Severe Hypothermia in a Patient withCerebral Relapse of Whipple's Disease

Abstract. : The diagnosis of cerebral relapse of Whipple's disease in a 67-year-old patient was made after he presented with somnolence and severe hypothermia 4 months after discontinuing treatment with cotrimoxazole. Hypothermia is a rare hypothalamic manifestation of cerebral Whipple's diseas

Multifocal Vasculopathy Due to Varicella-Zoster Virus (VZV): Serial Analysis of VZV DNA and Intrathecal Synthesis of VZV Antibody in Cerebrospinal Fluid

Recognition of multifocal vasculopathy due to varicella-zoster virus (VZV) is often problematic. We describe a human immunodeficiency virus—infected patient who had progressive central nervous system disease for >3 months. Both VZV DNA and antibody were detected in cerebrospinal fluid (CSF) specimens; serial polymerase chain reaction analyses confirmed the diagnosis and guided the duration of therapy. Reduced ratios of VZV antibody in serum to that in CSF were also demonstrate

Blockage of longitudinal flow in Meniere's disease: A human temporal bone study

Conclusion: Blockage of the endolymphatic duct is a significant finding in Meniere's disease. The position of the utriculo-endolymphatic valve (UEV) and blockage of the ductus reuniens in the temporal bones were not found to be directly indicative of Meniere's disease. Objective: Comparison of blockage of the longitudinal flow of endolymph between ears affected by Meniere's disease and normal ears. Methods: We examined 21 temporal bones from 13 subjects who had Meniere's disease and 21 normal temporal bones from 12 controls. Results: The endolymphatic duct was blocked in five (23%) ears affected by Meniere's disease (p = 0.016). The utricular duct was blocked in 16 (76%) ears affected by Meniere's disease and 11 (52%) normal ears (p = 0.112). The saccular duct was blocked in 6 (28%) of ears affected by Meniere's disease and 16 (76%) normal ears (p = 0.001). The ductus reuniens was blocked in 10 (47%) ears affected by Meniere's disease and 10 (47%) normal ears (p = 1.000)

Visualization and assessment of saccular duct and endolymphatic sinus

Conclusion: The saccular duct and endolymphatic sinus run in the bony groove, before reaching the orifice of the vestibular aqueduct. We first clinically visualized this sulciform groove using three-dimensional (3D) cone beam CT images. This strategy can be useful to assess the condition of the saccular duct and endolymphatic sinus concerning the longitudinal flow system of endolymph. Objective: To assess the saccular duct and endolymphatic sinus in the endolymphatic system in order to advance clinical studies on inner ear dysfunction. Methods: The sulciform groove of the saccular duct and endolymphatic sinus of human subjects was analyzed by cone beam CT and compared with that of a cadaver. Results: We could obtain reconstructed 3D CT images of the sulciform groove of the saccular duct and endolymphatic sinus using several CT window levels

A Bast-like valve in the pigeon?

The first description of the presence of a utriculo-endolymphatic valve in human fetuses was given by Bast in 1928. Since then this valve-like structure is called Bast’s valve. Its exact function has not yet been established. The general opinion is that it has a protective function by having the possibility to separate the superior endolymphatic compartments of the labyrinth from the inferior compartment. Phylogenetically seen birds are the first vertebrates with a cochlear duct and a distinct inferior and superior part of the labyrinth. A structure in the pigeon inner ear, resembling Bast’s valve in mammals, is described

Radiological feature heterogeneity supports etiological diversity among patient groups in Meniere's disease

We aimed to determine the prevalence of radiological temporal bone features that in previous studies showed only a weak or an inconsistent association with the clinical diagnosis of Meniere's disease (MD), in two groups of MD patients (n = 71) with previously established distinct endolymphatic sac pathologies; i.e. the group MD-dg (ES degeneration) and the group MD-hp (ES hypoplasia). Delayed gadolinium-enhanced MRI and high-resolution CT data were used to determine and compare between and within (affected vs. non-affected side) groups geometric temporal bone features (lengths, widths, contours), air cell tract volume, height of the jugular bulb, sigmoid sinus width, and MRI signal intensity alterations of the ES. Temporal bone features with significant intergroup differences were the retrolabyrinthine bone thickness (1.04 ± 0.69 mm, MD-hp; 3.1 ± 1.9 mm, MD-dg; p < 0.0001); posterior contour tortuosity (mean arch-to-chord ratio 1.019 ± 0.013, MD-hp; 1.096 ± 0.038, MD-dg; p < 0.0001); and the pneumatized volume (1.37 [0.86] cm3, MD-hp; 5.25 [3.45] cm3, MD-dg; p = 0.03). Features with differences between the affected and non-affected sides within the MD-dg group were the sigmoid sinus width (6.5 ± 1.7 mm, affected; 7.6 ± 2.1 mm, non-affected; p = 0.04) and the MRI signal intensity of the endolymphatic sac (median signal intensity, affected vs. unaffected side, 0.59 [IQR 0.31-0.89]). Radiological temporal bone features known to be only weakly or inconsistently associated with the clinical diagnosis MD, are highly prevalent in either of two MD patient groups. These results support the existence of diverse-developmental and degenerative-disease etiologies manifesting with distinct radiological temporal bone abnormalities

Degenerative Veränderungen im alternden Innenohr, mit besonderer Berücksichtigung der vasculären Veränderungen, in Flächenpräparaten der menschlichen Cochlea dargestellt

