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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Normal brain development throughout childhood and adolescence is usually characterized by decreased cortical thickness in the frontal regions as well as region-specific patterns of increased white matter myelination and volume. We investigated total cerebral volumes, neocortical surface area, and neocortical thickness in 16 children with a neural tube defect, spina bifida myelomeningocele (SB), and 16 age-matched typically developing controls using a semi-automated, quantitative approach to MRI-based brain morphometry. The results revealed no significant group differences in total cerebral volume. However, group differences were observed in the global distribution of distinct tissue classes within the cerebrum: the SB group demonstrated a significant 15% reduction in total white matter and a 69% increase in cerebrospinal fluid, with no differences in total gray matter. Group comparisons of neocortical surface area assessments were significantly smaller in the occipital regions for SB, with no significant group differences in the frontal regions. Group comparisons of cortical thickness measurements demonstrated reduced cortical thickness in all regions except the frontal regions, where the SB group exhibited an increase relative to the PC group. Although regional patterns of thinning may be associated with the mechanical effects of hydrocephalus, the overall reduction in white matter and increased neocortical thickness in the frontal regions suggest that SB reflects a long-term disruption of brain development that extends far beyond the neural tube defect in the first weeks of gestation.
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PMID:Neocortical reorganization in spina bifida. 1833 24

Spina bifida meningomyelocele with hydrocephalus (SBM) is commonly associated with anomalies of the corpus callosum (CC). We describe MRI patterns of regional CC agenesis and relate CC anomalies to functional laterality based on a dichotic listening test in 90 children with SBM and 27 typically developing controls. Many children with SBM (n = 40) showed regional CC anomalies in the form of agenesis of the rostrum and/or splenium, and a smaller number (n = 20) showed hypoplasia (thinning) of all CC regions (rostrum, genu, body, and splenium). The expected right ear advantage (REA) was exhibited by normal controls and children with SBM having a normal or hypoplastic splenium. It was not shown by children with SBM who were left handed, missing a splenium, or had a higher level spinal cord lesion. Perhaps the right hemisphere of these children is more involved in processing some aspects of linguistic stimuli.
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PMID:Auditory interhemispheric transfer in relation to patterns of partial agenesis and hypoplasia of the corpus callosum in spina bifida meningomyelocele. 1876 72

The present study rates the value of different investigative procedures used to diagnose a congenital hydrocephalus internus of the dog. Six dogs, aged between two and ten months, were presented in our clinic with neurologic signs because of a congenital hydrocephalus internus. After taking a neurologic examination and further diagnostic studies they were euthanized and dissected. The neurologic examination did not help to predict the exact location of the lesion in the brain. Very high amplitudes and low frequencies are the characteristic electroencephalographic pattern of congenital hydrocephalus internus; they occurred in all electroencephalograms (EEGs). Radiologic changes like calvarial enlargement or thinning of the bony walls could be seen only in patients whose brain volumes had increased before the closure of the cranial sutures. The CT images of all dogs showed the dilatations of the cerebral ventricles in their entire size. Examination of the cerebrospinal fluid did not yield uniform findings. Consequently, EEG, conventional diagnostic radiography and computed tomography of the skull are the most important studies for diagnosing a primary hydrocephalus internus. However, the total extent of the lesion can be confirmed only by computed tomography. This is of special interest in case of planning and controlling therapies.
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PMID:[Congenital hydrocephalus internus of the dog: clinical, computed tomographic and pathomorphological features of six cases]. 1924 28

We report a case of a 22-year-old man presenting with a fourth ventricular tumour and associated chronic obstructive hydrocephalus likely leading to thinning of the cranium. Intraoperatively, he developed an epidural hematoma secondary to a fracture of the temporal bone by application of the three-point skull fixator. This is the second reported adult case of an iatrogenic epidural hematoma secondary to pin-site complications. We advocate the careful placement of the pins and suggest the tightening force be catered individually especially in pediatric patients or patients with chronic hydrocephalus.
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PMID:Pin-site epidural hematoma in an adult case of chronic hydrocephalus with associated thinning of the cranium. 1930 83

