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Hydrocephalus
Kinds of Hydrocephalus Selected AbstractsAUTOMIC FAILURE AND NORMAL PRESSURE HYDROCEPHALUS IN A PATIENT WITH CHRONIC DEMYELINATING INFLAMMATORY NEUROPATHYJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2002M. Laurà A 75-year-old man with HCV hepatitis developed at the age of 70 presented with rest and action tremor localized at both hands and progressive cognitive impairment with memory loss. Four years later he begun to complain of progressive fatigue, occasional falls, numbness at the extremities and orthostatic hypotension. One month after admission, he rapidly worsened with inability to walk, mainly because of autonomic failure. Neurological examination revealed gait disturbances, including a wide base of support and short stride, slurred speech, reduction of upward gaze, rest and action tremor at both hands, intrinsic hand muscle and anterior tibialis muscle wasting and weakness on both sides, absent deep tendon reflexes, loss of vibration sense at lower limbs, and bilateral pes cavus. Routine laboratory studies, autoantibodies, thyroid function, neoplastic markers and immunoelectrophoresis were normal. Cryoglobulins were absent, whereas CSF protein content was increased (142 mg/dl). Autonomic nervous system investigation detected severe orthostatic hypotension. Nerve conduction studies showed absent sensory potentials and a marked reduction of compound motor action potential amplitudes and of motor conduction velocities. A sural nerve biopsy revealed remarkable onion bulb-like changes, endoneurial and perivascular infiltrations of inflammatory cells. Psychometric tests showed mild cognitive impairment. Brain MRI was consistent with normotensive hydrocephalus. The findings indicated the presence of chronic inflammatory demyelinating polyneuropathy, autonomic nervous system involvement and normal pressure hydrocephalus. A condition of multiple system atrophy (MSA) might be taken into account, even if somatic peripheral nerve involvement may rarely occur in MSA. Moreover the normal pressure hydrocephalus could be due to the high protein content in CSF (Fukatsu R et al., 1997). [source] Vision in children with hydrocephalusDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 10 2006Susann Andersson MD Hydrocephalus in children has many aetiologies, and can cause multiple ophthalmic and visual disorders. This study sets out to detect and quantify visual and visuoperceptual dysfunction in children who have received surgical treatment for hydrocephalus with and without myelomeningocele, and to relate the results to the associated diagnoses and results from a comparison group. Seventy-five school-aged children (41 males, 34 females) with surgically-treated hydrocephalus and 140 comparison children (76 males, 64 females) matched for age and sex underwent comprehensive ophthalmologic examination. Median age at examination was 9 years and 4 months (range 7y 4mo- 12y 10mo). Visual function deficits were identified in 83% (62/75) of the children with hydrocephalus. Visual impairment (binocular visual acuity <0.3) was found in 15% (11/73; comparison group 0%) but in none with myelomeningocele. Strabismus was found in 69% (51/74; comparison group 4% [5/140], p<0.001), and refractive errors were found in 67% (47/70; comparison group 20% [28/140], p<0.001). Cognitive visual dysfunction was identified in 59% (38/64; comparison group 3% [4/140], p<0.001). These disorders were identified in various combinations and comprised impaired ability to plan movement through depth (e.g. going down a stair), impaired simultaneous perception, impaired perception of movement, impaired orientation, and (least frequently) impaired recognition. In this study, children with hydrocephalus associated with myelomeningocele were least commonly affected. Visual disorders were most frequent in those with epilepsy, cerebral palsy, and/or cognitive disability. [source] Aquaporin 4 changes in rat brain with severe hydrocephalusEUROPEAN JOURNAL OF NEUROSCIENCE, Issue 11 2006Xiaoyan Mao Abstract Hydrocephalus is characterized by impaired cerebrospinal fluid (CSF) flow with enlargement of the ventricular cavities of the brain and progressive damage to surrounding tissue. Bulk water movement is altered in these brains. We hypothesized that increased expression of aquaporins, which are water-permeable channel proteins, would occur in these brains to facilitate water shifts. We used quantitative (real-time) RT-PCR, Western blotting and immunohistochemistry to evaluate the brain expression of aquaporins (AQP) 1, 4, and 9 mRNA and protein in Sprague,Dawley rats rendered hydrocephalic by injection of kaolin into cistern magna. AQP4 mRNA was significantly up-regulated in parietal cerebrum and hippocampus 4 weeks and 9 months after induction of hydrocephalus (P < 0.05). Although Western blot analysis showed no significant change, there was more intense perivascular AQP4 immunoreactivity in cerebrum of hydrocephalic brains at 3,4 weeks after induction. We did not detect mRNA or protein changes in AQP1 (located in choroid plexus) or AQP9 (located in select neuron populations). Kir4.1, a potassium channel protein linked to water flux, exhibited enhanced immunoreactivity in the cerebral cortex of hydrocephalic rats; the perineuronal distribution was entirely different from that of AQP4. These results suggest that brain AQP4 up-regulation might be a compensatory response to maintain water homeostasis in hydrocephalus. [source] Transverse Sinus Thrombosis Presenting With Acute Hydrocephalus: A Case ReportHEADACHE, Issue 2 2008Lampis C. Stavrinou