Hydrocephalus

Hydrocephalus may be an acquired or congenital condition. Clinical signs often reflect the level of brain involvement. In young dogs, the presence of a dome-shaped head and/or persistent fontanel are suggestive of hydrocephalus. Ventriculoperitoneal shunting is often used for definitive treatment of hydrocephalus.     

Attention processes in children with shunted hydrocephalus versus attention deficit-hyperactivity disorder.

Children with congenital hydrocephalus, children with attention deficit-hyperactivity disorder, and normal controls were evaluated with measures of focused attention (Visual Orienting and Detection Task), sustained attention (continuous performance test), and attention shifting (Wisconsin Card Sorting Test). Components from these tasks have been linked to attention systems mediated by anterior or posterior brain networks. Children with congenital hydrocephalus showed an inability to focus and shift attention, which specifically implicated impairment of the disengage and move components of the posterior brain attention system. Children with attention deficit- hyperactivity disorder displayed the expected performance patterns on measures of focused attention once their difficulties with sustained attention were taken into account. However, they showed problems with shifting and sustaining attention, which are commonly associated with the anterior brain attention system. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Frontal foramina in pediatric skull in cases of congenital hydrocephalus

PURPOSE: To describe a new observation, frontal calvarial foramina, in pediatric patients with congenital hydrocephalus secondary to central nervous system malformation. MATERIALS AND METHODS: Frontal foramina were initially identified in three female patients with Chiari II malformation. Subsequently, head computed tomographic (CT) scans in 99 patients with congenital hydrocephalus were retrospectively reviewed. CT scans in a control group of 116 patients without hydrocephalus were also retrospectively reviewed. RESULTS: Frontal foramina were found in eight of 61 (13%) patients with Chiari II malformation, in one child with Dandy-Walker malformation, and in one child with occipital horn dilatation (colpocephaly), but not in control patients. Sequential CT examinations in three patients with frontal foramina depicted gradual closure after ventriculoperitoneal shunt placement. CONCLUSION: Frontal foramina may represent an abnormality variably expressed in certain central nervous system malformations that cause congenital hydrocephalus. The presence of frontal foramina palpated or visualized on plain radiographs may help in the diagnosis of congenital hydrocephalus and central nervous system malformation.     

Cranial axial computerized tomography in children (author's transl)

This report on computerized tomography in 400 children confirms the high diagnostic value of the new method in neuropediatrics. 31 brain tumours were diagnosed in children. Tumors present themselves as lesions of latered density compared to that of brain matter. Intravenous injection of contrast media increases the density of various lesions as tumors, angiomas, acute infarctions and abscess. Infarctions are low density lesions whereas hemorrhage is a high density lesion and both are thus safely differentiated. Edema may be visualized in the vicinity of tumours in brain injuries and in encephalitis. The normal ventricular system, hydrocephalus, cerebral malformations and subdural hygroma are easily demonstrated. Diseases of the orbits may also be detected.     

Effect of hyperosmotic solutions on human brain tumour vasculature

Reversible opening of the blood-brain barrier (BBB) has been used to increase delivery of chemotherapeutic agents into brain tumours, but it is complicated and requires general anaesthesia. Without affecting the normal BBB, and avoiding the complications of BBB modification by hyperosmotic solution, we tried an adequate minimal BBB disruption in brain tumours. Although the effect of BBB disruption on normal brain has been described, there are no reports of the effect of an impaired BBB on microcirculation. In this study, four patients underwent surgical resection of a glioblastoma multiforme (GM; n = 1), astrocytoma (n = 2), or metastatic brain tumour (n = 1). Epicerebral microcirculation was observed in the operative field. Serial fluorescein microangiograms of the tumour and peritumoural area were obtained before and after BBB disruption was introduced intra-operatively by retrograde infusion of mannitol introducing a catheter via the temporal superficial artery back to the carotid bifurcation. On the initial microangiogram, staining by the fluorescein dye was observed in the GM and metastatic tumour but not in the astrocytoma; no extravasation of fluorescein dye was observed in the peritumoural areas. After BBB disruption, fluorescein perfusion increased and extravasation of fluorescein dye from the venules was observed in the GM and the metastatic tumour and in the peritumoural area of both lesions; BBB disruption started from venules in the peritumoural area without affecting the normal brain. However, such effects were not observed in the astrocytomas after BBB disruption nor in normal brain tissue in any patient. It appears that the integrity of the BBB is less stable in the peritumoural area of GM and metastatic brain tumours than it is in astrocytomas or normal brain. Osmotic BBB disruption may offer a method for achieving global delivery of therapeutic agents to brain tumours and peritumoural areas.     

