Hydrocephalus secondary to diffuse villous hyperplasia of the choroid plexus. Case report and review of the literature

Diffuse villous hyperplasia of the choroid plexus, which is distinct from bilateral choroid plexus papillomas, is extremely rare and is often associated with hydrocephalus due to the overproduction of cerebrospinal fluid (CSF). The authors describe an infant with hydrocephalus, diagnosed by computerized tomography scanning, who developed ascites following placement of a ventriculoperitoneal shunt and, subsequently, demonstrated excessive CSF production when the shunt was externalized. The patient was later successfully treated by placement of a ventriculoatrial shunt. Magnetic resonance imaging demonstrated diffuse villous hyperplasia of the choroid plexus as the cause of the patient's hydrocephalus. The literature on diffuse villous hyperplasia of the choroid plexus is reviewed.     

Nocardia abscess of the choroid plexus: clinical and pathological case report

OBJECTIVE: Cerebral Nocardia abscesses are rare, accounting for approximately 1 to 2% of all cerebral abscesses. Prompt aggressive surgical treatment involving craniotomy and excision of these lesions has been advocated by many authors, because these lesions have significantly higher morbidity and mortality rates than do most other cerebral abscesses. We report an atypical presentation of cerebral nocardiosis localized to the choroid plexus of the lateral ventricle. CLINICAL PRESENTATION: A 56-year-old man presented with a 3-week history of fever, cough, and progressive headache and an ensuing 3-day history of progressive lethargy, confusion, and gait ataxia. Radiographic studies demonstrated a loculated contrast-enhancing left lateral ventricular lesion with significant perilesional parenchymal edema that was thought preoperatively to be a neoplasm. INTERVENTION: The patient underwent a craniotomy for resection of the lesion. Intraoperatively, a reddish gray lesion with purulent exudate was encountered within the left lateral ventricle intimately adherent to the choroid plexus as well as to the ependyma and subependymal veins. A frozen section demonstrated an organizing abscess wall. The lesion was resected in its entirety, and multiple cultures were sent for analysis. CONCLUSION: Microbiology cultures grew Nocardia asteroides. A course of intravenous antibiotics was started, which included trimethoprim-sulfamethoxazole, amikacin, and ceftriaxone. Two weeks after surgery, at the time of discharge, the patient's neurological status had improved considerably. Although Nocardia abscesses have been documented to occur throughout the central nervous system, the presentation of a lesion confined to the choroid plexus of the lateral ventricle with significant parenchymal edema is unusual and demonstrates that Nocardia abscesses must be considered in the differential diagnosis of a contrast-enhancing intraventricular mass lesion involving the choroid plexus.     

Diffuse craniospinal seeding from a benign fourth ventricle choroid plexus papilloma. Case report

Choroid plexus papillomas can metastasize to the subarachnoid space, but extensive metastasis has only been reported when the tumors are malignant. The authors report a case of diffuse, extensive metastasis to the craniospinal leptomeninges from a benign fourth ventricular choroid plexus papilloma in an adult. This 19-year-old woman presented with a 2-year history of headache, blurred vision, diplopia, and ataxia. Magnetic resonance imaging of the brain and spinal cord revealed obstructive hydrocephalus caused by a 4-cm, partially calcified, inhomogeneously enhancing tumor of the fourth ventricle that was displacing the pons, medulla oblongata, and cerebellum. Innumerable cystic lesions of varying size were also seen in the cranial and spinal leptomeninges. Histological examination of the resected fourth ventricular tumor and of a few of the leptomeningeal lesions revealed a benign choroid plexus papilloma and leptomeningeal choroid plexus cysts. This singular case of diffuse and extensive metastasis to the craniospinal leptomeninges from a histologically benign fourth ventricular papilloma adds to the available information about the biological potential of these tumors and expands the differential diagnosis of posterior fossa lesions with subarachnoid metastasis.     

The effects of acute phencyclidine treatment on neuropeptide Y (NPY) neuronal system in the rat arcuate nucleus studied by immunocytochemistry and in situ hybridization

The clinical, radiological and pathological findings of four cases of primary intraventricular haemorrhage secondary to choroid plexus arteriovenous malformations (AVM) are described and the relevant literature reviewed. In three of the cases the diagnosis was confirmed or made at autopsy. The fourth case survived to undergo craniotomy followed by radiosurgery with excellent results. All AVMs originated in the choroid plexus of the lateral ventricle, and autopsy confirmation required a high degree of suspicion and the systematic microscopic examination of serial coronal sections of the ventricle with the clot in situ.     

