Nervous system involvement in von Hippel-Lindau disease: pathology and mechanisms.

abstract：:Leukocyte adhesion molecules to endothelium plays an important role in the pathogenesis of inflammatory diseases, including HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP). To help define the role of adhesion molecules in HAM/TSP, we studied the expression of lymphocyte function-associated antige...

journal_title：Acta neuropathologica

authors： Umehara F,Izumo S,Takeya M,Takahashi K,Sato E,Osame M

更新日期：1996-01-01 00:00:00

abstract：:Histological, ultrastructural and morphometrical observations on Clarke's column were carried out in 18 patients with sporadic amyotrophic lateral sclerosis (ALS) and 15 age-matched control subjects. Of the 18 ALS patients 6 had been on a respirator before death. Bunina bodies were found in the neuronal cytoplasm in 7...

journal_title：Acta neuropathologica

authors： Takahashi H,Oyanagi K,Ohama E,Ikuta F

更新日期：1992-01-01 00:00:00

abstract：:Neuropeptides have turned out to be promising new parameters, in addition to the routinely performed histochemical diagnosis, of Hirschsprung's disease (HD). Studies of the peptidergic innervation of the affected intestinal segment of patients with HD have demonstrated a marked reduction in the density of several neur...

journal_title：Acta neuropathologica

authors： Berger A,Kofler B,Santic R,Zipperer E,Sperl W,Hauser-Kronberger C

更新日期：2003-01-01 00:00:00

abstract：:In the present work the effect of Piromen is studied on the formation of the glial-mesenchymal scar in the experimentally injuried cortex of hamsters. The uniform effect of the bacterial polysaccharide is suggested in a strong and lasting stimulation of proliferating reactive cells in the traumatic area. The excessive...

journal_title：Acta neuropathologica

authors： von Cramon D

更新日期：1975-01-01 00:00:00

abstract：:There is evidence that apoptotic cell death mechanisms contribute to muscle fibre loss in dystrophinopathies, but little knowledge about the activators of the final degrading caspase cascade in muscle fibre apoptosis. As mitochondria-related activation of this caspase cascade, through e.g. APAF-1, could not be proven ...

journal_title：Acta neuropathologica

authors： Tews DS

更新日期：2005-02-01 00:00:00

abstract：:Alpha-synuclein (alphaS) and ubiquitin (Ub) are shared constituents of glial cytoplasmic inclusions (GCIs) and Lewy bodies (LBs), both composed of fibrillary structures. Staining profiles of GCIs were investigated with triple immunofluorescence involving immunostaining for alphaS and Ub, both amplified with catalyzed ...

journal_title：Acta neuropathologica

authors： Sakamoto M,Uchihara T,Nakamura A,Mizutani T,Mizusawa H

更新日期：2005-10-01 00:00:00

abstract：:Charcot-Marie-Tooth disease type 1A (CMT1A) is a common hereditary demyelinating neuropathy caused by a duplication of the gene for the myelin protein PMP22, resulting in overexpression of PMP22 in young patients. Although genetically well defined, the pathogenesis of the hereditary demyelinating neuropathy CMT1A is s...

journal_title：Acta neuropathologica

authors： Hanemann CO,Gabreëls-Festen AA,Stoll G,Müller HW

更新日期：1997-10-01 00:00:00

abstract：:Mutations in the sarcomeric protein titin, encoded by TTN, are emerging as a common cause of myopathies. The diagnosis of a TTN-related myopathy is, however, often not straightforward due to clinico-pathological overlap with other myopathies and the prevalence of TTN variants in control populations. Here, we present a...

journal_title：Acta neuropathologica

authors： Rees M,Nikoopour R,Fukuzawa A,Kho AL,Fernandez-Garcia MA,Wraige E,Bodi I,Deshpande C,Özdemir Ö,Daimagüler HS,Pfuhl M,Holt M,Brandmeier B,Grover S,Fluss J,Longman C,Farrugia ME,Matthews E,Hanna M,Muntoni F,Sarkozy

更新日期：2021-01-15 00:00:00

abstract：:Seven patients aged 29 to 76 years with various clinical subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP) were investigated. Sural nerve biopsies were performed between 7 months and 19 years after onset of disease. Quantitative electron microscopy revealed involvement of primary unmyelinated fibers...

journal_title：Acta neuropathologica

authors： Gibbels E,Kentenich M

更新日期：1990-01-01 00:00:00

abstract：:We report two sporadic cases of tauopathy with unusual neuropathological features. The ages of the patients at death were 86 and 74 years, and the disease durations were 4 and 3 years, respectively. The former patient showed progressive dementia and amyotrophy (autopsy revealed that severe cervical spondylosis was res...

journal_title：Acta neuropathologica

authors： Sakai K,Piao YS,Kikugawa K,Ohara S,Hasegawa M,Takano H,Fukase M,Nishizawa M,Kakita A,Takahashi H

更新日期：2006-09-01 00:00:00

abstract：:The transmissible spongiform encephalopathies (TSEs) or prion diseases of animals are characterised by CNS spongiform change, gliosis and the accumulation of disease-associated forms of prion protein (PrP(d)). Particularly in ruminant prion diseases, a wide range of morphological types of PrP(d) depositions are found ...

journal_title：Acta neuropathologica

authors： Jeffrey M,McGovern G,Sisó S,González L

更新日期：2011-01-01 00:00:00

abstract：:A 77-year-old man suffered intermittent hemiconvulsions of unknown etiology on the left side for a period of about 5 weeks. At the autopsy, there was marked neuronal loss, severe proliferation of astrocytes and spongiform changes in the right cerebral cortex. The cerebral white matter showed loosening with astroglial ...