Genuine giant-cell tumour of bone; a combined cytological, histopathological and ultrastructural study

Three patients with a giant-cell tumour of bone were studied clinically, roentgenologically and morphologically, using cytological, histopathological and electron microscopic methods. The tumours were composed of giant cells possessing a great number of mitochondria and stromal cells exhibiting prominent endoplasmic reticulum and Golgi complex. Mitotic activity was only found among the stromal cells. Autophagic vacuoles, lysosomal bodies and degenerative changes of varying severity were found mainly or exclusively among the giant cells, which may indicate that these cells represent an involutionary form of tumour cell. The giant-cell tumours of bone are believed to arise from undifferentiated cells of the bone marrow. Fine-needle aspiration biopsy represents a valuable complement to histopathological examination in the identification of giant cell lesions and diagnosis of genuine cell tumours of bone.     

Whole-body [18F]FDG PET in the management of metastatic brain tumours

BACKGROUND: To determine its roles in the diagnosis and the systemic evaluation of metastatic brain tumours, whole-body positron emission tomography (PET) using [18F]FDG was performed in 20 consecutive patients. METHODS: All patients were thought to be suffering or needing to be differentiated from metastatic brain tumours. Nine patients had multiple brain lesions; six were older and showed a rim-enhancing lesion with surrounding oedema; seven had homogeneously enhancing periventricular lesion(s) on computed tomography (CT) and/or magnetic resonance (MR) imaging, thought to be central nervous system lymphomas. Two patients had skull mass(es) and two patients had a solid mass suspected to be, respectively, a haemorrhagic metastasis and a metastatic malignant melanoma. All of them received whole-body [18F]FDG PET and conventional systemic work-up for metastasis in order to compare the results of the two methods. RESULTS: Metastatic brain tumours were diagnosed on whole-body [18F]FDG PET in eleven patients who had extracranial and intracranial hypermetabolic lesions. In nine of these, a conventional work-up also detected primary lesions which on whole-body [18F]FDG PET were seen to be hypermetabolic foci. Systemic lymph node metastases were detected by whole-body [18F]FDG PET only in two patients and histological diagnosis was possible by biopsy of lymph nodes rather than of brain lesions. In the remaining nine patients who had only intracranial hypermetabolic foci, histological diagnosis was made by craniotomy or stereotactic biopsy. It was confirmed that seven of nine patients were suffering from a primary brain tumour and two from metastatic carcinoma. None of the nine showed evidence of systemic cancer on conventional work-up. Histological diagnoses of the primary brain tumours were four cases of primary central nervous system lymphoma and one each of multifocal glioblastoma, Ewing's sarcoma, and cavernous angioma. Patients felt no discomfort during the whole-body [18F]FDG PET procedure and there were no complications. The false negative rate in [18F]FDG PET and in conventional work-up was 15.4% and 30.7% respectively. There were no false positives on either [18F]FDG PET or conventional work-up. CONCLUSION: It is suggested that whole-body [18F]FDG PET is a safe, reliable, and convenient method for the diagnosis and systemic evaluation of patients thought to be suffering or needing to be differentiated from a metastatic brain tumour.     