Choroid plexus cyst of the left lateral ventricle with intermittent blockage of the foramen of Monro, and initial invagination into the III ventricle in a child

A cyst of the choroid plexus of the left lateral ventricle with intermittent blockage of the foramen of Monro and initially with invagination of the III ventricle in a child is described. In a 6-week-old boy a ventriculoatrial shunt was implanted for correction of an active asymmetrical hydrocephalus of unknown origin. When he was 3 months of age a water-soluble contrast CT ventriculography revealed a noncolloid cyst localised predominantly in the upper portion of the III ventricle. At that time the ventricular catheter obstructed with choroid plexus was removed; new bilateral catheters in a parieto-occipital region were implanted. In the course of the next 4 years, first the atrial catheter had to be extracted and then the peritoneal catheter was changed, in both cases because of obstruction. Periods of normal life alternated with periods of transient and intermittent symptoms of increased intracranial pressure, papilloedema, and myoclonic jerks. Repeated computed tomography (CT) and magnetic resonance imaging (MRI) showed stabilised hydrocephalus with an enlarged left lateral ventricle. When the boy was 16 years old MRI revealed a choroid plexus cyst in the left lateral ventricle 2 cm in diameter, with a ballvalve type of obstruction of the foramen of Monro. CT stereoendoscopic resection of the wall of a large cyst filled with cerebrospinal fluid was performed, and two additional adnexal small cysts were coagulated using the bipolar coagulator, Diomed 25 laser and scissors; the symptoms then regressed, except for superior bilateral altitudinal anopsia. Light and electron microscopy of the cyst wall is reported. The cyst was composed of collagenic connective tissue lined with a basal lamina lacking in epithelial cells. The preoperative and postoperative MRI are presented. Choroid plexus cysts localised in the anterior part of lateral ventricles are very rare, and all reported cases have been in male patients. According to the literature our case is only the third ever described in a child.     

Transchoroidal approach to the third ventricle: an anatomic study of the choroidal fissure and its clinical application

OBJECTIVE: We review the anatomic features of the lateral ventricle, the foramen of Monro, the third ventricle, and the choroidal fissure, and we describe the transchoroidal approach to the third ventricle. This approach consists of opening the taenia fornicis of the choroidal fissure in the body of the lateral ventricle and approaching the third ventricle between the two internal cerebral veins. This route allows further posterior enlargement of the foramen of Monro without sacrificing any neural structures. When necessary, the anterior septal vein can be sacrificed. METHODS: Twenty adult cadaveric brains and four adult cadaveric heads were studied, using a magnification ranging from 3 times to 40 times, after perfusion of the arteries and veins with colored latex. RESULTS: The choroidal fissure is a natural cleft between the thalamus and the fornix, and it is identified by following the choroid plexus in the lateral ventricle. The choroid plexus in the body of the lateral ventricle originates from the tela choroidea of the roof of the third ventricle and is apparently attached to the fornix by the taenia fornicis and to the thalamus by the taenia choroidea. The taenia is actually the ependyma that covers the internal wall of the ventricular cavity and the choroid plexus. CONCLUSION: An understanding of the choroidal fissure is fundamental for use of the transchoroidal approach. Unlike transforaminal, subchoroidal, subforniceal, and interforniceal approaches to the third ventricle, which sacrifice some neural or vascular structures, the transchoroidal approach follows a natural route, and certainly it is one of the options to be considered when entry into the third ventricle is required.     

Differential distribution of nicotinamide adenine dinucleotide phosphate-diaphorase and neural nitric oxide synthase in the rat choroid plexus. A histochemical and immunocytochemical study

