Cerebral Palsy

INTRODUCTION: Studies of developmental deficits in face recognition, or developmental prosopagnosia, have shown that individuals who have not suffered brain damage can show face recognition impairments coupled with normal object recognition (Duchaine and Nakayama, 2005; Duchaine et al., 2006; Nunn et al., 2001). However, no developmental cases with the opposite dissociation – normal face recognition with impaired object recognition – have been reported. The existence of a case of non-face developmental visual agnosia would indicate that the development of normal face recognition mechanisms does not rely on the development of normal object recognition mechanisms. METHODS: To see whether a developmental variant of non-face visual object agnosia exists, we conducted a series of web-based object and face recognition tests to screen for individuals showing object recognition memory impairments but not face recognition impairments. Through this screening process, we identified AW, an otherwise normal 19-year-old female, who was then tested in the lab on face and object recognition tests. RESULTS: AW’s performance was impaired in within-class visual recognition memory across six different visual categories (guns, horses, scenes, tools, doors, and cars). In contrast, she scored normally on seven tests of face recognition, tests of memory for two other object categories (houses and glasses), and tests of recall memory for visual shapes. Testing confirmed that her impairment was not related to a general deficit in lower-level perception, object perception, basic-level recognition, or memory. DISCUSSION: AW’s results provide the first neuropsychological evidence that recognition memory for non-face visual object categories can be selectively impaired in individuals without brain damage or other memory impairment. These results indicate that the development of recognition memory for faces does not depend on intact object recognition memory and provide further evidence for category-specific dissociations in visual recognition. Copyright © 2010 Elsevier Srl. All rights reserved.

Damage to human neurological system cells resulting from exposure to mycotoxins confirms a previously controversial public health threat for occupants of water-damaged buildings. Leading scientific organizations disagree about the ability of inhaled mycotoxins in the indoor environment to cause adverse human health effects. Damage to the neurological system can result from exposure to trichothecene mycotoxins in the indoor environment. This study demonstrates that neurological system cell damage can occur from satratoxin H exposure to neurological cells at exposure levels that can be found in water-damaged buildings contaminated with fungal growth. The constant activation of inflammatory and apoptotic pathways at low levels of exposure in human brain capillary endothelial cells, astrocytes, and neural progenitor cells may amplify devastation to neurological tissues and lead to neurological system cell damage from indirect events triggered by the presence of trichothecenes.

Abstract We report the case of a 30-year-old woman with delayed postanoxic encephalopathy and visual field disturbance caused by strangulation. Although she was normal up to the sixth day after strangulation,she developed quadrantic hemianopia on the seventh day. The results of magnetic resonance imaging (MRI) showed high-intensity T2 and fluid-attenuated inversion recovery (FLAIR) signals in the bilateral striatum; on the basis of these findings,she was diagnosed with delayed postanoxic encephalopathy. Associated with quadrantic hemianopia,an area with low-intensity FLAIR signals was noted in the subcortical region of the right occipital cortex. Single-photon emission computed tomography (SPECT) revealed decreased blood flow in the right occipital lobe and striatum. By day 41 after strangulation,the low-intensity area in the subcortical region of the right occipital cortex disappeared,and high-intensity FLAIR signals were observed in the right occipital cortex. The quadrantic hemianopia and occipital lesion that were revealed by MRI regressed 4 months later. Respiratory dysfunction or circulatory dysfunction causes ischemia of the entire brain; however,strangulation does not lead to disturbances in the blood flow in the regions supplied by the vertebrobasilar artery. However,in the case of the present patient,a lesion was noted in the occipital lobe after strangulation. It has been reported that the autonomic control in the vertebrobasilar artery is weak,and the control of blood pressure in this artery is limited. In the case of this patient,not only the ischemia resulting from the stricture of the artery and the trachea,but also congestion resulting from disturbances in the venous blood flow might be associated with the brain damage and might have thus led to the development of the occipital lesion. (Received: November 17,2009,Accepted: February 12,2010).