MD We report on a 32-year-old woman who presented with headache of a 10-day duration, due to acute hydrocephalus. This was a result of a tumefactive lesion of the posterior fossa, which was later proven to be a cerebellar venous infarct caused by unilateral transverse sinus thrombosis. Cerebral dural sinus thrombosis should be considered in the differential diagnosis of new onset of headache. [source] Parent's involvement in decisions when their child is admitted to hospital with suspected shunt malfunction: study protocolJOURNAL OF ADVANCED NURSING, Issue 10 2009Joanna Smith Abstract Title., Parent's involvement in decisions when their child is admitted to hospital with suspected shunt malfunction: study protocol. Aim., This paper outlines the protocol for a study aimed at exploring parent's involvement during professional,parent interactions and decisions about their child's care in the context of suspected shunt malfunction. Background., Hydrocephalus is a long-term condition treated primarily by the insertion of a shunt that diverts fluid from the brain to another body compartment. Shunts frequently malfunction, and parents of children with shunted hydrocephalus are responsible for recognizing and responding to shunt complications. Parents feel that interactions with professionals when they seek healthcare advice for their child do always not encourage active participation in care decisions. Methods., The study design is based on qualitative methodologies: a combination of conversation analysis applied to consultation recordings of professional,parent interactions when a child is admitted to hospital with suspected shunt malfunction, and semi-structured follow-up interviews with the same participants within 2 weeks of the consultation. Participants., This is a prospective study and participants will be purposefully selected. Parents of children who have been admitted to hospital with suspected shunt malfunction and healthcare professionals responsible for the initial assessment of the child will be invited to participate. Discussion., The study will identify how decisions about a child's care are negotiated between parents and healthcare professionals at key stages of the care pathway. In addition, examining interactions between healthcare professionals and parents may identify approaches that support or hinder parents in contributing to the decision-making processes when they seek advice from healthcare professionals. [source] Tumor suppressor gene Co-operativity in compound Patched1 and suppressor of fused heterozygous mutant miceMOLECULAR CARCINOGENESIS, Issue 5 2009Jessica Svärd Abstract Dysregulation of the Hedgehog signaling pathway is central to the development of certain tumor types, including medulloblastoma and basal cell carcinoma (BCC). Patched1 (Ptch1) and Suppressor of fused (Sufu) are two essential negative regulators of the pathway with tumor suppressor activity. Ptch1+/, mice are predisposed to developing medulloblastoma and rhabdomyosarcoma, while Sufu+/, mice develop a skin phenotype characterized by basaloid epidermal proliferations. Here, we have studied tumor development in Sufu+/,Ptch1+/, mice to determine the effect of compound heterozygosity on the onset, incidence, and spectrum of tumors. We found significantly more (2.3-fold) basaloid proliferations in Sufu+/,Ptch1+/, compared to Sufu+/, female, but not male, mice. For medulloblastoma, the cumulative 1-yr incidence was 1.5-fold higher in Sufu+/,Ptch1+/, compared to Ptch1+/, female mice but this strong trend was not statistically significant. Together this suggests a weak genetic interaction of the two tumor suppressor genes. We noted a few rhabdomyosarcomas and pancreatic cysts in the Sufu+/,Ptch1+/, mice, but the numbers were not significantly different from the single heterozygous mice. Hydrocephalus developed in ,20% of the Ptch1+/, and Sufu+/,Ptch1+/, but not in Sufu+/, mice. Interestingly, most of the medulloblastomas from the Sufu+/,Ptch1+/, mice had lost expression of the remaining Ptch1 wild-type allele but not the Sufu wild-type allele. On the contrary, Sufu as well as Gli1 and Gli2 expression was upregulated in the medulloblastomas compared to adult cerebellum in Ptch1+/, and Sufu+/,Ptch1+/, mice. This suggests that Sufu expression may be regulated by Hedgehog pathway activity and could constitute another negative feedback loop in the pathway. © 2008 Wiley-Liss, Inc. [source] Prenatal diagnosis in a family with X-linked hydrocephalusPRENATAL DIAGNOSIS, Issue 10 2005Maria Panayi Abstract The neural cell adhesion molecule L1 is a transmembrane glycoprotein belonging to the immunoglobulin superfamily of cell adhesion molecules (CAMs). Its expression is essential during embryonic development of the nervous system and it is involved in cognitive function and memory. Mutations in the L1CAM gene are responsible for four related L1 disorders; X-linked hydrocephalus/HSAS (Hydrocephalus as a result of Stenosis of the Aqueduct of Sylvius), MASA (Mental retardation, Aphasia, Shuffling gait, and Adducted thumbs) syndrome, X-linked complicated spastic paraplegia type I (SPG1) and X-linked Agenesis of the Corpus Callosum (ACC). These four disorders represent a clinical spectrum that varies both between and within families. The main clinical features of this spectrum are Corpus callosum hypoplasia, mental Retardation, Adducted thumbs, Spastic paraplegia and Hydrocephalus (CRASH syndrome). Since there is no biochemically assayed disease marker, molecular analysis of the L1CAM gene is the only means of confirming a clinical diagnosis. Most L1CAM mutations reported to date are point mutations (missense, nonsense, splice site) and only a few patients with larger rearrangements have been documented. We have characterised a rare intragenic deletion of the L1CAM gene in a sample of DNA extracted from a chorionic villus biopsy (CVB) performed at 12 weeks' gestation. Copyright © 2005 John Wiley & Sons, Ltd. [source] The Management of Cerebrospinal Fluid Leaks in Patients at Risk for High-Pressure Hydrocephalus,THE LARYNGOSCOPE, Issue 2 2005Ricardo L. Carrau MD Abstract Objectives/Hypothesis: The transnasal endoscopic approach has become the preferred technique for the surgical management of patients with cerebrospinal fluid (CSF) leaks of the anterior, sellar, and parasellar skull base. The literature has reported an 85% to 100% success rate for the endoscopic repair of CSF leaks, which compares favorably with that reported after transcranial repair. Despite an adequate repair, a subpopulation of patients remain at high risk for recurrence of the CSF leak attributable to undiagnosed high-pressure hydrocephalus. Patients at high risk for high-pressure hydrocephalus include those who have had a subarachnoid hemorrhage as a result of trauma (accidental or surgical) or stroke and those with spontaneous CSF leaks. Study Design: With the goal of reducing the risk of recurrence, the authors developed a protocol for the identification and management of patients with CSF leaks who are at risk for high-pressure hydrocephalus. Methods: The protocol includes endoscopic repair, temporary CSF diversion, measurement of CSF pressure after the repair, and immediate ventriculoperitoneal shunting if necessary. Results: During the period of September 1999 to April 2002, the authors repaired 25 CSF leaks through an endonasal endoscopic approach. Nineteen patients were considered at high risk for high-pressure hydrocephalus. Using the protocol described, the authors identified six patients (31%) with CSF leaks that could be associated with undiagnosed high-pressure hydrocephalus. All CSF leaks were successfully repaired using a transnasal endoscopic repair. Six patients with high-pressure hydrocephalus underwent ventriculoperitoneal shunting after repair of the CSF Leak. No recurrence has been observed at a follow-up ranging from 24 to 84 months (median period, 30 mo). Conclusion: Patients with high-pressure hydrocephalus may be identified in a prospective fashion to prevent recurrence or persistence of the CSF leaks. The presence or absence of high-pressure CSF may be established by means of direct CSF pressure measurement through lumbar puncture postoperatively. This allows early intervention and prevention of recurrence. [source] Insights into the Pathogenesis of Hydrocephalus from Transgenic and Experimental Animal ModelsBRAIN PATHOLOGY, Issue 3 2004Leslie Crews Hydrocephalus is a progressive brain disorder characterized by abnormalities in the flow of cerebrospinal fluid (CSF) and ventricular dilatation that leads to cerebral atrophy, and if left untreated, can be fatal. Genetic mutations, congenital malformations, infectious diseases, intracerebral hemorrhages and tumors are common conditions resulting in hydrocephalus. Although the causes of obstructive hydrocephalus are better understood, the mechanisms resulting in chronic, progressive communicating congenital and acquired hydrocephalus are less well understood. In this regard, recent studies in transgenic (tg) mice suggest that increased expression of cytokines such as TGF-,1 might play an important role by disrupting the vascular extracellular matrix (ECM) remodeling, promoting hemorrhages, and altering the reabsorption of CSF. In this context, the main objective of this manuscript is to provide an overview on the cellular and molecular mechanisms of hydrocephalus based on studies derived from tg and experimental animal models. [source] Cellular Damage and Prevention in Childhood HydrocephalusBRAIN PATHOLOGY, Issue 3 2004Marc R. Del Bigio The literature concerning brain damage due to hydrocephalus, especially in children and animal models, is reviewed. The following conclusions are reached: 1Hydrocephalus has a deleterious effect on brain that is dependent on magnitude and duration of ventriculomegaly and modified by the age of onset. 2Animal models have many histopathological similarities to humans and can be used to understand the pathogenesis of brain damage. 3Periventricular axons and myelin are the primary targets of injury. The pathogenesis has similarities to traumatic and ischemic white matter injury. Secondary changes in neurons reflect compensation to the stress or ultimately the disconnection. 4Altered efflux of extracellular fluid could result in accumulation of waste products that might interfere with neuron function. Further research is needed in this as well as the blood-brain barrier in hydrocephalus. 5Some, but not all, of the changes are preventable by shunting CSF. However, axon loss cannot be reversed, therefore shunting in a given case must be considered carefully. 