abstract：:The mechanisms underlying neurodegenerative diseases are the outcome of pathological alterations of evolutionary conserved molecular and cellular cascades. For this reason, Drosophila and C. elegans serve as useful model systems to study various aspects of neurodegenerative diseases. Here, we introduce the advantageou...

journal_title：Acta neuropathologica

authors： Shemesh OA,Spira ME

更新日期：2010-08-01 00:00:00

abstract：:Altered levels of cerebrospinal fluid (CSF) peptides related to Alzheimer's disease (AD) are associated with pathologic AD diagnosis, although cognitively normal subjects can also have abnormal levels of these AD biomarkers. To identify novel CSF biomarkers that distinguish pathologically confirmed AD from cognitively...

journal_title：Acta neuropathologica

authors： Hu WT,Chen-Plotkin A,Arnold SE,Grossman M,Clark CM,Shaw LM,Pickering E,Kuhn M,Chen Y,McCluskey L,Elman L,Karlawish J,Hurtig HI,Siderowf A,Lee VM,Soares H,Trojanowski JQ

更新日期：2010-06-01 00:00:00

abstract：:Activating beta-catenin mutations with aberrant cytoplasmic and nuclear protein accumulation are hallmarks of adamantinomatous craniopharyngiomas (adaCP). These tumours tend to be associated with unfavourable and occasionally disastrous sequelae, as they invade adjacent brain structures such as the hypothalamus. The p...

journal_title：Acta neuropathologica

authors： Hölsken A,Buchfelder M,Fahlbusch R,Blümcke I,Buslei R

更新日期：2010-05-01 00:00:00

abstract：:Abnormal TDP-43 aggregation is a prominent feature in the neuropathology of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. Mutations in TARDBP, the gene encoding TDP-43, cause some cases of ALS. The normal function of TDP-43 remains incompletely understood. To better understand TDP-43 biolo...

journal_title：Acta neuropathologica

authors： Kraemer BC,Schuck T,Wheeler JM,Robinson LC,Trojanowski JQ,Lee VM,Schellenberg GD

更新日期：2010-04-01 00:00:00

abstract：:Autopsy findings of a patient, with sialidosis type I phenotype carrying V217M/G243R mutations in the lysosomal sialidase gene and biochemically defined isolated sialidase deficiency, who died of intractable lymphoma at the age of 32 years, are described. Perikaryal expansion of cytoplasm was evident, mostly in motor ...

journal_title：Acta neuropathologica

authors： Uchihara T,Ohashi K,Kitagawa M,Kurata M,Nakamura A,Hirokawa K,Kasuga T,Kobayashi T

更新日期：2010-01-01 00:00:00

abstract：:The choroid plexus is an epithelial-endothelial vascular convolute within the ventricular system of the vertebrate brain. It consists of epithelial cells, fenestrated blood vessels, and the stroma, dependent on various physiological or pathological conditions, which may contain fibroblasts, mast cells, macrophages, gr...

journal_title：Acta neuropathologica

authors： Wolburg H,Paulus W

更新日期：2010-01-01 00:00:00

abstract：:Inflammatory myopathy with abundant macrophages (IMAM) and dermatomyositis (DM) are considered to represent related disorders, since they share inflammatory infiltrates and skin alterations. In order to get more insight into these disorders, we addressed the cellular composition of the inflammatory infiltrates in musc...

journal_title：Acta neuropathologica

authors： Brunn A,Hans VJ,Vogelgesang S,Deckert M

更新日期：2009-12-01 00:00:00

abstract：:Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrP(Sc), and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenot...

journal_title：Acta neuropathologica

authors： Parchi P,Strammiello R,Notari S,Giese A,Langeveld JP,Ladogana A,Zerr I,Roncaroli F,Cras P,Ghetti B,Pocchiari M,Kretzschmar H,Capellari S

更新日期：2009-11-01 00:00:00

abstract：:Separation of pilocytic astrocytoma from diffuse astrocytomas frequently poses problems mostly related to small sample size. Precise classification and grading are essential due to different therapeutic strategies prompted by diagnoses of pilocytic astrocytoma WHO grade I, diffuse astrocytomas WHO grade II or anaplast...

