Comparison of spinal deformity in children with Chiari I malformation with and without syringomyelia: matched cohort study.

PURPOSE: To describe curve patterns in patients with Chiari malformation I (CIM) without syringomyelia, and compare to patients with Chiari malformation with syringomyelia (CIM + SM). METHODS: Review of medical records from 2000 to 2013 at a single institution was performed to identify CIM patients with scoliosis. Patients with CIM were matched (1:1) by age and gender to CIM + SM. Radiographic curve patterns, MRI-based craniovertebral junction parameters, and associated neurological signs were compared between the two cohorts. RESULTS: Eighteen patients with CIM-associated scoliosis in the absence of syringomyelia were identified; 14 (78 %) were female, with mean age of 11.5 ± 4.5 years. Mean tonsillar descent was 9.9 ± 4.1 mm in the CIM group and 9.1 ± 3.0 mm in the CIM + SM group (p = 0.57). Average syrinx diameter in the CIM + SM group was 9.0 ± 2.7 mm. CIM patients demonstrated less severe scoliotic curves (32.1° vs. 46.1°, p = 0.04), despite comparable thoracic kyphosis (43.7° vs. 49.6°, p = 0.85). Two (11 %) patients with CIM demonstrated thoracic apex left deformities compared to 9/18 (50 %) in the CIM + SM cohort (p = 0.01). Neurological abnormalities were only observed in the group with syringomyelia (6/18, or 33 %; p = 0.007). CONCLUSION: In the largest series specifically evaluating CIM and scoliosis, we found that these patients appear to present with fewer atypical curve features, with less severe scoliotic curves, fewer apex left curves, and fewer related neurological abnormalities than CIM + SM. Notably, equivalent thoracic kyphosis was observed in both groups. Future studies are needed to better understand pathogenesis of spinal deformity in CIM with and without SM.

Two-dimensional movement control using electrocorticographic signals in humans.

We show here that a brain-computer interface (BCI) using electrocorticographic activity (ECoG) and imagined or overt motor tasks enables humans to control a computer cursor in two dimensions. Over a brief training period of 12-36 min, each of five human subjects acquired substantial control of particular ECoG features recorded from several locations over the same hemisphere, and achieved average success rates of 53-73% in a two-dimensional four-target center-out task in which chance accuracy was 25%. Our results support the expectation that ECoG-based BCIs can combine high performance with technical and clinical practicality, and also indicate promising directions for further research.

The Children's Cancer and Leukaemia Group guidelines for the diagnosis and management of dysembryoplastic neuroepithelial tumours.

Dysembryoplastic neuroepithelial tumours (DNETs) were incorporated into the new World Health Organization classification of brain tumours as part of the group of glioneuronal tumours in 1993. Large series of patients with DNETs and pharmaco-resistant epilepsy have been reported. DNETs are most often located in the temporal lobe, occurring in both mesial and lateral temporal locations. DNETs have also been reported in the insular cortex, brain stem, cerebellum, occipital lobe and striatum. Approximately 40% of DNETs are cystic, and solitary nodular, multinodular or diffuse forms have been recognized. Approximately 30% of DNETs are associated with subtle cortical dysplastic changes in the adjacent cortex. DNET nodules usually look like oligodendroglioma, whilst between the nodules it may be possible to recognize vertical columns of neurons surrounded by oligodendrocyte-like cells. Cytologically, oligodendroglial-like cells of DNETs are distinguished from oligodendroglioma by larger nuclei with frequent nuclear indentations and multiple, small nucleoli, whilst oligodendrogliomas consistently show nuclear roundness with one or two occasional nucleoli. Very rare cases of malignant transformation have been reported. DNETs are hypodense on CT and demonstrate decreased signal on the T1-weighted images and a hyper-intense signal on T2-weighted MRI. DNETs associated with pharmaco-resistant epilepsy should be removed early to achieve seizure freedom and prevent tumour progression. The surgical approach should be that of an extended lesionectomy, i.e. excision of the lesion and the abnormal dysplastic cortex around it. Use of MRI-based image guidance (neuronavigation) as a surgical tool to identify this area of abnormal cortex is very helpful to ensure that the extended lesionectomy includes any visibly dysplastic cortex. It is not advocated to use a stereotactic biopsy only, as this may generate an unrepresentative tissue sample consisting of an oligodendroglial component only and may lead to an incorrect diagnosis.

Intracranial ependymomas of childhood: current management strategies.

