1/19. Isolated large third-trimester intracranial cyst on fetal ultrasound: fact or fiction?OBJECTIVE: To distinguish the fact from artifact of an isolated, large, intracranial cyst on prenatal sonography (PSG). BACKGROUND: The use of PSG is rapidly increasing with most obstetric ultrasounds occurring in general community settings like small hospitals and clinics with personnel who have variable training, experience, and interest levels. In contrast, most PSG articles and books are produced in large subspecialty centers with concentrated referral bases plus both highly-trained and experienced personnel. DESIGN/methods: We report a series of 2 normal newborn patients who had a large prenatal unilateral intracranial cyst diagnosed by PSG in the 10 years between July of 1989 and 1999 at a rural community hospital. The newborns had imaging studies at birth and their neurodevelopmental progress was followed for several years. Textbook, bibliography and computerized medline (1966-present) searches including prenatal ultrasound, observer variation, diagnostic errors, reproducibility of results, sensitivity and specificity, accuracy, central nervous system, false-positive, prenatal diagnosis, and brain were examined starting in August 1996 for reports. RESULTS: There were 4079 obstetric ultrasounds performed in 3.5 years, January 1996 through July 1999 at this rural community facility. This rate extrapolates to a total of 11 654 obstetric ultrasounds over the 10-year study period in which the 2 cases of intracranial cyst artifact occurred. Thus, the incidence of 2 intracranial cyst artifacts was estimated as 2/11 654 PSG, a .0002% false-positive rate. CONCLUSIONS: This is the first report of the occurrence of PSG artifacts in a community facility. Artifact is a real problem and needs to be specified in differential diagnoses. There are ways to decrease sonographic artifact-or at least to recognize it-so our estimates at a community hospital for its occurrence are presented with the relevant technical and ethical issues. None of these issues have been previously reported in the pediatric literature. Our false-positive rate for large intracranial cyst compares favorably with other reports. Our estimate may inflate our denominator by reporting scans rather than the number of fetuses scanned, and our numerator may miss cases that moved from the community. confusion differentiating PSG artifact from reality often occurs when interpreting static or frozen real-time images. The signs that sonogram images may be artifacts include defects that: extend outside the fetal body; change shape, size and echogenecity with different scan planes; are not seen on all examinations; and are isolated in an otherwise normal fetus. Failure to offer quality PSG in clinical settings where it is available restricts access of pregnant women to the diagnosis of fetal anomalies, and therefore restricts access to the options of pregnancy termination, fetal therapy like fetal surgery, and delivery options of timing, setting, and mode. We suggest a multidisciplinary approach to prenatal abnormalities like isolated third trimester unilateral intracranial cyst in both primary and tertiary care settings aids interpretation followed by expectant conservative management without elaborate, risky, or terminal interventions.- - - - - - - - - - ranking = 1keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
2/19. Merosin-deficient congenital muscular dystrophy with mental retardation and cerebellar cysts unlinked to the LAMA2, FCMD and MEB loci.We report a case of congenital muscular dystrophy with secondary merosin deficiency, structural involvement of the central nervous system and mental retardation in an 8-year-old girl from a consanguineous family. She had early-onset hypotonia, generalized muscle wasting, with weakness especially of the neck muscles, joint contractures, mental retardation and high creatine kinase. Muscle biopsy showed dystrophic changes with partial deficiency of the laminin alpha(2) chain. Cranial magnetic resonance imaging revealed multiple small cysts in the cerebellum, without cerebral cortical dysplasia or white matter changes. The laminin alpha(2) chain (6q2), Fukuyama type congenital muscular dystrophy (9q31-q33) and muscle-eye-brain disease (1p32-p34) loci were all excluded by linkage analysis. We suggest that this case represents a new entity in the nosology of congenital muscular dystrophy.- - - - - - - - - - ranking = 1keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
