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PubMed2001

A case of rapidly progressive multiple system degeneration: morphological findings and pathogenetic implications.

A 62-year-old woman was referred to our psychiatric hospital by the municipal health office, because she was in such a neglected condition that she was a danger to herself. Initially, it was suspected that she was suffering from dementia or psychosis. X-rays led to the suspicion of bronchial carcinoma. Consequently, the mental changes were interpreted as probable effects of metastases to the brain. There was not enough time, however, to check the patient thoroughly to find the actual cause of her altered personality. The patient developed a high fever (up to 42 degrees C). A few days later, she died of cardiorespiratory failure with severe abscess-forming obstructive pneumonia. An autopsy confirmed the bronchial carcinoma. However, metastases were only found in the hilar lymph nodes. No metastases were detectable in the CNS, either macroscopically or microscopically. The neuropathological examination of the brain revealed multiple system degeneration. The striking microscopic findings (a large number of typical apoptotic figures visible with the light microscope in ganglion cells, lack of cytoplasmic inclusion bodies in the oligodendroglia and an unusually strong monocytic reaction (so-called reactive satellitosis) indicated that the disease course had been very rapid. Perhaps these were early steps in the evolution of a multisystem atrophy. A kind of time-lapse effect, as it were, revealed simultaneously individual details of the pathogenetic course, which would have disappeared in the usual long course of the disease and thus could not have been observed, even with the most up-to-date molecular methods. It is very likely that this was a paraneoplastic syndrome.

Brain↗
PubMed1994

Nodular cutaneous lupus mucinosis associated with atrophie blanche-like lesions in a patient with systemic lupus erythematosus.

BACKGROUND: Multiple, pale, atrophic depressions surrounded by erythema developed on nodular cutaneous mucinosis lesions in a patient with systemic lupus erythematosus. OBSERVATIONS: A 22-year-old man developed multiple nodular erythematous lesions on the upper arms, back, and chest during the course of systemic lupus erythematosus. Histologically, these nodular lesions were diagnosed as lupus mucinosis. Three years later, the patient began to have atrophic depressed lesions on several nodular mucinosis lesions. These lesions did not develop on normal appearing skin. A biopsy specimen from one of these lesions showed occlusion of blood vessels with thickening of their walls and perivascular dense lymphocytic infiltration. No leukocytoclastic vasculitis was found. Hyaline degeneration was present in some parts of the fatty tissue. CONCLUSION: The atrophic depressed lesions were atrophie blanche-like and induced by the vascular occlusion. Since these lesions developed only on the lupus mucinosis lesions, mucin deposition and vascular changes may be closely related.

Adult↗
PubMed2006

IBZM tool: a fully automated expert system for the evaluation of IBZM SPECT studies.

PURPOSE: Visual reading of [(123)I]IBZM SPECT scans depends on the experience of the interpreter. Therefore, semi-quantification of striatal IBZM uptake is commonly considered mandatory. However, semi-quantification is time consuming and prone to error, particularly if the volumes of interest (VOIs) are positioned manually. Therefore, the present paper proposes a new software tool ("IBZM tool") for fully automated and standardised processing, evaluation and documentation of [(123)I]IBZM SPECT scans. METHODS: The IBZM tool is an easy-to-use SPM toolbox. It includes automated procedures for realignment and summation of multiple frames (motion correction), stereotactic normalisation, scaling, VOI analysis of striatum-to-reference ratio R, classification of R and standardised display. In order to evaluate the tool, which was developed at the University of Hamburg, the tool was transferred to the University of Hannover. There it was applied to 27 well-documented subjects: eight patients with multi-system atrophy (MSA), 12 patients with Parkinson's disease (PD) and seven controls. The IBZM tool was compared with manual VOI analysis. RESULTS: The sensitivity and specificity of the IBZM tool for the differentiation of the MSA subjects from the controls were 100% and 86%, respectively. The IBZM tool provided improved statistical power compared with manual VOI analysis. CONCLUSION: The IBZM tool is an expert system for the detection of reduced striatal D(2) availability on [(123)I]IBZM SPECT scans. The standardised documentation supports visual and semi-quantitative evaluation, and it is useful for presenting the findings to the referring physician. The IBZM tool has the potential for widespread use, since it appears to be fairly independent of the performance characteristics of the particular SPECT system used. The tool is available free of charge.

Adult↗
PubMedDate not supplied

Nutrition and translocation.

