Neurological Impairment (neurological + impairment)

Distribution by Scientific Domains
Distribution within Medical Sciences

Kinds of Neurological Impairment

  • severe neurological impairment

  • Selected Abstracts

    EFNS guidelines on management of neurological problems in liver transplantation

    M. Guarino
    Neurological impairment after orthotopic liver transplantation (OLT) is common and represents a major source of morbidity and mortality. The diagnosis and management of neurological problems occurring after OLT are difficult and evidence-based guidelines for this task are currently lacking. A Task Force was set up under the auspices of the European Federation of Neurological Societies to devise guidelines to prevent and manage neurological problems in OLT. We selected six major neurological problems and approached them combining an evidence-based scientific literature analysis with a search of consensus by means of a Delphi process. Search results were translated into a series of recommendations constituting a basis for better care of patients with neurological complications after OLT. [source]

    Mechanical ventilation for respiratory failure in children with severe neurological impairment: is it futile medical treatment?

    Aim, To assess outcome for children with severe neurological impairment receiving invasive mechanical ventilation for respiratory failure. Method, Medical charts for all such children treated in our intensive care unit (ICU) between January 2003 and July 2008 were reviewed. Outcomes were compared with those for children with moderate neurological impairment. Results, Twenty-two children with severe neurological impairment were included (nine females, 13 males; median age 7y 10mo; range 4mo,17y). The median duration of mechanical ventilation was 16 days. Six children had an uneventful 1-year survival, the others required reintubation or readmission to the ICU, or died. Eleven children were still alive 1 year after discharge from the ICU. Nine patients died of respiratory failure. None of the children in the severe group died of a heart defect. Eleven children with moderate neurological impairment were included (eight females, three males; median age 1y 1mo, range 4mo,13y). Four children had an uneventful 1-year survival. Eight children were still alive 1 year after discharge from the ICU. Two of the three non-survivors died of their heart defects. Interpretation, Mechanical ventilation for respiratory failure in children with severe neurological impairment is complex and associated with limited survival. However, it cannot be regarded as futile medical treatment. Further studies are urgently needed for the rational guidance of clinical decision-making. [source]

    Cerebral palsy and newborn care: I, II, and III (1981)

    Fiona Stanley MD
    Another in our series of commentaries on notable papers from the DMCN archives. The full papers are available at Kiely JL, Paneth N, Stein Z, Susser M. Cerebral palsy and newborn care. I: Secular trends in cerebral palsy. Dev Med Child Neurol 1981; 23: 533,38. Kiely JL, Paneth N, Stein Z, Susser M. Cerebral palsy and newborn care. II: Mortality and neurological impairment in low-birthweight infants. Dev Med Child Neurol 1981; 23: 650,59. Kiely JL, Paneth N, Stein Z, Susser M. Cerebral palsy and newborn care. III: Estimated prevalence rates of cerebral palsy under differing rates of mortality and impairment of low-birthweight infants. Dev Med Child Neurol 1981; 23: 801,07. [source]

    A visual skills inventory for children with neurological impairments

    D L McCulloch OD PhD FAAO
    Children with neurological impairments often have visual deficits that are difficult to quantify. We have compared visual skills evaluated by carers with results of a comprehensive visual assessment. Participants were 76 children with mild to profound intellectual and/or motor impairment (33 males, 43 females; age range 7mo,16y; mean age 5y 1mo [SD 4y 2mo]) who completed a visual skills inventory before attending a special vision clinic. The inventory included 16 questions about visual skills and responses to familiar situations. Responses were augmented by taking a structured clinical history, compared with visual evoked potential (VEP) and/or acuity card measures of visual acuity, and examined using exploratory factor analysis. Acuity ranged from normal to no light perception, and was positively associated with responses to individual questions. After excluding four uninformative questions, an association between the remaining questions and two significant independent factors was found. Factor 1 was associated with questions about visual recognition (e.g. ,Does your child see a small silent toy?') and these items were correlated with both the VEP and acuity card thresholds. Factor 2 was associated primarily with questions about visually mediated social interactions (e.g. ,Does he/she return your silent smile?'). Evaluation of visual skills in children with neurological impairment can provide valid information about the quality of children's vision. Questions with the highest validity for predicting vision are identified. [source]

    Total esophagogastric dissociation in adult neurologically impaired patients with severe gastroesophageal reflux: an alternative approach

    E. J. Hazebroek
    SUMMARY Patients with neuromuscular impairment, such as cerebral palsy or myotonic dystrophy, often suffer from oropharyngeal neuromuscular incoordination and severe gastresophageal reflux (GER). In 1997, Bianchi proposed total esophagogastric dissociation (TEGD) as an alternative to fundoplication and gastrostomy to eliminate totally the risk of recurrence of GER in neurologically impaired children. Little information exists about the best management for adult patients with severe neurological impairment in whom recurrent GER develops after failed fundoplication. We present our experience in three adult patients with neurological impairment in whom TEGD with Roux-en-Y esophagojejunostomy and feeding gastrostomy was performed for permanent treatment of GER. [source]

    Severe Epilepsy in X-Linked Creatine Transporter Defect (CRTR-D)