3/19. Multiple cysts in the cerebral white matter: a rare complication of whole brain radiation therapy.We describe a patient with multiple cysts in the cerebral white matter, several years after whole brain radiation therapy (WBRT) for a solitary cerebellar metastasis of a lung carcinoma. MR images of the brain show diffuse white matter changes, 1 year after radiation, and cyst formation in the white matter, starting 3.5 years later. We conclude that cysts in the cerebral white matter can be a late stage of white matter damage after whole brain radiation therapy in long-term survivors with brain metastases.- - - - - - - - - - ranking = 2.2069980571473keywords = brain (Clic here for more details about this article) |
4/19. Endoscopic treatment for a huge neonatal prepontine-suprasellar arachnoid cyst: a case report.A huge prepontine-suprasellar arachnoid cyst was identified by fetal sonography and magnetic resonance imaging. It was successfully treated with a two-stage operation using a neuroendoscope. The first operation during the neonatal period consisted of implantation of a cyst-peritoneal shunt. In the second operation, the cyst was fenestrated with the aid of a neuroendoscope. The cyst membrane was seen to cover the foramen magnum and to extend continuously to the ventral surface of the brain stem. At the foramen magnum, it was pulsating synchronously with the pulsation of the vertebral artery, which was suggestive of the mechanism of cyst growth. A ventriculo-peritoneal shunt was inserted to replace the cyst-peritoneal shunt. Endoscopic cyst fenestration is a less invasive alternative for the treatment of arachnoid cysts and can also be used for young children. In that case, however, special care should be taken to avoid complications, especially if the cyst exerts considerable pressure on the critical areas of the brain.- - - - - - - - - - ranking = 0.55174951428683keywords = brain (Clic here for more details about this article) |
5/19. Lateral supratentorial endodermal cyst: case report and review of literature.OBJECTIVE AND IMPORTANCE: Epithelial cysts of the central nervous system are unusual entities, with the majority reported to occur in the spinal canal. More unusual is the intracranial presentation, which shows a predilection for midline localization in the posterior fossa, brainstem, and suprasellar regions. This report discusses the differential diagnosis, pathogenesis, radiographic presentation, and therapeutic considerations of a laterally positioned cerebral convexity endodermal cyst. CLINICAL PRESENTATION: A 49-year-old right-handed man presented with approximately a 30-month history of short- and long-term memory difficulties. A magnetic resonance imaging study revealed a large, nonenhancing, extra-axial cystic lesion overlying the right lateral frontal convexity. INTERVENTION: A right frontoparietal craniotomy exposed a large extra-axial cyst with an opaque, yellowish-white membrane containing a mucoid fluid. Histological analysis disclosed a layer of unremarkable, ciliated columnar epithelium with a basement membrane that stained positive for cytokeratin, periodic acid-Schiff, and alcian blue. No evidence of either a muscular or cartilaginous layer around the mucosa was present to further delineate neurenteric versus bronchogenic origin of the cyst. CONCLUSION: This case involved the occurrence of a solitary endodermal cyst as an extra-axial mass localized over the lateral frontal lobe. The lateral supratentorial localization of this lesion illustrates the need for consideration of the pathogenesis of this entity as well as its diagnostic differentiation from other cystic abnormalities in this region. A review of the histochemistry of endodermal, neuroepithelial, and other cerebral cysts is presented.- - - - - - - - - - ranking = 1keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
6/19. Cyst-like cerebral lesions in tuberous sclerosis.Known brain manifestations of tuberous sclerosis (TSC) are cortical sclerotic tubera, giant cell astrocytomas, subependymal calcified nodules in the lateral walls of the lateral ventricles, and white matter heterotopias. In addition, small cyst-like lesions in the white matter have been described. We report on three TSC patients with hitherto undescribed large cyst-like cerebral lesions in subcortical and white matter locations. We emphasize that cystoid brain degeneration is a rare but typical cerebral manifestation of TSC and suggest that, in patients with such lesions, TSC should be taken into consideration.- - - - - - - - - - ranking = 0.55174951428683keywords = brain (Clic here for more details about this article) |
