"Reducing body"-like inclusions in skeletal muscle in childhood-onset acid maltase deficiency.

abstract：:Psammoma bodies at the perivascular area in five cases of meningioma were examined with the electron microscope. In general, meningocytic cells invest the outer aspect of blood vessels, which are constituted by multilayered basal laminae, collagen fibers, microfibrils, and pericytes. Remnants of degenerated cells are ...

journal_title：Acta neuropathologica

authors： Kubota T,Hirano A,Sato K,Yamamoto S

更新日期：1985-01-01 00:00:00

abstract：:Most cases of frontotemporal lobar degeneration (FTLD) are characterized by abnormal intracellular accumulation of either tau or TDP-43 protein. However, in ~10% of cases, composed of a heterogenous collection of uncommon disorders, the molecular basis remains to be uncertain. We recently discovered that the pathologi...

journal_title：Acta neuropathologica

authors： Mackenzie IR,Munoz DG,Kusaka H,Yokota O,Ishihara K,Roeber S,Kretzschmar HA,Cairns NJ,Neumann M

更新日期：2011-02-01 00:00:00

abstract：:This study was undertaken to elucidate, using the Golgi method, the neuropathological change in the brain of the macular mutant mouse, whose hemizygote (Ml/y) is considered to be a model of Menkes kinky hair disease (MKHD). The hemizygote mice gradually lost weight after 10 days of age and died with emaciation and sei...

journal_title：Acta neuropathologica

authors： Kawasaki H,Onaga A,Yamano T,Shimada M,Nishimura M

更新日期：1987-01-01 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：:The calcium-activated protease calpain cleaves a variety of biologically important proteins and serves, therefore, as a key regulator of many cellular functions. Activation of both main isoforms, calpain 1 and calpain 2, was demonstrated previously in Alzheimer's disease. In this report, antibodies specifically recogn...

journal_title：Acta neuropathologica

authors： Adamec E,Mohan P,Vonsattel JP,Nixon RA

更新日期：2002-07-01 00:00:00

abstract：:A description is given of three cases of ganglioneuromas, which originated from ganglia of the sympathetic chain of hamsters. Other reports of such tumors in this species are reviewed and the literature relative to hamster ganglioneuromas presented. In addition, the possible genesis of origin from adult ganglion cells...

journal_title：Acta neuropathologica

authors： Rustia M,Cardesa A

更新日期：1975-10-01 00:00:00

abstract：:The essential role of the cellular prion protein (PrPC) in prion disorders such as Creutzfeldt-Jakob disease is well documented. Moreover, evidence is accumulating that PrPC may act as a receptor for protein aggregates and transduce neurotoxic signals in more common neurodegenerative disorders, such as Alzheimer's dis...

journal_title：Acta neuropathologica

authors： Watts JC,Bourkas MEC,Arshad H

更新日期：2018-02-01 00:00:00

abstract：:Considering the possibility of a paracellular route for edema resolution we studied the microvasculature of the subependymal and subcortical white matter in hydrocephalic rats. Normal adult rats were used as controls. After injection of kaolin suspension into the cisterna magna, the animals were killed at intervals of...

journal_title：Acta neuropathologica

authors： Nakagawa Y,Cervós-Navarro J,Artigas J

更新日期：1984-01-01 00:00:00

abstract：:Widespread damage to axons in the white matter of the brain is a well-recognised consequence of non-missile head injury. This diffuse axonal injury is characterised by a gradual swelling of the axon associated with an accumulation of cellular organelles and proteins. We have investigated the relationship between the s...

journal_title：Acta neuropathologica

authors： Wilkinson AE,Bridges LR,Sivaloganathan S

更新日期：1999-08-01 00:00:00

abstract：:We described a new type of cytoplasmic inclusion in the choroidal epithelial cells of humans. The inclusions usually appeared as brown, round or elongated bodies with or without an inner core, ranging in size from 1.3 to 7.0 micron. Histochemically, they contained polysaccharides, proteins and compound lipids. Ultrast...

journal_title：Acta neuropathologica

authors： Ohama E,Takeda S,Ikuta F

更新日期：1988-01-01 00:00:00

abstract：:Focal cortical dysplasias (FCD) which represent a composite group of cortical malformations are increasingly recognized as morphological substrate for severe therapy-refractory epilepsy in children and young adults. However, presurgical evaluation remains challenging as not all FCD variants can be reliably detected by...

journal_title：Acta neuropathologica

authors： Mühlebner A,Coras R,Kobow K,Feucht M,Czech T,Stefan H,Weigel D,Buchfelder M,Holthausen H,Pieper T,Kudernatsch M,Blümcke I

更新日期：2012-02-01 00:00:00

abstract：:Observations have been made on the structure of the paranodal region at nodes of Ranvier in the sural nerve of patients with diabetic sensory polyneuropathy. The structure of the paranodes was examined with particular attention to the definition and assessment of axoglial dysjunction, which has been claimed to be a ch...