    EPILEPSIA, Issue 6 2007
    Maria Margherita Mancardi
    Disorders of creatine synthesis or its transporter resulting in neurological impairment with mental retardation and epilepsy have only been recognized in recent years. To date, the epileptic disorder observed in creatine transporter deficiency (CRTR-D) has been described as a mild phenotype with infrequent seizures and favorable response to common antiepileptic drugs. We report on a 5 year-old boy with known speech delay who presented with severe and refractory epilepsy. After extensive investigations, metabolite analysis and brain 1H-MRS suggested CRTR-D, which was confirmed by the detection of a known pathogenic mutation in the SLC6A8 gene (c.1631C>T; p.Pro544Leu). [source]

    Epilepsy Can Be Diagnosed When the First Two Seizures Occur on the Same Day

    EPILEPSIA, Issue 9 2000
    Peter Camfield
    Summary: Purpose: Experts have suggested that when the first two (or more) unprovoked seizures occur on the same day, they should be considered as a single event and the diagnosis of epilepsy await a further seizure. We have studied the subsequent clinical course of children with their first two seizures on the same day ("same day" group) compared with children with their first two seizures separated by more than one day ("different day" group). Method: The Nova Scotia childhood epilepsy database documented all newly diagnosed children with epilepsy from 1977 to 1985 with follow-up in 1990 and 1991. Epilepsy was defined as two or more unprovoked seizures regardless of the interval between seizures provided that consciousness fully returned between seizures. All patients had their first seizure between the ages of 1 month and 16 years. Seizure types were restricted to partial, generalized tonic-clonic, and partial with secondary generalization. Results: Of the 490 children with partial or generalized tonic-clonic seizures and follow-up of more than 2 years, 70 had their first two or more seizures on the same day and 420 had their first two seizures on different days. Eighty percent (56 of 70) of the "same day" group subsequently had one or more further seizures with (n = 14) or without (n = 42) medication; 80.9% (340 of 420) of the "different day" group had one or more further seizures with (n = 115) or without (n = 225) medication. Seizure types were nearly identical. Cause was the same (except for fewer idiopathic "genetic" cases in the "same day" group: 1 of 70 vs. 42 of 420; p = 0.02). Rates of mental handicap and previous febrile seizures were the same. Children in the "same day" group were younger on average (60 vs. 84 months; p = 0.001) and were somewhat more likely to have neurological impairment. Outcome after 7 years average follow-up was the same: 58% of the "same day" group and 56% of the "different day" group were in remission. Conclusion: If two or more unprovoked seizures (with normal consciousness between) occur on the same day, the child appears to have epilepsy and will have a clinical course identical to that of the child with a longer time interval between the first two seizures. [source]

    Brain barrier dysfunction in Cuban Epidemic Optic Neuropathy

    A. González-Quevedo Monteagudo
    Background and purpose:, There are practically no references to cerebrospinal fluid(CSF) studies in tropical or nutritional neuropathies. In the present paper we present the results of CSF studies in patients with Cuban Epidemic Optic Neuropathy (CEON) during epidemic and endemic periods, with an appraisal as to the contribution of brain barriers, function in the pathophysiology of this disease. Methods:, Two hundred and five patients with CEON were studied during the epidemic period (1992,1993) and 12 patients outside the outbreak (1995,1997). CSF protein determination and electrophoresis were carried out, as well as serum and CSF albumin and immunoglobulin G (IgG) quantitation for calculating IgG and Qalb indexes, in order to evaluate intrathecal IgG synthesis and the permeability of the blood,CSF barrier (B-CSF B). Results:, One fourth of the patients had increased permeability of the B-CSF B, but damage was more frequent between 16 and 60 days from onset of disease, disappearing after 120 days. B-CSF B dysfunction was more prevalent in patients with severe neurological impairment, although it was not related to the severity of ophthalmological damage. The group of patients studied outside of the outbreak (endemic period) showed similar results. Discussion:, The possible association of increased permeability of the B-CSF B with oxidative stress, which lies on the basis of this epidemic outbreak, is discussed. [source]

    EFNS guideline on neuroimaging in acute stroke.

    Report of an EFNS task force
    Neuroimaging techniques are necessary for the evaluation of stroke, one of the leading causes of death and neurological impairment in developed countries. The multiplicity of techniques available has increased the complexity of decision making for physicians. We performed a comprehensive review of the literature in English for the period 1965,2005 and critically assessed the relevant publications. The members of the panel reviewed and corrected an initial draft, until a consensus was reached on recommendations stratified according to the European Federation of Neurological Societies (EFNS) criteria. Non-contrast computed tomography (CT) scan is the established imaging procedure for the initial evaluation of stroke patients. However, magnetic resonance imaging (MRI) has a higher sensitivity than CT for the demonstration of infarcted or ischemic areas and depicts well acute and chronic intracerebral hemorrhage. Perfusion and diffusion MRI together with MR angiography (MRA) are very helpful for the acute evaluation of patients with ischemic stroke. MRI and MRA are the recommended techniques for screening cerebral aneurysms and for the diagnosis of cerebral venous thrombosis and arterial dissection. For the non-invasive study of extracranial vessels, MRA is less portable and more expensive than ultrasonography but it has higher sensitivity and specificity for carotid stenosis. Transcranial Doppler is very useful for monitoring arterial reperfusion after thrombolysis, for the diagnosis of intracranial stenosis and of right-to-left shunts, and for monitoring vasospasm after subarachnoid hemorrhage. Currently, single photon emission computed tomography and positron emission tomography have a more limited role in the evaluation of the acute stroke patient. [source]

