1/44. Impairment of social and moral behavior related to early damage in human prefrontal cortex.The long-term consequences of early prefrontal cortex lesions occurring before 16 months were investigated in two adults. As is the case when such damage occurs in adulthood, the two early-onset patients had severely impaired social behavior despite normal basic cognitive abilities, and showed insensitivity to future consequences of decisions, defective autonomic responses to punishment contingencies and failure to respond to behavioral interventions. Unlike adult-onset patients, however, the two patients had defective social and moral reasoning, suggesting that the acquisition of complex social conventions and moral rules had been impaired. Thus early-onset prefrontal damage resulted in a syndrome resembling psychopathy.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/44. Ethical issues in clinical practice: cases and analyses.Given the severity and complexity of the neurologic disorders that can affect infants and children, pediatric neurologists frequently encounter complex and challenging ethical issues. This discussion uses five cases to illustrate common ethical issues and dilemmas that are confronted by pediatric neurologists. These cases involve the provision of artificial nutrition and hydration to patients in vegetative or minimally conscious states, the use of mechanical ventilation for patients with spinal muscular atrophy, controversial therapies for autistic spectrum disorders, the use of medical information obtained over the internet, and genetic testing in children with suspected Huntington's disease. The ethical concepts discussed in these cases include autonomy, confidentiality, futility, surrogate decision-making, the best-interest principle, and the withholding and withdrawal of treatment.- - - - - - - - - - ranking = 2keywords = complex (Clic here for more details about this article) |
3/44. Lack of neuronal damage in atypical absence status epilepticus.PURPOSE: Whether status epilepticus of nonconvulsive epileptic seizures is harmful still remains controversial. To investigate this, the presence and/or extent of neuronal damage in patients with absence status epilepticus (ASE) and patients with complex partial status epilepticus (CPSE) was examined and compared. methods: Neuron-specific enolase (NSE) in CSF was examined in the patients with ASE and compared with that of the patients having CPSE. Clinical aspects of these patients also were investigated. RESULTS: CSF NSE levels in ASE patients were lower than those of CPSE patients and were considered as the normal values. No clinical symptoms indicated neuronal damage in the ASE patients. CONCLUSIONS: This study suggests that ASE does not induce neuronal damage. serum NSE is not always correlated to CSF NSE, and determination of serum NSE levels may be an inappropriate method of estimating neuronal damage in some cases of ASE.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
4/44. Epileptic spasms in clusters without hypsarrhythmia in infancy.Spasms are defined as epileptic seizures characterized by brief axial contraction, in flexion, extension or mixed, symmetric or asymmetric, lasting from a fraction of a second to 1-2s, and are associated with a slow-wave transient or sharp and slow-wave complex, followed or not by voltage attenuation. Epileptic spasms usually appear in clusters and are age-dependent. This type of epileptic spasms associated with the particular EEG pattern, hypsar rhythmia, constitutes the basis for the diagnosis of West syndrome. The question is, how to nosologically define those patients who clearly present epileptic spasms in clusters without modified or typical hypsarrhythmia and with or without focal paroxysmal discharges on the interictal EEG. In the present series, the four patients show that epileptic spasms in clusters may occur in infancy, without hypsarrhythmia. They all presented the following features: normal neuropsychological development before onset of epileptic spasms, clusters of epileptic spasms, focal clinical and/or EEG abnormalities, normal neuroradiological imaging, neurometabolic investigations and karyotypes. In three of the patients, seizures were refractory to AEDs. Epileptic spasms in clusters without hypsarrhythmia that start in the first year of life represent a subtype of infantile spasms that generally are refractory to AEDs. It is not yet clear whether it should be considered as a variant of West syndrome or not [Published with Video sequence].- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
5/44. From what to where: neuropsychological evidence for implicit interactions between object- and space- based attention.Three experiments examined nonspatial extinction in G.K., a patient with bilateral parietal damage. Experiment 1 demonstrated nonspatial extinction (poor detection of a weak relative to a stronger perceptual group), even when the stronger group was less complex than the weaker group. Experiment 2 showed improved report of a letter falling at the location of the stronger group, but explicit judgments of the location of the letter were at chance. Experiment 3 replicated the object-cuing benefit, though G.K. could not discriminate whether a letter fell at the same location as the stronger perceptual group. The data indicate coupling between object- and space-based attention, so that spatial attention is drawn to the location occupied by the winner of object-based competition for selection. In this case, what cues where. This coupling operates implicitly, even when explicit location judgments are impaired.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
6/44. hyperammonemia with complex urinary tract anomaly: a case report.hyperammonemia has been reported rarely in the pediatric age group in systemically ill patients. All cases resulted from infections with urea splitting organisms, which are more common among patients who have undergone surgical procedures on the urinary tract. The authors report for the first time in the pediatric literature, one patient who presented with hyperammonemic encephalopathy that resulted from urinary tract infection with staphylococcus epidermidis and corynebacterium sp.- - - - - - - - - - ranking = 4keywords = complex (Clic here for more details about this article) |