6Experimental work has so far failed to show any benefit in reducing CSF production. Pharmacologic protection of the brain, at least as a temporary measure, holds some promise but more pre-clinical research is required. [source] Chronic Hydrocephalus in AdultsBRAIN PATHOLOGY, Issue 3 2004Richard J Edwards Chronic hydrocephalus is a complex condition, the incidence of which increases with increasing age. It is characterised by the presence of ventricular enlargement in the absence of significant elevations of intracranial pressure. The clinical syndrome may develop either as a result of decompensation of a "compensated" congenital hydrocephalus, or it may arise de novo in adult life secondary to a known acquired disturbance of normal CSF dynamics. The latter may be due to late onset acqueductal stenosis or disruption of normal CSF absorptive pathways following subarachnoid hemorrhage or meningitis ("secondary" normal pressure hydrocephalus (NPH)). In some cases the cause of the hydrocephalus remains obscure ("idiopathic" NPH). In all forms of chronic hydrocephalus the clinical course of the disease is heavily influenced by changes in the brain associated with aging, in particular cerebrovascular disease. Recent research has challenged previously held tenets regarding the CSF circulatory system and this in turn has led to a radical rethinking of the pathophysiological basis of chronic hydrocephalus. [source] Medical problems in adolescents with myelomeningocele (MMC): an inventory of the Swedish MMC population born during 1986,1989ACTA PAEDIATRICA, Issue 3 2007I Olsson Abstract Aim: To describe the prevalence of myelomeningocele (MMC) and the medical needs of adolescents, 15,18 years, with MMC in Sweden, at a time when they are on the threshold of adulthood, leaving paediatrics. Methods: In a retrospective study, we identified all adolescents with MMC, born during 1986,1989 and living in Sweden on July 1, 2004. An inventory was agreed upon with questions concerning their medical problems and need for medical care. Results: There were 175 persons 15,18 years of age, born with MMC or lipoMMC (prevalence 3.8 per 10 000). Hydrocephalus was seen in 86%, 31% had been operated because of tethered cord syndrome, and 6% for Chiari malformation symptoms. The majority had motor impairments. Clean intermittent catheterisation for bladder emptying was used by 85%, and 59% used enemas on a regular basis because of the neurogenic bowel dysfunction. Renal dysfunction was seen in 1.7% of the adolescents. Conclusion: Lifelong follow-up by many specialists, among others neurologists and neurosurgeons, urotherapists and urologists, orthopaedic surgeons and orthotists, is necessary for individuals with MMC. The complex medical situation, often in combination with cognitive difficulties, makes it necessary to coordinate medical services for this increasing group of adults with multiple impairments. [source] Pitfalls in the Diagnosis of Cerebellar InfarctionACADEMIC EMERGENCY MEDICINE, Issue 1 2007Sean I. Savitz MD Abstract Background Cerebellar infarctions are an important cause of neurologic disease. Failure to recognize and rapidly diagnose cerebellar infarction may lead to serious morbidity and mortality due to hydrocephalus and brain stem infarction. Objectives To identify sources of preventable medical errors, the authors obtained pilot data on cerebellar ischemic strokes that were initially misdiagnosed in the emergency department. Methods Fifteen cases of misdiagnosed cerebellar infarctions were collected, all seen, or reviewed by the authors during a five-year period. For each patient, they report the presenting symptoms, the findings on neurologic examination performed in the emergency department, specific areas of the examination not performed or documented, diagnostic testing, the follow-up course after misdiagnosis, and outcome. The different types of errors leading to misdiagnosis are categorized. Results Half of the patients were younger than 50 years and presented with headache and dizziness. All patients had either incomplete or poorly documented neurologic examinations. Almost all patients had a computed tomographic scan of the head interpreted as normal, and most of these patients underwent subsequent magnetic resonance imaging showing cerebellar infarction. The initial incorrect diagnoses included migraine, toxic encephalopathy, gastritis, meningitis, myocardial infarction, and polyneuropathy. The overall mortality in this patient cohort was 40%. Among the survivors, about 50% had disabling deficits. Pitfalls leading to misdiagnosis involved the clinical evaluation, diagnostic testing, and establishing a diagnosis and disposition. Conclusions This study demonstrates how the diagnosis of cerebellar infarction can be missed or delayed in patients presenting to the emergency department. [source] Quality of evidence for the present Swedish child health surveillance programmeACTA PAEDIATRICA, Issue 2000S Bremberg The present Swedish health surveillance programme includes 15 examinations by a nurse, 5 examinations by a physician, 7 assessments of development, 2 assessments of hearing and 1 assessment of visual acuity. The WHO criteria for evaluation of screening programmes can be applied to the Swedish health surveillance programme. These criteria state that the health problem must be important, that there should be an early phase during which the condition is only detectable by medical professionals and that treatment at an early phase should favourably affect the prognosis. The quality of evidence for fulfilment of these criteria has been graded I-III. Grade II-2 refers to evidence obtained from well-designed cohort or case-control analytical studies. The following disorders might be affected by health surveillance at child health centres: amblyopia, ADHD/DAMP, failure to thrive, cerebral palsy, congenital heart failure, congenital luxation of hip, hearing impairment (severe or moderate), mental retardation, retentio testis and hydrocephalus. None of these conditions fulfils the WHO criteria with quality of evidence grade II-2 or better. Thus, the evidence for the present Swedish health surveillance programme is problematic. [source] Phenotypic differences in the brains and limbs of mutant mice caused by