Transient axonal injury in the absence of demyelination: a correlate of clinical disease in acute experimental autoimmune encephalomyelitis.


: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 and Lewis rats with a slowly progressive myelin degeneration due to proteolipid protein (PLP) overexpression. EAE was triggered either by the transfer of encephalitogenic T-cells alone or by the co-transfer of T-cells with demyelinating antibodies. Inducible nitric oxide synthase (iNOS) expression in perivascular macrophages was associated with a transient functional disturbance of axons, reflected by the focal and reversible accumulation of amyloid precursor protein. Clinical disease correlated with the numbers of APP positive axon spheroids. Demyelination was associated with a further increase of iNOS expression in macrophages and with a higher degree of axonal injury. Our studies suggest that nitric oxide and its metabolites contribute to axonal pathology and possibly also to subsequent neurological dysfunction in EAE.


Acta Neuropathol


Acta neuropathologica


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




Has Abstract


2006-06-01 00:00:00












  • The effects of 5-minute ischemia in Mongolian gerbils: I. Blood-brain barrier, cerebral blood flow, and local cerebral glucose utilization changes.

    abstract::Changes in morphology, behavior of the blood-brain barrier (BBB), regional cerebral blood flow (rCBF), and local cerebral glucose utilization (LCGU) were assessed and correlated in Mongolian gerbils following 5 min cerebral ischemia, produced by bilateral clamping of the common carotid arteries. The morphological chan...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Suzuki R,Yamaguchi T,Kirino T,Orzi F,Klatzo I

    更新日期:1983-01-01 00:00:00

  • Neuropathologic measurements in focal cortical dysplasias: validation of the ILAE 2011 classification system and diagnostic implications for MRI.

    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

    pub_type: 杂志文章


    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

  • Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA.

    abstract::The current classification of human sporadic prion diseases recognizes six major phenotypic subtypes with distinctive clinicopathological features, which largely correlate at the molecular level with the genotype at the polymorphic codon 129 (methionine, M, or valine, V) in the prion protein gene and with the size of ...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Parchi P,de Boni L,Saverioni D,Cohen ML,Ferrer I,Gambetti P,Gelpi E,Giaccone G,Hauw JJ,Höftberger R,Ironside JW,Jansen C,Kovacs GG,Rozemuller A,Seilhean D,Tagliavini F,Giese A,Kretzschmar HA

    更新日期:2012-10-01 00:00:00

  • p35nck5a and cyclin-dependent kinase 5 colocalize in Lewy bodies of brains with Parkinson's disease.

    abstract::We examined the immunohistochemical localization of p35nck5a, the regulatory subunit of cyclin-dependent kinase 5 (cdk5), in brains obtained postmortem from patients with Parkinson's disease (PD) and controls. We found p35nck5a immunoreactivity in Lewy bodies (LB) in the substantia nigra, locus ceruleus, and neocortex...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Nakamura S,Kawamoto Y,Nakano S,Akiguchi I,Kimura J

    更新日期:1997-08-01 00:00:00

  • Pituitary growth hormone cell adenoma with cytoplasmic tubular aggregates in the capillary endothelium.

    abstract::Electron microscopy revealed the presence of cytoplasmic tubular aggregates in the capillary endothelium of a sparsely granulated growth hormone cell adenoma removed surgically from a 25-year-old female patient with acromegaly. To our knowledge, this is the second publication describing these structures in hypophysial...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Kovacs K,Horvath E,Pritzker KP,Schwartz ML

    更新日期:1977-01-31 00:00:00

  • [Musculi longus capitis et splenius of the rat and innervating motoneurons (author's transl)].

    abstract::The nuclei for the nerves of a dorsal (m. splenius) and a ventral (m. longus capitis) neck muscle of the rat were retrogradely labeled by applying horseradish peroxidase (HRP) to the respective cut muscle nerves. Motoneurons of both muscles were analyzed for their localization, diameter of perikarya, and area of dendr...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Stillhard G

    更新日期:1981-01-01 00:00:00

  • Preponderance of sonic hedgehog pathway activation characterizes adult medulloblastoma.