journal_title：Acta neuropathologica

authors： Korshunov A,Meyer J,Capper D,Christians A,Remke M,Witt H,Pfister S,von Deimling A,Hartmann C

更新日期：2009-09-01 00:00:00

abstract：:Wolfram syndrome or DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness) is a neurodegenerative disorder characterized by diabetes mellitus and optic atrophy as well as diabetes insipidus and deafness in many cases. We report the post-mortem neuropathologic findings of a patient with Wolfram syn...

journal_title：Acta neuropathologica

authors： Hilson JB,Merchant SN,Adams JC,Joseph JT

更新日期：2009-09-01 00:00:00

abstract：:Oxidative stress has been implicated in the pathogenesis of a number of diseases including Alzheimer's disease (AD). The oxidative stress hypothesis of AD pathogenesis, in part, is based on beta-amyloid peptide (Abeta)-induced oxidative stress in both in vitro and in vivo studies. Oxidative modification of the protein...

journal_title：Acta neuropathologica

authors： Sultana R,Perluigi M,Butterfield DA

更新日期：2009-07-01 00:00:00

abstract：:When 22 members of the BrainNet Europe (BNE) consortium assessed 31 cases with alpha-synuclein (alphaS) immunoreactive (IR) pathology applying the consensus protocol described by McKeith and colleagues in 2005, the inter-observer agreement was 80%, being lowest in the limbic category (73%). When applying the staging p...

journal_title：Acta neuropathologica

authors： Alafuzoff I,Ince PG,Arzberger T,Al-Sarraj S,Bell J,Bodi I,Bogdanovic N,Bugiani O,Ferrer I,Gelpi E,Gentleman S,Giaccone G,Ironside JW,Kavantzas N,King A,Korkolopoulou P,Kovács GG,Meyronet D,Monoranu C,Parchi P,Park

更新日期：2009-06-01 00:00:00

abstract：:Vascular smooth muscle cells are involved in deposition of amyloid in brain blood vessels. Accumulation of amyloid-beta peptide (Abeta) in cultured brain vascular smooth muscle cells that overexpress human amyloid-beta precursor protein (APP) Swedish, is strongly enhanced by exposure to iron ions. We studied cellular ...

journal_title：Acta neuropathologica

authors： Frackowiak J,Potempska A,Mazur-Kolecka B

更新日期：2009-05-01 00:00:00

abstract：:With an incidence of approximately 350 in 100,000, stroke is the third leading cause of death and a major cause of disability in industrialized countries. At present, although progress has been made in understanding the molecular pathways that lead to ischemic cell death, the current clinical treatments remain poorly ...

journal_title：Acta neuropathologica

authors： Kriz J,Lalancette-Hébert M

更新日期：2009-05-01 00:00:00

abstract：:Neuronal accumulation of oligomeric amyloid-beta (Alphabeta) is considered the proximal cause of neuronal demise in Alzheimer disease (AD) patients. Blood-borne macrophages might reduce Abeta stress to neurons by immigration into the brain and phagocytosis of Alphabeta. We tested migration and export across a blood-br...

journal_title：Acta neuropathologica

authors： Zaghi J,Goldenson B,Inayathullah M,Lossinsky AS,Masoumi A,Avagyan H,Mahanian M,Bernas M,Weinand M,Rosenthal MJ,Espinosa-Jeffrey A,de Vellis J,Teplow DB,Fiala M

更新日期：2009-02-01 00:00:00

abstract：:Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive...

journal_title：Acta neuropathologica

authors： Kobayashi A,Arima K,Ogawa M,Murata M,Fukuda T,Kitamoto T

更新日期：2008-11-01 00:00:00

abstract：:In recent years, we have used a variety of tau immunological markers combined with the dye thiazin red (TR), an accurate marker to differentiate the fibrillar from the nonfibrillar state of both amyloid-beta and tau in Alzheimer's disease (AD). In this study, we used TR as a potential diagnostic marker of AD in frozen...