7/19. Pathogenesis and diagnostic pitfalls of ventricular diverticula: case report and review of the literature.OBJECTIVE AND IMPORTANCE: Ventricular diverticula are local herniations of the ventricular system occurring in association with severe, long-standing hydrocephalus. Variability in the extent and location of these ventricular pouches may produce misleading radiological conformations and raise diagnostic challenges. In this report, we discuss the pathogenesis and differential diagnosis of ventricular diverticula on the basis of a case illustration. CLINICAL PRESENTATION: We report the case of a 38-year-old woman presenting with hydrocephalus secondary to an obstructive lesion of the third ventricle. A large, dilated structure compressing the brainstem was present in the posterior fossa. INTERVENTION: The posterior fossa lesion was identified as an atrial diverticulum by injection of contrast medium into the right lateral ventricle, which led to immediate enhancement of the infratentorial cavity. Ventricular drainage and removal of the third ventricular lesion, a colloid cyst, allowed progressive collapse of the diverticulum and symptom resolution. CONCLUSION: This report discusses the pathogenesis of ventricular diverticula and demonstrates that ventriculography can be used to differentiate atrial diverticula from other posterior fossa lesions when time-consuming multiplanar magnetic resonance imaging cannot be afforded on an emergency basis.- - - - - - - - - - ranking = 0.27587475714342keywords = brain (Clic here for more details about this article) |
8/19. Delleman (oculocerebrocutaneous) syndrome: few variations in a classical case.Delleman syndrome involves a group of congenital abnormalities affecting the eye, skin and central nervous system. It is a rare and sporadic disorder. We report on a 4-year-old male child who presented to us with oculocerebrocutaneous syndrome featuring: focal alopecia on the left side of the scalp, left periorbital skin appendages, a left-sided orbital dermoid, a large left-sided intracranial cyst,optic atrophy. About 35 such cases have been reported in the literature so far. To our knowledge optic atrophy associated with oculocerebrocutaneous syndrome has not been reported previously. Our patient had only a single seizure and his IQ was normal.- - - - - - - - - - ranking = 0.72412524285658keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/19. Fetal subependymal cysts with normal neonatal outcome. A case report.The authors report the prenatal sonographic diagnosis of subependymal cysts, confirmed by in utero MRI, in an apparently uneventful pregnancy. The sonographic appearance of the lesions, the obstetric management and the postnatal follow-up to one year are described. Special attention is paid to prenatal factors of neurological morbidity, rather than intrapartum and postnatal, thus emphasizing the importance of early and sequential evaluation of the developing fetal brain. Diagnosis, clinical significance and outcome of prenatal subependymal cysts are necessary for parental counselling and obstetric management. Furthermore, the detection of a prenatal brain injury may have remarkable medico-legal implications.- - - - - - - - - - ranking = 0.55174951428683keywords = brain (Clic here for more details about this article) |
10/19. Giant supratentorial enterogenous cyst: report of a case, literature review, and discussion of pathogenesis.OBJECTIVE AND IMPORTANCE: To describe a histologically well-documented adult case of a giant supratentorial enterogenous cyst (EC). Fewer than 15 cases of supratentorial ECs are on record: 8 associated with the brain hemispheres or the overlying meninges, 4 with the sellar region, and 2 with the optic nerve. CLINICAL PRESENTATION: A 31-year-old woman complained of long-standing mild left brachial and crural motor deficit precipitated by headache and signs of intracranial hypertension. magnetic resonance imaging revealed a huge cyst overlying the frontoparietal brain. INTERVENTION: Symptoms were relieved by evacuation of the cyst content by means of a Rickam's reservoir, and the lesion was subsequently removed in toto. Histological and immunohistochemical examination of the cyst wall clearly established the enterogenous nature of its epithelium. Follow-up for up to 2 years after intervention showed no sign of recurrence, and symptoms, including treatment-resistant seizures in the postoperative period, have entirely subsided. CONCLUSION: Supratentorial ECs, distinctly rare in adult patients, may in some cases present as giant lesions. Total removal seems to be curative once careful examination has eliminated the possibility of a metastasis from an unknown primary. A correct histological diagnosis is important because, in contrast to other benign cysts of similar location and size, ECs may be prone to intraoperative dissemination.- - - - - - - - - - ranking = 0.55174951428683keywords = brain (Clic here for more details about this article) |
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