TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation.

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：:Aluminum was observed in the nucleolus, interchromatin granules, rough endoplasmic reticulum, free ribosomes, euchromatin, and the heterochromatin of the neuron. The association of aluminum with the first four r-RNA-containing cellular components and with the last two DNA-containing chromatins suggests the association...

journal_title：Acta neuropathologica

authors： Wen GY,Wisniewski HM

更新日期：1985-01-01 00:00:00

abstract：:Using both tumor specimen and cultured tumor cells, we have studied the differentiation of a pineocytoma by light and electron microscopy (EM) and immunohistochemical demonstration of glial, neuronal and neuroendocrine markers. Only interstitial cells were labeled with anti-glial fibrillary acidic protein and anti-S10...

journal_title：Acta neuropathologica

authors： Fèvre-Montange M,Jouvet A,Privat K,Korf HW,Champier J,Reboul A,Aguera M,Mottolese C

更新日期：1998-05-01 00:00:00

abstract：:Progressive myopathy developed in two women who consumed ipecac syrup containing emetine hydrochloride to induce vomiting as part of their anorexia nervosa. Muscle biopsy specimens were characterized by severe disruption of the sarcomeres. The ultrastructural spectrum extended from "Z-band streaming" to the formation ...

journal_title：Acta neuropathologica

authors： Halbig L,Gutmann L,Goebel HH,Brick JF,Schochet S

更新日期：1988-01-01 00:00:00

abstract：:Cornelia de Lange syndrome (CDLS) is a rare multisystemic malformative syndrome of uncertain etiology characterized by severe psychomotor and mental retardation. Here we report the neuropathological analysis of a 35-year-old patient who displayed the classical clinical symptomatology of CDLS. A congenital dysgenesis o...

journal_title：Acta neuropathologica

authors： Vuilleumier N,Kövari E,Michon A,Hof PR,Mentenopoulos G,Giannakopoulos P,Bouras C

更新日期：2002-09-01 00:00:00

abstract：:By unknown mechanisms, the symptoms of amyotrophic lateral sclerosis (ALS) seem to spread along neuroanatomical pathways to engulf the motor nervous system. The rate at which symptoms spread is one of the primary drivers of disease progression. One mechanism by which ALS symptoms could spread is by a prion-like propag...

journal_title：Acta neuropathologica

authors： Ayers JI,Fromholt S,Koch M,DeBosier A,McMahon B,Xu G,Borchelt DR

更新日期：2014-12-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a lethal disease characterized by motor neuron degeneration and associated with aggregation of nuclear RNA-binding proteins (RBPs), including FUS. How FUS aggregation and neurodegeneration are prevented in healthy motor neurons remain critically unanswered questions. Here, we use...

journal_title：Acta neuropathologica

authors： Marrone L,Drexler HCA,Wang J,Tripathi P,Distler T,Heisterkamp P,Anderson EN,Kour S,Moraiti A,Maharana S,Bhatnagar R,Belgard TG,Tripathy V,Kalmbach N,Hosseinzadeh Z,Crippa V,Abo-Rady M,Wegner F,Poletti A,Troost D,A

更新日期：2019-07-01 00:00:00

abstract：:Glucosylceramide lipidosis results from a defective lysosomal degradation of this glycolipid. Lipid degradation is controlled by two components, the enzyme beta-glucocerebrosidase and a sphingolipid activator protein. While most Gaucher cases are due to mutations within the gene that codes for the lysosomal enzyme, on...

journal_title：Acta neuropathologica

authors： Pàmpols T,Pineda M,Girós ML,Ferrer I,Cusi V,Chabás A,Sanmarti FX,Vanier MT,Christomanou H

更新日期：1999-01-01 00:00:00

abstract：:We present here an unusual case of papillary neuroepithelial tumor of the pineal region. The patient was a 29-year-old female who presented with headaches. A computed tomography scan revealed a tumorous lesion at the pineal region and hydrocephalus. The resected tumor was composed of columnar and cuboidal cells showin...

journal_title：Acta neuropathologica

authors： Shibahara J,Todo T,Morita A,Mori H,Aoki S,Fukayama M

更新日期：2004-10-01 00:00:00

abstract：:An unusual case of cerebellar neurocytoma with rhabdomyomatous differentiation in a 6-year-old boy is reported. Immunocytochemical and ultrastructural features of the tumour were studied. Abortive synapse formation, the presence of clear vesicles and synaptophysin immunoreactivity of the tumour cells indicated its int...