journal_title：Acta neuropathologica

authors： Luna-Muñoz J,Peralta-Ramirez J,Chávez-Macías L,Harrington CR,Wischik CM,Mena R

更新日期：2008-11-01 00:00:00

abstract：:X-linked lissencephaly with abnormal genitalia (XLAG) is a rare disorder caused by mutations in the aristaless-related homeobox (ARX) gene, located on Xp22.13. Arx-null mice show loss of tangential migration of GABAergic interneurons, presumably being related to caudal ganglionic eminence tangential migration. In the ...

journal_title：Acta neuropathologica

authors： Okazaki S,Ohsawa M,Kuki I,Kawawaki H,Koriyama T,Ri S,Ichiba H,Hai E,Inoue T,Nakamura H,Goto Y,Tomiwa K,Yamano T,Kitamura K,Itoh M

更新日期：2008-10-01 00:00:00

abstract：:Although it is known that deletions or mutations of the SMN1 gene on chromosome 5 cause decreased levels of the SMN protein in subjects with proximal autosomal recessive spinal muscular atrophy (SMA), the exact sequence of pathological events leading to selective motoneuron cell death is not fully understood yet. In t...

journal_title：Acta neuropathologica

authors： Simic G

更新日期：2008-09-01 00:00:00

abstract：:In this study, we used immunohistochemistry to screen for alpha-synuclein pathology in the brains of 241 individuals without clinical evidence of neurologic disease, and discovered 36 cases (15%) with incidental Lewy bodies (LBs) and one case, a 96-year-old woman (0.4%), with inclusions similar to those seen in multip...

journal_title：Acta neuropathologica

authors： Fujishiro H,Ahn TB,Frigerio R,DelleDonne A,Josephs KA,Parisi JE,Eric Ahlskog J,Dickson DW

更新日期：2008-09-01 00:00:00

abstract：:Sensitive detection of alpha-synuclein (alpha-syn) pathology is important in the diagnosis of disorders like Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy and in providing better insights into the etiology of these diseases. Several monoclonal antibodies that selectively react with aggreg...

journal_title：Acta neuropathologica

authors： Waxman EA,Duda JE,Giasson BI

更新日期：2008-07-01 00:00:00

abstract：:Collections of human postmortem brains gathered in brain banks have underpinned many significant developments in the understanding of central nervous system (CNS) disorders and continue to support current research. Unfortunately, the worldwide decline in postmortem examinations has had an adverse effect on research ti...

journal_title：Acta neuropathologica

authors： Bell JE,Alafuzoff I,Al-Sarraj S,Arzberger T,Bogdanovic N,Budka H,Dexter DT,Falkai P,Ferrer I,Gelpi E,Gentleman SM,Giaccone G,Huitinga I,Ironside JW,Klioueva N,Kovacs GG,Meyronet D,Palkovits M,Parchi P,Patsouris E,

更新日期：2008-05-01 00:00:00

abstract：:Amyloid-beta (Abeta) peptide pathology in Alzheimer's disease (AD) comprises extracellular plaques and cerebral amyloid angiopathy (CAA). In Parkinson's disease (PD), alpha-synuclein forms intraneuronal Lewy bodies (LBs), and cortical LBs are thought to play a major role in cognitive decline designated as PD with deme...

journal_title：Acta neuropathologica

authors： Lashley T,Holton JL,Gray E,Kirkham K,O'Sullivan SS,Hilbig A,Wood NW,Lees AJ,Revesz T

更新日期：2008-04-01 00:00:00

abstract：:The incidence of CNS lymphoma has increased significantly in the past 30 years, primarily in the elderly and immunocompromised. While T-cell lymphomas comprise 15-20% of systemic lymphomas, they comprise less than 4% of primary CNS lymphomas, suggesting that they may be under-recognized compared to their systemic coun...

