The study of spinal abnormalities associated with cloacal exstrophy

The treatment of spinal abnormalities with cloacal exstrophy is controversial. Ten cases of this complex treated at Hyogo Prefectural Kobe Children's Hospital between 1991 and 2010 are presented. In our series, all 10 patients had tethered spinal cords. In addition, there were 3 terminal myelocystoceles, 2 meningoceles, 7 lipomas, 5 thickened filums and 3 syrinxes. Eight of 10 patients underwent surgery, and no patient deteriorated. All 3 patients with terminal myelocystocele had lower extremity weakness, but motor functions in two patients improved after surgery. All 4 patients with lipoma and/or thickened filum were asymptomatic. Two of them were conservatively treated, and they remain asymptomatic. Terminal myelocystocele and symptomatic syrinx should be surgically treated.     

Perceptual characteristics of peripheral vision in children with autism

The present study examined the recognition of numerical stimuli briefly presented in the peripheral and the central (foveal) visual fields of children with autism. The participants were 5 children with high-functioning Autism Spectrum Disorder (ASD) and 10 typically developing (TD) children of similar chronological age. The stimuli were number strings presented on a personal computer screen for 160 msec in the position of the fixation point (foveal condition) or in the peripheral visual field (retinal eccentricity of 16 degrees) in one of the eight radial positions selected randomly (peripheral condition). The participants' task was to report the number of stimuli. The results showed that there were no significant differences in the mean response times between the TD and ASD groups. However, the mean percentage of correct answers in the ASD group was significantly higher than in the TD group for the peripheral condition. These results suggest that the effective visual field of the ASD group is expanded compared with the TD group. We discussed the relationships between perceptual characteristics and cognitive processes particular to ASD children.     

GC/MS-based metabolomic analysis of cerebrospinal fluid (CSF) from glioma patients

Metabolomics has recently undergone rapid development; however, metabolomic analysis in cerebrospinal fluid (CSF) is not a common practice. We analyzed the metabolite profiles of preoperative CSF samples from 32 patients with histologically confirmed glioma using gas chromatography/mass spectrometry (GC/MS). We assessed how alterations in the metabolite levels were related to the World Health Organization (WHO) tumor grades, tumor location, gadolinium enhancement on magnetic resonance imaging (MRI), and the isocitrate dehydrogenase (IDH) mutation status. Sixty-one metabolites were identified in the CSF from glioma patients using targeted, quantitative and non-targeted, semi-quantitative analysis. The citric and isocitric acid levels were significantly higher in the glioblastoma (GBM) samples than in the grades I–II and grade III glioma samples. In addition, the lactic and 2-aminopimelic acid levels were relatively higher in the GBM samples than in the grades I–II glioma samples. The CSF levels of the citric, isocitric, and lactic acids were significantly higher in grade I–III gliomas with mutant IDH than in those with wild-type IDH. The tumor location and enhancement obtained using MRI did not significantly affect the metabolite profiles. Higher CSF levels of lactic acid were statistically associated with a poorer prognosis in grades III–IV malignant gliomas. Our study suggests that the metabolomic analysis of CSF from glioma patients may be useful for predicting the glioma grade, metabolic state, and prognosis of gliomas.     

Diffusion-weighted imaging predicts postoperative persistence in meningioma patients with peritumoural abnormalities on magnetic resonance imaging.

OBJECTIVE: While diffusion-weighted magnetic resonance imaging (MRI) has been used to study malignant brain tumours, this modality has not been used to study MRI abnormalities surrounding meningiomas. METHODS: We examined intensity and apparent diffusion coefficient (ADC) on diffusion weighted imaging (DWI) for predicting postoperative persistence of MRI abnormalities surrounding meningiomas as well as characterizing the tumours. RESULTS: Of 36 meningiomas who underwent gross total resection, 27 (75%) showed hyperintensity on DWI at b=1100s/mm(2). No atypical meningiomas were hypointense on DWI. Of the 26 supratentorial meningiomas, 18 (69.0%) had associated MRI abnormality. No significant correlation was seen between tumour intensity on DWI and existence of surrounding MRI abnormality. Meningothelial meningiomas showed a relatively low prevalence of MRI abnormalities surrounding tumour (30%). Of 11 patients who underwent sequential MRI, all MRI abnormalities surrounding tumour showing isointensity and high ADC on preoperative DWI disappeared after surgery (from 3 weeks to 10 months). All MRI abnormalities surrounding tumour showing hyperintensity and low ADC on preoperative DWI persisted on final follow-up MRI (from 6 months to 20 months). CONCLUSION: The postoperative course of MRI abnormality surrounding tumour might be predictable from the intensity and ADC on preoperative DWI. Since MRI abnormalities associated with meningiomas can cause preoperative neurologic deficits. We hypothesise that abnormalities with restricted diffusion will be more likely to be associated with a preoperative deficit, and more likely to remain after removal of the causative meningioma.     

A case of cerebellar capillary hemangioma with multiple cysts

Intracranial capillary hemangiomas are very rare, though several spinal capillary hemangiomas have recently been reported. We report here a case of intracranial capillary hemangioma with multiple cysts and review the current literature of similar cases. A 4-month-old girl was referred to our hospital for treatment of hydrocephalus and a cerebellar mass lesion. She presented with hemangiomas distributed widely over the body, as well as disseminated hemangiomas in the pleura, liver, spleen, pancreas, kidneys and vagina. Pathological examination of the specimen from the vagina confirmed the diagnosis of a capillary hemangioma made at another hospital. Radiological examination of the brain revealed a cystic mass lesion in the left cerebellar hemisphere with subsequent obstructive hydrocephalus. The cysts extended upward into the bilateral ventricle. Following neuroendoscopic fenestration of the cysts, resection of the left cerebellar mass was performed. Histological examination of the lesion demonstrated similarly sized capillaries, and the pathological diagnosis was confirmed as capillary hemangioma. There was no recurrence postoperatively. Our patient was treated successfully by surgical resection and neuroendoscopic procedures. Surgical intervention may therefore be indicated in intracranial capillary hemangiomas.