journal_title：Acta neuropathologica

authors： Pal L,Santosh V,Gayathri N,Das S,Das BS,Jayakumar PN,Shankar SK

更新日期：1998-03-01 00:00:00

abstract：:The suprascapular nerve from 14 horses, which had no clinical evidence of spinatus muscle atrophy, were obtained to determine whether the nerve was sub-clinically compressed at the scapular edge. The nerves were divided into three portions, proximal and distal to the scapular edge and as it reflected around it. In nin...

journal_title：Acta neuropathologica

authors： Duncan ID,Schneider RK,Hammang JP

更新日期：1987-01-01 00:00:00

abstract：:The distribution of the calcium-binding protein calretinin was investigated by immunohistochemistry in the hippocampus, the subicular areas, and the entorhinal cortex in patients with Alzheimer's disease and in control subjects. By double immunolabelling, the calretinin immunoreactivity was compared to the immunoreact...

journal_title：Acta neuropathologica

authors： Brion JP,Résibois A

更新日期：1994-01-01 00:00:00

abstract：:Six medulloblastomas were studied by electron microscopy. Two features were found which seem to be constant and essential characteristics of medulloblastoma. First, cell junctions are abundant between tumor cells. These are mostly desmosome-like but other, closer junctions, were also seen. Second, the capillary endoth...

journal_title：Acta neuropathologica

authors： Hassoun J,Hirano A,Zimmerman HM

更新日期：1975-10-27 00:00:00

abstract：:Histamine dihydrochloride (10 micrograms of 500 micrograms/ml) was infused during 1 min into the lateral cerebral ventricle of rats, which resulted in a significant stimulation of pinocytosis in the endothelial cells. Systemic injections of mepyramine or metiamide could not prevent this activation. In contrast, raniti...

journal_title：Acta neuropathologica

authors： Dux E,Dóczi T,Joó F,Szerdahelyi P,Siklós L

更新日期：1988-01-01 00:00:00

abstract：:A quantitative study was performed on spinal cord lesions in seven patients with X-linked recessive spinal and bulbar muscular atrophy. The myelinated fiber density of the lateral corticospinal tracts at the T7 cord level was well preserved for both large and small myelinated fibers. On the other hand, neurons in the ...

journal_title：Acta neuropathologica

authors： Terao S,Sobue G,Li M,Hashizume Y,Tanaka F,Mitsuma T

更新日期：1997-01-01 00:00:00

abstract：:Endoneurial cell response and type of nerve fibre damage were studied after perineural injections of 7% phenol-aqua and pure glycerol. Our previous studies have shown that phenol and glycerol induce different types of nerve fibre degeneration after intraneural injections: phenol dissolves axons and Schwann cells insid...

journal_title：Acta neuropathologica

authors： Westerlund T,Vuorinen V,Röyttä M

更新日期：2004-10-01 00:00:00

abstract：:Brain slices are used extensively for biochemical, electrophysiological and molecular investigations. However, only the time frame for electrophysiological and biochemical investigations has as yet been defined. The goal of the present study was to investigate the time course of nuclear structure in live brain slices....

journal_title：Acta neuropathologica

authors： Kasischke K,Büchner M,Ludolph AC,Riepe MW

更新日期：2001-05-01 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：:The authors describe in biopsies from 6 cases of Werdnig-Hoffmann disease, including 2 of the more benign type, the ultrastructural typical aspects of denervation. They compare their findings with those of other workers. The striking points are the great variation in the diameter of the muscle fibres and the myofibril...

journal_title：Acta neuropathologica

authors： Szliwowski HB,Drochmans P

更新日期：1975-01-01 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：:Inflammatory processes have been implicated in the formation of senile plaques in the cerebral cortex of patients with dementia of the Alzheimer type (DAT), since several inflammation-induced proteins are present within these plaques. The relation between inflammatory components and other amyloid beta protein (A beta)...