    Candesartan pretreatment is cerebroprotective in a rat model of endothelin-1-induced middle cerebral artery occlusion

    Adam P. Mecca
    Endogenous levels of angiotensin II (Ang II) are increased in the cortex and hypothalamus following stroke, and Ang II type 1 receptor blockers (ARBs) have been shown to attenuate the deleterious effects in animal stroke models using middle cerebral artery (MCA) intraluminal occlusion procedures. However, the endothelin-1 (ET-1)-induced middle cerebral artery occlusion (MCAO) model of cerebral ischaemia is thought to more closely mimic the temporal events of an embolic stroke. This method provides rapid occlusion of the MCA and a gradual reperfusion that lasts for 16,22 h. The aim of the present study was to evaluate whether systemic administration of an ARB prior to ET-1-induced MCAO would provide cerebroprotection during this model of ischaemic stroke. Injection of 3 ,l of 80 ,m ET-1 adjacent to the MCA resulted in complete occlusion of the vessel that resolved over a period of 30,40 min. Following ET-1-induced MCAO, rats had significant neurological impairment, as well as an infarct that consisted of 30% of the ipsilateral grey matter. Systemic pretreatment with 0.2 mg kg,1 day,1 candesartan for 7 days attenuated both the infarct size and the neurological deficits caused by ET-1-induced MCAO without altering blood pressure. This study confirms the cerebroprotective properties of ARBs during ischaemic stroke and validates the ET-1-induced MCAO model for examination of the role of the brain renin,angiotensin system in ischaemic stroke. [source]

    Oxidative stress in NPC1 deficient cells: protective effect of allopregnanolone

    Stefania Zampieri
    Abstract Niemann-Pick C disease (NPC) is an autosomal recessive neurodegenerative disorder caused by the abnormal function of NPC1 or NPC2 proteins, leading to an accumulation of unesterified cholesterol and glycosphingolipids (GSLs) in the lysosomes. The mechanisms underlying the pathophysiology in NPC disease are not clear. Oxidative damage is implicated in the pathophysiology of different neurological disorders and the effect of GSL accumulation on the intracellular redox state has been documented. Therefore, we determined whether the intracellular redox state might contribute to the NPC disease pathophysiology. Because the treatment of NPC mice with allopregnanolone (ALLO) increases their lifespan and delays the onset of neurological impairment, we analysed the effect of ALLO on the oxidative damage in human NPC fibroblasts. Concentrations of reactive oxygen species (ROS) and lipid peroxidation were higher in fibroblasts from NPC patients than in fibroblasts from normal subjects. Fibroblasts from NPC patients were more susceptible to cell death through apoptosis after an acute oxidative insult. This process is mediated by activation of the NF-,B signalling pathway. Knockdown of NPC1 mRNA both in normal fibroblasts and in human SH-SY5Y neuroblastoma cells caused increased ROS concentrations. ALLO treatment of fibroblasts from NPC patients or NPC1 knockdown cells reduced the levels of ROS and lipid peroxidation and prevented peroxide-induced apoptosis and NF-kB activation. Thus, these findings suggest that oxidative stress might contribute to the NPC disease and ALLO might be beneficial in the treatment of the disease, at least in part, due to its ability to restore the intracellular redox state. [source]

    Ricochet of a Bullet in the Spinal Canal: A Case Report and Review of the Literature on Bullet Migration

    Audrey Farrugia M.D.
    Abstract:, Ricochet of a bullet in the spinal canal is well known by neurosurgeons but relatively not a common event in usual medico-legal autopsy practice. This article presents a homicide case of a penetrating gunshot injury of the lumbar spine through the T12-L1 intervertebral foramen with active movement of the projectile within the spinal canal to the L5-S1 level. This case illustrates a bullet intradural and intramedullary active movement because of a ricochet of the body of T12 with active redirection of the path. In the current literature, different types of migration in caudal or cranial direction, intradural, or intramedullary are reported. If spontaneous migration of T10 to S1 seems to be more frequent, some authors reported a C1 to S2 migration. Such migration could be asymptomatic or induce neurological impairment. The medico-legal consequences of these migrations within the spinal canal are described. [source]

    Predictability of FTY720 efficacy in experimental autoimmune encephalomyelitis by in vivo macrophage tracking: Clinical implications for ultrasmall superparamagnetic iron oxide-enhanced magnetic resonance imaging

