Cerebral and cervical arterial abnormalities are the most common non-cutaneous anomaly in PHACE syndrome, but the location and type of arterial lesions that occur have not been systematically ...assessed in a large cohort. Our aim was to characterize the phenotypic spectrum of arteriopathy, assess the frequency with which different arteries are involved, and evaluate spatial relationships between arteriopathy, brain structural lesions, and hemangiomas in PHACE syndrome.
Intracranial MRA and/or CTA images from 70 children and accompanying brain MR images in 59 patients with arteriopathy and PHACE syndrome were reviewed to identify the type and location of arterial lesions and brain abnormalities. Five categories of arteriopathy were identified and used for classification: dysgenesis, narrowing, nonvisualization, primitive embryonic carotid-vertebrobasilar connections, and anomalous arterial course or origin. Univariate logistic regression analyses were performed to test for associations between arteriopathy location, hemangiomas, and brain abnormalities.
By study design, all patients had arterial abnormalities, and 57% had >1 form of arteriopathy. Dysgenesis was the most common abnormality (56%), followed by anomalous course and/or origin (47%), narrowing (39%), and nonvisualization (20%). Primitive embryonic carotid-vertebrobasilar connections were present in 20% of children. Hemangiomas were ipsilateral to arteriopathy in all but 1 case. The frontotemporal and/or mandibular facial segments were involved in 97% of cases, but no other specific associations between arteriopathy location and hemangioma sites were detected. All cases with posterior fossa anomalies had either ICA anomalies or persistent embryonic carotid-basilar connections.
The arteriopathy of PHACE syndrome commonly involves the ICA and its embryonic branches, ipsilateral to the cutaneous hemangioma, with dysgenesis and abnormal arterial course the most commonly noted abnormalities. Brain abnormalities are also typically ipsilateral.
Abstract
Electrophysiology and neuroimaging provide conflicting evidence for the neural contributions to target detection. Scalp electroencephalography (EEG) studies localize the P3b event-related ...potential component mainly to parietal cortex, whereas neuroimaging studies report activations in both frontal and parietal cortices. We addressed this discrepancy by examining the sources that generate the target-detection process using electrocorticography (ECoG). We recorded ECoG activity from cortex in 14 patients undergoing epilepsy monitoring, as they performed an auditory or visual target-detection task. We examined target-related responses in 2 domains: high frequency band (HFB) activity and the P3b. Across tasks, we observed a greater proportion of electrodes that showed target-specific HFB power relative to P3b over frontal cortex, but their proportions over parietal cortex were comparable. Notably, there was minimal overlap in the electrodes that showed target-specific HFB and P3b activity. These results revealed that the target-detection process is characterized by at least 2 different neural markers with distinct cortical distributions. Our findings suggest that separate neural mechanisms are driving the differential patterns of activity observed in scalp EEG and neuroimaging studies, with the P3b reflecting EEG findings and HFB activity reflecting neuroimaging findings, highlighting the notion that target detection is not a unitary phenomenon.
The inherent biology of neural stem cells (NSCs) endows them with capabilities that not only circumvent many of the limitations of other gene transfer vehicles, but that enable a variety of novel ...therapeutic strategies heretofore regarded as beyond the purview of neural transplantation. Most neurodegenerative diseases are characterized not by discrete, focal abnormalities but rather by extensive, multifocal, or even global neuropathology. Such widely disseminated lesions have not conventionally been regarded as amenable to neural transplantation. However, the ability of NSCs to engraft diffusely and become integral members of structures throughout the host CNS, while also expressing therapeutic molecules, may permit these cells to address that challenge. Intriguingly, while NSCs can be readily engineered to express specified foreign genes, other intrinsic factors appear to emanate spontaneously from NSCs and, in the context of reciprocal donor-host signaling, seem to be capable of neuroprotective and/or neuroregenerative functions. Stem cells additionally have the appealing ability to 'home in' on pathology, even over great distances. Such observations help to advance the idea that NSCs - as a prototype for stem cells from other solid organs - might aid in reconstructing the molecular and cellular milieu of maldeveloped or damaged organs.