This study used NADPH diaphorase (NADPHd) histochemistry and neuronal nitric oxide synthase immunocytochemistry to examine the localization of nitric oxide synthase in the choroid plexus of the lateral ventricles and the fourth ventricle of rat brain. That the NADPHd reaction product in choroid plexus was specific to nitric oxide synthase was evaluated: (i) by comparison to immunocytochemical labelling for nitric oxide synthase; and (ii) by comparing NADPHd histochemical staining in choroid plexus and brain (rich in nitric oxide synthase-positive and NADPHd-positive neurons) in the presence or absence of iodonium diphenyl or dichlorophenolindophenol, two potent albeit non-selective inhibitors of nitric oxide synthase activity. In brain, NADPHd histochemistry homogeneously stained neuronal cell bodies, axons and dendrites, while it produced particulate cytoplasmic staining of all epithelial cells in the choroid plexuses of the lateral and fourth ventricles. Within the choroid plexus of the lateral ventricles, NADPHd-positive nerve fibres were also observed around blood vessels and coursing among the epithelial cells. The distribution of immunoreactivity for nitric oxide synthase in brain and in nerve fibres in the choroid plexuses of the lateral ventricles resembled the distribution of histochemical labelling for NADPHd. Choroid plexus epithelial cells were, however, devoid of nitric oxide synthase immunoreactivity. Consistent with this, iodonium diphenyl and dichlorophenolindophenol (0.1 mM) inhibited NADPHd histochemical staining in brain neurons and in choroid plexus nerve fibres, but not in choroid plexus epithelial cells. These results demonstrate that the choroid plexus of the lateral ventricles in rat brain is innervated by nitric oxide synthase-positive nerve fibres. These nitric oxide synthase-positive nerve fibres may have an important role in the regulation of cerebrospinal fluid balance. Although choroid plexus epithelial cells contain an enzyme with NADPHd activity, this enzyme is not nitric oxide synthase.     

Involvement of phospholipids in apolipoprotein B modification during low density lipoprotein oxidation

Metastatic tumors to the choroid plexus are rare. We report a case of renal cell carcinoma metastasizing to the choroid plexus of the lateral ventricular atrium. This tumor was shown by magnetic resonance imaging, followed by craniotomy and histologic confirmation. Computed tomography of the abdomen showed the primary tumor. We also reviewed 40 years of the literature and found 14 cases of metastasis to the choroid plexus. We discuss the clinical features of this intraventricular metastasis.     

Primary choroid plexus papilloma of the foramen magnum--case report

A 50-year-old male presented with a choroid plexus papilloma in the foramen magnum manifesting as dysesthesia in the right hand and severe headache. Magnetic resonance imaging clearly showed that the tumor was located in the cerebellomedullary cistern, without extension into the fourth ventricle. However, differentiation from hemangioblastoma or foramen magnum tumor was difficult by neuroimaging. Intraoperative observation found the tumor was located extraventricularly and attached to the choroid plexus of the foramen of Magendie. The tumor was grossly totally resected. Histological examination proved the tumor was a choroid plexus papilloma without malignancy. His neurological deficits resolved almost completely.     

Simplified assessment of asphyxia at birth

A choroid plexus papilloma of the lateral ventricle presenting in early infancy and producing symptoms purely by secretion of cerebrospinal fluid with formation of a large cyst is presented. Initial conservative management by cyst-peritoneal shunt was followed by late recurrence of symptoms due to reformation of the cyst when the shunt blocked. Excision of the tumour afforded definitive relief.     

Lipofuscin pigment in cerebellar Purkinje neurones and choroid plexus epithelial cells of macaque monkeys with Plasmodium knowlesi cerebral malaria: an electron microscopical observation

Experimental infection of rhesus monkeys (Macaca mulatta) with a virulent (W1) strain of Plasmodium knowlesi resulted in cerebral malaria. Electron microscopical examination of the brain revealed large numbers of intracytoplasmic lipofuscin pigment deposits in cerebellar Purkinje neurones and choroid plexus epithelium of the lateral ventricle. This lesion may be part of the nervous system response to ischaemic hypoxia.     

The microvascularization of the choroid plexus of the lateral ventricles in the goat (Capra hircus)

The goat choroid plexus angioarchitecture of the lateral ventricles was studied under the SEM using the method of "microvascular corrosion casts". The whole plexus is semilunar shaped and directed in an antero-posterior, latero-median fashion. In the plexus the lateral extremity is larger than the median one. All the components of the vascular bed (arteries, veins and capillaries) of the choroid plexus have interesting morpho-structural features. In particular, the capillaries are more developed than the other components and they are variously located on both sides of the plexus. The capillary network has a various organization in different zones of each side of the plexus.     