    Martin Rausch PhD
    Abstract Purpose To examine the efficacy of FTY720 as a new agent to reduce inflammatory activity in an animal model of multiple sclerosis (MS) by in vivo macrophage tracking. Material and Methods FTY720 was used for treatment of rats in a model of chronic relapsing experimental autoimmune encephalomyelitis (EAE) at an oral dose of 0.3 mg/kg/day. Magnetic resonance imaging (MRI) based on in vivo tracking of macrophages labeled with ultrasmall superparamagnetic iron oxide (USPIO) nanoparticles, immunohistological staining (IHC), and neurological readouts was used to study the burden of disease in treated and untreated animals. Results While untreated animals showed severe paralysis of the hind paws, intense accumulation of macrophages in brain tissue, and areas of blood-brain barrier (BBB) disruption, FTY720-treated animals displayed no signs of inflammatory activity or neurological impairment. These observations were made for both acute phase and first relapse. Conclusion Tracking of macrophages by MRI provides direct evidence of the immunomodulatory efficacy of FTY720 in the EAE model and correlates well with neurological symptoms and histology. J. Magn. Reson. Imaging 2004;20:16,24. © 2004 Wiley-Liss, Inc. [source]

    Clinical,Magnetic Resonance Imaging Correlations in Multiple Sclerosis

    Robert Zivadinov MD
    ABSTRACT Conventional magnetic resonance imaging (MRI) has routinely been used to improve the accuracy of multiple sclerosis (MS) diagnosis and monitoring, detect the effects of diseasemodifying therapy, and refine the utility of clinical assessments. However, conventional MRI measures, such as the use of lesion volume and count of gadolinium-enhancing and T2 lesions, have insufficient sensitivity and specificity to reveal the true degree of pathological changes occurring in MS. Newer metrics of MRI analysis, including T1-weighted hypointense lesions (black holes) and central nervous system (CNS) atrophy measures, are able to capture a more global picture of the range of tissue alterations caused by inflammation, demyelination, axonal loss, and neurodegeneration. There is mounting evidence that these MRI measures correlate well with existing and developing neurological impairment and disability. In so doing, these MRI techniques can help elucidate the mechanisms underlying the pathophysiology and natural history of MS. The current understanding is that T1 black holes and CNS atrophy more accurately reflect the neurodegenerative and destructive components of the MS disease process. Therefore, the shortand long-term studies that aim to measure the degree and severity of the neurodegenerative MS disease process should incorporate these MRI metrics as part of their standard routine MRI protocols. [source]

    Fundoplication in children with gastro-oesophageal reflux disease

    AW Norrashidah
    Objectives: The associations between gastro-oesophageal reflux (GOR), chronic respiratory symptoms and gastrointestinal complications have been well described. The aim of this study was to compare the characteristics of children in whom the main indication for fundoplication was respiratory disease with children who had gastrointestinal indications for surgery. Methods: A retrospective review of 79 children who underwent fundoplication between January 1995 and December 1999. Results: Forty-nine of the children (62%) had a respiratory indication for fundoplication. Children with neurological impairment tended to have a respiratory rather than a gastrointestinal indication for surgery. Congenital anomalies were present in 47%. Fundoplication in older children was more likely to be for a gastrointestinal indication. Children with neurological impairment were more likely to have a gastrostomy compared to children with normal neurological status (P < 0.01). Children with a respiratory indication were more likely to have three or more diagnostic investigations (P < 0.001). Ninety-two per cent of children with a respiratory indication and 90% with a gastrointestinal indication for fundoplication had at least one positive test for GOR (barium meal or 24-h oesophageal pH monitoring). Oesophagoscopy showed reflux oesophagitis in 46/61. Eighty-five per cent of the children had complete resolution of their symptoms after fundoplication. Conclusions: Neurological comorbidity was common in children who had surgery for gastro-oesophageal reflux disease, whether for gastrointestinal or respiratory indications. The majority of fundoplications were performed for respiratory indications. [source]

    Selective activation of the sacral anterior roots for induction of bladder voiding

    Narendra Bhadra
    Abstract Aim We investigated the efficacy of selective activation of the smaller diameter axons in the sacral anterior roots for electrically induced bladder voiding. Materials and Methods Acute experiments were conducted in five adult dogs. The anterior sacral roots S2 and S3 were implanted bilaterally with tripolar electrodes. Pressures were recorded from the bladder and from the proximal urethra and the external urethral sphincter. A detector and flow meter monitored fluid flow. A complete sacral dorsal rhizotomy was carried out. The effects of two types of pulse trains at 20 Hz were compared; quasitrapezoidal pulses (500 µsec with 500 µsec exponential decay) and interrupted rectangular (100 µsec, 2 sec on/2 sec off). Before rhizotomy, rectangular pulse trains (100 µsec) to activate all fibers were also applied. The experimental design was block randomized before and after rhizotomy. Results Quasitrapezoidal pulses showed block of sphincter activation with average minimum current for maximum suppression of 1.37 mA. All pulse types evoked average bladder pressures above the basal sphincter closure pressure. The pressure patterns in the proximal urethra closely followed the bladder pressures. Before dorsal rhizotomy, stimulation evoked a superadded increase in sphincter pressures with slow rise time. After rhizotomy, the sphincter pressure patterns followed the bladder pressures during selective activation and voiding occurred during stimulation with quasitrapezoidal trains and in between bursts with interrupted rectangular stimulation. Conclusions Selective activation of sacral ventral roots combined with dorsal rhizotomy may provide a viable means of low-pressure continuous voiding in neurological impairment. Neurourol. Urdynam. © 2005 Wiley-Liss, Inc. [source]