journal_title：Acta neuropathologica

authors： Verbeek MM,Otte-Höller I,Wesseling P,Ruiter DJ,de Waal RM

更新日期：1996-01-01 00:00:00

abstract：:Gelatinase A is an enzyme capable of cleaving soluble beta-amyloid protein (beta AP), and may function as an alpha-secretase to produce secretory forms of amyloid precursor protein. We examined gelatinase A immunoreactivity in the brains and posterior roots of neurologically normal, lacunar stroke, Alzheimer disease (...

journal_title：Acta neuropathologica

authors： Yamada T,Miyazaki K,Koshikawa N,Takahashi M,Akatsu H,Yamamoto T

更新日期：1995-01-01 00:00:00

abstract：:Upon brain reperfusion following ischemia, there is widespread inhibition of neuronal protein synthesis that is due to phosphorylation of eukaryotic initiation factor 2alpha (eIF2alpha), which persists in selectively vulnerable neurons (SVNs) destined to die. Other investigators have shown that expression of mutant eI...

journal_title：Acta neuropathologica

authors： Page AB,Owen CR,Kumar R,Miller JM,Rafols JA,White BC,DeGracia DJ,Krause GS

更新日期：2003-07-01 00:00:00

abstract：:A mixed lymphoblastic T cell lymphoma and gonadotroph cell pituitary adenoma occurred 25 years after first resection of the adenoma. Within 1 year the lymphoma overgrew the adenoma, but was still restricted to the sellar region. Histologically, lymphoma and adenoma components were tightly admixed. Possible pathogeneti...

journal_title：Acta neuropathologica

authors： Kuhn D,Buchfelder M,Brabletz T,Paulus W

更新日期：1999-03-01 00:00:00

abstract：:Immunohistochemical analysis of inflammatory cell density and infiltrate subpopulations in 42 meningiomas was performed. Evaluation of infiltrating cell density was carried out by cell counting. Meningothelial and fibroblastic meningiomas contained an average of 3% mononuclear cells; the few lymphocytes were localized...

journal_title：Acta neuropathologica

authors： Becker I,Roggendorf W

更新日期：1989-01-01 00:00:00

abstract：:Paediatric high grade glioma (pHGG) (World Health Organisation astrocytoma grades III and IV) remains poor prognosis tumours, with a median survival of only 15 months following diagnosis. Current investigation of anti-angiogenic strategies has focused on adult glioblastoma multiforme (GBM) with phase III trials target...

journal_title：Acta neuropathologica

authors： Smith SJ,Tilly H,Ward JH,Macarthur DC,Lowe J,Coyle B,Grundy RG

更新日期：2012-07-01 00:00:00

abstract：:We studied the densities of utrophin and dystrophin at the motor end-plates of patients with myasthenia gravis (MG) using immunohistochemical analysis. The densities were compared with those found in patients with amyotrophic lateral sclerosis, Lambert-Eaton myasthenic syndrome and normal controls. Utrophin was reduce...

journal_title：Acta neuropathologica

authors： Ito H,Yoshimura T,Satoh A,Takino H,Tsujihata M,Nagataki S

更新日期：1996-07-01 00:00:00

abstract：:Involvement of the choroid plexus by lymphoma (Hodgkin) is a hitherto undescribed complication. We report herein the case of a 49-year-old man who developed, shortly before death, neurologic symptoms seemingly related to extensive involvement of choroid plexus and surrounding ventricular structures by lymphoma (Hodgki...

journal_title：Acta neuropathologica

authors： Sanchez JE,Garcia JH,Kwee H

更新日期：1977-02-28 00:00:00

abstract：:Detailed neuropathologic and immunohistologic analysis of a case of serologically and polymerase chain reaction-confirmed human immunodeficiency virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is reported in a 73-year-old North American black woman. In addition to the usual neuropath...

journal_title：Acta neuropathologica

authors： Wu E,Dickson DW,Jacobson S,Raine CS

更新日期：1993-01-01 00:00:00

abstract：:Hyper- and hypothyroidism repercussions on soma, nucleus and cytoplasm increases were studied by electron microscopy on 7-, 14-, 21-, and 35-day-old rats. Adult normal animals were compared with adult ones made hypothyroid until they were 35 days old. Also heat value restriction of food intake effects were compared wi...

journal_title：Acta neuropathologica

authors： Rebière A,Dainat J

更新日期：1978-09-15 00:00:00