Abstract The goal of this study was to characterize long-term social and functional outcomes in adults treated for idiopathic normal pressure hydrocephalus (NPH). Data for 252 patients treated ...medically or surgically for idiopathic NPH were obtained through the Hydrocephalus Association Database Project. Data on post-surgical outcomes including improvement in symptoms, the need for in-home care, ability to drive, and employment status were analyzed. Most patients (73.7%) surveyed were treated with a shunt, an endoscopic third ventriculostomy (ETV), or both. More patients who underwent surgery reported driving and being employed compared to those who did not have surgery. Most shunt patients had improvements in gait (81.1%), urinary incontinence (55.9%), and dementia (64.4%). Overall, shunt patients reported more dramatic improvements in quality of life as compared to ETV patients (72.2% versus 55.6%). Treating idiopathic NPH with cerebrospinal fluid diversion facilitates a return to independence through improved functional and social outcomes.
The inherent biology of neural stem cells (NSCs) endows them with capabilities that not only circumvent many of the limitations of other gene transfer vehicles, but that enable a variety of novel ...therapeutic strategies heretofore regarded as beyond the purview of neural transplanation. Most neurodegenerative diseases are characterized not by discrete, focal abnormalities but rather by extensive, multifocal, or even global neuropathology. Such widely disseminated lesions have not conventionally been regarded as amenable to neural transplantation. However, the ability of NSCs to engraft diffusely and become integral members of structures throughout the host CNS, while also expressing therapeutic molecules, may permit these cells to address that challenge. Intriguingly, while NSCs can be readily engineered to express specified foreign genes, other intrinsic factors appear to emanate spontaneously from NSCs and, in the context of reciprocal donor-host signaling, seem to be capable of neuroprotective and/or neuroregenerative functions. Stem cells additionally have the appealing ability to `home in' on pathology, even over great distances. Such observations help to advance the idea that NSCs - as a prototype for stem cells from other solid organs - might aid in reconstructing the molecular and cellular milieu of maldeveloped or damaged organs.
We reported previously that astroglia cultured from aquaporin-4-deficient (AQP4⁻/⁻) mice migrate more slowly in vitro than those from wild-type (AQP4⁺/⁺) mice (J. Cell Sci. 2005;118, 5691-5698). ...Here, we investigate the migration of fluorescently labeled AQP4⁺/⁺ and AQP4⁻/⁻ astroglia after implantation into mouse brains in which directional movement was stimulated by a planar stab wound 3 mm away from the axis of the injection needle. Two days after cell injection we determined the location, elongation ratio, and orientation of labeled cells. Migration of AQP4⁺/⁺ but not AQP4⁻/⁻ cells toward the stab was greater than away from the stab. AQP4⁺/⁺ astroglia moved on average 1.5 mm toward the stab compared with 0.6 mm for AQP4⁻/⁻ cells. More than 25% of the migrating AQP4⁺/⁺ cells but <3% of AQP4⁻/⁻ cells appeared elongated (axial ratio>2.5). In transwell assays, AQP4⁺/⁺ astroglia migrated faster than AQP4⁻/⁻ cells in a manner dependent on pore size. At 8 h, ~50% of AQP4⁺/⁺ cells migrated through 8-μm diameter pores, whereas equivalent migration of AQP4⁻/⁻ cells was found for 12-μm diameter pores. These results provide in vivo evidence for AQP4-dependent astroglial migration and suggest that modulation of AQP4 expression or function might alter glial scarring--Auguste, K. I., Jin S., Uchida K., Yan D., Manley G. T., Papadopoulos M. C., Verkman A. S. Greatly impaired migration of implanted aquaporin-4-deficient astroglial cells in mouse brain toward a site of injury.
Evidence-based reviews support the use of venous thromboembolism (VTE) prophylaxis in the form of compression devices and/or stockings for patients undergoing craniotomy. In patients undergoing ...craniotomy with motor mapping for glioma, the contralateral lower extremity should remain visible so that motor responses can be accurately identified. As a consequence, these patients could be placed at a higher risk to develop VTE. The authors have quantified the incidence of VTE in patients undergoing craniotomy with motor mapping and have shown that there is no increased risk of developing a VTE in the contralateral lower extremity when compression devices are not used.