7/44. Seeing without the occipito-parietal cortex: Simultagnosia as a shrinkage of the attentional visual field.Following bi-parietal lesions patient AT showed a severe inability to relocate her attention within a visual field which perimetry proved to be near-normal. An experimental approach with tasks testing visuo-spatial attention demonstrated a shrinkage of A.T.'s attentional visual field. With her visual attention narrowed to a kind of functional tunnel vision, the patient exhibited simultanagnosia (Wolpert, 1924), a symptom previously described in 1909 by Balint under the label of Psychic paralysis of "Gaze". In striking contrast AT showed an efficient and effortless perception of complex natural scenes, which, according to recent work in normal subjects, necessitate few if any attentional resources.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
8/44. Naming impairments following recovery from herpes simplex encephalitis: category-specific?An apparently clear case of category-specific naming impairment selectively affecting animals was detected in a patient who had recovered from herpes simplex encephalitis. However, subsequent investigation demonstrated that these category-specific effects could be eliminated by controlling simultaneously for three factors in picture naming: word frequency, concept familiarity, and visual complexity. The results emphasize the importance of controlling for all factors pertinent to picture naming when attempting to demonstrate category specificity in picture naming. Further testing indicated that deficits were also apparent when naming to definition was required, and some impairment in the ability to answer questions about objects and living things was also noted. Theoretical implications of these data are considered.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
9/44. Developmental plasticity after right hemispherectomy in an epileptic adolescent with early brain injury.OBJECTIVES: The authors present the case of an adolescent affected with refractory epilepsy due to a neonatal ischemic infarction of the right medial cerebral artery. Hemiplegic since the first months of life, she began presenting motor partial seizures associated with drop attacks at 4.5 years; these were initially well controlled by antiepileptic drugs, but at 10 years seizures appeared again and became refractory. Thus, at 14 years and 10 months, she was submitted to a right hemispherectomy that made her rapidly seizure free. In the post-surgical follow-up lasting 5 years, neuropsychological serial assessments showed an impressive progressive improvement of cognitive skills, namely, visuospatial abilities. This case seems to challenge the widely spread feeling that functional catch-up in brain-injured children could only occur early in life. In effect, the astonishing recovery especially of visuospatial skills in our case occurred in adolescence after a late surgical intervention of right hemispherectomy. methods: Different neuropsychological aspects are discussed. The reorganisation process recovered the spatial and linguistic abilities as well as the verbal and visuospatial memory; however, there was a persistent impairment of complex spatial and perceptual skills as well as recall abilities. Despite the deficit of complex visual stimuli processing, the patient showed a good performance in the recognition of unknown faces. CONCLUSIONS: Probably, the absence of seizures in the first 4 years of life could have allowed a generally adequate compensatory reorganisation, successively masked by the persistent and diffuse epileptic disorder. The seizure control produced by surgery eventually made evident the effectiveness of the brain reorganisation.- - - - - - - - - - ranking = 2keywords = complex (Clic here for more details about this article) |
10/44. The fusiform face area is not sufficient for face recognition: evidence from a patient with dense prosopagnosia and no occipital face area.We tested functional activation for faces in patient D.F., who following acquired brain damage has a profound deficit in object recognition based on form (visual form agnosia) and also prosopagnosia that is undocumented to date. Functional imaging demonstrated that like our control observers, D.F. shows significantly more activation when passively viewing face compared to scene images in an area that is consistent with the fusiform face area (FFA) (p < 0.01). Control observers also show occipital face area (OFA) activation; however, whereas D.F.'s lesions appear to overlap the OFA bilaterally. We asked, given that D.F. shows FFA activation for faces, to what extent is she able to recognize faces? D.F. demonstrated a severe impairment in higher level face processing--she could not recognize face identity, gender or emotional expression. In contrast, she performed relatively normally on many face categorization tasks. D.F. can differentiate faces from non-faces given sufficient texture information and processing time, and she can do this is independent of color and illumination information. D.F. can use configural information for categorizing faces when they are presented in an upright but not a sideways orientation and given that she also cannot discriminate half-faces she may rely on a spatially symmetric feature arrangement. Faces appear to be a unique category, which she can classify even when she has no advance knowledge that she will be shown face images. Together, these imaging and behavioral data support the importance of the integrity of a complex network of regions for face identification, including more than just the FFA--in particular the OFA, a region believed to be associated with low-level processing.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
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