    Symptom cluster and physical activity in relapsing-remitting multiple sclerosis

    Robert W. Motl
    Abstract We compared the explanatory power of two symptom clusters that consisted of either three or five symptoms as correlates of physical activity in individuals with relapsing-remitting multiple sclerosis (RRMS; N,=,218). The data were primarily analyzed using covariance modeling in Mplus 3.0. A symptom cluster of fatigue, depression, and pain had a moderate, negative relationship with physical activity, and this relationship was comparable in magnitude with a symptom cluster of fatigue, depression, pain, perceived cognitive dysfunction, and poor sleep quality. The relationships were attenuated after controlling for exercise history and neurological impairment. Such findings further support the consideration of a narrowly defined cluster of three symptoms as an independent correlate of physical activity in persons with RRMS. © 2010 Wiley Periodicals, Inc. Res Nurs Health 33:398,412, 2010 [source]

    Role of transverse bands in maintaining paranodal structure and axolemmal domain organization in myelinated nerve fibers: Effect on longevity in dysmyelinated mutant mice

    Amanda J. Mierzwa
    Abstract The consequences of dysmyelination are poorly understood and vary widely in severity. The shaking mouse, a quaking allele, is characterized by severe central nervous system (CNS) dysmyelination and demyelination, a conspicuous action tremor, and seizures in ,25% of animals, but with normal muscle strength and a normal lifespan. In this study we compare this mutant with other dysmyelinated mutants including the ceramide sulfotransferase deficient (CST,/,) mouse, which are more severely affected behaviorally, to determine what might underlie the differences between them with respect to behavior and longevity. Examination of the paranodal junctional region of CNS myelinated fibers shows that "transverse bands," a component of the junction, are present in nearly all shaking paranodes but in only a minority of CST,/, paranodes. The number of terminal loops that have transverse bands within a paranode and the number of transverse bands per unit length are only moderately reduced in the shaking mutant, compared with controls, but markedly reduced in CST,/, mice. Immunofluorescence studies also show that although the nodes of the shaking mutant are somewhat longer than normal, Na+ and K+ channels remain separated, distinguishing this mutant from CST,/, mice and others that lack transverse bands. We conclude that the essential difference between the shaking mutant and others more severely affected is the presence of transverse bands, which serve to stabilize paranodal structure over time as well as the organization of the axolemmal domains, and that differences in the prevalence of transverse bands underlie the marked differences in progressive neurological impairment and longevity among dysmyelinated mouse mutants. J. Comp. Neurol. 518:2841,2853, 2010. © 2010 Wiley-Liss, Inc. [source]

    ORIGINAL RESEARCH,WOMEN'S SEXUAL HEALTH: Genital Sensation and Sexual Function in Women Bicyclists and Runners: Are Your Feet Safer than Your Seat?

    Marsha K. Guess MD
    ABSTRACT Introduction., Bicycling is associated with neurological impairment and impotence in men. Similar deficits have not been confirmed in women. Aim., To evaluate the effects of bicycling on genital sensation and sexual function in women. Methods., Healthy, premenopausal, competitive women bicyclists and runners (controls) were compared. Main Outcome Measures., (1) Genital vibratory thresholds (VTs) were determined using the Medoc Vibratory Sensation Analyzer 3000. (2) Sexual function and sexually related distress were assessed by the Dennerstein Personal Experience Questionnaire (SPEQ) and the Female Sexual Distress Scale (FSDS). Results., Forty-eight bicyclists and 22 controls were enrolled. The median age was 33 years. The bicyclists were older, had higher body mass indices (BMIs), were more diverse in their sexual orientation, and were more likely to have a current partner. Bicyclists rode an average of 28.3 ± 19.7 miles/day (range 4,100), 3.8 ± 1.5 days/week, for an average of 2.1 ± 1.8 hours/ride. The mean number of years riding was 7.9 ± 7.1 years (range 0.5,30). Controls ran an average of 4.65 ± 2.1 miles/day (range 1.5,8) and 5.0 ± 1.2 days/week. On bivariate analysis, bicyclists had significantly higher VTs than runners, indicating worse neurological function at all sites (P < 0.05). Multivariate analysis found significant correlations between higher VTs and bicycling at the left and right perineum, posterior vagina, left and right labia. Increasing VTs at the clitoris, anterior vagina, and urethra were associated with age. In bicyclists, there were no correlations between VTs and miles biked per week, duration of riding, or BMI. Composite SPEQ scores indicated normal sexual function in all sexually active subjects. Neither group suffered from sexually related distress. Conclusion., There is an association between bicycling and decreased genital sensation in competitive women bicyclists. Negative effects on sexual function and quality of life were not apparent in our young, healthy premenopausal cohort. Guess MK, Connell K, Schrader S, Reutman S, Wang A, LaCombe J, Toennis C, Lowe B, Melman A, and Mikhail MK. Genital sensation and sexual function in women bicyclists and runners: Are your feet safer than your seat? J Sex Med 2006;3:1018,1027. [source]

    Delineation of Early Changes in Cases with Progressive Supranuclear Palsy-Like Pathology.

