Intraoperative nerve action and compound motor action potential recordings in patients with obstetric brachial plexus lesions.

OBJECT: A typical finding in supraclavicular exploration of infants with severe obstetric brachial plexus lesions (OBPLs) is a neuroma-in-continuity with the superior trunk and/or a root avulsion at C-5, C-6, or C-7. The operative strategy in these cases is determined by the intraoperative assessment of the severity of the lesion. Intraoperative nerve action potential (NAP) and evoked compound motor action potential (CMAP) recordings have been shown to be helpful diagnostic tools in adults, whereas their value in the intraoperative assessment of infants with OBPLs remains to be determined. METHODS: Intraoperative NAPs and CMAPs were systematically recorded from damaged and normal nerves of the upper brachial plexus in a consecutive series of 95 infants (mean age 175 days) with OBPLs. A total of 599 intraoperative NAP and 836 CMAP recordings were analyzed. The severity of the nerve lesions was graded as normal, axonotmesis, neurotmesis, or root avulsion, based on surgical, clinical, histological, and radiographic criteria. RESULTS: The correlation of NAP and CMAP recordings with the severity of the lesion was assessed. The specificity of an absent NAP or CMAP to predict a severe lesion (neurotmesis or avulsion) was > 0.9. However, the sensitivity of an absent NAP or CMAP for predicting a severe lesion was low (typically < 0.3). The severity of the nerve lesion was related to CMAP and NAP amplitudes. Cutoff points useful for intraoperative decision making could not be found to differentiate between lesion types in individual patients. CONCLUSIONS: Intraoperative NAP and CMAP recordings do not assist in decision making in the surgical treatment of infants with OBPLs. The authors' findings in infants cannot be generalized to adults.

The X-linked mental retardation protein oligophrenin-1 is required for dendritic spine morphogenesis.

Of 11 genes involved in nonspecific X-linked mental retardation (MRX), three encode regulators or effectors of the Rho GTPases, suggesting an important role for Rho signaling in cognitive function. It remains unknown, however, how mutations in Rho-linked genes lead to MRX. Here we report that oligophrenin-1, a Rho-GTPase activating protein that is absent in a family affected with MRX, is required for dendritic spine morphogenesis. Using RNA interference and antisense RNA approaches, we show that knock-down of oligophrenin-1 levels in CA1 neurons in rat hippocampal slices significantly decreases spine length. This phenotype can be recapitulated using an activated form of RhoA and rescued by inhibiting Rho-kinase, indicating that reduced oligophrenin-1 levels affect spine length by increasing RhoA and Rho-kinase activities. We further demonstrate an interaction between oligophrenin-1 and the postsynaptic adaptor protein Homer. Our findings provide the first insight into how mutations in a Rho-linked MRX gene may compromise neuronal function.

A specialized vascular niche for adult neural stem cells.

Stem cells reside in specialized niches that regulate their self-renewal and differentiation. The vasculature is emerging as an important component of stem cell niches. Here, we show that the adult subventricular zone (SVZ) neural stem cell niche contains an extensive planar vascular plexus that has specialized properties. Dividing stem cells and their transit-amplifying progeny are tightly apposed to SVZ blood vessels both during homeostasis and regeneration. They frequently contact the vasculature at sites that lack astrocyte endfeet and pericyte coverage, a modification of the blood-brain barrier unique to the SVZ. Moreover, regeneration often occurs at these sites. Finally, we find that circulating small molecules in the blood enter the SVZ. Thus, the vasculature is a key component of the adult SVZ neural stem cell niche, with SVZ stem cells and transit-amplifying cells uniquely poised to receive spatial cues and regulatory signals from diverse elements of the vascular system.