The pathological diagnosis of nerve biopsies: a practical approach

The approach to the neuropathological assessment of nerve biopsies is the main focus of this review. Nerve biopsies are invasive diagnostic procedures resulting in a permanent neurological deficit, and are therefore carried out only following an in-depth clinical assessment including laboratory, imaging, electrophysiological, and where appropriate also genetic studies. This review will outline the key diagnostic approaches and will discuss neuropathies relevant in clinical practice, caused by vasculitis, inflammatory demyelination, dysproteinaemic, amyloid, toxic agents, and neuropathies due to genetic conditions

Prion disease: experimental models and reality

The understanding of the pathogenesis and mechanisms of diseases requires a multidisciplinary approach, involving clinical observation, correlation to pathological processes, and modelling of disease mechanisms. It is an inherent challenge, and arguably impossible to generate model systems that can faithfully recapitulate all aspects of human disease. It is, therefore, important to be aware of the potentials and also the limitations of specific model systems. Model systems are usually designed to recapitulate only specific aspects of the disease, such as a pathological phenotype, a pathomechanism, or to test a hypothesis. Here, we evaluate and discuss model systems that were generated to understand clinical, pathological, genetic, biochemical, and epidemiological aspects of prion diseases. Whilst clinical research and studies on human tissue are an essential component of prion research, much of the understanding of the mechanisms governing transmission, replication, and toxicity comes from in vitro and in vivo studies. As with other neurodegenerative diseases caused by protein misfolding, the pathogenesis of prion disease is complex, full of conundra and contradictions. We will give here a historical overview of the use of models of prion disease, how they have evolved alongside the scientific questions, and how advancements in technologies have pushed the boundaries of our understanding of prion biology

The role of prion-like mechanisms in neurodegenerative diseases

The prototype of transmissible neurodegenerative proteinopathies is prion disease, characterised by aggregation of abnormally folded conformers of the native prion protein. A wealth of mechanisms have been proposed to explain the conformational conversion from physiological protein into misfolded, pathological form, mode of toxicity, propagation from cell to cell and regional spread. There is increasing evidence that other neurodegenerative diseases, most notably Alzheimer's disease (Aβ and tau), Parkinson's disease (α-synuclein), frontotemporal dementia (TDP43, tau or FUS) and motor neurone disease (TDP43), exhibit at least some of the misfolded prion protein properties. In this review, we will discuss to what extent each of the properties of misfolded prion protein is known to occur for Aβ, tau, α-synuclein and TDP43, with particular focus on self-propagation through seeding, conformational strains, selective cellular and regional vulnerability, stability and resistance to inactivation, oligomers, toxicity and summarise the most recent literature on transmissibility of neurodegenerative disorders

Neurological update: gliomas and other primary brain tumours in adults.

The emerging understanding of molecular changes in a wide range of brain tumours has led to a significant shift in how these tumours are diagnosed, managed and treated. This article will provide a hands-on overview of the relevant biomarkers and their association with newly defined biological tumour entities

Microcystic Cerebral Neoplasm in a Nilgai Antelope (Boselaphus tragocamelus): Putative Microcystic Meningioma

Tumours of the nervous system are rare in wild and captive mammals. In this report, we describe an intracranial, solid, space-occupying lesion originating from the meninges in a Nilgai antelope (Boselaphus tragocamelus). Histologically, the tumour had a conspicuous microcystic appearance with features similar to the histological subtype of microcystic meningioma described in humans. This is the first such tumour reported in this species

Microstructure devices for water evaporation

This paper was presented at the 2nd Micro and Nano Flows Conference (MNF2009), which was held at Brunel University, West London, UK. The conference was organised by Brunel University and supported by the Institution of Mechanical Engineers, IPEM, the Italian Union of Thermofluid dynamics, the Process Intensification Network, HEXAG - the Heat Exchange Action Group and the Institute of Mathematics and its Applications.Evaporation of liquids is of major interest for many topics in process engineering. One of these is chemical process engineering, where evaporation of liquids and generation of superheated steam is mandatory for numerous processes. Generally, this is performed by use of classical pool boiling and evaporation process equipment, providing relatively limited performance, or by other systems like falling-film evaporators. Due to the advantages of microstructure devices especially in chemical process engineering the interest in microstructure evaporators and steam generators have been increased through the last decade. In this publication different microstructure devices used for evaporation and generation of steam will be described. Starting with simple liquid-heated devices, different types of electrically powered devices containing micro channels as well as non-channel microstructures will be shown. While evaporation of liquids in crossflow and counterflow or co-current flow micro channel devices is possible, it is, in many cases, not possible to obtain superheated steam due to certain boundary conditions. Thus, a new design was proposed to obtain complete evaporation and superheating of the generated steam

GRAVITY: The AO-Assisted, Two-Object Beam-Combiner Instrument

We present the proposal for the infrared adaptive optics (AO) assisted, two-object, high-throughput, multiple-beam-combiner GRAVITY for the VLTI. This instrument will be optimized for phase-referenced interferometric imaging and narrow-angle astrometry of faint, red objects. Following the scientific drivers, we analyze the VLTI infrastructure, and subsequently derive the requirements and concept for the optimum instrument. The analysis can be summarized with the need for highest sensitivity, phase referenced imaging and astrometry of two objects in the VLTI beam, and infrared wavefront-sensing. Consequently our proposed instrument allows the observations of faint, red objects with its internal infrared wavefront sensor, pushes the optical throughput by restricting observations to K-band at low and medium spectral resolution, and is fully enclosed in a cryostat for optimum background suppression and stability. Our instrument will thus increase the sensitivity of the VLTI significantly beyond the present capabilities. With its two fibers per telescope beam, GRAVITY will not only allow the simultaneous observations of two objects, but will also push the astrometric accuracy for UTs to 10 micro-arcsec, and provide simultaneous astrometry for up to six baselines.Comment: 12 pages, to be published in the Proceedings of the ESO Workshop on "The Power of Optical/IR Interferometry: Recent Scientific Results and 2nd Generation VLTI Instrumentation", eds. F. Paresce, A. Richichi, A. Chelli and F. Delplancke, held in Garching, Germany, 4-8 April 200

Extensive spherical amyloid deposition presenting as a pituitary tumor

A 71-yr-old man was admitted for further evaluation and trans-sphenoidal surgery of a pituitary tumor. He complained of impotence and decreased libido over a period of about 40 yr. Thirty-eight yr ago he was treated for bilateral gynecomastia with galactorrhea. Endocrinological investigation at presentation revealed only mild hyperprolactinemia and hypogonadotropic hypogonadism. Pituitary magnetic resonance imaging (MRI) showed a tumor up to 2.5 cm in diameter with infiltration of the sphenoid sinus and right cavernous sinus. The tumor exhibited a heterogeneous hyperintense signal on T1-weighted images and hypointense signal on T2-weighted images. Standard trans-sphenoidal surgery was performed and a brownish mass was found inside the sella, which was removed. Histological examination of the mass revealed extensive spherical amyloid deposits with strongly positive immunohistochemical staining for prolactin. Therefore, a prolactinoma with extensive spherical amyloid deposition was diagnosed. Extensive spherical amyloid deposition is a rare finding in prolactin-secreting pituitary adenomas. So far, characteristic radiological findings by MRI have been described only twice. Due to characteristic MRI findings, the diagnosis of extensive intrasellar amyloid deposition can be entertained pre-operatively. Trans-sphenoidal surgical resection is essential to confirm the diagnosis histologically and because of the potential lack of tumor shrinkage under dopaminagonist therapy in this type of prolactinom