Isolated choroid plexus cysts--the need for routine offer of karyotyping

The management of isolated fetal choroid plexus cysts remains controversial. We have prospectively studied 15,565 pregnancies at two large obstetric units for the presence of choroid plexus cysts. In all cases where cysts were present at 19 weeks' gestation or greater, and were multiple, bilateral or solitary and greater than 5 mm maximum diameter, women were offered amniocentesis or placental biopsy, irrespective of the presence or absence of other abnormalities. Choroid plexus cysts were present in 152 (0.98 per cent) of cases. Four cases (2.6 per cent) of autosomal trisomy (three of trisomy 18, one of trisomy 21) were detected on prenatal karyotyping. In all cases, choroid plexus cysts were the only detectable prenatal anomaly. This study and a review of other large studies do not support the view that isolated choroid plexus cysts are a benign variant, the risk of trisomy being 1 in 82. Until further evidence is available, we recommend that cases of isolated fetal choroid plexus cysts at 19 weeks' gestation or greater should be offered prenatal karyotyping.     

A choroid plexus papilloma in an elasmobranch (Squalus acanthias)

A choroid papilloma in the choroid plexus of the ala of the fourth ventricle in a mature male elasmobranch, Squalus acanthias, was described. This is apparently the first report of a neoplasm of the central nervous system in a member of the class Chondrichthyes.     

Retinoid-binding proteins in the cerebellum and choroid plexus and their relationship to regionalized retinoic acid synthesis and degradation

The expression of cellular retinoic-acid-binding protein (CRABP) and cellular retinol-binding protein (CRBP), as well as their relationship to retinoic acid (RA) synthesis and degradation were examined in the developing mouse cerebellum and choroid plexus of the fourth ventricle. The choroid plexus, which expresses the RA-synthesizing retinaldehyde dehydrogenase RALDH-2, is likely to represent a diffusion source of RA for the closely apposed cerebellum, regulating its development. We found CRBP to be expressed in the choroid plexus and, in an in-vitro assay, addition of recombinant CRBP to RALDH-2 increased RA synthesis from retinaldehyde, with the amount of increase depending on the CRBP/retinaldehyde ratio. A technique that characterizes RA-binding proteins according to their isoelectric point showed both CRABP I and CRABP II to be present in the cerebellum and P19 cells, and only CRABP II to be present in the choroid plexus. With this technique, CRABP I could also be detected in the HL60 cell line. In addition to the two known acidic RA-binding proteins CRABP I and II, the cerebellum expressed a third RA-binding protein distinguishable by its neutral isoelectric point; the same binding protein was also detected in the olfactory bulb, kidney and testes. We used the RA-binding technique to follow the rate of elimination of bound RA from the cerebellum. A systemic injection of 0.3 micromols RA into postnatal day-1 mice was almost completely removed after 8 hours. These results suggest mechanisms by which the retinoid-binding protein may regulate the equilibrium of RA synthesis and catabolism in the cerebellum and choroid plexus.     

The xylose isomerase gene from Thermoanaerobacterium thermosulfurogenes allows effective selection of transgenic plant cells using D-xylose as the selection agent

Transthyretin, an extracellular thyroid-hormone-binding protein (THBP) in higher vertebrates, is synthesized and secreted by the choroid plexus of all classes of vertebrates, except fish and amphibians, and synthesized in the liver of endothermic animals. Here, we report the nucleotide sequence of the cDNA for a THBP found in plasma of bullfrog (Rana catesbeiana) tadpoles before the climax of metamorphosis. The amino acid sequence clearly shows this protein to be an amphibian transthyretin. The three-dimensional structure of bullfrog transthyretin was derived using homology modeling. Compared with transthyretins from other vertebrate species, bullfrog transthyretin is highly conserved at the thyroid hormone-binding sites and other important structural regions of the subunits. Bullfrog transthyretin mRNA was found in tadpole liver, but not in tadpole choroid plexus. Thus, during evolution, synthesis of transthyretin in the liver of metamorphosing amphibians preceded that in the choroid plexus of reptiles, birds and mammals. It was previously observed that the protein most abundantly synthesized and secreted by the choroid plexus in adult amphibians is a lipocalin [Achen, M. G., Harms, P. J., Thomas, T., Richardson, S. J., Wettenhall, R. E. H. & Schreiber, G. (1992) J. Biol. Chem. 267, 23170-23174], in contrast to transthyretin being the most abundantly synthesized and secreted protein in the choroid plexus of mammals, birds and reptiles. Lipocalin mRNA was found in large amounts in tadpole choroid plexus, but not livers.     