    abstract::Medulloblastoma (MB) represents approximately 4% of adult brain tumours, and as such is a poorly studied disease. Although many adult MB are treated using paediatric MB protocols, the reported outcomes are inferior to those observed in children. It remains unclear whether biologic differences underlie these clinical o...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Al-Halabi H,Nantel A,Klekner A,Guiot MC,Albrecht S,Hauser P,Garami M,Bognar L,Kavan P,Gerges N,Shirinian M,Roberge D,Muanza T,Jabado N

    更新日期:2011-02-01 00:00:00

  • Prominent cortical atrophy with neuronal loss as correlate of human immunodeficiency virus encephalopathy.

    abstract::A 25-year-old homosexual AIDS patient presented with progressive cognitive, motor and behavioral disturbances consistent with HIV encephalopathy. CT scans demonstrated progressive diffuse brain atrophy. Neuropathology showed predominant cortical changes including severe neuronal loss corroborated by morphometry. Only ...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Gray F,Haug H,Chimelli L,Geny C,Gaston A,Scaravilli F,Budka H

    更新日期:1991-01-01 00:00:00

  • 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::We studied a 27-year-old woman who died after a 6-year history of progressive dementia, dystonia, ataxia, apraxia, spasticity, choreoathetosis, visual and auditory hallucinations, and optic atrophy. Magnetic resonance imaging showed decreased intensity in the globus pallidus, substantia nigra, and dentate nuclei in T2...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Neumann M,Adler S,Schlüter O,Kremmer E,Benecke R,Kretzschmar HA

    更新日期:2000-11-01 00:00:00

  • Deletions on the long arm of chromosome 17 in pilocytic astrocytoma.

    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

    pub_type: 杂志文章


    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

  • Genetic Creutzfeldt-Jakob disease associated with the E200K mutation: characterization of a complex proteinopathy.

    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

    pub_type: 杂志文章


    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

  • Expression of the cytokine leukemia inhibitory factor and pro-apoptotic insulin-like growth factor binding protein-3 in Alzheimer's disease.

    abstract::Amyloid-beta (Abeta) deposition in cerebral blood vessel walls is one of the key features of Alzheimer's disease (AD). Abeta(1-40) carrying the "Dutch" mutation (DAbeta(1-40)) induces rapid degeneration of cultured human brain pericytes (HBP). To study the mechanisms of this Abeta-induced toxicity, a comparative cDNA ...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Rensink AA,Gellekink H,Otte-Höller I,ten Donkelaar HJ,de Waal RM,Verbeek MM,Kremer B

    更新日期:2002-11-01 00:00:00

  • Tuberous sclerosis and dysplasia of the corpus callosum. Case report of their combined occurrence in a newborn.

    abstract::A neuropathological study is presented of a case showing the association of tuberous sclerosis of the brain and dysplasia of the corpus callosum as well as omphalocele and malrotated colon. No signs of tuberous sclerosis were found in the internal organs. From a review of the literature this appears to be the fourth c...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Barth PG,Stam FC,von der Harten JJ

    更新日期:1978-04-26 00:00:00

  • Ultrastructural study of medullomyoblastoma.

    abstract::A case of cerebellar medullomyoblastoma in a young boy was investigated by electron microscopy. The neuroectodermal component shows the characteristics of a desmoplastic medulloblastoma. The mesodermal component consists of more or less differentiated cross-striated muscle cells. Undifferentiated muscle cells are very...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Walter GF,Brucher JM

    更新日期:1979-12-01 00:00:00

  • Depopulation of the ventromedial hypothalamic nucleus in the diabetic Chinese hamster.

    abstract::The relationship between diabetes and the size, density and area of the ventromedial hypothalamic nucleus (VMH) was studied in the genetically diabetic Chinese hamster. Matched diabetic and non-diabetic control chinese hamsters were perfused, the hypothalamus collected, sectioned and stained for light microscopy. The ...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Garris DR,Diani AR,Smith C,Gerritsen GC

    更新日期:1982-01-01 00:00:00

  • Intravenous injection of horseradish peroxidase in the rat stimulates corticosterone and adrenocorticotropic hormone release.

