Temporal crescent syndrome caused by a lateral ventricular glioependymal cyst: case report.

Temporal crescent syndrome is a monocular visual field defect involving the temporal crescent of one eye caused by a retrochiasmal lesion. The most anterior portion of the striate cortex is the only area where the retrochiasmal lesion produces a monocular visual field defect. The authors present the case of a 9-year-old boy who presented with mild headache. MRI revealed a cyst with cerebrospinal fluid signal intensity, occupying the body and trigone of the right lateral ventricle. Conservative treatment with regular clinical and radiological follow-up was chosen because neurological examination findings were normal. Three years later, the patient experienced blurred vision with a temporal crescent defect in the left eye. Endoscopic cyst fenestration was performed, and the pathological findings indicated a glioependymal cyst. After surgery, the monocular temporal crescent disorder was resolved. MRI indicated shrinkage of the cyst and improvement in the narrowing of the anterior calcarine sulcus. These findings suggested that the temporal crescent syndrome was caused by a lateral ventricular glioependymal cyst. This is the first known report of temporal crescent syndrome caused by a lateral ventricular glioependymal cyst. In patients with monocular temporal crescent disorder without intraocular disease, a retrochiasmal lesion in the most anterior portion of the striate cortex should be considered.

Awareness of folic acid use increases its consumption, and reduces the risk of spina bifida.

The majority of neural tube defects were believed to be folic acid (FA)-preventable in the 1990s. The Japanese government recommended women planning pregnancy to take FA supplements of 400 µg/d in 2000, but the incidence of spina bifida has not decreased. We aimed to evaluate the OR of having an infant with spina bifida for women who periconceptionally took FA supplements and the association between an increase in supplement use and possible promoters for the increase. This is a case-control study which used 360 case women who gave birth to newborns afflicted with spina bifida, and 2333 control women who gave birth to healthy newborns during the first 12 years of this century. They were divided into two 6-year periods; from 2001 to 2006 and from 2007 to 2012. Logistic regression analyses were conducted to compute OR between cases and controls. The adjusted OR of having an infant with spina bifida for supplement users was 0.48 in the first period, and 0.53 in the second period. The proportion of women who periconceptionally consumed supplements significantly increased from 10 % in the first period to 30 % in the second period. Awareness of the preventive role of FA was a promoter for an increase in supplement use, and thus an FA campaign in high school seems rational and effective. The failure of the current public health policy is responsible for an epidemic of spina bifida. Mandatory food fortification with FA is urgent and long overdue in Japan.

Choroid plexus papilloma in a girl with hypomelanosis of Ito.

The authors report a case of choroid plexus papilloma in a girl with hypomelanosis of Ito, and they review the literature in brief. Hypomelanosis of Ito is a rare neurocutaneous syndrome characterized by cutaneous hypopigmented whorls, streaks, and patches along lines of Blaschko. Most patients exhibit CNS manifestations, including psychomotor retardation, seizures, hypotonia, and ataxia. A 6-year-old girl with hypomelanosis of Ito was referred to the authors' hospital with bilateral tumors in the lateral ventricles. The right lateral ventricle tumor was surgically removed. Immunohistochemical investigations revealed the tumor to be a choroid plexus papilloma (WHO Grade I). A chromosomal investigation revealed that the tumor tissue demonstrated a large loss of heterozygosity at chromosome 10. The case reported here serves as a reminder that de novo brain tumors may arise in patients with chromosomal mosaicism.

Endoscopic transaqueductal placement of a single-catheter cyst-ventriculoperitoneal shunt in a neonate with Dandy-Walker malformation-associated hydrocephalus: case report.

A neonate with hydrocephalus associated with Dandy-Walker malformation was successfully treated with an endoscopic placement of a transaqueductal ventricular single catheter. The modified catheter was provided with additional fenestration on its proximal side to allow simultaneous drainage from both the supra- and infratentorial compartments. This technique is well known for isolated fourth ventricles, but has not been applied to hydrocephalus associated with Dandy-Walker malformation. The cyst-ventriculoperitoneal shunt effectively drained both compartments. The patient was doing well 18 months after the surgical procedure. Endoscopic transaqueductal shunt placement can be considered, especially in patients with aqueductal patency.

Giant glioependymal cyst in an infant.

The authors report the case of an infant with a giant glioependymal cyst. Although it has been suggested that these cysts originate from the tela choroidea, their origin remains controversial. This 35-month-old girl with truncal ataxia was referred to the authors' hospital. Magnetic resonance imaging revealed a giant cystic mass extending from the anterior to the posterior cranial fossa. Hydrocephalus was caused by obstruction of the sylvian aqueduct. Endoscopic fenestration of the cyst wall was performed. Histochemical and immunohistochemical staining identified the lesion as a glioependymal cyst. Magnetic resonance imaging performed 8 months later suggested that the cyst originated from the tela choroidea. At 5-year follow-up, there was no tumor recurrence and she had fully recovered. The origin of glioependymal cysts is discussed, and the authors suggest that their origin is the tela choroidea.

[Clinical and electroencephalographic analysis of epilepsy in children with hydrocephalus].

Clinical and electroencephalographic features of epilepsy and the prognosis of intelligence were investigated in 156 children with hydrocephalus. Of these 53 (34.0%) had epilepsy. The incidence and outcome of epilepsy were determined by the etiology of hydrocephalus. The incidence was high in children with hydrocephalus caused by intra-cranial infection and dysgenetic hydrocephalus without dysraphism. Furthermore, it was difficult to control the epileptic seizures in these patients. In contrast, children with dysgenetic hydrocephalus caused by dysraphism and simple hydrocephalus had lower incidence of epilepsy and epileptic seizures had been well controlled in these cases. In hydrocephalic children, localization-related epilepsy was the most common. Complex partial seizures and focal motor seizures were frequently observed. Common electroencephalographic findings were focal spikes or multi-focal spikes. The intelligence quotient was significantly lower in children with epilepsy than in those without epilepsy. All these findings suggest that the incidence of epilepsy may be a crucial prognostic factor in children with hydrocephalus.

The assessment of fetal brain morphology and circulation by transvaginal 3D sonography and power Doppler.

OBJECTIVE: The objective of this article is to describe the use of transvaginal 3D ultrasound in prenatal neuroimaging and to investigate its clinical usefulness. METHODS: Firstly, 18 fetuses with hydrocephalus, ventriculomegaly and/or space occupying lesion were examined by transvaginal 3D sonography, and fetal CNS abnormalities were evaluated. Multiplanar image analysis and volumetric assessment were performed off-line. Longitudinal volume changes of target structure were evaluated, and usefulness of transvaginal 3D ultrasound was evaluated. Secondly, in 56 normal cephalic fetuses of between 18 and 32 weeks, 3D Doppler volume acquisition and reconstruction of the intracranial vascular structure were performed. RESULTS: Longitudinal changing appearance in the same cutting section of the enlarged ventricle or cystic lesion could be demonstrated in all cases and volumetric assessment was also successful in all cases. Longitudinal objective data were useful in the brain assessment, consultation and counselling. 3D Doppler acquisition time was 5.6-26 seconds and symmetrical 3D reconstruction was successful in 51.8%. CONCLUSION: Transvaginal 3D imaging technology provides us with not only comprehensive intracranial images in exactly the right sections, but also with objective volume data. Prenatal information with objective data analyses is useful in consultation for both specialists and parents, and leads to proper management of CNS diseases.