Abstract The revised act on organ transplantation was passed in July 2009; it will be implemented in July 2010. This law allows organ donation from a brain-dead person with or without an Organ Donation Decision Card,if the family members permit this donation and the brain-dead person had not decided against it when conscious. In Japan,the legal definition of human death has not changed after the revision of the act on organ transplantation; therefore,brain death is considered human death for the purpose of organ transplantation. Certain minor revisions are required in the medical and legal criteria for diagnosis of brain death,especially for children under 6 years. Ancillary tests,including radioisotope measurement of cerebral blood flow and auditory brainstem evoked potentials,should be considered for younger children or in cases where comprehensive neurological examination was not possible because of injuries to the face,eyes,or ears. An increase in the number of organ donations from brain-dead persons because of the revised act will greatly increase the responsibility of and burden on hospitals and neurosurgeons that treat many emergency cases of severe brain injury and stroke. Adequate socioeconomic and systemic medical support should be provided to hospitals where organ donation is carried out.

Neuroimaging studies of cognitive control have identified two distinct networks with dissociable resting state connectivity patterns. This study, in patients with heterogeneous damage to these networks, demonstrates network independence through a double dissociation of lesion location on two different measures of network integrity: functional correlations among network nodes and within-node graph theory network properties. The degree of network damage correlates with a decrease in functional connectivity within that network while sparing the nonlesioned network. Graph theory properties of intact nodes within the damaged network show evidence of dysfunction compared with the undamaged network. The effect of anatomical damage thus extends beyond the lesioned area, but remains within the bounds of the existing network connections. Together this evidence suggests that networks defined by their role in cognitive control processes exhibit independence in resting data.

The histopathology of clinical isolates of Scedoporium apiospermum, S. boydii and S. aurantiacum, was evaluated in immunosuppressed mice. The organs most affected were brain, kidneys and spleen. S. aurantiacum produced more tissue damage than the other two species. AMB was ineffective in the treatment of murine infections caused by such isolates and posaconazole and voriconazole showed efficacy that correlated with the in vitro susceptibility data.

Chromium ions are frequently found in aquatic ecosystems and are known to be inducers of oxidative stress in fish solid tissues. The present study was designed to determine whether fish blood samples can be used to allow nonlethal diagnostic testing for chromium intoxication. First, we confirmed that 96h exposures to water containing 10.0mgL(-1) chromium ions, either Cr(3+) or Cr(6+), induced oxidative stress in brain of goldfish (Carassius auratus). Multiple blood parameters were then evaluated. Cr(6+) exposure triggered a 579% increase in the number of erythrocytes containing micronuclei, a frequently used marker of cellular toxicity. Leucocyte numbers were also perturbed by exposure to either Cr(3+) or Cr(6+) indicating that chromium ions could impair the immune system as well. The content of protein carbonyl groups, a marker of oxidative damage to proteins, was enhanced in fish plasma by exposure to either chromium ion and activities of catalase and lactate dehydrogenase also were affected. The data demonstrate that chromium ions induced oxidative stress in goldfish blood and were cytotoxic for erythrocytes. This indicates that analysis of plasma can be used as a good early nonlethal diagnostic marker of fish intoxication by transition metal ions. Copyright © 2010. Published by Elsevier Ltd.