    BRAIN PATHOLOGY, Issue 2 2009
    Astrocytes in Striatum are Primary Targets of Tau Phosphorylation, GFAP Oxidation
    Abstract Progressive supranuclear palsy (PSP) is a complex tauopathy usually confirmed at post-mortem in advanced stages of the disease. Early PSP-like changes that may outline the course of the disease are not known. Since PSP is not rarely associated with argyrophilic grain disease (AGD) of varible intensity, the present study was focused on AGD cases with associated PSP-like changes in an attempt to delineate early PSP-like pathology in this category of cases. Three were typical clinical and pathological PSP. Another case presented with cognitive impairment, abnormal behavior and two falls in the last three months. One case suffered from mild cognitive impairment, and two had no evidence of neurological abnormality. Neuropathological study revealed, in addition to AGD, increased intensity and extent of lesion in three groups of regions, striatum, pallidus/subthalamus and selected nuclei of the brain stem, correlating with neurological impairment. Biochemical studies disclosed oxidative damage in the striatum and amygdala. Together the present observations suggest (i) early PSP-like lesions in the striatum, followed by the globus pallidus/subthalamus and selected nuclei of the brain stem; (ii) early involvement of neurons and astrocytes, but late appearance of tufted astrocytres; and (iii) oxidative damage of glial acidic protein in the striatum. [source]

    Gestational Hypoxia Induces White Matter Damage in Neonatal Rats: A New Model of Periventricular Leukomalacia

    BRAIN PATHOLOGY, Issue 1 2004
    Olivier Baud
    In the premature infant, periventricular leukomalacia, usually related to hypoxic-ischemic white matter damage, is the main cause of neurological impairment. We hypothesized that protracted prenatal hypoxia might induce white matter damage during the perinatal period. Pregnant Sparague-Dawley rats were placed in a chamber supplied with hypoxic gas (10% O2 -90% N2) from embryonic day 5(E5) to E20. Neonatal rat brains were investigated by histology, immunocytochemistry, western blotting, in situ hybridization, DNA fragmentation analysis, and in vivo magnetic resonance imaging (MRI). Body weight of pups subjected to prenatal hypoxia was 10 to 30% lower from p0 to P14 than in controls. Specific white matter cysts wear detected between p0 and p7 in pups subjected to prenatal hypoxia, in addition to abnormal extra-cellular matrix, increased lipid peroxidation, white matter cell death detected by TUNEL and increased activated macrophage counts in white matter. Subsequently, gliotic scars and delayed myelination primarily involving immature oligodendrocytes were seen In vivo MRI with T1, T2, and diffusion sequences disclosed similar findings immediately after birth, showing strong correlations with histological abnormalities. We speculate that protracted prenatal hypoxia in rat induces abnormalities. We speculate that protracted prenatal hypoxia in rat induces white matter damage occurring through local inflammatory response and oxidative stress linked to re-oxygenation during the perinatal period. [source]

    Ocular manifestations in liver transplant recipients with familial amyloid polyneuropathy

    Ola Sandgren
    Abstract. Purpose:, To evaluate postoperative ocular involvement in Swedish liver transplant (LT) recipients with familial amyloid polyneuropathy (FAP). Methods:, Routine ophthalmological examinations were performed in 48 LT recipients, with particular attention given to amyloid deposition in the anterior segment and the vitreous body. Medical records were scrutinized for information regarding neurological impairment at the time of the LT. The diagnosis was secured in all cases by examining for amyloid deposits in biopsy specimens and positive genetic testing for amyloidogenic transthyretin (ATTR) Val30Met mutation. Results:, Six patients (12.5%) developed vitreous opacities within the post-LT observation period. The first opacities were seen 40 months after transplantation, 8 years after the onset of systemic disease. Four patients (8%) developed secondary glaucoma, the first of which was observed 18 months after the procedure and 6.5 years after the onset of disease. Sixteen patients (33%) developed deposits on the anterior surface of the lens. Scalloped pupillary margins were noted in 10 patients (21%). Conclusion:, The prevalence of eye complications increases with time after LT and regular follow-up is necessary, especially to disclose the development of glaucoma , a complication with insidious symptoms of which patients are normally unaware. [source]

    Barrett's esophagus and Cornelia de Lange Syndrome

    ACTA PAEDIATRICA, Issue 9 2010
    Francesco Macchini
    Abstract Aim:, To review the records of Cornelia de Lange Syndrome (CDLS) children, affected by Gastro-oesophageal reflux disease (GERD), to detect the presence of Barrett's Esophagus (BE). Methods:, A total of 62 CDLS patients were investigated for GERD (1 month,35 years). In all of them a pH-metry, an upper endoscopy with multiple biopsies and a complete radiologic digestive evaluation were carried out. BE was diagnosed in case of replacement of oesophageal mucosa by specialized intestinal-type columnar mucosa. Anti-reflux surgery was considered in case of persistence of BE after medical therapy. Follow-up (mean 3.5 years) consisted in endoscopy every 6 months . Results:, Gastro-oesophageal reflux disease was found in 50 CDLS patients (80%) and BE in six of them (12% of the GERD group, 9.6% of the entire population, mean age 17 years, range 6,32 years). A short segment BE was observed in three patients, a long one in two patients and an infiltrating adenocarcinoma of the lower oesophagus in one patient. Conclusions:, A higher frequency of BE in CDLS patients than in a normal population is found. A delayed diagnosis because of atypical GERD symptoms and an altered intestinal motility as a result of neurological impairment can be recognized as the main cause. [source]