Children with intracranial ependymomas have relatively poor outcomes despite the low-grade histology of these tumors and recent advances in diagnosis, microneurosurgical resection, and adjuvant therapy. Aggressive surgical resection and postoperative adjuvant therapy result in only a 5-year survival rate of 50%. In this paper, we provide a review of the current technical aspects of surgical resection, and focus on recent studies of the epidemiology, molecular markers, prognostic factors and adjuvant therapy for childhood intracranial ependymomas and discuss their implications for current and future management strategies.

The surgical treatment of spasticity.

Many neurosurgical procedures have been designed for or applied to the treatment of spasticity arising from different disorders, including cerebral palsy; traumatic, ischemic, or hypoxic brain injury, multiple sclerosis, and spinal cord injury. Neurosurgical procedures are primarily aimed at reducing spasticity by interrupting the stretch reflex at various sites along the spinal reflex arc or attempting to increase the centrally mediated inhibitory influence on the pool of motor neurons in the anterior horn. Surgical interventions for spasticity can be classified into peripheral ablative procedures, such as rhizotomy or peripheral neurectomy, and central ablative procedures, such as cordectomy, myelotomy, or stereotactic procedures. Non-ablative procedures include peripheral nerve or motor point blocks, the implantation of cerebellar or spinal stimulators, and the implantation of subdural catheters for infusion of pharmacologic agents to increase inhibitory activity. Several proposed mechanisms for spasticity are reviewed so that the rationale for the various surgical interventions for spasticity described may be better understood.

Decreased levels of C1q in cerebrospinal fluid of living Alzheimer patients correlate with disease state.

Recent reports that complement proteins comprising the classical pathway are associated with senile plaques suggest that activation of the classical complement cascade in Alzheimer's disease tissue results in bystander cell lysis and may contribute to AD neuropathology. Analysis of cerebrospinal fluid may prove to be a useful means of detecting changes in immunological activity in the brain. We use an enzyme-linked immunosorbent assay to measure levels of C1q, a subunit of the classical complement cascade, in the CSF of patients clinically diagnosed with possible or probable AD. Significantly lower levels of C1q were detected in the CSF of the Alzheimer group as compared to control CSF [AD: mu = 268 ng/ml, SD = 84; non-AD: mu = 340 ng/ml, SD = 76; F(1, 44) = 5.84, p = 0.02]. Diminished performance on global measures of mental status such as the Mini-Mental State Exam (R = 0.45; p = 0.0072) and Blessed's Information, Memory, and Concentration test (R = 0.42; p = 0.0138) showed high correlations with decreased C1q levels. More specific measures of cognitive function, such as word recall (R = 0.42; p = 0.012), word recognition (R = 0.52; p = 0.0017) and delayed recall (R = 0.45; p = 0.0062) memory tasks also correlated strongly with decreased C1q levels.

Analysis of brain injury following intrahippocampal administration of beta-amyloid in streptozotocin-treated rats.

It has been suggested that the vulnerability of the aged brain to Alzheimer's disease (AD) pathogenesis depends on a number of risk factors, including abnormal glycolytic metabolism and beta-amyloid accumulation. Intrahippocampal injections of beta-amyloid and related peptides were administered to chronically hyperglycemic rats to examine beta-amyloid toxicity and the interaction with imbalances of glucose metabolism. Chronic hyperglycemia was induced by systemic injection of streptozotocin (STZ) which selectively destroys pancreatic beta-islet cells. Ten days after intrahippocampal injection of synthetic beta-amyloid peptides (beta 1-42, beta 25-35, scrambled beta 25-35), lesion volume, blood glucose, and plasma corticosterone concentrations, beta 1-42 immunoreactivity and gliosis were assessed to determine peptide toxicity in the normoglycemic and hyperglycemic conditions. Glucose levels correlated with plasma corticosterone concentrations (r = 0.85) and increased lesion volume size (r = 0.36). Intrahippocampal peptide injections in normoglycemic subjects did not induce significant damage as compared to control injections of vehicle alone. STZ-treated groups demonstrated a trend for increased lesion volume size following injection of either vehicle, beta 1-42, or beta 25-35. The combination of the beta 1-42 peptide and streptozotocin-induced hyperglycemia was toxic and induced significantly larger lesions (p < 0.01) of the dorsal blade of the dentate gyrus as compared to injections of beta 1-42 into normoglycemic subjects.