journal_title：Acta neuropathologica

authors： Dulai MS,Park CY,Howell WD,Smyth LT,Desai M,Carter DM,Vogel H

更新日期：2008-03-01 00:00:00

abstract：:Little information is available regarding the morphological changes in the mitochondria in amyotrophic lateral sclerosis (ALS). In particular, mitochondrial changes in dorsal root ganglion cells have not yet been examined. We therefore conducted an electron microscopic examination of the mitochondria in dorsal root ga...

journal_title：Acta neuropathologica

authors： Sasaki S,Horie Y,Iwata M

更新日期：2007-12-01 00:00:00

abstract：:Pontocerebellar hypoplasia type 2 (PCH-2; MIM 277470), an autosomal recessive neurodegeneration with fetal onset, was studied in six autopsies with ages at death ranging between 1 and 22 years. Three patients were distantly related. A case of olivopontocerebellar hypoplasia (OPCH; MIM 225753) was studied for compariso...

journal_title：Acta neuropathologica

authors： Barth PG,Aronica E,de Vries L,Nikkels PG,Scheper W,Hoozemans JJ,Poll-The BT,Troost D

更新日期：2007-10-01 00:00:00

abstract：:Abuse of the club drugs Methamphetamine (Meth) and Ecstasy (MDMA) is an international problem. The seriousness of this problem is the result of what appears to be programmed cell death (PCD) occurring within the brain following their use. This follow up study focused on determining which cell types, neurons and/or gli...

journal_title：Acta neuropathologica

authors： Warren MW,Larner SF,Kobeissy FH,Brezing CA,Jeung JA,Hayes RL,Gold MS,Wang KK

更新日期：2007-09-01 00:00:00

abstract：:Astrocytic alpha-synuclein-immunoreactive inclusions have recently been noted to develop in sporadic Parkinson's disease (PD). Here, the presence of immunoreactive astrocytes is reported in 14 autopsy cases with clinically diagnosed PD and a neuropathological stage of 4 or higher. The labeled astrocytes occur preferen...

journal_title：Acta neuropathologica

authors： Braak H,Sastre M,Del Tredici K

更新日期：2007-09-01 00:00:00

abstract：:The finding of novel fibroblastic onion bulb-like structures in peripheral nerves is reported for the first time in avian riboflavin deficiency. Day old broiler meat chickens were fed a riboflavin deficient diet (1.8 mg/kg) and were killed on postnatal days 6, 11, 16, 21 and 31, whereas control chickens were fed a con...

journal_title：Acta neuropathologica

authors： Cai Z,Blumbergs PC,Finnie JW,Manavis J,Thompson PD

更新日期：2007-08-01 00:00:00

abstract：:Defects of major histocompatibility complex (MHC) class I antigen-processing machinery (APM) components have been shown to contribute to immune escape of malignant cells. We investigated the expression of APM components in astrocytomas without detectable defects in HLA class I antigen expression and correlated it with...

journal_title：Acta neuropathologica

authors： Mehling M,Simon P,Mittelbronn M,Meyermann R,Ferrone S,Weller M,Wiendl H

更新日期：2007-08-01 00:00:00

abstract：:Frontotemporal lobar degeneration is the term used to describe the non-Alzheimer clinical syndromes of frontotemporal dementia, semantic dementia and progressive non-fluent aphasia, regardless of the underlying neuropathological features. Considerable progress has been made in recent years in our understanding of the ...

journal_title：Acta neuropathologica

authors： Pickering-Brown SM

更新日期：2007-07-01 00:00:00

abstract：:The presence of many neurofibrillary tangles (NFTs) in the central nervous system is a hallmark of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) in people living in the Kii peninsula of Japan and in the island of Guam. To determine whether or not ALS and PDC are on a spectrum of a single ...

journal_title：Acta neuropathologica

authors： Mimuro M,Kokubo Y,Kuzuhara S

更新日期：2007-06-01 00:00:00

abstract：:Recently, 43-kDa TAR DNA-binding protein (TDP-43) was identified as a component of ubiquitinated inclusions (UIs) in sporadic amyotrophic lateral sclerosis (SALS). To clarify whether TDP-43 immunoreactivity is present in neuronal inclusions in familial ALS (FALS), we examined immunohistochemically the brains and spina...