journal_title：Acta neuropathologica

authors： Thomas PK,Beamish NG,Small JR,King RH,Tesfaye S,Ward JD,Tsigos C,Young RJ,Boulton AJ

更新日期：1996-12-01 00:00:00

abstract：:Histopathological, immunocytochemical, and electron microscopical investigations were carried out in a man with a protracted history of spastic paraparesis, adrenal insufficiency and hypogonadism. Pathological findings were identical with those of the few previously reported cases of adrenomyeloneuropathy (AMN) includ...

journal_title：Acta neuropathologica

authors： Probst A,Ulrich J,Heitz PU,Herschkowitz N

更新日期：1980-01-01 00:00:00

abstract：:The Trembler mouse suffers from an hereditary demyelinating neuropathy. Schwann cell myelination of peripheral nerve fibres in the Trembler mouse is abnormal. Myelination of central nerve fibres in the deeper layers of white matter of the spinal cord is normal. At the junction between the peripheral nervous system and...

journal_title：Acta neuropathologica

authors： Harrison BM

更新日期：1977-04-29 00:00:00

abstract：:Uniform neuropathological changes are described in eight cases of the progressive encephalopathy syndrome with edema, hypsarrhythmia and optic atrophy (PEHO syndrome). Two of the autopsied patients were sisters and two other cases were familial. Macroscopically, cerebral and pronounced cerebellar atrophy was seen, the...

journal_title：Acta neuropathologica

authors： Haltia M,Somer M

更新日期：1993-01-01 00:00:00

abstract：:Aberrant peripheral nerve fiber bundles, i.e., masses of peripheral nerve fibers without perineural sheath cells, are found in the perivascular spaces of some spinal cords. The 514 spinal cords examined for these structures were separated into two groups; 507 spinal cords without definite necrotic lesions (group 1) an...

journal_title：Acta neuropathologica

authors： Kamiya M,Hashizume Y

更新日期：1989-01-01 00:00:00

abstract：:A 40-year-old man suffering from amyotrophic lateral sclerosis with symmetrical degeneration of the thalamus and the substantia nigra is reported. The distribution pattern of the thalamic degeneration in the present case was characteristic in that the Nucleus centralis was the severest affected of the thalamic nuclei...

journal_title：Acta neuropathologica

authors： Kosaka K,Mehraein P

更新日期：1978-12-15 00:00:00

abstract：:Research efforts during the last decade have deciphered the basic molecular mechanisms governing mitochondrial fusion and fission. We now know that in mammalian cells mitochondrial fission is mediated by the large GTPase dynamin-related protein 1 (Drp1) acting in concert with outer mitochondrial membrane (OMM) protein...

journal_title：Acta neuropathologica

authors： Oettinghaus B,Licci M,Scorrano L,Frank S

更新日期：2012-02-01 00:00:00

abstract：:A simple and reproducible method for cryopreservation of brain tissue from patients with Alzheimer's disease is described. Fresh brain slices (1 cm thick) obtained less than 6 h postmortem are placed in sealed plastic bags, sandwiched between 0.3-cm-thick aluminium sheets, and frozen by placing the entire "sandwich" b...

journal_title：Acta neuropathologica

authors： Nochlin D,Mackenzie AP,Bryant EM,Norwood TH,Sumi SM

更新日期：1993-01-01 00:00:00

abstract：:Frontotemporal dementia (FTD) is a prevalent neurodegenerative disease of heterogeneous histopathology. Neuropathological subtypes are identified on the basis of the presence or absence of tau- or ubiquitin-positive neuronal inclusions. Our recent work has established four disease stages that are independent of neurop...

journal_title：Acta neuropathologica

authors： Kersaitis C,Halliday GM,Kril JJ

更新日期：2004-12-01 00:00:00

abstract：:A limited cortical resection including the rolandic fissure and the pre- and postcentral cortical regions was carried out in a patient suffering from epilepsia partialis continua resistant to antiepileptic drugs. The histological examination revealed several foci of very large neurons distributed with no laminar organ...

journal_title：Acta neuropathologica

authors： Ferrer I,Pineda M,Tallada M,Oliver B,Russi A,Oller L,Noboa R,Zújar MJ,Alcántara S

更新日期：1992-01-01 00:00:00

abstract：:Brain tumors are the most common solid tumors of childhood, and the genetic drivers and optimal therapeutic strategies for many of the different subtypes remain unknown. Here, we identify that bithalamic gliomas harbor frequent mutations in the EGFR oncogene, only rare histone H3 mutation (in contrast to their unilate...