journal_title：Acta neuropathologica

authors： Yamanouchi H,Matsui R,Tomonaga M,Sakurai H,Yoshimura M,Shimada H

更新日期：1988-01-01 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 human glioma cell line, SA146, was initiated on precoated extracellular matrix from a stereotactic biopsy of a glioblastoma. We report modulation in the expression of glial fibrillary acidic protein (GFAP) by SA146 passed in vitro before or after xenogenic transplantation into nude mice. Immunofluorescence data show...

journal_title：Acta neuropathologica

authors： Gomès D,de Néchaud B,Maunoury R,Moura Neto V,Brigaudeau C,Labrousse F,Dupouey P

更新日期：1997-10-01 00:00:00

abstract：:In looking for a possible influence of nuclear inclusions (NIs) on neurodegeneration in human brains, we quantified morphological features of pontine neurons of three unrelated cases of neuronal intranuclear inclusion disease (NIID) and five control cases. Cross-sectional area of each neuronal nucleus and the indices ...

journal_title：Acta neuropathologica

authors： Uchihara T,Tanaka J,Funata N,Arai K,Hattori T

更新日期：2003-02-01 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：:MicroRNA (miRNA) expression was assessed in human cerebral cortical gray matter (GM) and white matter (WM) in order to provide the first insights into the difference between GM and WM miRNA repertoires across a range of Alzheimer's disease (AD) pathology. RNA was isolated separately from GM and WM portions of superior...

journal_title：Acta neuropathologica

authors： Wang WX,Huang Q,Hu Y,Stromberg AJ,Nelson PT

更新日期：2011-02-01 00:00:00

abstract：:In adult mice, administration of the anticonvulsive drug phenytoin caused focal swellings along the Purkinje cell axon correlated with ataxia and incoordination of movements. In our model, we used murine cerebellar slice cultures to study the influence of phenytoin on postnatal Purkinje cell axon differentiation. Almo...

journal_title：Acta neuropathologica

authors： Tauer U,Knoth R,Volk B

更新日期：1998-06-01 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：:The nature and distribution of vascular changes in acute Wernicke's encephalopathy (WE) were analyzed in three autopsy cases. Lesions of the lateral vestibular nucleus of the medulla oblongata (three cases) and lateral ventricular wall (one case) were examined by reconstruction of 200 serial sections, and the capillar...

journal_title：Acta neuropathologica

authors： Okeda R,Taki K,Ikari R,Funata N

更新日期：1995-01-01 00:00:00

abstract：:Mutations in the fused in sarcoma (FUS) gene are linked to a form of familial amyotrophic lateral sclerosis (ALS), ALS6. The FUS protein is a major component of the ubiquitin-positive neuronal cytoplasmic inclusions in both ALS6 and some rare forms of frontotemporal lobar degeneration (FTLD). The latter are now collec...

journal_title：Acta neuropathologica

authors： Aoki N,Higashi S,Kawakami I,Kobayashi Z,Hosokawa M,Katsuse O,Togo T,Hirayasu Y,Akiyama H

更新日期：2012-09-01 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：:Long-term epidemiological studies indicate that environmental factors play a causative role in high-incidence amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) in the western Pacific. An increased risk for disease is acquired in youth and remains for life. The low concentrations of calcium and magnesi...

journal_title：Acta neuropathologica

authors： Garruto RM,Shankar SK,Yanagihara R,Salazar AM,Amyx HL,Gajdusek DC

更新日期：1989-01-01 00:00:00

abstract：:This study concerns an autopsy case of motor neuron disease with dementia (MND-D) that exhibited unusual clinical and neuropathological findings. The patient was a Japanese man without any relevant family history who was 60 years old at the time of death. His clinical manifestation included character change at the age...

journal_title：Acta neuropathologica

authors： Sudo S,Fukutani Y,Matsubara R,Sasaki K,Shiozawa M,Wada Y,Naiki H,Isaki K

更新日期：2002-05-01 00:00:00

abstract：:The neuropathologic findings in the brain of a National Aeronautics and Space Administration (NASA) engineer, who died subsequent to nitrogen inhalation, are reported. Findings which are unusual in other forms of anoxia included relative preservation of neurons in the Sommer sector of the hippocampus and a pattern of ...

journal_title：Acta neuropathologica

authors： Hunter S,Ballinger WE Jr,Greer M

更新日期：1985-01-01 00:00:00

abstract：:The topographic distribution of brain atrophy was quantified by image analysis of fixed coronal brain slices from four patients dying with cortical Lewy body disease (CLBD) all with Alzheimer-type pathology and compared to that in four other patients of similar age and gender dying with Alzheimer's disease (AD) alone....

journal_title：Acta neuropathologica

authors： Mann DM,Snowden JS

更新日期：1995-01-01 00:00:00

abstract：:A study was undertaken to determine the pathological significance of previously unrecognized intracytoplasmic eosinophilic inclusions (IEIs) in ependymoma. The study group consisted of 58 ependymomas, all of which were pathologically characterized and graded according to the 1993 WHO classification. Electron microscop...

journal_title：Acta neuropathologica

authors： Kawano N,Ohba Y,Nagashima K

更新日期：2000-02-01 00:00:00

abstract：:Intracranial solitary fibrous tumors (SFTs) are typically dural-based, CD34-positive neoplasms of uncertain histogenesis. We examined ten cases of meninges obtained at autopsy from patients with no history of neurological illness, head trauma, or neurosurgical intervention, and ten cases of typical meningiomas with at...

journal_title：Acta neuropathologica

authors： Cummings TJ,Burchette JL,McLendon RE

更新日期：2001-10-01 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：: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