MOS-SF-36 in evaluating health-related quality of life in alcohol-dependent patients

OBJECT: It is possible to diagnose hydrocephalus prenatally based on the morphological appearance of the fetus on neurodiagnostic images; however, the prognosis of this disease shows wide variation. The authors previously proposed a classification system for the prediction of postnatal outcome based on progression of hydrocephalus and affected brain development, known as the "Perspective Classification of Congenital Hydrocephalus (PCCH)." In this study the authors have used their classification system to analyze long-term follow-up results obtained in each clinicoembryological stage of fetal hydrocephalus. METHODS: Sixty-one fetuses with hydrocephalus were examined to predict postnatal outcome by using this newly developed classification. The authors' recently developed method of using heavily T2-weighted imaging with a superconducting magnet clearly delineated the cerebrospinal fluid (CSF) space and the malformed brain and spinal cord. Imaging was achieved in less than 1 second per slice and required no sedation of the fetus. The technique appears to be simple and good at delineating intrauterine anatomy. Hydrocephalus was diagnosed in two fetuses at PCCH embryological Stage I (8-21 gestational weeks), in 28 fetuses at Stage II (22-31 weeks), and in 31 fetuses at Stage III (32-40 weeks). Among these 61 fetuses, clinicopathological typing showed that 19 had primary hydrocephalus (nine in Stage II and 10 in Stage III), 34 had dysgenetic hydrocephalus (two in Stage I, 16 in Stage II, and 16 in Stage III), and eight had secondary hydrocephalus (three in Stage II and five in Stage III). When the hydrocephalic state developed during PCCH Stage I or II, the prognosis was very poor, and only one of 18 fetuses with dysgenetic hydrocephalus and none of three fetuses with secondary hydrocephalus had an acceptable postnatal outcome. Even within the same category or subtype of fetal hydrocephalus, such as primary hydrocephalus in its simple form, or hydrocephalus with spina bifida aperta (myeloschisis), the postnatal outcomes differed depending on the time of onset of hydrocephalus. When the diagnosis of hydrocephalus was made during PCCH Stage II, the fetuses had a poorer postnatal outcome compared with those at Stage III (p < 0.05). CONCLUSIONS: It is emphasized that postnatal prognosis is not simply a function of the form of the diagnosis but is also dependent on the progression of hydrocephalus and the degree to which that process affects neuronal development. Early decompressive procedures, conventionally performed after but, hopefully, performed before birth, are indicated to obtain the optimal postnatal prognosis of fetuses with hydrocephalus diagnosed at PCCH Stage II.     

Hydrocephalus in children: neurosurgical and neuroimaging concerns

The neuroimaging of hydrocephalus from the perspective of the pediatric neurosurgeon is discussed. Processes with new developments in therapy and imaging are described, including congenital causes of hydrocephalus, unilateral hydrocephalus, trapped fourth ventricle, and benign external hydrocephalus.     

Heterologous antibodies in human and rat's glioblastoma

IgG from immunized rabbits was labelled with Fluorescein Isothiocyanate, and conjugated in vitro with human and with rat glioblastoma. After absorption of the antisera with normal brain and liver, a heavy concentration of antibody was found in the rat tumour cells. A great amount of antibody was also found in the human neoplastic cells. The incorporation of this antibody in glioma cells of different rats developing new tumours indicates that transplantation antigens of the H-2 type are not the only ones capable of inducing specific antibody attachment.     

Desmoplastic tumors with multiple differentiation. A new entity. Six cases

Desmoplastic tumours with divergent differentiation are principally located in the abdomen and develop locally or regionally. They occur in adolescents or young adults and are characterized at histology by a proliferation of undifferentiated small cells surrounded by a dense stroma. Only immuno-histo-chemistry provides the diagnosis. Since their chemosensitivity is rare and often partial the outcome is usually lethal. Six new cases of this recently described entity are presented here. The authors are in favour of a multidisciplinary and aggressive management, combining intensive polychemotherapy, extensive surgical exerisis and total abdominal radiotherapy.     

