Clarke's column in sporadic amyotrophic lateral sclerosis.

abstract：:PICALM, a clathrin adaptor protein, plays important roles in clathrin-mediated endocytosis in all cell types. Recently, genome-wide association studies identified single nucleotide polymorphisms in PICALM gene as genetic risk factors for late-onset Alzheimer disease (LOAD). We analysed by western blotting with several...

journal_title：Acta neuropathologica

authors： Ando K,Brion JP,Stygelbout V,Suain V,Authelet M,Dedecker R,Chanut A,Lacor P,Lavaur J,Sazdovitch V,Rogaeva E,Potier MC,Duyckaerts C

更新日期：2013-06-01 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：:The Norrbottnian type of Gaucher disease is characterized by infantile or juvenile onset and variable degrees of neurological symptoms, some of which develop only after splenectomy. A full neuropathological description of this type of Gaucher disease has not been reported previously. The brains of five patients were e...

journal_title：Acta neuropathologica

authors： Conradi NG,Sourander P,Nilsson O,Svennerholm L,Erikson A

更新日期：1984-01-01 00:00:00

abstract：:Charcot-Marie-Tooth disease type 1 B (CMT1B) is a demyelinating neuropathy caused by mutations in the myelin protein zero (P0) gene (MPZ). A few cases of CMT1B were recently found to be characterized by focally folded myelin sheaths in nerve biopsy specimens; the significance of this association is unknown. Here, we d...

journal_title：Acta neuropathologica

authors： Fabrizi GM,Taioli F,Cavallaro T,Rigatelli F,Simonati A,Mariani G,Perrone P,Rizzuto N

更新日期：2000-09-01 00:00:00

abstract：:A cerebral biopsy was performed in a 39-year-old male patient with subacute paraparesis who later developed severe dementia and moderate cerebellar involvement. The histological examination showed a marked neuronal loss, severe neurofibrillary degeneration, and a great number of senile plaques. No PAS-positive plaques...

journal_title：Acta neuropathologica

authors： Ferrer I,Aymami A,Rovira A,Grau Veciana JM

更新日期：1983-01-01 00:00:00

abstract：:Essential mixed cryoglobulinemia, which can cause hypersensitivity vasculitis, was observed in five patients with peripheral neuropathy. Three cases presented with multifocal neuropathies and two cases with symmetrical polyneuropathy. One had cryoglobulinemia with IgM monoclonal gammopathy IgG polyclonal gammopathy, a...

journal_title：Acta neuropathologica

authors： Vital C,Deminière C,Lagueny A,Bergouignan FX,Pellegrin JL,Doutre MS,Clement A,Beylot J

更新日期：1988-01-01 00:00:00

abstract：:Cryostat sections of 12 gliomas and of 3 peritumoral brain tissue samples were investigated for mononuclear cell infiltration by immunohistochemistry, concentrating on cells expressing monocyte/macrophage markers. Only low numbers of T cells were detected in the tumors, whereas in average 20%-30% of all cells present ...

journal_title：Acta neuropathologica

authors： Morimura T,Neuchrist C,Kitz K,Budka H,Scheiner O,Kraft D,Lassmann H

更新日期：1990-01-01 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：:The colocalization of beta amyloid protein with the enzymes acetyl- and butyrylcholinesterase was assessed using immunocytochemistry for beta amyloid protein and a sensitive histochemical technique for cholinesterases. In non-demented aged and Alzheimer's disease brains, double-stained sections for cholinesterases and...

journal_title：Acta neuropathologica

authors： Morán MA,Mufson EJ,Gómez-Ramos P

更新日期：1993-01-01 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：:This report illustrates a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) masquerading as neurofibromatosis due to multifocal enlargements of spinal nerve roots. The patient initially complained of intermittent numbness of the hands and leg weakness at age 62. Nerve conduction velocities were ...

journal_title：Acta neuropathologica

authors： Pytel P,Rezania K,Soliven B,Frank J,Wollmann R

更新日期：2003-02-01 00:00:00

abstract：:Four melanin pigment-containing intracranial tumors were found in three Long-Evans rats in the course of experimental oncogenesis by transplacental ethylnitrosourea (ENU). One of them was a leptomeningeal melanoma. Aside from the presence of scattered melanin-pigmented cells, the other three had the typical histologic...

journal_title：Acta neuropathologica

authors： Spence AM,Rubinstein LJ,Conley FK,Herman MM

更新日期：1976-05-18 00:00:00

abstract：:Intracellular filamentous tau pathology is the defining feature of tauopathies, which form a subset of neurodegenerative diseases. We have analyzed pathological tau in Alzheimer's disease, and in frontotemporal lobar degeneration associated with tauopathy to include cases with Pick bodies, corticobasal degeneration, p...

journal_title：Acta neuropathologica

authors： Taniguchi-Watanabe S,Arai T,Kametani F,Nonaka T,Masuda-Suzukake M,Tarutani A,Murayama S,Saito Y,Arima K,Yoshida M,Akiyama H,Robinson A,Mann DMA,Iwatsubo T,Hasegawa M

更新日期：2016-02-01 00:00:00

abstract：:Evidence of misfolded wild-type superoxide dismutase 1 (SOD1) has been detected in spinal cords of sporadic ALS (sALS) patients, suggesting an etiological relationship to SOD1-associated familial ALS (fALS). Given that there are currently a number of promising therapies under development that target SOD1, it is of cri...

journal_title：Acta neuropathologica

authors： Ayers JI,Diamond J,Sari A,Fromholt S,Galaleldeen A,Ostrow LW,Glass JD,Hart PJ,Borchelt DR