journal_title：Acta neuropathologica

authors： Tan CF,Eguchi H,Tagawa A,Onodera O,Iwasaki T,Tsujino A,Nishizawa M,Kakita A,Takahashi H

更新日期：2007-05-01 00:00:00

abstract：:Silver-staining methods are helpful for histological identification of pathological deposits. In spite of some ambiguities regarding their mechanism and interpretation, they are widely used for histopathological diagnosis. In this review, four major silver-staining methods, modified Bielschowsky, Bodian, Gallyas (GAL)...

journal_title：Acta neuropathologica

authors： Uchihara T

更新日期：2007-05-01 00:00:00

abstract：:Neuregulins belong to a family of multipotent growth-promoting proteins, and have been shown to have a crucial role in accumulating acetylcholine receptor at neuromuscular junctions. A functional role of neuregulins in muscle regeneration has not yet been identified. Using reverse transcription (RT)-PCR, Western blot ...

journal_title：Acta neuropathologica

authors： Hirata M,Sakuma K,Okajima S,Fujiwara H,Inashima S,Yasuhara M,Kubo T

更新日期：2007-04-01 00:00:00

abstract：:Old age is associated with a deficient recovery from stroke, but the cellular mechanisms underlying such phenomena are poorly understood. To address this issue, focal cerebral ischemia was produced by reversible occlusion of the right middle cerebral artery in 3- and 20-month-old male Sprague-Dawley rats. Aged rats sh...

journal_title：Acta neuropathologica

authors： Popa-Wagner A,Badan I,Walker L,Groppa S,Patrana N,Kessler C

更新日期：2007-03-01 00:00:00

abstract：:Primary extradural ependymomas are rare neoplasms usually of the myxopapillary type. Reports on malignant primary extradural ependymal tumors are exceptionally rare. We here report on a 3-year-old boy with Schinzel-Giedion syndrome (SGS), who presented with lumbar spina bifida occulta and a progressive extraspinal les...

journal_title：Acta neuropathologica

authors： Beschorner R,Wehrmann M,Ernemann U,Bonin M,Horber V,Oehl-Jaschkowitz B,Meyermann R,Dufke A

更新日期：2007-03-01 00:00:00

abstract：:We performed a comparative study to assess cerebral amyloid angiopathy and ApoE genotype in cases of Alzheimer's disease (AD). Ten ApoE 3,3 and ten ApoE 4,4 AD brains, as well as ten normal control brains, were selected after matching for age, sex, and duration of disease. Sections of middle frontal and inferior parie...

journal_title：Acta neuropathologica

authors： Trembath D,Ervin JF,Broom L,Szymanski M,Welsh-Bohmer K,Pieper C,Hulette CM

更新日期：2007-01-01 00:00:00

abstract：:Electrophysiological studies in subjects with HIV/AIDS demonstrate subtle changes in the visual pathway even in the absence of visual symptoms. But the pathological correlate of the electrophysiological abnormalities is largely unknown. This study attempts to correlate pathological changes in the retina and intraorbit...

journal_title：Acta neuropathologica

authors： Mahadevan A,Satishchandra P,Prachet KK,Sidappa NB,Ranga U,Santosh V,Yasha TC,Desai A,Ravi V,Shankar SK

更新日期：2006-10-01 00:00:00

abstract：:Disturbance of intracellular trafficking plays a major role in several neurodegenerative disorders including Alzheimer or Parkinson's disease. The Chediak-Higashi syndrome (CHS), a life-threatening autosomal recessive disease with frequent mutations in the LYST gene, and its animal model, the beige mouse, are both cha...

journal_title：Acta neuropathologica

authors： Rudelius M,Osanger A,Kohlmann S,Augustin M,Piontek G,Heinzmann U,Jennen G,Russ A,Matiasek K,Stumm G,Schlegel J

更新日期：2006-09-01 00:00:00