differences of Gli3 gene expression levelsCONGENITAL ANOMALIES, Issue 2 2001Ichiro Naruse ABSTRACT, The genetic polydactyly/arhinencephaly mouse, Pdn/Pdn, exhibits severe polydactyly both in the fore-and hindlimbs, agenesis of the olfactory bulbs, corpus callosum, anterior commissure, and hydrocephalus. A candidate gene for the Pdn mouse has been speculated to be Gli3, because Pdn has been considered to be an allele of Xt whose responsible gene has been clarified to be Gli3. Recently, it has been cleared that retro-transposons are inserted into nitron 3, upstream of zinc finger domain, of the Gli3 gene in the Pdn mouse, resulting to the severe suppression of Gli3 gene expression in Pdn/Pdn embryos. Meanwhile, XtJ/XtJ mice exhibit more severe polydactyly than that of Pdn/Pdn. Arhinencephaly and microholoprosencephaly including agenesis of the olfactory bulbs, corpus callosum, anterior commissure, hippocampal commissure, habenular commissure, and posterior commissure, and moreover, the cerebral cortical plates and hippocampus are not formed in the XtJ/XtJ mice. The XtJ/XtJ mouse has a large deletion in Gli3 structural gene and shows null expression. From these corroborations, we speculated that the differences in the Gli3 gene expression levels resulted in the phenotypic differences between the Pdn/Pdn and XtJ/XtJ mice. [source] Lifestyle, participation, and health-related quality of life in adolescents and young adults with myelomeningoceleDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 11 2009LAURIEN M BUFFART PHD This study aimed to describe participation and health-related quality of life (HRQoL) in adolescents and young adults with myelomeningocele and to explore their relationships with lifestyle-related factors. Fifty-one individuals with a mean age of 21 years 1 month (SD 4y 6mo) years participated (26 males, 25 females; 82% hydrocephalus, 55% wheelchair-dependent). Participation was assessed using the Life Habits Questionnaire, and HRQoL was assessed using the Medical Outcomes Study 36-item Short-form Health Survey. Physical activity was measured using an accelerometry-based activity monitor, fitness (peak oxygen uptake) was measured during a maximal exercise test, and the sum of four skin-folds was assessed to indicate body fat. Relationships were studied using logistic regression analyses. Of the participants, 63% had difficulties in daily activities and 59% in social roles. Participants perceived lower physical HRQoL than a Dutch reference population. Participants with higher levels of physical activity and fitness had fewer difficulties in participating in daily activities (odds ratio [OR]=8.8, p=0.02 and OR=29.7, p=0.02 respectively) and a higher physical HRQoL (OR=4.8, p=0.02 and OR=30.2, p=0.006 respectively), but not mental HRQoL. Body fat was not related to participation or HRQoL. In conclusion, a large proportion of individuals with myelomeningocele had difficulties in participation and perceived low physical HRQoL. Higher levels of physical activity and fitness were related to fewer difficulties in participation and higher physical HRQoL. [source] Smooth ocular pursuit in Chiari type II malformationDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 4 2007Michael S Salman MRCP PhD Chiari type II malformation (CII) is a congenital anomaly of the cerebellum and brainstem, both important structures for processing smooth ocular pursuit. CII is associated with myelomeningocele and hydrocephalus. We investigated the effects of CII on smooth pursuit (SP) eye movements, and determined the effects of spinal lesion level, number of shunt revisions, nystagmus, and brain dysmorphology on SP. SP was recorded using an infrared eye tracker in 21 participants with CII (11 males, 10 females; age range 8-19y, mean 14y 3mo [SD 3y 2mo]). Thirty-eight healthy children (21 males, 17 females) constituted the comparison group. Participants followed a visual target moving sinusoidally at ± 10° amplitude, horizontally and vertically at 0.25 or 0.5Hz. SP gains, the ratio of eye to target velocities, were abnormal in the CII group with nystagmus (n= 8). The number of shunt revisions (range 0-10), brain dysmorphology, or spinal lesion level (n= 15 for lower and n= 6 for upper spinal lesion level) did not correlate with SP gains. SP is impaired in children with CII and nystagmus. Abnormal pursuit might be related to the CII dysgenesis or to effects of hydrocephalus. The lack of effect of shunt revisions and abnormal tracking in participants with nystagmus provide evidence that it is related primarily to the cerebellar and brainstem malformation. [source] Vision in children with hydrocephalusDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 10 2006Susann Andersson MD Hydrocephalus in children has many aetiologies, and can cause multiple ophthalmic and visual disorders. This study sets out to detect and quantify visual and visuoperceptual dysfunction in children who have received surgical treatment for hydrocephalus with and without myelomeningocele, and to relate the results to the associated diagnoses and results from a comparison group. Seventy-five school-aged children (41 males, 34 females) with surgically-treated hydrocephalus and 140 comparison children (76 males, 64 females) matched for age and sex underwent comprehensive ophthalmologic examination. Median age at examination was 9 years and 4 months (range 7y 4mo- 12y 10mo). Visual function deficits were identified in 83% (62/75) of the children with hydrocephalus. Visual impairment (binocular visual acuity <0.3) was found in 15% (11/73; comparison group 0%) but in none with myelomeningocele. Strabismus