更新日期：2016-12-01 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：:Ubiquitin-positive Lewy neurites and Lewy bodies are found in idiopathic Parkinson's disease (PD) and diffuse Lewy body disease (DLBD). We found that, in three patients with PD and one with DLBD, microtubule-associated protein 5 (MAP5) immunostaining was consistently present in both Lewy neurites and Lewy bodies throu...

journal_title：Acta neuropathologica

authors： Gai WP,Blumbergs PC,Blessing WW

更新日期：1996-01-01 00:00:00

abstract：:The identification of the alpha-synuclein gene on chromosome 4q as a locus for familial Lewy-body parkinsonism and of alpha-synuclein as a component of Lewy bodies has heralded a new era in the study of Parkinson's disease. We have identified a large family with Lewy body parkinsonism linked to a novel locus on chromo...

journal_title：Acta neuropathologica

authors： Gwinn-Hardy K,Mehta ND,Farrer M,Maraganore D,Muenter M,Yen SH,Hardy J,Dickson DW

更新日期：2000-06-01 00:00:00

abstract：:We describe features of a patient that broadens the clinical and pathological spectrum of neurofilament inclusion disease (NFID). The patient was a 52-year-old man with a 5--6 year history of progressive, asymmetrical spastic weakness of the upper and lower extremities; L-DOPA-unresponsive parkinsonism; and SPECT evid...

journal_title：Acta neuropathologica

authors： Josephs KA,Uchikado H,McComb RD,Bashir R,Wszolek Z,Swanson J,Matsumoto J,Shaw G,Dickson DW

更新日期：2005-04-01 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：: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：:Pilocytic astrocytomas are the most common astrocytic tumors of childhood and differ clinically and histopathologically from those astrocytomas that affect adults. Studies of adult astrocytic tumors have revealed allelic losses on chromosomes 10, 17p, 19q and alterations in the epidermal growth factor receptor (EGFR) ...

journal_title：Acta neuropathologica

authors： von Deimling A,Louis DN,Menon AG,von Ammon K,Petersen I,Ellison D,Wiestler OD,Seizinger BR

更新日期：1993-01-01 00:00:00

abstract：:During corticogenesis, neurons adopt different migration pathways to reach their final position. The precursors of pyramidal neurons migrate radially, whereas most of the GABA-containing interneurons are generated in the ventral telencephalon and migrate tangentially into the neocortex. Then, they use a radial migrati...

journal_title：Acta neuropathologica

authors： Marcorelles P,Laquerrière A,Adde-Michel C,Marret S,Saugier-Veber P,Beldjord C,Friocourt G

更新日期：2010-10-01 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：:Brain metastases (BM) are common in cancer patients and are associated with high morbidity and poor prognosis, even after intensive multimodal therapy including resection, radiotherapy (stereotactic radiosurgery or whole brain radiotherapy) and chemotherapy. However, advances in the understanding of the pathobiology o...

journal_title：Acta neuropathologica

authors： Preusser M,Capper D,Ilhan-Mutlu A,Berghoff AS,Birner P,Bartsch R,Marosi C,Zielinski C,Mehta MP,Winkler F,Wick W,von Deimling A

更新日期：2012-02-01 00:00:00

abstract：:Histochemical and electron microscopic studies of the brains inclusive of the leptomeninges containing large blood vessels from 7 patients with mucopolysaccharidosis (MPS) I, II, IIIA and V showed marked increase in mesenchymal elements and the generalized presence of characteristic lesions around cerebral veins and a...

journal_title：Acta neuropathologica

authors： Dekaban AS,Constantopoulos G

更新日期：1977-07-15 00:00:00

abstract：:In this study we evaluated the relationship between polyglucosan bodies and peripheral nerve lesions. The biopsied sural nerve from a patient with late-onset chronic sensori-motor neuropathy showed many intra-axonal polyglucosan bodies and segmental demyelination/remyelination. The formation of Schwann cell hyperplasi...

journal_title：Acta neuropathologica

authors： Matsumuro K,Izumo S,Minauchi Y,Inose M,Higuchi I,Osame M

更新日期：1993-01-01 00:00:00

abstract：:Neuroaxonal spheroids became evident microscopically after the autopsy of a 45-year-old woman with pigmentation of the globus pallidus suggesting Hallervorden-Spatz disease. In our opinion the fine floccular pigment seen electron-microscopically in many of the axonal spheroids is melanin, an end product of catecholami...

journal_title：Acta neuropathologica

authors： Hartmann HA,White SK,Levine RL

更新日期：1983-01-01 00:00:00

abstract：:Glioblastoma (GBM), the most common malignant brain tumor, is among the most lethal neoplasms, with a median survival of approximately 1 year. Prognosis is poor since GBMs possess a strong migratory and highly invasive potential, making complete surgical resection impossible. Reduced expression of carboxypeptidase E (...

journal_title：Acta neuropathologica

authors： Höring E,Harter PN,Seznec J,Schittenhelm J,Bühring HJ,Bhattacharyya S,von Hattingen E,Zachskorn C,Mittelbronn M,Naumann U

更新日期：2012-07-01 00:00:00

abstract：:Acute arterial hypertension was induced in male Wistar rats using two experimental techniques: (1) i.v. injection of Aramine and (2) infusion of physiological saline as a bolus via internal carotid artery. Horseradish peroxidase (HRP) was injected i.v. prior to both experimental procedures and subsequently localized i...

journal_title：Acta neuropathologica

authors： Nagy Z,Mathieson G,Hüttner I

更新日期：1979-10-01 00:00:00

abstract：:HTLV-III-like particles were observed within a cell process surrounded by a myelin sheath, in the brain of a 4-year-old boy with AIDS encephalopathy. Similar particles were also observed in the mononuclear macrophage-like cells. ...

journal_title：Acta neuropathologica

authors： Kato T,Dembitzer HM,Hirano A,Llena JF

更新日期：1987-01-01 00:00:00