Alpha-synuclein accumulation in a case of neurodegeneration with brain iron accumulation type 1 (NBIA-1, formerly Hallervorden-Spatz syndrome) with widespread cortical and brainstem-type Lewy bodies.

abstract：:The microtubule-associated protein tau accumulates as cytoplasmic inclusions in Alzheimer's disease (AD), Pick's disease (PiD), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). We investigated the immunoreactivity of tau-positive structures using a panel of antibodies to epitopes spanning the ...

journal_title：Acta neuropathologica

authors： Arai T,Ikeda K,Akiyama H,Tsuchiya K,Iritani S,Ishiguro K,Yagishita S,Oda T,Odawara T,Iseki E

更新日期：2003-05-01 00:00:00

abstract：:An unresolved issue in the study of demyelinating disease is whether blood-brain barrier damage is dependent upon the migration of inflammatory cells into the central nervous system (CNS). In a study of experimental autoimmune encephalomyelitis (EAE) in rabbits, a freeze-dried, paraffin-embedded tissue technique was e...

journal_title：Acta neuropathologica

authors： Simmons RD,Buzbee TM,Linthicum DS,Mandy WJ,Chen G,Wang C

更新日期：1987-01-01 00:00:00

abstract：:The central distal axonopathy induced in dogs by the administration of high doses of clioquinol is contrasted with the central-peripheral distal axonopathy precipitated by intoxication with 2,5-hexanedione. Mature, pure-bred Beagle dogs received a daily oral dose of 400 mg/kg of clioquinol for up to 7 months, or 1 ml ...

journal_title：Acta neuropathologica

authors： Krinke G,Schaumburg HH,Spencer PS,Thomann P,Hess R

更新日期：1979-08-01 00:00:00

abstract：:Glycerol (an atoxic alcohol) and phenol (a toxic monohydroxybenzene) are currently used as neurolytic blocking agents to relieve pain or spasticity. In the present study we compared the endoneurial response of anhydrous glycerol and 7% phenol-aqua after intraneural injection into rat sciatic nerve, using electron micr...

journal_title：Acta neuropathologica

authors： Westerlund T,Vuorinen V,Röyttä M

更新日期：2001-07-01 00:00:00

abstract：:Hydrocephalic neonates were observed in a small breeding colony of rats. Normal rats from this colony were obtained and brother-sister mated for seven generations. The overall prevalence of hydrocephalics was approximately 23%; however, in one subline, the prevalence approached 50%. Breeding data suggested the trait t...

journal_title：Acta neuropathologica

authors： Kohn DF,Chinookoswong N,Chou SM

更新日期：1981-01-01 00:00:00

abstract：:Pick's disease is characterized morphologically by severe atrophy of the frontal and temporal lobes and the presence in the cerebral cortex of degenerative neuronal lesions referred to as Pick bodies. In the present study, we analyzed the regional and laminar distribution of Pick bodies in a series of 16 Pick's diseas...

journal_title：Acta neuropathologica

authors： Hof PR,Bouras C,Perl DP,Morrison JH

更新日期：1994-01-01 00:00:00

abstract：:Patients with von Hippel-Lindau disease carry a germline mutation of the Von Hippel-Lindau (VHL) tumor-suppressor gene. We discuss the molecular consequences of loss of VHL gene function and their impact on the nervous system. Dysfunction of the VHL protein causes accumulation and activation of hypoxia inducible facto...

journal_title：Acta neuropathologica

authors： Vortmeyer AO,Falke EA,Gläsker S,Li J,Oldfield EH

更新日期：2013-03-01 00:00:00

abstract：:Autopsy findings of a patient, with sialidosis type I phenotype carrying V217M/G243R mutations in the lysosomal sialidase gene and biochemically defined isolated sialidase deficiency, who died of intractable lymphoma at the age of 32 years, are described. Perikaryal expansion of cytoplasm was evident, mostly in motor ...

journal_title：Acta neuropathologica

authors： Uchihara T,Ohashi K,Kitagawa M,Kurata M,Nakamura A,Hirokawa K,Kasuga T,Kobayashi T

更新日期：2010-01-01 00:00:00

abstract：:Focal cortical dysplasia (FCD) is a localized malformation of cortical development and is the commonest cause of severe childhood epilepsy in surgical practice. Children with FCD are severely disabled by their epilepsy, presenting with frequent seizures early in life. The commonest form of FCD in children is character...