was found in 69% (51/74; comparison group 4% [5/140], p<0.001), and refractive errors were found in 67% (47/70; comparison group 20% [28/140], p<0.001). Cognitive visual dysfunction was identified in 59% (38/64; comparison group 3% [4/140], p<0.001). These disorders were identified in various combinations and comprised impaired ability to plan movement through depth (e.g. going down a stair), impaired simultaneous perception, impaired perception of movement, impaired orientation, and (least frequently) impaired recognition. In this study, children with hydrocephalus associated with myelomeningocele were least commonly affected. Visual disorders were most frequent in those with epilepsy, cerebral palsy, and/or cognitive disability. [source] Outcome of craniopharyngioma in children: long-term complications and quality of lifeDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 4 2004Andrea Poretti MB BS Childhood craniopharyngiomas are histologically benign tumours arising from remnants of Rathke's pouch in the hypothalamic,pituitary region. The two common treatment approaches are primary total resection or limited resection followed by radiotherapy. To study the outcome after a primary surgical approach, we followed 25 consecutive patients (10 females, 15 males) under 16 years of age who were treated in a single institution with a management policy of radical tumour excision (mean age at diagnosis 9 years 2 months, SD 4 years 3 months; range 2 years 9 months to 15 years 11 months). Mean follow-up after primary surgery was 11 years 3 months (SD 7 years 7 months). Tumour control, and neurological, endocrine, and hypothalamic complications and their impact on health-related quality of life were assessed (medical follow-up, semi-structured interview, and questionnaires). Results of tumour control were generally good, however, local failure was observed in 6 of 25 patients, and severe late-treatment complications decreased quality of life for many long-time survivors. Endocrine deficiency occurred in 24/25, visual complications in 16/24, neurological complications in 8/24, obesity in 14/23, increased daytime sleepiness in 6/21, and significant school problems in 10/20. Patients with craniopharyngioma rated their health-related quality of life as considerably lower than healthy controls; the domains of social and emotional functioning were particularly affected. Parents'ratings were considerably lower than those of the patients. Poor functional outcome was associated with large tumours infiltrating or displacing the hypothalamus, the occurrence of hydrocephalus, and young age at diagnosis, but also with multiple operations due to tumour recurrence. Alternative treatment strategies should be considered, especially in very young patients with large tumours. [source] A case of hydrocephalus occlusus presenting as bipolar disorderACTA PSYCHIATRICA SCANDINAVICA, Issue 2 2005T. Reisch Objective:, This case highlights the fact that manic and depressive symptoms can be related to hydrocephalus occlusus even in the absence of neurological symptoms. Method:, Single case report. Results:, A 22-year-old male patient presented with a 2-year history of manic and depressive symptoms. He was admitted to psychiatric in-patient care fulfilling sufficient criteria of bipolar disorder presenting with a hypomanic state. No neurological symptoms could be detected. Three months later, a MRI of the brain showed a hydrocephalus occlusus because of a space-occupying lesion of 5 mm in the lamina tecti obstructing the aqueduct of Silvius. The MRI also showed parahippocampal changes, which were probably related to the hydrocephalus. After the implantation of a ventriculo-peritoneal shunt, manic symptoms resolved, but the patient continued to suffer from adynamic symptoms. Follow-up MRIs over 3 years showed no progression of the lesion of unknown etiology. Conclusions:, In this case, early routine neuroimaging might have reduced long-term brain damage. The case underlines that even in the absence of neurological symptoms, brain imaging in bipolar disorder might be crucial. The feasibility of routine brain imaging in bipolar patients is discussed. [source] Hemispheric Surgery in Children with Refractory Epilepsy: Seizure Outcome, Complications, and Adaptive FunctionEPILEPSIA, Issue 1 2007Sheikh Nigel Basheer Summary:,Purpose: To describe seizure control, complications, adaptive function and language skills following hemispheric surgery for epilepsy. Methods: Retrospective chart review of patients who underwent hemispheric surgery from July 1993 to June 2004 with a minimum follow-up of 12 months. Results: The study population comprised 24 children, median age at seizure onset six months and median age at surgery 41 months. Etiology included malformations of cortical development (7), infarction (7), Sturge-Weber Syndrome (6), and Rasmussen's encephalitis (4). The most frequent complication was intraoperative bleeding (17 transfused). Age <2 yr, weight <11 kg, and hemidecortication were risk factors for transfusion. Postoperative complications included aseptic meningitis (6), and hydrocephalus (3). At median follow-up of 7 yr, 79% of patients are seizure free. Children with malformations of cortical development and Rasmussen's encephalitis were more likely to have ongoing seizures. Overall adaptive function scores were low, but relative strengths in verbal abilities were observed. Shorter duration of epilepsy prior to surgery was related significantly to better adaptive functioning. Conclusions: Hemispheric surgery is an effective therapy for refractory epilepsy in children. The most common complication