It is now clear that atrophy of the intestinal mucosa can occur rapidly after injury and that the degree of atrophy is quantitatively related to the severity of injury. Such atrophy can be associated with translocation of bacteria and endotoxin, which can trigger the hypermetabolic response and induce a septic state, which may ultimately lead to multiple system organ failure. Early enteral feeding following trauma can prevent atrophy of the intestinal mucosa and is associated with a decrease in the hypermetabolic response and the incidence of septic complications and diarrhea. Enteral feeding of complete diets can also improve outcome with other forms of intestinal injury such as gamma irradiation or cytotoxic drugs. In contrast, total parenteral nutrition and elemental enteral diets are associated with atrophy of the intestinal mucosa and increased translocation. The addition of glutamine and/or fiber to elemental diets may decrease translocation in some but not all circumstances. It is now clear that enteral nutrition can influence the incidence and severity of translocation, which in turn can potentially reverse or prevent the adverse effects of injury and inflammation in traumatic injury and serious illness.

Enteral Nutrition↗
PubMed2004

Congenital unilateral ureteropelvic junction obstruction of the rat: a useful animal model for human ureteropelvic junction obstruction?

OBJECTIVES: To investigate the expression of endothelin-1 (ET-1) and adrenomedullin (ADM) in the renal pelvis, stenotic ureteropelvic junction, and ureter of 20 male Wistar rats with congenital unilateral ureteropelvic junction obstruction; the normal contralateral kidneys served as controls. The molecular pathophysiology of congenital ureteropelvic junction obstruction is still unclear. The implication of altered peptidergic innervation is under discussion. Our study group has recently been able to demonstrate a significant increase in ET-1 and a significant decrease in ADM in prestenotic and stenotic tissue, but not in the remainder of the ureter, compared with controls. METHODS: Twenty animals were killed, and samples of the renal pelvis, ureteropelvic junction, upper ureter, middle part of the ureter, and lower ureter were immediately snap-frozen and stored in liquid nitrogen. Total RNA was extracted, and subsequently 1 microg of RNA was reversely transcribed. mRNA expression of ET-1 and ADM was determined semiquantitatively using on-line polymerase chain reaction. The expression of glyceraldehyde-3-phosphate dehydrogenase (GAPDH) was determined to relate the specific mRNA expression to the expression of a housekeeping gene. RESULTS: We found a significant increase in the expression of ET-1 in the obstructed junctions related to GAPDH (P <0.001). The expression of ADM, however, revealed no statistically significant differences. No differences at all could be detected in the tissue samples from the rest of the ureter. CONCLUSIONS: Alterations in the local production of peptidergic neurotransmitters, especially ET-1, may contribute to the molecular pathogenesis of ureteropelvic junction obstruction. Results previously obtained in the stenotic tissue from children were confirmed in the stenotic tissue from the rat model. We hypothesize that the alterations are disease-, but not age-specific.

Abnormalities, Multiple↗
PubMed1971

Multiple mucosal neuromas, pheochromocytoma, medullary carcinoma of the thyroid and marfanoid body build with muscle wasting: reexamination of a syndrome of neural crest malmigration.

Initially described in part by Wagenmann and by Froboese almost 50 years age, the syndrome of a) multiple mucosal neuromas, b) pheochromocytoma, c) medullary carcinoma of the thyroid and d) marafanoid build with muscle wasting of the limbs, was enlarged by Williams and Pollock, Gorlin et al, Schimke et al and Levy et al.

Adolescent↗
PubMed1999

Infratentorial atrophy on magnetic resonance imaging and disability in multiple sclerosis.

Loss of tissue volume in the central nervous system may provide an index of fixed neurological dysfunction in multiple sclerosis. Recent magnetic resonance studies have shown a modest relationship between clinical disability rating scores and transverse sectional area of the cervical spinal cord. To explore further the relationship between atrophy and disability in multiple sclerosis, we estimated the volumes of infratentorial structures from MRIs in a cross-sectional study of 41 patients, 21 with relapsing-remitting multiple sclerosis and 20 with secondary progressive multiple sclerosis. We used the Cavalieri method of modern design stereology with point counting to estimate the volume of brainstem, cerebellum and upper cervical spinal cord from three-dimensional MRIs acquired with an MPRAGE (Magnetization-prepared Rapid Acquisition Gradient Echo) sequence. The volume of the upper (C1-C3) cervical spinal cord was significantly correlated with a composite spinal cord score derived from the appropriate Functional Scale scores of the Expanded Disability Status Scale (r = -0.50, P < 0.01). The cerebellar (r = 0.49, P < 0.01) and brainstem (r = 0.34, P < 0.05) volumes correlated with the Scripp's Neurological Disability Rating Scale scores. The upper cervical cord volumes (r = -0.39, P < 0.01), but not the brainstem or cerebellar volumes, were significantly associated with disease duration. MRI-estimated structural volumes may provide a simple index of axonal and/or myelin loss, the presumed pathological substrates of irreversible impairment and disability in multiple sclerosis.