journal_title：Acta neuropathologica

authors： Mondal G,Lee JC,Ravindranathan A,Villanueva-Meyer JE,Tran QT,Allen SJ,Barreto J,Gupta R,Doo P,Van Ziffle J,Onodera C,Devine P,Grenert JP,Samuel D,Li R,Metrock LK,Jin LW,Antony R,Alashari M,Cheshier S,Whipple NS,

更新日期：2020-06-01 00:00:00

abstract：:We describe three cases of early- (cases 1-3, 28-39 years) and one of late-onset (case 4, 76 years) Alzheimer's disease (AD) with 'cotton wool' plaques (CWPs) but without a family history indicating autosomal dominant inheritance. The early-onset cases, but not the late-onset case, showed remarkable aggression, disinh...

journal_title：Acta neuropathologica

authors： Yokota O,Terada S,Ishizu H,Ujike H,Ishihara T,Namba M,Hayashi Y,Nishinaka T,Namba R,Nakashima H,Uéda K,Checler F,Kuroda S

更新日期：2003-10-01 00:00:00

abstract：:Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by the expansion of a polyglutamine tract in the androgen receptor (AR). The mechanism by which expansion of polyglutamine in AR causes muscle atrophy is unknown. Here, we investigated pathological pathways underlying muscle atrophy in SBMA kn...

journal_title：Acta neuropathologica

authors： Rocchi A,Milioto C,Parodi S,Armirotti A,Borgia D,Pellegrini M,Urciuolo A,Molon S,Morbidoni V,Marabita M,Romanello V,Gatto P,Blaauw B,Bonaldo P,Sambataro F,Robins DM,Lieberman AP,Sorarù G,Vergani L,Sandri M,Pennuto

更新日期：2016-07-01 00:00:00

abstract：:A complex neuropathological study of two cases of Niemann-Pick disease (NPD) type C (NPDC) revealed some novel features in the chemical pathology of the neuronal storage. Lipid histochemistry showed the presence of a lipid which met the criteria of a neuronal glycosphingolipid. Sphingomyelin (SM) was not detected in t...

journal_title：Acta neuropathologica

authors： Elleder M,Jirásek A,Smíd F,Ledvinová J,Besley GT

更新日期：1985-01-01 00:00:00

abstract：:Extracellular deposition of amyloid β-protein (Aβ) in amyloid plaques and intracellular accumulation of abnormally phosphorylated τ-protein (p-τ) in neurofibrillary tangles (NFTs) represent pathological hallmark lesions of Alzheimer's disease (AD). Both lesions develop in parallel in the human brain throughout the pre...

journal_title：Acta neuropathologica

authors： Gomes LA,Hipp SA,Rijal Upadhaya A,Balakrishnan K,Ospitalieri S,Koper MJ,Largo-Barrientos P,Uytterhoeven V,Reichwald J,Rabe S,Vandenberghe R,von Arnim CAF,Tousseyn T,Feederle R,Giudici C,Willem M,Staufenbiel M,Thal DR

更新日期：2019-12-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are relentlessly progressive neurodegenerative disorders with overlapping clinical, genetic and pathological features. Cytoplasmic inclusions of fused in sarcoma (FUS) are the hallmark of several forms of FTLD and ALS patients with mutati...

journal_title：Acta neuropathologica

authors： Mitchell JC,McGoldrick P,Vance C,Hortobagyi T,Sreedharan J,Rogelj B,Tudor EL,Smith BN,Klasen C,Miller CC,Cooper JD,Greensmith L,Shaw CE

更新日期：2013-02-01 00:00:00

abstract：:A significant reduction in the myelinated nerve fiber population was observed during quantitative electron-microscopic examination of peripheral nerves in chronic alloxan diabetic rats. Dystrophic axonal abnormalities and regenerating fibers were more numerous in diabetics than age-matched controls. Schwann cells show...

journal_title：Acta neuropathologica

authors： Powell HC,Myers RR

更新日期：1984-01-01 00:00:00

abstract：:The isolated paired helical filaments (PHF) that occur in the neurofibrillary tangles of Alzheimer's disease were assayed to determine if they contained N-acetyl-glucosamine and N-acetyl-galactosamine residues. The enzyme-linked lectin assay was used to detect their total content in the PHF preparation. The assay empl...

journal_title：Acta neuropathologica

authors： Sparkman DR,Hill SJ,White CL 3rd

更新日期：1990-01-01 00:00:00

abstract：:The expression of glial fibrillary acidic protein (GFAP), vimentin, S-100 protein (S-100), HNK-1, myelin basic protein (MBP) and fibronectin was investigated immunohistochemically in 51 ethylnitrosourea (ENU)-induced neurinomas of the rat. Additionally, 90 transplantation tumors derived from ENU-induced neurinomas and...

journal_title：Acta neuropathologica

authors： Vogeley KT,Bilzer T,Reifenberger G,Wechsler W

更新日期：1991-01-01 00:00:00