Evidence for tangential migration disturbances in human lissencephaly resulting from a defect in LIS1, DCX and ARX genes.

abstract：:The cholinergic basal forebrain is divided into four subregions (Ch1-4), and cholinergic neuronal loss in the nucleus basalis of Meynert (Ch4) has been correlated with cognitive impairments in both Alzheimer's disease (AD) and dementia with Lewy bodies (DLB). However, the Ch1-2 regions, which provide the major choline...

journal_title：Acta neuropathologica

authors： Fujishiro H,Umegaki H,Isojima D,Akatsu H,Iguchi A,Kosaka K

更新日期：2006-02-01 00:00:00

abstract：:Meningiomas are among the most frequent intracranial tumors. The secretory variant of meningioma is characterized by glandular differentiation, formation of intracellular lumina and pseudopsammoma bodies, expression of a distinct pattern of cytokeratins and clinically by pronounced perifocal brain edema. Here we descr...

journal_title：Acta neuropathologica

authors： Reuss DE,Piro RM,Jones DT,Simon M,Ketter R,Kool M,Becker A,Sahm F,Pusch S,Meyer J,Hagenlocher C,Schweizer L,Capper D,Kickingereder P,Mucha J,Koelsche C,Jäger N,Santarius T,Tarpey PS,Stephens PJ,Andrew Futreal P,

更新日期：2013-03-01 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：:We investigated whether the brainstem is affected by the pathologic process of sporadic Creutzfeldt-Jakob disease (sCJD), with particular attention to brainstem atrophy, neuronal loss, pyramidal tract degeneration, and prion protein (PrP) deposition, in 33 patients with sCJD. Brainstem atrophy, particularly in the pon...

journal_title：Acta neuropathologica

authors： Iwasaki Y,Hashizume Y,Yoshida M,Kitamoto T,Sobue G

更新日期：2005-06-01 00:00:00

abstract：:A murine model of Tay-Sachs disease, the prototype of the GM2 gangliosidoses, was produced through the targeted disruption of the Hexa gene encoding the subunit of alpha-hexosaminidase A. The mice were completely devoid of beta-hexosaminidase A activity and accumulated GM2 ganglioside in the CNS in an age-dependent ma...

journal_title：Acta neuropathologica

authors： Taniike M,Yamanaka S,Proia RL,Langaman C,Bone-Turrentine T,Suzuki K

更新日期：1995-01-01 00:00:00

abstract：:A 25-year-old homosexual AIDS patient presented with progressive cognitive, motor and behavioral disturbances consistent with HIV encephalopathy. CT scans demonstrated progressive diffuse brain atrophy. Neuropathology showed predominant cortical changes including severe neuronal loss corroborated by morphometry. Only ...

journal_title：Acta neuropathologica

authors： Gray F,Haug H,Chimelli L,Geny C,Gaston A,Scaravilli F,Budka H

更新日期：1991-01-01 00:00:00

abstract：:Structural proteins of cultured neurofibromatosis (NF) tumor and skin cells were studied with reference to control skin fibroblasts. In polyacrylamide gel electrophoresis (PAGE)/fluorography the banding patterns of the cell lysates were markedly similar. NF tumor cells, however, produced a 60 kD band with a stronger a...

journal_title：Acta neuropathologica

authors： Peltonen J,Näntö-Salonen K,Aho HJ,Kouri T,Virtanen I,Penttinen R

更新日期：1984-01-01 00:00:00

abstract：:The pro-inflammatory and blood-brain barrier (BBB) effects of intratumoral (IT) injection of human recombinant tumor necrosis factor-alpha (rTNF-alpha) were studied in the Fischer rat RT-2 glioma model. Animals received a single stereotaxic injection of either 6 x 10(4) U rTNF-alpha or excipient (vehicle) into the cen...

journal_title：Acta neuropathologica

authors： Wright JL,Merchant RE

更新日期：1997-01-01 00:00:00

abstract：:A case of Creutzfeldt-Jakob disease (CJD) with a rare mutation of the prion protein (PrP) gene (PRNP) at codon 208 (R208H) is described. By comparison with two preceding reports, the case described here displayed two distinct biochemical and neuropathological features. Western blot analysis of brain homogenates showed...

journal_title：Acta neuropathologica

authors： Roeber S,Krebs B,Neumann M,Windl O,Zerr I,Grasbon-Frodl EM,Kretzschmar HA

更新日期：2005-04-01 00:00:00

abstract：:The occurrence of oligodendrocytes within astrocytes ("emperipolesis") has been described in demyelinating lesions in cases of multiple sclerosis and also in other non-demyelinating disorders. We found that this finding was common in the cerebral white matter of patients with Creutzfeldt-Jakob disease (CJD). Eight con...