Activities of Daily Living↗
PubMed2005

Steroids and brain atrophy in multiple sclerosis.

In this review, we focus on different pathogenetic mechanisms of corticosteroids that induce short- and long-term brain volume fluctuations in a variety of systemic conditions and disorders, as well as on corticosteroid-induced immunomodulatory, immunosuppressive and anti-inflammatory mechanisms that contribute to the slowdown of brain atrophy progression in patients with multiple sclerosis (MS). It appears that chronic low-dose treatment with corticosteroids may contribute to irreversible loss of brain tissue in a variety of autoimmune diseases. This side effect of steroid therapy is probably mediated by steroid-induced protein catabolism mechanism. Evidence is mounting that high-dose corticosteroids may induce reversible short-term brain volume changes due to loss of intracellular water and reduction of abnormal vascular permeability, without there having been axonal loss. Other apoptotic and selective inhibiting mechanisms have been proposed to explain the nature of corticosteroid-induced brain volume fluctuations. It has been shown that chronic use of high dose intravenous methylprednisolone (IVMP) in patients with MS may limit brain atrophy progression over the long-term via different immunological mechanisms, including downregulation of adhesion molecule expression on endothelial cells, decreased cytokine and matrix metalloproteinase secretion, decreased autoreactive T-cell-mediated inflammation and T-cell apoptosis induction, blood-brain barrier closure, demyelination inhibition and, possibly, remyelination promotion. Studies in nonhuman primates have confirmed that short-term brain volume fluctuations may be induced by corticosteroid treatment, but that they are inconsistent, potentially reversible and probably dependent upon individual susceptibility to the effects of corticosteroids. Further longitudinal studies are needed to elucidate pathogenetic mechanisms contributing to brain volume fluctuations in autoimmune diseases and multiple sclerosis.

Animals↗
PubMed2002

[Brain atrophy and cognitive disorder in multiple sclerosis].

Multiple sclerosis (MS) is an autoimmune disease of the central nervous system causing severe disability via the progressive damage of white matter. Beyond physical signs cognitive dysfunction might be present as well. The aim of this study was to investigate the frequency and characteristics of brain atrophy and cognitive alterations. Significant cortical and subcortical atrophy was found on brain MRI of 30 MS patients included in this study comparing to healthy controls. Abnormal findings were detected in more than 60% of patients using a cognitive test battery. Generally, verbal abstraction, visuospatial orientation, attention, short-term memory was impaired and the psychomotor speed was decreased, even in the early stage of the disease. Depression-related complaints were found in 57% of this population. The Kurtzke scale, the atrophy of corpus callosum and widening of 3rd ventricle and Sylvian fissures were related to impaired cognitive performances. The authors would like to call attention to the early cognitive deficit and the need of treatment in MS.

Adult↗
PubMed1979

Paroxysmal dysesthesia in multiple sclerosis.

Seven cases of multiple sclerosis with paroxysmal dysesthesias of an upper extremity were reported. This seizure characteristically is a purely sensory one induced by movements and is not accompanied by convulsions or a disorder of voluntary movements. In other ways it shares some features with other paroxysmal manifestations seen in MS. Clinical observations with subsequent neurophysiologic considerations have led to the hypothesis that paroxysmal dysesthesias are segmental symptoms with their foci lying in or about the spinal cord.

Adult↗
PubMed2004

Transcriptional profile of a myotube starvation model of atrophy.

Skeletal muscle wasting is a pervasive phenomenon that can result from a wide range of pathological conditions as well as from habitual muscular inactivity. The present work describes a cell-culture condition that induces significant atrophy in skeletal muscle C2C12 myotubes. The failure to replenish differentiation media in mature myotubes leads to rapid atrophy (53% in diameter), which is referred to here as starvation. Affymetrix microarrays were used to develop a transcriptional profile of control (fed) vs. atrophied (nonfed) myotubes. Myotube starvation was characterized by an upregulation of genes involved in translational inhibition, amino acid biosynthesis and transport, and cell cycle arrest/apoptosis, among others. Downregulated genes included several structural and regulatory elements of the extracellular matrix as well as several elements of Wnt/frizzled and TGF-beta signaling pathways. Interestingly, the characteristic transcriptional upregulation of the ubiquitin-proteasome system, calpains, and cathepsins known to occur in multiple in vivo models of atrophy were not seen during myotube starvation. With the exception of the downregulation of extracellular matrix genes, serine protease inhibitor genes, and the upregulation of the translation initiation factor PHAS-I, this model of atrophy in cell culture has a transcriptional profile quite distinct from any study published to date with atrophy in whole muscle. These data show that, although the gross morphology of atrophied muscle fibers may be similar in whole muscle vs. myotube culture, the processes by which this phenotype is achieved differ markedly.

Animals↗