The Pak4 serine/threonine kinase regulates cytoskeletal organization, and controls cell growth, proliferation, and survival. Deletion of Pak4 in mice results in embryonic lethality prior to embryonic day 11.5. Pak4 knockout embryos exhibit abnormalities in the nervous system, the heart, and other tissues. In this study a conditional deletion of Pak4 was generated in order to study the function of Pak4 in the development of the brain. Nervous system-specific conditional deletion of Pak4 was accomplished by crossing mice with a floxed allele of Pak4 with transgenic mice expressing Cre recombinase under the control of the nestin promoter. The conditional Pak4 knockout mice were born normally, but displayed growth retardation and died prematurely. The brains showed a dramatic decrease in proliferation of cortical and striatal neuronal progenitor cells. In vitro analyses revealed a reduced proliferation and self-renewing capacity of neural progenitor cells isolated from Pak4 knockout brains. The mice also exhibited cortical thinning, impaired neurogenesis and loss of neuroepithelial adherens junctions. By the time the mice died, by 4weeks after birth, severe hydrocephalus could also be seen. These results suggest that Pak4 plays a critical role in the regulation of neural progenitor cell proliferation and in establishing the foundation for development of the adult brain.
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PMID:A key role for Pak4 in proliferation and differentiation of neural progenitor cells. 2138 68

Cervical myelomeningocele (MMC) is an uncommon congenital malformation of the spinal cord and accounts for a small proportion of neural tube defects. These lesions mostly occur in the dorsal part of the body. Only a single case of an anterior cervical MMC has been previously reported. The authors report a second case of anterior cervical MMC diagnosed when the patient began to experience symptoms of bilateral hand weakness in adulthood. In this patient, MR imaging of the cervical spine showed an anterior cervical MMC at the C6-7 level with hydrocephalus, thinning of the genu and trunk of the corpus callosum, maldevelopment of the cerebellar tonsils, and expansion of the fourth ventricle, posterior cranial fossa, and subarachnoid space. A CT scan and a 3D CT reconstruction of the cervical spine clearly demonstrated contiguous fusions of multiple lower-cervical vertebrae and neural arches, which was consistent with Type III Klippel-Feil syndrome. The patient was advised to undergo operative treatment to prevent the progression of her neurological deficit. However, after being notified of the potential neurological risks, the patient declined surgery and opted for conservative treatment with a hard neck collar. At 4 months' follow-up, the patient's neurological deficit remains stable with the MMC left untreated. The authors presume that the possible pathogenesis of anterior cervical MMC may greatly differ from that of posterior lesions. This lesion could also be associated with multiple other spinal abnormalities, which highlights the importance of comprehensive preoperative radiological examinations.
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PMID:Anterior cervical myelomeningocele: a rare malformation of the spinal cord. 2215 Feb 40

The Ro1 model of hydrocephalus represents an excellent model for studying the pathogenesis of hydrocephalus due to its complete penetrance and inducibility, enabling the investigation of the earliest cellular and histological changes in hydrocephalus prior to overt pathology. Hematoxylin and eosin staining, immunofluorescence and electron microscopy were used to characterize the histopathological events of hydrocephalus in this model. Additionally, a broad battery of behavioral tests was used to investigate behavioral changes in the Ro1 model of hydrocephalus. The earliest histological changes observed in this model were ventriculomegaly and disorganization of the ependymal lining of the aqueduct of Sylvius, which occurred concomitantly. Ventriculomegaly led to thinning of the ependyma, which was associated with periventricular edema and areas of the ventricular wall void of cilia and microvilli. Ependymal denudation was subsequent to severe ventriculomegaly, suggesting that it is an effect, rather than a cause, of hydrocephalus in the Ro1 model. Additionally, there was no closure of the aqueduct of Sylvius or any blockages within the ventricular system, even with severe ventriculomegaly, suggesting that the Ro1 model represents a model of communicating hydrocephalus. Interestingly, even with severe ventriculomegaly, there were no behavioral changes, suggesting that the brain is able to compensate for the structural changes that occur in the pathogenesis of hydrocephalus if the disorder progresses at a sufficiently slow rate.
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PMID:Morphological and behavioral changes in the pathogenesis of a novel mouse model of communicating hydrocephalus. 2229 10