journal_title：Acta neuropathologica

authors： Yasin SA,Ali AM,Tata M,Picker SR,Anderson GW,Latimer-Bowman E,Nicholson SL,Harkness W,Cross JH,Paine SM,Jacques TS

更新日期：2013-08-01 00:00:00

abstract：:A 56-day-old infant with alpha-neuraminidase deficiency, whose clinical features included severe edema of extremities and ascites which resembled those in severe infantile sialidosis, was autopsied. Perforation, whose pathogenesis was unclear, was found on the descending portion of the duodenum. Light and electron mic...

journal_title：Acta neuropathologica

authors： Yamano T,Shimada M,Matsuzaki K,Matsumoto Y,Yoshihara W,Okada S,Inui K,Yutaka T,Yabuuchi H

更新日期：1986-01-01 00:00:00

abstract：:The original version of this article unfortunately contained a mistake. The Panel A in the published figure 5 is incorrect. The corrected Figure 5 is placed in the following page. ...

journal_title：Acta neuropathologica

authors： Saijo E,Metrick MA 2nd,Koga S,Parchi P,Litvan I,Spina S,Boxer A,Rojas JC,Galasko D,Kraus A,Rossi M,Newell K,Zanusso G,Grinberg LT,Seeley WW,Ghetti B,Dickson DW,Caughey B

更新日期：2020-01-01 00:00:00

abstract：:Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease associated with repetitive mild impact traumatic brain injury from contact sports. Recently, a consensus panel defined the pathognomonic lesion for CTE as accumulations of abnormally hyperphosphorylated tau (p-tau) in neurons (neurofibrillary tangle...

journal_title：Acta neuropathologica

authors： Shively SB,Edgerton SL,Iacono D,Purohit DP,Qu BX,Haroutunian V,Davis KL,Diaz-Arrastia R,Perl DP

更新日期：2017-03-01 00:00:00

abstract：:Electrophysiological studies in subjects with HIV/AIDS demonstrate subtle changes in the visual pathway even in the absence of visual symptoms. But the pathological correlate of the electrophysiological abnormalities is largely unknown. This study attempts to correlate pathological changes in the retina and intraorbit...

journal_title：Acta neuropathologica

authors： Mahadevan A,Satishchandra P,Prachet KK,Sidappa NB,Ranga U,Santosh V,Yasha TC,Desai A,Ravi V,Shankar SK

更新日期：2006-10-01 00:00:00

abstract：:It is conventionally accepted that during the early stages of Wallerian degeneration of myelinated peripheral nerve fibres Schmidt-Lanterman incisures represent the sites at which the myelin sheath, together with enclosed axoplasm, is segmented into myelin ovoids. This mechanism is considered by some authors to be fac...

journal_title：Acta neuropathologica

authors： Ghabriel MN,Allt G

更新日期：1979-11-01 00:00:00

abstract：:Muscle fiber abnormalities are described in three cases of neonatal maple syrup disease. There were important variations in fiber diameters. Lesions consisted in focal or diffuse destruction of myofibrils. In view of recent biochemical and clinical data, a direct relation between elevated branched-chain amino acid lev...

journal_title：Acta neuropathologica

authors： Ferrière G,de Castro M,Rodriguez J

更新日期：1984-01-01 00:00:00

abstract：:The close correlation between abnormally low pre-mortem cerebrospinal fluid (CSF) concentrations of amyloid-β1-42 (Aβ(1-42)) and plaque burden measured by amyloid imaging as well as between pathologically increased levels of CSF tau and the extent of neurodegeneration measured by MRI has led to growing interest in usi...

journal_title：Acta neuropathologica

authors： Shaw LM,Vanderstichele H,Knapik-Czajka M,Figurski M,Coart E,Blennow K,Soares H,Simon AJ,Lewczuk P,Dean RA,Siemers E,Potter W,Lee VM,Trojanowski JQ,Alzheimer's Disease Neuroimaging Initiative.

