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Zilli  T.  Allal  A. S. 《Neurological sciences》2011,32(1):151-153
Guillain-Barré syndrome (GBS) is an acute demyelinating polyradiculoneuropathy normally associated with a preceding infection, but sometimes it can be linked to a subjacent malignancy. We report an unusual case of GBS occurring as the first clinical manifestation of an esophageal adenocarcinoma in a 65-year-old patient. A GBS neuropathy of undetermined origin may be associated with an underlying tumor and esophageal cancer has to be considered in the differential diagnosis.  相似文献   

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Congenital bilateral perisylvian syndrome (CBPS) is a syndrome of cortical malformation characterized by faciopharyngoglossomasticatory diplegia. We report on two cases of CBPS with associated esophageal malformations and a poor mental and motor development. The association of CBPS and esophageal malformations may indicate a subgroup of patients with a very early prenatal injury, characterised by a bad prognosis due to severe cortical disorganization. However, it can not be excluded that the association of CBPS and esophageal malformation is purely coincidental.  相似文献   

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Background The advent of drugs that inhibit transient lower esophageal sphincter relaxation (TLESR) necessitates accurate identification and scoring. We assessed the intra‐ and inter‐assessor variability of the existing objective criteria for TLESR, improving them where necessary. Methods Two 3‐h postprandial esophageal manometric and pH recordings were performed in 20 healthy volunteers. Each recording was duplicated. The recordings were analyzed by five experienced observers for TLESRs based on their expert opinion. TLESRs were also analyzed for the presence of the original four criteria as well as inhibition of the crural diaphragm (ID), a prominent after‐contraction (AC), acid reflux and an esophageal common cavity. Key Results The overall inter‐ and intra‐observer agreements for TLESRs scored, according to observer’s expert opinion, were 59% (range 56–67%) and 74% (60–89%), respectively. When TLESRs were restricted to those fulfilling the original criteria, these agreements fell to 46% (40–53%) and 60% (44–67%), respectively. Cleaning the recordings by removal of technically flawed sections improved agreements by 5%. Inclusion of additional criteria (ID and AC) resulted in inter‐ and intra‐observer agreements of 62% (52–70%) and 69% (53–79%), respectively. A consensus analysis performed collectively by three observers and based on the new criteria (original ± ID and AC) resulted in 84% agreement between the paired recordings. Conclusions & Inferences The original criteria for the definition of TLESRs allows for substantial inter‐ and intra‐observer variability, which can be reduced by incorporation of additional objective criteria. However, the highest level of intra‐observer agreement can be achieved by consensus analysis.  相似文献   

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Background The gastric sling muscle has not been investigated for possible sensory innervation, in spite of the key roles the structure plays in lower esophageal sphincter (LES) function and gastric physiology. Thus, the present experiment used tracing techniques to label vagal afferents and survey their projections in the lesser curvature. Methods Sprague–Dawley rats received injections of dextran biotin into the nodose ganglia. Fourteen days postinjection, animals were euthanized and their stomachs were processed to visualize the vagal afferent innervation. In different cases, neurons, muscle cells, or interstitial cells of Cajal (ICC) were counterstained. Key Results The sling muscle is innervated throughout its length by vagal afferent intramuscular arrays (IMAs) associated with ICC. In addition, the distal antral attachment site of the sling muscle is innervated by a novel vagal afferent terminal specialization, an antral web ending. The muscle wall of the distal antrum is also innervated by conventional IMAs and intraganglionic laminar endings, the two types of mechanoreceptors found throughout stomach smooth muscle. Conclusions & Inferences The innervation of sling muscle by IMAs, putative stretch receptors, suggests that sling sensory feedback may generate vago‐vagal or other reflexes with vagal afferent limbs. The restricted distribution of afferent web endings near the antral attachments of sling fibers suggests the possibility of specialized mechanoreceptor functions linking antral and pyloric activity to the operation of the LES. Dysfunctional sling afferents could generate LES motor disturbances, or normative compensatory sensory feedback from the muscle could compromise therapies targeting only effectors.  相似文献   

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