Brevetoxins are persistent, bioaccumulative, lipophilic polyether neurotoxins synthesized by Karenia brevis, a harmful algal bloom (HAB) dinoflagellate. Although some marine organisms accumulate potentially harmful levels of brevetoxins, little is known about neurotoxic effects in wild populations. Here, tissue (i.e., liver, kidney, muscle, intestine, gill, brain) brevetoxin levels (as ng PbTx-3 eq/g) and four neurochemical biomarkers (monoamine oxidase, MAO; cholinesterase, ChE; muscarinic cholinergic receptor, mAChR; N-methyl-d-aspartic acid receptor, NMDAR) were compared between eleven lemon sharks collected during a K. brevis bloom and eighteen lemon sharks not exposed to a bloom (controls) in a case-control manner. Brevetoxin levels in tissues were significantly higher in HAB-exposed sharks when compared to controls, and tissue levels (e.g., 277-3112ng/g in livers, 429-2833ng/g in gills) in HAB-exposed sharks were comparable to levels detected in a shark (e.g., 1223ng/g in liver, 930ng/g in gill) that died presumably of toxin exposure. Further, there were significant correlations between brain brevetoxin levels and ChE activity (r=-0.41; p<0.05), MAO activity (r=-0.37; p<0.05), mAChR levels (r=0.55; p<0.01), and NMDAR levels (r=-0.49; p<0.01). There were no relationships between neurochemical biomarkers and metals (total mercury, methylmercury, selenium). Overall, these results in tissues from free-ranging lemon sharks indicate that ecologically relevant exposures to brevetoxins may cause significant changes in brain neurochemistry. As disruptions to neurochemistry precede structural and functional damage to the nervous system, these results suggest that relevant exposures to HABs may be causing sub-clinical effects in lemon sharks and raise further questions about the ecological and physiological impacts of HABs on marine biota. Copyright © 2010 Elsevier B.V. All rights reserved.

Traumatic brain injury (TBI) is an acute event resulting from external force to the brain and is a major cause of death and disability associated with high health care costs in the western world. Additional injuries, originating from the secondary molecular events after the initial intensive care, may be limited by the use of objective biomarkers to provide the best treatment and patient prediction outcome. In this study, hexapeptide ligand libraries (HLL) have been used for the enrichment of suggested protein biomarkers for TBI in cerebrospinal fluid (CSF). HLL have the potential to enrich low abundant proteins and simultaneously reduce the high abundant proteins, rendering a sample with significantly reduced dynamic range. The CSF proteome from two TBI inflicted patients have been extensively mapped using a large initial sample volume obtained by extraventricular drainage. Shotgun proteomics, in combination with isoelectric focusing (IEF) and nano-LC-MS/MS, identified 339 unique proteins (MudPIT scoring p</=0.05) with a protein overlap of 130 between the patients. As much as 45% of the proteins reported in the literature to be associated with degenerative/regenerative processes occurring after a trauma to the head were identified. Out of the most prominent potential protein biomarkers, such as neuron specific enolase, glial fibrillary acidic protein, myelin basic protein, creatine kinase B-type and S-100beta, all except myelin basic protein were detected in the study. This study shows the possibility of using HLL as a tool for screening of low abundant protein biomarkers in human CSF. Copyright © 2010 Elsevier B.V. All rights reserved.

Although normalization of brain images is critical to the analysis of structural damage across individuals, loss of tissue due to focal lesions presents challenges to the available normalization algorithms. Until recently, cost function masking, as advocated by Brett and colleagues (2001), was the accepted method to overcome difficulties encountered when normalizing damaged brains; however, development of the unified segmentation approach for normalization in SPM5 (Ashburner & Friston, 2005) offered an alternative. Crinion et al. (2007) demonstrated this approach produced normalization results without cost function masking that appeared to be robust to lesion effects when tested using the same simulated lesions studied by Brett et al. (2001). The present study sought to confirm the validity of this approach in brains with focal damage due to vascular events. To do so, we examined outcomes of normalization using unified segmentation with and without cost function masking in 49 brain images with chronic stroke. Lesion masks were created using two approaches (precise and rough drawings of lesion boundaries), and normalization was implemented with both smoothed and unsmoothed versions of the masks. We found that failure to employ cost function masking produced less accurate results in real and simulated lesions, compared to masked normalization, both in terms of deformation field displacement and voxelwise intensity differences. Additionally, unmasked normalization led to significant underestimation of lesion volume relative to all four masking conditions, especially in patients with large lesions. Taken together, these findings suggest cost function masking is still necessary when normalizing brain images with chronic infarcts. Copyright © 2010. Published by Elsevier Inc.