One hundred eighty consecutive cases (1997-2000) of craniotomy with motor mapping for glioma were retrospectively reviewed to determine the incidence and location of VTEs during the early postoperative course. Intraoperative VTE prophylaxis in all patients consisted of ipsilateral (that is, ipsilateral to the hemisphere being mapped) lower-extremity mechanical prophylaxis (antiembolism stocking plus compression device). Postoperatively, all patients received bilateral mechanical prophylaxis. Patients were observed until discharge and received clinical follow up. Venous thromboembolism, classified as deep venous thrombosis (DVT) or pulmonary embolism (PE) occurring within 6 weeks postoperatively, was confirmed by Doppler ultrasonography, spiral computerized tomography scanning, or both. The average duration of postoperative hospitalization was 5 days (range 2-59 days). Six patients (3.3%) experienced VTE. Of those, in four (2.2%) the DVT was localized to the contralateral (three patients) or ipsilateral (one patient) lower extremity. Two other patients (1.1%) only had PE. There were no deaths from thromboembolic complications and no statistically significant predisposition to VTE in the contralateral lower extremity among patients not receiving intraoperative prophylaxis.
The incidence of VTE in patients undergoing craniotomy with motor mapping is comparable to that in patients receiving bilateral lower-extremity mechanical VTE prophylaxis. The practice of leaving the contralateral lower extremity free from intraoperative prophylaxis does not appear to place patients at a higher risk for developing VTE. There appears to be no preferential distribution of VTE in contralateral lower extremities that do not receive immediate preoperative and intraoperative mechanical prophylaxis.
Expandable cylindrical cages (ECCs) have been utilized successfully to reconstruct the thoracic and lumbar spine. Their advantages include ease of insertion, reduced endplate trauma, direct ...application/maintenance of interbody distraction force, and one-step kyphosis correction. The authors present their experience with ECCs in the reconstruction of the cervical spine in patients with various pathological conditions.
Data obtained in 22 patients were reviewed retrospectively. A standard anterior cervical corpectomy was performed in all cases. Local vertebral body bone was harvested for use as graft material. Patients underwent pre- and postoperative assessment involving the visual analog scale (VAS), Nurick grading system for determining myelopathy disability, and radiographic studies to determine cervical kyphosis/lordosis and cage subsidence. Fusion was defined as the absence of motion on flexion-extension x-ray films. Sixteen patients presented with spondylotic myelopathy, two with osteomyelitis, two with fracture, one with tumor metastasis, and one with severe stenosis. Fourteen patients underwent supplemental posterior spinal fusion, seven underwent single-level corpectomy, and 15 patients underwent multilevel corpectomy. No perioperative complications occurred. The mean follow-up period was 22 months. In 11 patients with preexisting kyphosis (mean deformity +19 degrees), the mean correction was 22 degrees. There was no statistically significant difference in subsidence between single- and multilevel corpectomy or between 360 degrees fusion and anterior fusion alone. The VAS scores improved by 35%, and the Nurick grade improved by 31%. The fusion rate was 100%.
The preliminary results support the use of ECCs in the cervical spine in the treatment of patients with various disease processes. No significant subsidence was noted, and pain and functional scores improved in all cases. Expandable cylindrical cages appear to be well suited for cervical reconstruction and for correcting sagittal malalignment.
In recent years, it has become evident that the developing and even the adult mammalian CNS contain a population of undifferentiated, multipotent cell precursors, neural stem cells, the plastic ...properties of which might be of advantage for the design of more effective therapies for many neurological diseases. This article reviews the recent progress in establishing rodent and human clonal neural stem cell lines, their biological properties, and how these cells can be utilized to correct a variety of defects, with prospects for the near future to harness their behaviour for neural stem cell-based treatment of diseases in humans.
PDF