Telemedicine in the care of the end-stage renal disease patient

Juvenile pleomorphic adenoma of the parotid gland represents an extremely rare tumour entity and is comparable to congenital tumours of the salivary glands concerning its embryonal structure. The clinical detection of the tumour in a 7-year-old girl does not exclude that the tumour had developed either earlier or immediately after the birth. The high cellularity and the evidence of primitive epithelial and myoepithelial cellular structures do not justify its classification as a malignant tumour. However the presence of embryonal tissue structures associated with the end of the third month of embryogenesis is characterized by more solid cell formations in partly verticulate arrangement. The absence of further differentiation into lobular structures and differentiated duct or acinic cell formations may be due to cell arrest. Differential diagnosis of juvenile pleomorphic adenoma must distinguished it from other congenital salivary gland tumours (e.g. congenital basal cell adenoma, hybrid basal cell adenoma-adenoid cystic carcinoma, sialoblastoma, salivary gland anlage tumour.     

MR of the normal neonatal brain: assessment of deep structures

BACKGROUND AND PURPOSE: Recent work has shown that up to 50% of patients with congenital muscular dystrophies (CMDs) have abnormalities of the brain that can be detected by brain MR imaging. We attempted to determine whether brain MR imaging is useful for the diagnosis and classification of patients with CMDs. METHODS: The brain MR studies of 12 patients with biopsy-proved CMDs were reviewed retrospectively. Using information available in the literature regarding associated brain anomalies as a guide, an attempt was made to classify the patients in terms of "pure" CMD, CMD with occipital agyria, Fukuyama CMD, muscle-eye-brain disease, or Walker-Warburg syndrome. RESULTS: All the patients were easily classified into one of four groups: pure CMD (four patients), Fukuyama CMD (four patients), muscle-eye-brain disease (two patients), or Walker-Warburg syndrome (two patients). Patients with pure CMD had diffuse central cerebral hypomyelination with mild pontine and cerebellar hypoplasia. Patients with Fukuyama CMD had diffuse central cerebral hypomyelination, cerebellar polymicrogyria (with or without cysts), frontal polymicrogyria, a variable degree of hypoplasia of the pons and cerebellar vermis, and a variable occipital cobblestone cortex. Patients with muscle-eye-brain disease had cerebellar polymicrogyria (with or without cysts), absence of the septum pellucidum, diffuse cerebral cortical dysplasia, pontine and cerebellar vermian hypoplasia, patchy hypomyelination, and variable callosal hypogenesis and hydrocephalus. Patients with Walker-Warburg syndrome had diffuse cerebral cobblestone cortex, absence of cerebral and cerebellar myelin, cerebellar polymicrogyria (with or without cysts), pontine and cerebellar vermal hypoplasia, hydrocephalus, and variable callosal hypogenesis. CONCLUSION: MR imaging shows distinctive brain anomalies that allows patients with CMD to be classified into four distinct groups that are consistent with known disorders.     

Autosomal recessive hydrocephalus with aqueductal stenosis

We report the case histories of three sisters with congenital hydrocephalus associated with stenosis of the cerebral aqueduct. The parents were a young consanguineous couple. In two cases hydrocephalus was detected before birth by ultrasonography. We consider these three cases to be of the rare autosomal form of hereditary hydrocephalus.     

Sustained attention in children with two etiologies of early hydrocephalus.