Temporal bones from 150 patients, ranging in age from fetuses and newborn to 97 years, were studied by the technique of microdissection and the use of surface specimens stained with OSO 4 . Hair cell and nerve degeneration were seen in the extreme basal turn of the cochlea even in children. In the fetal cochlea vascularization is very dense, in the newborn and infant somewhat less so. A gradual involution of blood vessels occurs postnatally and continues with maturity and aging. Involution is seen especially in the membranous wall of the cochlea and in the system of spiral vessels of the basilar membrane and vestibular lip. During the first decade the radiating arterioles and the outer spiral vessel in the basal turn are reduced to their adult size. In presbycusis material we observed a marked loss of capillaries and of some of the radiating arterioles in the spiral ligament. Other arterioles had thickened walls. This devascularization was accompanied by atrophy and acellularity of the spiral ligament and atrophy of the stria. Atrophy of the spiral vessels was seen, especially in the lower half of the basal turn. Most of the cochlear blood vessels have clearly distinguishable perivascular spaces. Vessels which had become occluded and disappeared left behind them intervascular strands and/or avascular channels . Such channels were seen to connect the perivascular space of one capillary with that of another, representing the perivascular space of the vessel which had atrophied. The hair cell and nerve degeneration seen in presbycusis may be caused, at least in part, by microangiopathy of this type. Similar vascular changes have been observed in retinal vessels. It is possible that the gradual reduction of blood supply through the disappearance of capillaries occurs in many tissues of the body and plays an important role in the aging process. Mit Hilfe der Oberflächenpräparation wurden von uns 150 menschliche Labyrinthe aller Altersgruppen untersucht. Es wurde eine Haarzellen-und Nervendegeneration schon im Kindesalter beobachtet. Blutgefäße in der Schnecke haben perivasculdre Spalten; Gefäße atrophieren and werden zu „avascular channels”, leeren Spaltrdumen oder Striingen. Bei Presbyakusis fällt neben der Haarzell- and Nervendegeneration in der Basalwindung der Schnecke die Gefäßatrophie im Ligamentum spirale und der Membrana basilaris auf. Dazu kommt noch eine beträchtliche Atrophie des Ligamentum spirale und der Stria vascularis. Bemerkenswert ist, daß die Devascularisation schon im Kindesalter beginnt.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47255/1/405_2004_Article_BF00373313.pd

Endotype-Phenotype Patterns in Meniere's Disease Based on Gadolinium-Enhanced MRI of the Vestibular Aqueduct

Two histopathological subtypes of Meniere's disease (MD) were recently described in a human post-mortem pathology study. The first subtype demonstrated a degenerating distal endolymphatic sac (ES) in the affected inner ear (subtype MD-dg); the second subtype (MD-hp) demonstrated an ES that was developmentally hypoplastic. The two subtypes were associated with different clinical disease features (phenotypes), suggesting that distinct endotype-phenotype patterns exist among MD patients. Therefore, clinical endotyping based on ES pathology may reveal clinically meaningful MD patient subgroups. Here, we retrospectively determined the ES pathologies of clinical MD patients (n = 72) who underwent intravenous delayed gadolinium-enhanced inner ear magnetic resonance imaging using previously established indirect radiographic markers for both ES pathologies. Phenotypic subgroup differences were evidenced; for example, the MD-dg group presented a higher average of vertigo attacks (ratio of vertigo patterns daily/weekly/other vs. monthly, MD-dg: 6.87: 1; MD-hp: 1.43: 1; p = 0.048) and more severely reduced vestibular function upon caloric testing (average caloric asymmetry ratio, MD-dg: 30.2% ± 30.4%; MD-hp: 13.5% ± 15.2%; p = 0.009), while the MD-hp group presented a predominantly male sex ratio (MD-hp: 0.06:1 [f/m]; MD-dg: 1.2:1 [f/m]; p = 0.0004), higher frequencies of bilateral clinical affection (MD-hp: 29.4%; MD-dg: 5.5%; p = 0.015), a positive family history for hearing loss/vertigo/MD (MD-hp: 41.2%; MD-dg: 15.7%; p = 0.028), and radiographic signs of concomitant temporal bone abnormalities, i.e., semicircular canal dehiscence (MD-hp: 29.4%; MD-dg: 3.6%; p = 0.007). In conclusion, this new endotyping approach may potentially improve the diagnosis, prognosis and clinical decision-making for individual MD patients

Fiscal redistribution around elections when democracy is not "the only game in town"

This paper seeks to examine the implications of policy intervention around elections on income inequality and fiscal redistribution. We first develop a simplified theoretical framework that allows us to examine election-cycle fiscal redistribution programs in the presence of a revolutionary threat from some groups of agents, i.e., when democracy is not “the only game in town”. According to our theoretical analysis, when democracy is not “the only game in town”, incumbents implement redistributive policies not only as a means of improving their reelection prospects, but also in order to signal that “democracy works”, thereby preventing a reversion to an autocratic status quo ante at a time of the current regime’s extreme vulnerability. Subsequently, focusing on 65 developed and developing countries over the 1975–2010 period, we report robust empirical evidence of pre-electoral budgetary manipulation in new democracies. Consistent with our theory, this finding is driven by political instability that induces incumbents to redistribute income—through tax and spending policies—in a relatively broader coalition of voters with the aim of consolidating the vulnerable newly established democratic regime