When considering the underlying pathophysiological mechanisms involved in idiopathic normal pressure hydrocephalus (iNPH), white matter is often the main locus of investigation. However, when an axon in the brain is damaged, degeneration of the neuron can occur proximally (dying back) and Alzheimer's disease (AD), associated with cortical thinning, is a common pathologic comorbidity with iNPH. We investigated differences in cortical thickness between CSF tap test (CSFTT) responders and non-responders in iNPH patients and compared patterns of cortical thickness in iNPH patients with that of AD patients. Thirty-two iNPH patients (16 CSFTT responders and 16 CSFTT non-responders) and 16 AD patients were imaged with MRI, including 3-dimensional volumetric images for cortical thickness analysis across the entire brain. Among the iNPH patients, CSFTT non-responders, when compared to responders, had statistically significant cortical thinning in the left superior frontal gyrus at the level of a false discovery rate (FDR) p<0.05, and tended to show widespread cortical thinning in most areas of the brain. Relative to the CSFTT responders, AD patients showed statistically significant cortical thinning in superior and medial frontal gyrus, left precentral gyrus, postcentral gyrus, paracentral lobule, precuneus, and superior parietal lobule after FDR correction (p<0.05). However, comparing patterns of cortical thinning between AD patients and CSFTT non-responders revealed no statistically significant differences. Differences in cortical thickness correlated with CSFTT response for iNPH patients may indicate a possibility for considering patterns of cortical thinning in patients with ventriculomegaly as potential brain imaging markers for the prediction of CSFTT responders. And, our findings suggest that comorbid AD pathology might be related to the cortical thinning patterns found in CSFTT non-responders. Larger studies, using normal controls and combinations of other biomarkers associated with AD, would be necessary to evaluate these hypotheses.
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PMID:Idiopathic normal-pressure hydrocephalus, cortical thinning, and the cerebrospinal fluid tap test. 2393 54

Optic nerve hypoplasia (ONH) is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65%) than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED).
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PMID:Optic nerve hypoplasia. 2408 63

Thickening of the corpus callosum is an important feature of development, whereas thinning of the corpus callosum can be the result of a number of diseases that affect development or cause destruction of the corpus callosum. Corpus callosum thickness reflects the volume of the hemispheres and responds to changes through direct effects or through Wallerian degeneration. It is therefore not only important to evaluate the morphology of the corpus callosum for congenital anomalies but also to evaluate the thickness of specific components or the whole corpus callosum in association with other findings. The goal of this pictorial review is raise awareness that the thickness of the corpus callosum can be a useful feature of pathology in pediatric central nervous system disease and must be considered in the context of the stage of development of a child. Thinning of the corpus callosum can be primary or secondary, and generalized or focal. Primary thinning is caused by abnormal or failed myelination related to the hypomyelinating leukoencephalopathies, metabolic disorders affecting white matter, and microcephaly. Secondary thinning of the corpus callosum can be caused by diffuse injury such as hypoxic-ischemic encephalopathy, human immunodeficiency virus (HIV) encephalopathy, hydrocephalus, dysmyelinating conditions and demyelinating conditions. Focal disturbance of formation or focal injury also causes localized thinning, e.g., callosal dysgenesis, metabolic disorders with localized effects, hypoglycemia, white matter injury of prematurity, HIV-related atrophy, infarction and vasculitis, trauma and toxins. The corpus callosum might be too thick because of a primary disorder in which the corpus callosum finding is essential to diagnosis; abnormal thickening can also be secondary to inflammation, infection and trauma.
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PMID:Corpus callosum thickness in children: an MR pattern-recognition approach on the midsagittal image. 2517 5


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