Look granulomatosis with polyangiitis (GPA) straight in the face: missed opportunities leading to a delayed diagnosis.

Granulomatosis with polyangiitis (GPA) is a systemic vasculitis with a potential to involve any organ system. It remains an important cause of kidney related morbidity and mortality. Early diagnosis can be difficult and requires high index of suspicion in all patients, but especially in cases with atypical presentation. We report a case with GPA, which was diagnosed only after new and advancing symptoms belied the original diagnosis of bilateral facial palsy and aortic mural thrombus.

Multiple vasodilator pathways from the pelvic plexus to the penis of the rat.

The main penile or cavernous nerve is usually regarded as the most important vasodilator projection in the rat. Although other descending pathways have been described, there is little detailed information on their importance. In this present report, we provide topographic and quantitative information on lateral and ventral penile branches and examine the vasodilator fibers which join the pudendal neurovascular bundle. Seventeen Sprague-Dawley rats were used. The techniques included injection of dye in the penis to label neurons in the pelvic plexus in combination with transection of the main penile nerve (MPN). NADPH diaphorase (NADPH-d) histochemistry was used to assess the effects of transection of vasodilator pathways on innervation of the penis and for in situ staining of the pelvic plexus. Distinct clusters of penile neurons are aggregated at the origin of several nerve tracts leaving the posterior margin of the major pelvic ganglion (MPG). Multiple NADPH-d+ fiber bundles coursed over the anterior surface of the prostate to reach the penis. Branches from these tracts joined the pudendal neurovascular bundle proximal to the hilum of the penis and provided innervation to the artery throughout its course in the pudendal canal. Consistent with the presence of multiple penile pathways, transection of the MPN reduced, but did not eliminate retrograde labeling of penile neurons in the MPG and only modestly decreased NADPH-d+ fibers in the penis. This study confirms that there are multiple pathways by which vasodilator fibers reach the penis. If a similar allocation of vasodilator output is present in man, preservation of finer branches of the pelvic plexus would be important in surgical procedures on the prostate.

Preganglionic fibers in the rat hypogastric nerve project bilaterally to pelvic ganglia.

Stimulation of the hypogastric nerve (HGN) often evokes bilateral responses in some pelvic organs. Retrograde labeling studies indicate that axons of postganglionic neurons often cross to the opposite side. However, there is little information available as to whether preganglionic fibers in the HGN have a contralateral projection to pelvic ganglia. A retrograde tracer was injected into the left major pelvic ganglion (MPG) in rats receiving various lesions of preganglionic nerves (HGN and pelvic nerve, PN). The lumbar spinal cord was then examined for location and number of dye-filled neurons. In a second approach, the incidence of synaptophysin immunoreactivity (SN-IR) perineuronal profiles (baskets) was examined in the MPG and in the accessory pelvic ganglia (APG) after nerve lesions. Labeled neuronal profiles were found in spinal cord nuclei (Lumbar1-2) after dye injection of the MPG in animals with an intact contralateral HGN. Cutting both HGNs virtually eliminated dye labeling in the lumbar cord, as did severing commissural branches (CB) between pelvic ganglia (leaving the contralateral HGN intact). Some SN-IR baskets were found in the left APG when only the contralateral HGN was intact, but baskets were rare when all four preganglionic nerves were cut. It could not be determined whether the HGN projects to the contralateral MPG, since SN-IR baskets were numerous in the MPG even when all four nerves were cut. This study has shown that some preganglionic fibers in the HGN synapse on neurons in contralateral pelvic ganglia. Both the APG and MPG receive contralateral innervation, but it is likely that neurons in the APG are the primary target of this input. Thus, in addition to crossing postganglionic fibers, a portion of the bilateral control of pelvic tissues is accomplished by preganglionic fibers which target autonomic neurons in contralateral ganglia.

Cytochrome oxidase staining in the major pelvic ganglion of the male rat.

Cytochrome oxidase staining was used as a marker of metabolic activity in neural elements in the rat major pelvic ganglion. Many neurons in the ventral pole of the ganglion have little cytochrome oxidase activity, while neurons in other locations show gradations in staining intensity. Punctate staining around principal neurons may represent preganglionic terminals, since it was greatly reduced after denervation of the ganglion. Image analysis was used to compare neuronal size to staining intensity. There was a negative correlation between cell size and staining intensity; the largest neurons were only lightly stained for cytochrome oxidase, while the medium and the small neurons showed a full range of metabolic activity. To study metabolic activity of an identified neuronal population, the seminal vesicles were injected with a retrograde tracer. The largest seminal vesicles neurons (1500 to 3200 microns2) had low enzyme activity, whereas the majority of neurons to this organ were smaller with gradations in staining. These results are indicative of the metabolic activity of the autonomic innervation to various pelvic tissues. Cytochrome oxidase histochemistry should prove valuable in assessing the demands placed on autonomic ganglia in differing functional and dysfunctional states.