1 crude ginseng extract suspended in saline daily for the next 30 days. Later, kinetic functions were evaluated on an elevated rotating rod. No long-term influences of the treatments were observed on this task. Significant positive influences of ginseng were observed in the position reversal task. The learning deficits observed in the saline control brain-damaged rats were significantly attenuated in the ginseng-treated animals. An analysis of trial 2 response accuracy across reversals revealed enhanced cognitive abilities (i.e. acquisition of a win-stay, lose-shift strategy) in both the brain damaged and sham control rats administered ginseng. Generally, administration of the higher dose resulted in better performance in the learning paradigm. The exact mechanism responsible for these promising results remains to be discovered. Several possible mechanisms are discussed.