49 research outputs found

    Anatomical evidence for an ascending somatosensory pathway to the telencephalon in crocodiles, Caiman crocodilus

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    A series of experiments in Caiman crocodilus that outlines an ascending somatosensory pathway to the telencephalon is described. This telencephalic somatosensory area was found to correspond to a region high in succinate dehydrogenase activity.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/46550/1/221_2004_Article_BF00237800.pd

    Succinate dehydrogenase activity in the telencephalon of crocodiles correlates with the projection areas of sensory thalamic nuclei

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    Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/22953/1/0000520.pd

    Periodontal Ehlers-Danlos Syndrome Is Caused by Mutations in C1R and C1S, which Encode Subcomponents C1r and C1s of Complement

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    Periodontal Ehlers-Danlos syndrome (pEDS) is an autosomal-dominant disorder characterized by early-onset periodontitis leading to premature loss of teeth, joint hypermobility, and mild skin findings. A locus was mapped to an approximately 5.8 Mb region at 12p13.1 but no candidate gene was identified. In an international consortium we recruited 19 independent families comprising 107 individuals with pEDS to identify the locus, characterize the clinical details in those with defined genetic causes, and try to understand the physiological basis of the condition. In 17 of these families, we identified heterozygous missense or in-frame insertion/deletion mutations in C1R (15 families) or C1S (2 families), contiguous genes in the mapped locus that encode subunits C1r and C1s of the first component of the classical complement pathway. These two proteins form a heterotetramer that then combines with six C1q subunits. Pathogenic variants involve the subunit interfaces or inter-domain hinges of C1r and C1s and are associated with intracellular retention and mild endoplasmic reticulum enlargement. Clinical features of affected individuals in these families include rapidly progressing periodontitis with onset in the teens or childhood, a previously unrecognized lack of attached gingiva, pretibial hyperpigmentation, skin and vascular fragility, easy bruising, and variable musculoskeletal symptoms. Our findings open a connection between the inflammatory classical complement pathway and connective tissue homeostasis

    Endocrinologic, neurologic, and visual morbidity after treatment for craniopharyngioma

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    Craniopharyngiomas are locally aggressive tumors which typically are focused in the sellar and suprasellar region near a number of critical neural and vascular structures mediating endocrinologic, behavioral, and visual functions. The present study aims to summarize and compare the published literature regarding morbidity resulting from treatment of craniopharyngioma. We performed a comprehensive search of the published English language literature to identify studies publishing outcome data of patients undergoing surgery for craniopharyngioma. Comparisons of the rates of endocrine, vascular, neurological, and visual complications were performed using Pearson’s chi-squared test, and covariates of interest were fitted into a multivariate logistic regression model. In our data set, 540 patients underwent surgical resection of their tumor. 138 patients received biopsy alone followed by some form of radiotherapy. Mean overall follow-up for all patients in these studies was 54 ± 1.8 months. The overall rate of new endocrinopathy for all patients undergoing surgical resection of their mass was 37% (95% CI = 33–41). Patients receiving GTR had over 2.5 times the rate of developing at least one endocrinopathy compared to patients receiving STR alone or STR + XRT (52 vs. 19 vs. 20%, χ2P < 0.00001). On multivariate analysis, GTR conferred a significant increase in the risk of endocrinopathy compared to STR + XRT (OR = 3.45, 95% CI = 2.05–5.81, P < 0.00001), after controlling for study size and the presence of significant hypothalamic involvement. There was a statistical trend towards worse visual outcomes in patients receiving XRT after STR compared to GTR or STR alone (GTR = 3.5% vs. STR 2.1% vs. STR + XRT 6.4%, P = 0.11). Given the difficulty in obtaining class 1 data regarding the treatment of this tumor, this study can serve as an estimate of expected outcomes for these patients, and guide decision making until these data are available

    Closure of Dural Defects after Anterior Clinoid and Optic Canal Roof Removal: Technical Note

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    A technique using vascularized pericranium to close dural defects after anterior clinoid and/or optic canal roof removal is described. This approach is simple, inexpensive, and uses autologous tissue. This method has provided satisfactory dural closure and has avoided cerebrospinal fluid leaks or extradural accumulation of cerebrospinal fluid

    The transsylvian approach to middle cerebral artery bifurcation/trifurcation aneurysms

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    A transsylvian approach to treat aneurysms located at the bifurcation/trifurcation of the middle cerebral artery (MCA) is described. This surgical route traces a distal MCA branch retrogradely into the Sylvian fissure where-upon a secondary MCA branch and the main stem of the MCA (Ml) are identified before aneurysm clipping. The advantages and disadvantages of this approach are discussed. The transsylvian route should be considered as an alternative to the traditional pterional approach, which utilizes proximal to distal dissection of the MCA, in situations when the M1 is long or when the pterional approach is inadvisable.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/31745/1/0000684.pd

    A simple method for distal catheter lengthening of ventriculoatrial shunts

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