更新日期：2011-05-01 00:00:00

abstract：:An extensive laminar spongiosis was found in the outer part of the dentate gyrus in an 84-year-old patient. An old cavitary infarct in the parahippocampal gyrus disconnected the dentate gyrus from the entorhinal area. This finding prompted us to seek laminar spongiosis in Alzheimer's disease, where the neuronal loss i...

journal_title：Acta neuropathologica

authors： Duyckaerts C,Colle MA,Seilhean D,Hauw JJ

更新日期：1998-04-01 00:00:00

abstract：:Focal cortical dysplasias (FCDs) are increasingly recognized as one of the most common causes of pharmaco-resistant epilepsies. FCDs were recently divided into various clinico-pathological subtypes due to distinct imaging, electrophysiological, and outcome characteristics. In this review, we will overview the internat...

journal_title：Acta neuropathologica

authors： Najm IM,Tassi L,Sarnat HB,Holthausen H,Russo GL

更新日期：2014-07-01 00:00:00

abstract：:Methylmercury (MeHg) poisoning (20 mg/kg body weight) in embryonic mice resulted in significant reductions of mitotic indices in the neuroepithelial germinal cells of the telencephalon at the ventricular surface 4 to 12 h following intoxication. After 24 h, no significant difference in the mitotic indices was observed...

journal_title：Acta neuropathologica

authors： Choi BH

更新日期：1991-01-01 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：:Axonal degeneration contributes to the transient and permanent neurological deficits seen in multiple sclerosis, an inflammatory disease of the central nervous system. To study the immunological mechanisms causing axonal degeneration, we induced experimental autoimmune encephalomyelitis (EAE) in wildtype Lewis rats an...

journal_title：Acta neuropathologica

authors： Aboul-Enein F,Weiser P,Höftberger R,Lassmann H,Bradl M

更新日期：2006-06-01 00:00:00

abstract：:We studied three siblings and one unrelated patient with cerebrotendinous xanthomatosis (CTX). Of two unrelated patients, we examined biopsies of sural nerve, soleus muscle, and achilles tendon. We also performed neurophysiologic investigations. Another patient died, and a postmortem examination of both brain and spin...

journal_title：Acta neuropathologica

authors： Pop PH,Joosten E,van Spreeken A,Gabreëls-Festen A,Jaspar H,ter Laak H,Vos A

更新日期：1984-01-01 00:00:00

abstract：:This report describes three brothers belonging to a consanguineous family suffering from a progressive neurological disorder associated with L-2-hydroxyglutaric aciduria. Clinically this disorder is characterized by childhood onset, pyramidal signs, cerebellar and pseudobulbar syndromes and epilepsy. Pathological exam...

journal_title：Acta neuropathologica

authors： Larnaout A,Hentati F,Belal S,Ben Hamida C,Kaabachi N,Ben Hamida M

更新日期：1994-01-01 00:00:00

abstract：:Canine distemper virus (CDV) causes severe immunosuppression and neurological disease in dogs, associated with demyelination, and is a model for multiple sclerosis in man. In the early stage of the infection, demyelination is associated with viral replication in the white matter. In acute demyelinating lesions there i...

journal_title：Acta neuropathologica

authors： Vandevelde M,Zurbriggen A

更新日期：2005-01-01 00:00:00

abstract：:An ultrastructural study was performed on normal and Benzo(a)-pyrene(B(a)P)-transformed fetal mouse brain cells. Early subcultures of a strain initiated from whole brain presented three cell types in vitro: astroglial, poorly differentiated glial, and spongioblastic types. After B(a)P-treatment, there was an exclusive...

journal_title：Acta neuropathologica

authors： Tripier MF,Markovits P,Papadopoulo D,Toga M

更新日期：1979-08-01 00:00:00

abstract：:Severe hypoxic-ischemic cerebral damage was produced in 8-day-old rats following permanent bilateral carotid artery occlusion and 15 min of ischemia. Cellular damage consisted of early necrosis and appearance of cells with apoptotic-like morphology (karyorrhectic cells) and cells with granular chromatin degeneration i...

journal_title：Acta neuropathologica

authors： Ferrer I,Pozas E,López E,Ballabriga J

更新日期：1997-12-01 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：:The aim of the present work was to investigate whether nestin, a member of the intermediate filament family, is immunohistochemically expressed in the non-tumoral human hypophysis and pituitary neoplasms. Twenty-three normal pituitaries and 125 pituitary neoplasms were included. The tissues were formalin-fixed and par...

journal_title：Acta neuropathologica

authors： Rotondo F,Kovacs K,Horvath E,Bell CD,Lloyd RV,Scheithauer BW

更新日期：2006-03-01 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：:Damage to axons in acute multiple sclerosis (MS) lesions is now well established but the mechanisms of this damage remain obscure. Here we have applied a panel of antibodies that identify cell populations and proteins contained in them with a view to detecting those cells and proteins that are localised particularly c...

journal_title：Acta neuropathologica

authors： Diaz-Sanchez M,Williams K,DeLuca GC,Esiri MM

更新日期：2006-04-01 00:00:00