journal_title：Acta neuropathologica

authors： Shintaku M,Yutani C

更新日期：2004-09-01 00:00:00

abstract：:Remyelination in multiple sclerosis (MS) lesions often remains incomplete despite the presence of oligodendrocyte progenitor cells (OPCs). Amongst other factors, successful remyelination depends on the phagocytic clearance of myelin debris. However, the proteins in myelin debris that act as potent and selective inhibi...

journal_title：Acta neuropathologica

authors： Syed YA,Zhao C,Mahad D,Möbius W,Altmann F,Foss F,González GA,Sentürk A,Acker-Palmer A,Lubec G,Lilley K,Franklin RJM,Nave KA,Kotter MRN

更新日期：2016-02-01 00:00:00

abstract：:Numbers of synapses were counted in columns of neuropil orientated at right angles to the pia in frontal and temporal regions from postmortem brains. In the frontal region of 28 patients aged 26-90 years there were 1.2 X 10(8) mm3 (SD 0.03 X 10(8], and in the temporal of 18 patients aged 16-90 years there were 1.4 X 1...

journal_title：Acta neuropathologica

authors： Gibson PH

更新日期：1983-01-01 00:00:00

abstract：:With increasing longevity, the number of older schizophrenic patients is growing. Previous criteria used the age of symptom onset to differentiate between the late manifestations of early-onset schizophrenia and late-onset schizophreniform disorders. Current DSM-IV or ICD 10 nomenclatures do not differentiate between ...

journal_title：Acta neuropathologica

authors： Casanova MF,Stevens JR,Brown R,Royston C,Bruton C

更新日期：2002-04-01 00:00:00

abstract：:In comparison to the levels in age and gender-matched controls, reduced levels of pathological amyloid-β protein in cerebrospinal fluid routinely precede the onset of Alzheimer's disease-related symptoms by several years, whereas elevated soluble abnormal tau fractions (phosphorylated tau, total tau protein) in cerebr...

journal_title：Acta neuropathologica

authors： Braak H,Zetterberg H,Del Tredici K,Blennow K

更新日期：2013-11-01 00:00:00

abstract：:Pathways for drainage of interstitial fluid and cerebrospinal fluid from the rat brain were investigated by the injection of 2-5 microliters Indian ink into cerebral white and grey matter and into the subarachnoid space over the vertex of the left frontal lobe. Animals were killed by formalin or glutaraldehyde perfusi...

journal_title：Acta neuropathologica

authors： Zhang ET,Richards HK,Kida S,Weller RO

更新日期：1992-01-01 00:00:00

abstract：:Gliomatosis cerebri (GC), a rare and deadly CNS neoplasm characterized by involvement of at least three cerebral lobes, predominantly affects adults. While a few small series have reported its occurrence in children, little is known about the molecular characteristics of pediatric GC. We reviewed clinical, radiologica...

journal_title：Acta neuropathologica

authors： Broniscer A,Chamdine O,Hwang S,Lin T,Pounds S,Onar-Thomas A,Shurtleff S,Allen S,Gajjar A,Northcott P,Orr BA

更新日期：2016-02-01 00:00:00

abstract：:To determine the role of advanced glycation endproducts (AGE) in the pathogenesis of familial amyotrophic lateral sclerosis (ALS) with superoxide dismutase-1 (SOD1) mutation, we investigated the immunohistochemical localization of N(epsilon)-carboxymethyl-lysine (CML), one of the major AGE structures, in spinal cords ...

journal_title：Acta neuropathologica

authors： Shibata N,Hirano A,Kato S,Nagai R,Horiuchi S,Komori T,Umahara T,Asayama K,Kobayashi M

更新日期：1999-03-01 00:00:00

abstract：:The E200K mutation is the most frequent prion protein gene (PRNP) mutation detected worldwide that is associated with Creutzfeldt-Jakob disease (CJD) and thought to have overlapping features with sporadic CJD, yet detailed neuropathological studies have not been reported. In addition to the prion protein, deposition o...

journal_title：Acta neuropathologica

authors： Kovacs GG,Seguin J,Quadrio I,Höftberger R,Kapás I,Streichenberger N,Biacabe AG,Meyronet D,Sciot R,Vandenberghe R,Majtenyi K,László L,Ströbel T,Budka H,Perret-Liaudet A

更新日期：2011-01-01 00:00:00

abstract：:A 64-year-old woman developed septicemia and a generalized peripheral neuropathy while being ventilated postoperatively. No cause for the neuropathy could be found during life. At autopsy she was found to have infective endocarditis and multifocal inflammatory lesions in the central and peripheral nervous systems, con...

journal_title：Acta neuropathologica

authors： Pamphlett R,Walsh J

更新日期：1989-01-01 00:00:00

abstract：:The occurrence and topographic analysis of granulovacuolar degeneration (GVD) in the hippocampal cortex of mentally normal controls (75 cases) and patients with Alzheimer's dementia (AD; 17 cases which included Alzheimer's disease and senile dementia of Alzheimer type), multi-infarct dementia (MID; 16 cases), Pick's d...