Several studies have shown that children with spina bifida meningomyelocele (SBM) and hydrocephalus have attention problems on parent ratings and difficulties in stimulus orienting associated with a posterior brain attention system. Less is known about response control and inhibition associated with an anterior brain attention system. Using the Gordon Vigilance Task (Gordon, 1983), we studied error rate, reaction time, and performance over time for sustained attention, a key anterior attention function, in 101 children with SBM, 17 with aqueductal stenosis (AS; another condition involving congenital hydrocephalus), and 40 typically developing controls (NC). In SBM, we investigated the relation between cognitive attention and parent ratings of inattention and hyperactivity and explored the impact of medical variables. Children with SBM did not differ from AS or NC groups on measures of sustained attention, but they committed more errors and responded more slowly. Approximately one-third of the SBM group had attention symptoms, although parent attention ratings were not associated with task performance. Hydrocephalus does not account for the attention profile of children with SBM, which also reflects the distinctive brain dysmorphologies associated with this condition. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

The origin of experimental brain tumours: a sequential study

A sequential study of rat brains treated transplacetally with the neurotropic carcinogen ethylnitrosourea reveals small foci of cell proliferations from the age of 8 weeks. These lesions consist mainly of undifferentiated cells of the subependymal plate type. They occur in those areas in which gliomas develop and represent the earliest, histologically detectable, changes in the development of brain tumours.     

Cyclin D1, another molecule of the year?

AIM: Testicular germ cell tumours may present as metastases in cervical lymph nodes, yet the primary tumours remain clinically occult. The aim of the study is to alert pathologists and clinicians to this uncommon but important presentation and highlight the clues and the diagnostic adjuncts to its correct diagnosis. METHODS: The clinical, cytological, histological, and immunohistochemical features of two patients with germ cell tumour initially presenting as cervical lymphadenopathy were described and analysed. RESULTS: Both patients were young adult males, who were found to have metastatic undifferentiated carcinoma on fine needle aspiration of the enlarged cervical lymph nodes. The tumour cells in both cases were positive for placental alkaline phosphatase (PLAP) and negative for epithelial membrane antigen (EMA). CONCLUSIONS: Clinicians and pathologists should be aware of the possibility of germ cell tumour when encountering a young adult male with metastatic poorly differentiated carcinoma. Positivity for PLAP and negativity for EMA are helpful adjuncts in arriving at the correct diagnosis.     

Hydrocephalus in one twin after massive reverse flow in the umbilical artery in the 2nd trimester

The case of a twin pregnancy with severe diastolic reverse flow in the umbilical artery of one of the twins at 23 weeks' gestation is reported. After delivery at 29 weeks' gestation, a congenital internal hydrocephalus was diagnosed in this twin. The most probable aetiology is an intrauterine periventricular haemorrhage. The severe diastolic reverse flow in the umbilical artery seems to represent a highly pathological flow pattern of prefinal degree, possibly leading to cerebral defects. The pathophysiology of the Doppler findings and the pathogenesis of the internal hydrocephalus are discussed.     

Prenatal diagnosis of orbital heterotopic brain tissue

Biopsy of fetal tissues is a relatively new procedure for the diagnosis of congenital malformations. The authors report the first case in which this technique has been applied to an orbital mass, in which heterotopic brain tissue was diagnosed by prenatal biopsy and excised in infancy. The wider implications of such intrauterine procedures are discussed.     

Space-Based Inhibition of Return in Children With Spina Bifida.

Inhibition of return (IOR) refers to an increase in time to react to a target in a previously attended location. Children with spina bifida meningomyelocele (SBM) and hydrocephalus have congenital dysmorphology of the midbrain, a brain region associated with the control of covert orienting in general and with IOR in particular. The authors studied exogenously cued covert orienting in 8- to 19-year-old children and adolescents (84 with SBM and 37 age-matched, typically developing controls). The exogenous cue was a luminance change in a peripheral box that was 50% valid for the upcoming target location. Compared with controls, children with SBM showed attenuated IOR in the vertical plane, a deficit that was associated with midbrain dysmorphology in the form of tectal beaking but not with posterior brain volume loss. The data add to the emerging evidence for SBM deficits in attentional orienting to salient information. (PsycINFO Database Record (c) 2010 APA, all rights reserved)