    Pathogenesis, diagnosis and treatment of neuromyelitis optica: Changing concept of an old disease

    Zsolt Illes
    Abstract The concept of neuromyelitis optica (NMO) has changed considerably during the past few years. The identification of autoantibodies in the sera generated against the water channel aquaporin 4 (AQP4) has increased the specificity of diagnosis and modified guidelines. A pathogenic role of anti-AQP4 antibodies has recently been indicated; they evoke the pathological and clinical hallmarks of the disease on transfer and cause necrosis of astrocytes expressing AQP4. The diagnosis of NMO is based on clinical, neuroimaging and serological criteria. Early therapy preventing relapses is mandatory, because neurological impairment accumulated during relapses is the main cause of permanent disability. In a number of cases defined as NMO spectrum diseases, the clinical manifestation is spatially limited. Such events of separate myelitis or relapsing/bilateral optic neuritis challenge diagnosis and might delay proper therapy. Here, current concepts of diagnosis and treatment of NMO and NMO spectrum diseases are summarized. Diagnostic and treatment decisions in different clinical situations are shown by discussion of cases. (Clin. Exp. Neuroimmunol. doi: 10.1111/j.1759-1961.2010.00011.x, 2010) [source]

    Factors of significance for mobility in children with myelomeningocele

    ACTA PAEDIATRICA, Issue 2 2003
    S Norrlin
    Aim: To investigate neurological impairment, hand function and cognitive function in a group of children with myelomeningocele, in order to identify factors of significance for independent mobility and the physical assistance required for mobility in daily activities. Methods: The study material comprised 32 children, aged 6,11 y and without mental retardation. Mobility was assessed with the Pediatric Evaluation of Disability Inventory, scored as caregiver assistance. Statistical differences and correlations between the caregiver assistance scores and the selected variables were analysed. Results: Nine children scored independent mobility. The independent children had better hand coordination (p= 0.004) and walking ability (p= 0.01), lower cele levels (p= 0.011), higher performance IQ (p= 0.027), better visuospatial function (p= 0.029) and executive function (p= 0.037) than the others. The caregiver assistance scores were lower for both the children with early and severe symptoms of brainstem dysfunction and the children with scoliosis. Statistically significant correlations were found between the need for caregiver assistance and reduced walking ability, high cele level, poor hand strength and coordination, visuospatial function, executive function and performance IQ. In the subgroup of children who needed a wheelchair or walking aids, hand strength was the only variable significantly correlated with caregiver assistance (rs= 0.703, p= 0.000). Conclusion: Most of the children were dependent on others in daily activities. Impaired hand function and cognitive function were significant for mobility, and this has implications for the therapy programme in children with myelomeningocele. [source]

    A visual skills inventory for children with neurological impairments

    D L McCulloch OD PhD FAAO
    Children with neurological impairments often have visual deficits that are difficult to quantify. We have compared visual skills evaluated by carers with results of a comprehensive visual assessment. Participants were 76 children with mild to profound intellectual and/or motor impairment (33 males, 43 females; age range 7mo,16y; mean age 5y 1mo [SD 4y 2mo]) who completed a visual skills inventory before attending a special vision clinic. The inventory included 16 questions about visual skills and responses to familiar situations. Responses were augmented by taking a structured clinical history, compared with visual evoked potential (VEP) and/or acuity card measures of visual acuity, and examined using exploratory factor analysis. Acuity ranged from normal to no light perception, and was positively associated with responses to individual questions. After excluding four uninformative questions, an association between the remaining questions and two significant independent factors was found. Factor 1 was associated with questions about visual recognition (e.g. ,Does your child see a small silent toy?') and these items were correlated with both the VEP and acuity card thresholds. Factor 2 was associated primarily with questions about visually mediated social interactions (e.g. ,Does he/she return your silent smile?'). Evaluation of visual skills in children with neurological impairment can provide valid information about the quality of children's vision. Questions with the highest validity for predicting vision are identified. [source]

    One-year longitudinal evaluation of sensorimotor functions in APP751SL transgenic mice

    C. Le Cudennec
    Intracerebral amyloid-beta (A,) peptide deposition is considered to play a key role in Alzheimer's disease and is designated as a principal therapeutic target. The relationship between brain A, levels and clinical deficits remains, however, unclear, both in human patients and in animal models of the disease. The purpose of the present study was to investigate, in a transgenic mouse model of brain amyloidosis, the consequences of A, deposition on basic neurological functions using a longitudinal approach. Animals were phenotyped at different ages corresponding to graded neuropathological stages (from no extracellular A, deposition to high amyloid loads). Sensory functions were evaluated by assessing visual and olfactory abilities and did not show any effects of the amyloid precursor protein (APP) transgene. Motor functions were assessed using multiple experimental paradigms. Results showed that motor strength was considerably reduced in APP transgenic mice compared with control animals. No deficit was noted in a motor coordination test although APP transgenic mice displayed decreased locomotion on a stationary beam. Hypolocomotion was also observed in the standard open-field test. Measures of anxiety obtained in the elevated plus-maze show some evidence of hyperanxiety in 15-month-old transgenic mice. Some of the neurological impairments showed by APP mice had an early onset and worsened with progressive aging, in parallel to gradual accumulation of A, in brain parenchyma. Relationships between neuropathologically assessed amyloid loads and behavioral deficits were further explored, and it was observed that motor strength deficits were correlated with cortical amyloid burden. [source]