was bleeding. Duration of epilepsy prior to surgery is an important factor in determining adaptive outcome. [source] Lumbar infusion testing in the selection of patients with normal pressure hydrocephalus for surgeryEUROPEAN JOURNAL OF NEUROLOGY, Issue 2 2009A. Brean No abstract is available for this article. [source] Assessment of idiopathic normal pressure patients in neurological practice: the role of lumbar infusion testing for referral of patients to neurosurgeryEUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2008A. Brean Background and purpose:, In neurological practice patients with tentative idiopathic normal pressure hydrocephalus (iNPH) usually are referred to neurosurgery based on clinical and radiological findings. Hydrodynamic assessment using lumbar infusion testing might be helpful in selecting patients. To retrospectively analyse lumbar infusion tests done in neurological practice in iNPH patients to see how infusion test results relate to the clinical course and shunt response. Materials and methods:, Sixty-three consecutive patients with Possible/Probable iNPH were tested during a 1-year period. The pre-operative lumbar infusion tests were assessed according to two strategies: (i) Determining the resistance to cerebrospinal fluid (CSF) outflow (Rout). (ii) Quantification of the CSF pressure (CSFP) pulsatility during lumbar infusion (Qpulse). The results were related to the prospectively followed clinical course and shunt response after 12 months. Results:, The lumbar infusion-derived parameters Rout and Qpulse related weakly. Shunt response after 12 months was not related to Rout, but was highly related to the Qpulse. False negative results of lumbar infusion testing were observed in 16% of the patients. Discussion:, In neurological practice lumbar infusion testing may be useful for determining which patients to refer to neurosurgery. Our data favour determination of CSFP pulsatility (Qpulse) rather than Rout for prediction of shunt response. [source] Ventricular cerebrospinal fluid neurofilament protein levels decrease in parallel with white matter pathology after shunt surgery in normal pressure hydrocephalusEUROPEAN JOURNAL OF NEUROLOGY, Issue 3 2007M. Tullberg Normal pressure hydrocephalus (NPH) is characterized by disturbed cerebrospinal fluid (CSF) dynamics and white matter lesions (WML). Although the morphology of these lesions is described, little is known about the biochemistry. Our aim was to explore the relationship between ventricular CSF markers, periventricular WML and postoperative clinical outcome in patients with NPH. We analysed lumbar and ventricular concentrations of 10 CSF markers, 12 clinical symptoms and signs, magnetic resonance imaging (MRI) periventricular white matter hyperintensities (PVH) and ventricular size before and 3 months after shunt surgery in 35 patients with NPH. Higher ventricular CSF neurofilament protein (NFL), an axonal marker, correlated with more extensive PVH. A larger postoperative reduction in NFL correlated with larger reduction in PVH and a more pronounced overall improvement. Albumin ratio, HMPG, NPY, VIP and GD3 increased postoperatively whereas NFL, tau and HVA decreased. Variations in ventricular size were not associated with CSF concentrations of any marker. We conclude that NPH is characterized by an ongoing periventricular neuronal dysfunction seen on MRI as PVH. Clinical improvement after shunt surgery is associated with CSF changes indicating a restitution of axonal function. Other biochemical effects of shunting may include increased monoaminergic and peptidergic neurotransmission, breakdown of blood brain barrier function, and gliosis. [source] rFVIIa, for acute rebleeding of a cerebral cavernous malformationEUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2007K. Engelhardt Recurrent bleeding episodes of cavernomas especially in the brainstem can cause progressive neurological deficits. Therefore brainstem cavernomas are still a therapeutic dilemma and a treatment challenge for the neuro critical care community. We report a 39-year-old woman with spontaneous ataxia diplopia and vomiting, who has been treated for multiple intracerebral cavernomas during the last 10 years. A cerebral computed tomography (cCT) revealed a re-bleeding cavernoma in the left cerebral peduncle with consecutive obstructive hydrocephalus. As a result of the difficult anatomical location, no surgical approach was possible. As an off-label treatment, recombinant activated factor VII (rFVIIa) was administered to prevent possible further bleeding and especially further sequelae. The patient recovered well and no adverse events and especially no further bleeding of the cavernoma were observed. To our knowledge, this is the first report of the safe and successful use of rFVIIa to treat re-bleeding episodes in cavernomas. Further clinical studies are needed to specify the future potential of rFVIIa. [source] Expression of nerve growth factor in cerebrospinal fluid of congenital hydrocephalic and normal childrenEUROPEAN JOURNAL OF NEUROLOGY, Issue 8 2005F. Mashayekhi Cerebrospinal fluid (CSF) is secreted by the choroids plexuses and has the potential to act as a signaling pathway for physiological control as it has been demonstrated to contain molecules such as interleukins, leukoterins, neuropeptides, growth transforming factor-beta (TGF- ,) and nerve growth factor (NGF), which are present at specific times during development. In this study, CSF from hydrocephalic and normal children were analysed using SDS-PAGE followed by silver staining. In order to obtain semi-quantitative estimates of the relative