journal_title：Acta neuropathologica

authors： Xu M,Shibayama H,Kobayashi H,Yamada K,Ishihara R,Zhao P,Takeuchi T,Yoshida K,Inagaki T,Nokura K

更新日期：1992-01-01 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 hemizygote of the macular mutant mice, which is clinically and neuropathologically considered to be a model of Menkes kinky hair disease (MKHD), were injected intraperitoneally four times with 10, 20, 20 and 30 micrograms of cupric chloride on days 4, 6, 8 and 10 after birth, respectively. Their cerebral and cereb...

journal_title：Acta neuropathologica

authors： Yamano T,Shimada M,Onaga A,Kawasaki H,Iwane S,Ono K,Nishimura M

更新日期：1988-01-01 00:00:00

abstract：:The intracellular localization of myoglobin(Mb) mRNA in the skeletal muscles of normal subjects and patients with Duchenne muscular dystrophy(DMD) or amyotrophic lateral sclerosis(ALS) was examined by in situ hybridization using a biotin-labeled cDNA probe. In cross sections of normal muscles, Mb mRNA signals were dem...

journal_title：Acta neuropathologica

authors： Mitsui T,Kawai H,Naruo T,Nishino H,Saito S

更新日期：1993-01-01 00:00:00

abstract：:We studied the muscle biopsy from an asymptomatic patient with high serum creatine kinase values. Subsarcolemmal and intermyofibrillar granular inclusions were seen at the light microscopy level. Ultrastructural observation showed clusters of cylindrical spirals (CS). CS are nonspecific, morphological finding, so far ...

journal_title：Acta neuropathologica

authors： Rapuzzi S,Prelle A,Moggio M,Rigoletto C,Ciscato P,Comi G,Francesca F,Scarlato G

更新日期：1995-01-01 00:00:00

abstract：:A 50-year-old carpenter died in Western Pennsylvania of rabies on January 4, 1979. He had been hospitalized in an intensive care unit for 28 days. The diagnosis was made postmortem from light and electron microscopic examination of central nervous system tissue. Immunofluorescence studies confirmed the diagnosis later...

journal_title：Acta neuropathologica

authors： Lamas CC,Martinez AJ,Baraff R,Bajwa J

更新日期：1980-01-01 00:00:00

abstract：:Knockout of genes encoding metabotropic glutamate receptor 5 (mGluR5) or its endogenous regulators, such as Norbin, induce a schizophrenia-like phenotype in rodents, suggesting dysregulation of mGluR5 in schizophrenia. Human genetic and pharmacological animal studies support this hypothesis, but no studies have explor...

journal_title：Acta neuropathologica

authors： Matosin N,Fernandez-Enright F,Fung SJ,Lum JS,Engel M,Andrews JL,Huang XF,Weickert CS,Newell KA

更新日期：2015-07-01 00:00:00

abstract：:The unfolded protein response (UPR) is a stress response of the endoplasmic reticulum (ER) to a disturbance in protein folding. The so-called ER stress sensors PERK, IRE1 and ATF6 play a central role in the initiation and regulation of the UPR. The accumulation of misfolded and aggregated proteins is a common characte...

journal_title：Acta neuropathologica

authors： Scheper W,Hoozemans JJ

更新日期：2015-09-01 00:00:00

abstract：:Multiple system atrophy (MSA) is characterized by the presence of distinctive glial cytoplasmic inclusions (GCIs) within oligodendrocytes that contain the neuronal protein alpha-synuclein (aSyn) and the oligodendroglia-specific phosphoprotein TPPP/p25α. However, the role of oligodendroglial aSyn and p25α in the format...

journal_title：Acta neuropathologica

authors： Mavroeidi P,Arvanitaki F,Karakitsou AK,Vetsi M,Kloukina I,Zweckstetter M,Giller K,Becker S,Sorrentino ZA,Giasson BI,Jensen PH,Stefanis L,Xilouri M

更新日期：2019-09-01 00:00:00

abstract：:The effects of ageing, cachexia and neoplasms on striated muscle were examined in histological sections of an autopsy material. Paraffin sections were examined from four separate muscles of 30 previously healthy subjects who died suddenly, from eight cases with cachexia and from 16 cases with cachexia and neoplasms. I...

journal_title：Acta neuropathologica

authors： Lindboe CF,Torvik A

更新日期：1982-01-01 00:00:00

abstract：:Muscle biopsy samples from five patients with cytoplasmic body myopathy (CBM) were investigated by immunohistochemical (antibodies to desmin, actin, dystrophin, spectrin, alpha actinin and utrophin), immunoelectron microscopic (antibodies to desmin, actin and dystrophin) and biochemical (desmin, dystrophin, actin and ...

journal_title：Acta neuropathologica

authors： Caron A,Viader F,Lechevalier B,Chapon F

更新日期：1995-01-01 00:00:00