Bronchiolitis obliterans in a patient with HIV infection

We sought to determine the relationship between an isolated choroid plexus cyst diagnosed antenatally and fetal aneuploidy in an unselected population at a district general hospital. Over a 5-year period all women attending for a detailed anomaly scan at 18-20 weeks' gestation were screened for evidence of a fetal choroid plexus cyst. All cases of choroid plexus cyst were recorded prospectively. The size, position and number of the cysts were noted and associated abnormalities seen on ultrasound were also recorded. Cases of choroid plexus cyst associated with fetal aneuploidy were noted. A total of 13,690 women were screened, and 84 cases of choroid plexus cyst were identified (0.6%). Of these, 41% underwent prenatal karyotyping by amniocentesis; 78 of 84 cases (93%) were isolated. Six had other markers for aneuploidy, and three of these fetuses had trisomy 18. All cases of isolated choroid plexus cyst resulted in chromosomally normal neonates. This was confirmed by either normal antenatal karyotype or postnatal examination by the pediatricians. The size, position and number of cysts did not appear to influence the risk of aneuploidy. We conclude that the risk of aneuploidy for a case of isolated choroid plexus cyst in an unselected population appears to be very low, and in this series was 0%. In this setting, we suggest detailed ultrasound examination is essential, rather than routine karyotyping.     

Glut1 glucose transporter in the primate choroid plexus endothelium

The objective of the present study was to define the cellular location of the Glut1 glucose transporter in the primate choroid plexus. Immunogold electron microscopy indicated that Glut1 epitopes were associated primarily with choroid plexus endothelial cells. Digitized analyses of electron microscopic images provided quantitative estimates of the relative number of Glut1 glucose transporter epitopes on luminal and abluminal endothelial cell membranes within the choroid plexuses. We recorded a high density of Glut1 in the microvascular endothelium of primate choroid plexus, which was consistent in vervet monkeys (5-10 Glut1 gold particles per micrometer of endothelial cell plasma membrane), as well as in baboons (5-20 Glut1 gold particles per micrometer of capillary plasma membrane). In the baboon choroid plexus, we observed that perivascular cells (presumed to be pericytes) were also Glut1-positive, but with substantially reduced activity compared with endothelial cells. Occasional Glut1-immunogold particles were also seen in the basolateral membranes of the choroid plexus cuboidal cells. Light microscopic immunocytochemistry confirmed the abundance of Glut1 immunoreactivity in choroid plexus endothelial cells of vervet monkeys and baboons. A similar pattern was observed in surgically resected human choroid plexus, suggesting differences between primates, including humans and laboratory animals. The only difference was that erythrocytes within the human choroid plexus exhibited a florid Glut1-positive response, but were weakly immunoreactive in nonhuman primates. The observation of high glucose transporter densities in choroid plexus endothelial cells is consistent with the suggestion that choroidal epithelia and capillaries provide a metabolic work capability for maintaining ionic gradients and secretory functions across the blood-CSF barriers.     

Duplication of 9P and hyperplasia of the choroid plexus: a pathologic, radiologic, and molecular cytogenetics study

Duplication of the short arm of chromosome 9 is a rare constitutional abnormality, and the presence of pathologically confirmed hyperplasia of the choroid plexus in one of two cases, and a choroid plexus papilloma in another, raises the possibility of a relationship between the 9p abnormality and abnormal growth of the choroid plexus. Molecular cytogenetic analysis using fluorescence in situ hybridization was used for detection of chromosome 9-derived material in various formalin-fixed choroid plexus abnormalities. Extra copies of chromosome 9-derived material was found in the hyperplastic choroid plexus and in a choroid plexus carcinoma. These findings suggest that there may be an association between duplication of chromosome 9 material and abnormal development of the choroid plexus.     

Role of the pneumo-encephalogram in the diagnosis of spontaneous subarachnoid haemorrhage

The authors have collected eighteen cases of primary intraventricular lesions revealed by a subarachnoid haemorrhage. Among these cases, the most important are the choroid plexus papillomas followed by ependymomas; some cases have no precise histological interpretation. Generally speaking, cerebral arteriography gives only indirect evidence of ventricular dilation and thus does not confirm the presence of a lesion. On the contrary, the pneumoencephalogram is consistantly positive, but does not give the differential diagnosis between intraventricular tumours and primary intraventricular haemorrhages. This test should therefore be given the same importance as spinal cord arteriography in the exploration of subarachnoid haemorrhages with negative cerebral angiograms.