amounts of 26 kDa protein, an image analyzer was used to determine the intensities of the band in the respective lanes in silver-stained gels. Quantification of the silver-stained gels from repeated experiments showed that the amount of 26 kDa protein was clearly increases in the hydrocephalic CSF when compared with the normal CSF. A Western blot analysis using anti-NGF antibody as a probe confirmed the presence of NGF. Using enzyme-linked immunosorbent assay (ELISA), it was shown that the level of NGF in the hydrocephalic CSF is higher than in normal CSF. It is concluded that NGF is not only a constant component of human CSF but could also be significantly involved in the pathophysiology of hydrocephalus. [source] Hematology and coagulation parameters predict outcome in Taiwanese patients with spontaneous intracerebral hemorrhageEUROPEAN JOURNAL OF NEUROLOGY, Issue 3 2005H.-Y. Fang Volume of intracerebral hemorrhage (ICH), Glasgow Coma Scale (GCS) score, peripheral edema around the hematoma, and hydrocephalus are good predictors of mortality in patients with spontaneous ICH from western countries. However, the significance of hematologic and biochemical parameters associated with spontaneous ICH has not been extensively studied. This study was designed to determine prognostic factors for spontaneous ICH in Taiwanese patients. We prospectively studied 109 consecutive patients with spontaneous ICH admitted to Changhua Christian Medical Center. Clinical and laboratory data were collected and analyzed. Mean age was 62.3 years. There were 63 men (58%) and 46 women (42%). Differences in GCS score, ICH score, and Acute Physiology and Chronic Health Evaluation II (APACHE II) score between the survival and non-survival groups were statistically significant. Laboratory data were statistically different using multivariate analysis for platelet count, prothrombin time, and white cell count. This is the first study providing information on predictors of spontaneous ICH mortality in Taiwanese patients. The prothrombin time and platelet count on the first day were good early predictors of mortality. This finding in ethnically Chinese patients appears to be different from the profile for patients from western countries. [source] Aquaporin 4 changes in rat brain with severe hydrocephalusEUROPEAN JOURNAL OF NEUROSCIENCE, Issue 11 2006Xiaoyan Mao Abstract Hydrocephalus is characterized by impaired cerebrospinal fluid (CSF) flow with enlargement of the ventricular cavities of the brain and progressive damage to surrounding tissue. Bulk water movement is altered in these brains. We hypothesized that increased expression of aquaporins, which are water-permeable channel proteins, would occur in these brains to facilitate water shifts. We used quantitative (real-time) RT-PCR, Western blotting and immunohistochemistry to evaluate the brain expression of aquaporins (AQP) 1, 4, and 9 mRNA and protein in Sprague,Dawley rats rendered hydrocephalic by injection of kaolin into cistern magna. AQP4 mRNA was significantly up-regulated in parietal cerebrum and hippocampus 4 weeks and 9 months after induction of hydrocephalus (P < 0.05). Although Western blot analysis showed no significant change, there was more intense perivascular AQP4 immunoreactivity in cerebrum of hydrocephalic brains at 3,4 weeks after induction. We did not detect mRNA or protein changes in AQP1 (located in choroid plexus) or AQP9 (located in select neuron populations). Kir4.1, a potassium channel protein linked to water flux, exhibited enhanced immunoreactivity in the cerebral cortex of hydrocephalic rats; the perineuronal distribution was entirely different from that of AQP4. These results suggest that brain AQP4 up-regulation might be a compensatory response to maintain water homeostasis in hydrocephalus. [source] Tissue-specific expression of Cre recombinase from the Tgfb3 locusGENESIS: THE JOURNAL OF GENETICS AND DEVELOPMENT, Issue 2 2008Liang-Tung Yang Abstract Tgfb3, a member of the TGF-, superfamily, is tightly regulated, both spatially and temporally, during embryogenesis. Previous mouse knockout studies have demonstrated that Tgfb3 is absolutely required for normal palatal fusion and pulmonary development. We have generated a novel tool to ablate genes in Tgfb3 -expressing cells by targeting the promoterless Cre-pgk-Neo cassette into exon 1 of the mouse Tgfb3 gene, which generates a functionally null Tgfb3 allele. Using the Rosa26 reporter assay, we demonstrate that Cre -induced recombination was already induced at embryonal day 10 (E10) in the ventricular myocardium, limb buds, and otic vesicles. At E14, robust recombination was detected in the prefusion palatal epithelium. Deletion of the TGF-, type I receptor Alk5 (Tgfbr1) specifically in Tgfb3 expressing cells using the Tgfb3-Cre driver line lead to a cleft palate phenotype similar to that seen in conventional Tgfb3 null mutants. In addition, Alk5/ Tgfb3-Cre mice displayed hydrocephalus, and severe intracranial bleeding due to germinal matrix hemorrhage. genesis 46:112,118, 2008. © 2008 Wiley-Liss, Inc. [source] Transverse Sinus Thrombosis Presenting With Acute Hydrocephalus: A Case ReportHEADACHE, Issue 2 2008Lampis C. Stavrinou MD We report on a 32-year-old woman who presented with headache of a 10-day duration, due to acute hydrocephalus. This was a result of a tumefactive lesion of the posterior fossa, which was later proven to be a cerebellar venous infarct caused by unilateral transverse sinus thrombosis. Cerebral dural sinus thrombosis should be considered in the differential diagnosis of new onset of headache. [source] | |||||||||||||||||||||||||||||||||||||