    Alterations of postsynaptic density proteins in the hippocampus of rat offspring from the morphine-addicted mother: Beneficial effect of dextromethorphan

    HIPPOCAMPUS, Issue 6 2006
    San Nan Yang
    Abstract Infants passively exposed to morphine or heroin through their addicted mothers usually develop characteristic withdrawal syndrome of morphine after birth. In such early life, the central nervous system exhibits significant plasticity and can be altered by various prenatal influences, including prenatal morphine exposure. Here we studied the effects of prenatal morphine exposure on postsynaptic density protein 95 (PSD-95), an important cytoskeletal specialization involved in the anchoring of the NMDAR and neuronal nitric oxide synthase (nNOS), of the hippocampal CA1 subregion from young offspring at postnatal day 14 (P14). We also evaluated the therapeutic efficacy of dextromethorphan, a widely used antitussive drug with noncompetitive antagonistic effects on NMDARs, for such offspring. The results revealed that prenatal morphine exposure caused a maximal decrease in PSD-95 expression at P14 followed by an age-dependent improvement. In addition, prenatal morphine exposure reduced not only the expression of nNOS and the phosphorylation of cAMP responsive element-binding protein at serine 133 (CREBSerine-133), but also the magnitude of long-term depression (LTD) at P14. Subsequently, the morphine-treated offspring exhibited impaired performance in long-term learning and memory at later ages (P28,29). Prenatal coadministration of dextromethorphan with morphine during pregnancy and throughout lactation could significantly attenuate the adverse effects as described above. Collectively, the study demonstrates that maternal exposure to morphine decreases the magnitude of PSD-95, nNOS, the phosphorylation of CREBSerine-133, and LTD expression in hippocampal CA1 subregion of young offspring (e.g., P14). Such alterations within the developing brain may play a role for subsequent neurological impairments (e.g., impaired performance of long-term learning and memory). The results raise a possibility that postsynaptic density proteins could serve an important role, at least in part, for the neurobiological pathogenesis in offspring from the morphine-addicted mother and provide tentative therapeutic strategy. © 2006 Wiley-Liss, Inc. [source]

    Parenting and attachment among toddlers with congenital anomalies: Examining the Strange Situation and attachment Q-sort

    Melissa Clements
    This study assessed parent and child predictors of attachment in a sample of 72 toddlers with neurological (e.g., cerebral palsy) and non-neurological (e.g., cleft lip and palate) birth defects and their mothers. Parenting quality (e.g., sensitivity) was expected to be more important in predicting the attachment relationship than type and severity of child medical condition. Parenting and indices of severity of child condition were measured via researcher observation. Attachment was measured via the Strange Situation and parent reported Attachment Q-sort. Parenting quality was better for children with more severe appearance disfigurements. Strange Situation and Q-sort assessments of attachment were not significantly related. Children with neurological impairments were at greater risk for developing insecure attachments than were children with non-neurological conditions. Parenting quality also directly predicted Strange Situation assessed attachment security and Q-sort comfort seeking/exploration but not standard Q-sort criterion scores. Parenting quality partially mediated the relation between child medical condition and attachment security. Results suggest child medical factors influence parenting, and thereby, child attachment. ©2002 Michigan Association for Infant Mental Health. [source]

    Minor neurological dysfunction, cognitive development and somatic development at the age of 3 to 11 years in very-low-birthweight infants with transient periventricular echodensities

    ACTA PAEDIATRICA, Issue 12 2006
    Abstract Aim: To determine, using strict exclusion criteria, whether transient periventricular echodensities (TPE) in very-low-birthweight infants lead to minor neurological dysfunction and problems in cognitive and somatic development in children without major neurological impairments. Methods: 23 children with TPE were matched to 23 children without TPE. Exclusion criteria were small for gestational age, microcephaly at birth, diplegia, asphyxia, psychomotor retardation, intraventricular haemorrhage grade III/IV, major surgical interventions and malformations. The Kaufman Assessment Battery for Children, Draw-a-Man Test and neuropaediatric examination were used for evaluation. Results: There were no differences in demographic data, growth and socio-economic status. Significant differences with lower results in the TPE group were found in fine motor skills and in the Draw-a-Man Test. In the Kaufman Assessment Battery for Children, all subscales were below average in the TPE group, except the sequential processing scale. In the control group, all subscales were within the average range. Conclusion: By using strict exclusion criteria to eliminate other risk factors for minimal neurological dysfunction and poor cognitive development, we were able to focus on the effect of TPE. TPE seem to affect cognitive development and cause minor neurological dysfunction. [source]