    abstract::Wistar rats were given intravenous (i.v.) horseradish peroxidase (HRP) in saline in doses commonly used to study vascular permeability and the blood-brain barrier. Samples of blood were taken from conscious animals via indwelling catheters at intervals up to 6 h after the HRP injection. Plasma concentrations of adreno...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Jezová D,Olsson Y

    更新日期:1986-01-01 00:00:00

  • Increased expression of the putative axon growth-repulsive extracellular matrix molecule, keratan sulphate proteoglycan, following traumatic injury of the adult rat spinal cord.

    abstract::Keratan sulphate proteoglycan (KSPG) is a developmentally regulated barrier molecule, directing axonal growth during central nervous system (CNS) formation. The possible re-expression and functional significance of KSPG in preventing axon regeneration following spinal cord injury (SCI) is poorly understood. In the pre...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Krautstrunk M,Scholtes F,Martin D,Schoenen J,Schmitt AB,Plate D,Nacimiento W,Noth J,Brook GA

    更新日期:2002-12-01 00:00:00

  • Differential radiosensitivity of neurons and neuroglia of the hippocampus in the adult rabbit.

    abstract::Adult rabbits were subjected to 4.5 Gy of whole-blody or brain alone gamma-irradiation, and their hippocampus was examined with the light and electron microscope. Pycnotic cells were found at the base of the granular layer of the dentate gyrus in the so-called subgranular zone, as soon as 3 h after irradiation, and we...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Guéneau G,Drouet J,Privat A,Court L

    更新日期:1979-12-01 00:00:00

  • Neuroaxonal pathology of central and peripheral nervous systems in cerebrotendinous xanthomatosis (CTX).

    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

    pub_type: 杂志文章


    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

  • Making sense of missense variants in TTN-related congenital myopathies.

    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

    pub_type: 杂志文章


    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

  • Hypoglycaemic neuropathy in BB/Wor rats treated with insulin implants: electron microscopic observations.

    abstract::Insulin-dependent diabetes mellitus is a chronic metabolic disease that causes long-term secondary complications such as neuropathy. The occurrence of diabetic neuropathy has generally been thought of as being associated with hyperglycaemia. However, in a previous light microscopic examination of plantar nerves in dia...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Mohseni S,Hildebrand C

    更新日期:1998-08-01 00:00:00

  • Infantile cerebello-optic atrophy. Neuropathology of the progressive encephalopathy syndrome with edema, hypsarrhythmia and optic atrophy (the PEHO syndrome).

    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

    pub_type: 杂志文章


    authors: Haltia M,Somer M

    更新日期:1993-01-01 00:00:00

  • Tumor necrosis factor-alpha, microglia and astrocytes in AIDS dementia complex.

    abstract::The pathogenesis of HIV-associated cognitive changes is poorly understood. Cytokines such as tumor necrosis factor-alpha (TNF-alpha) have been postulated to contribute to the mechanism of the neurological complications of HIV infection. One of the effects of TNF-alpha is to induce astrocyte proliferation in vitro. The...

    journal_title:Acta neuropathologica

    pub_type: 临床试验,杂志文章


    authors: Seilhean D,Kobayashi K,He Y,Uchihara T,Rosenblum O,Katlama C,Bricaire F,Duyckaerts C,Hauw JJ

    更新日期:1997-05-01 00:00:00

  • Ultrastructure of cerebellar capillary hemangioblastoma. III. Crystalloid bodies in endothelial cells.

    abstract::The endothelial cells of three cases of cerebellar capillary hemangioblastoma were studied by means of electron microscopy. Crystalloid bodies, not previously described in the vessels of the central nervous system (CNS), were found in 5%-10% of the endothelial cells, more often in the capillaries with small irregular ...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Ho KL

    更新日期:1985-01-01 00:00:00

  • Immunohistochemical, ultrastructural, biochemical and in vitro studies of a pineocytoma.

    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

    pub_type: 杂志文章


    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

  • Expression of glial fibrillary acidic protein in human glioma cell lines as detected by molecular hybridization.

    abstract::The expression of glial fibrillary acidic protein (GFAP) in continuous glioma and non-glioma cell lines was evaluated by molecular hybridization and immunocytochemistry. RNA transcripts from the GFAP gene were detected in two of six cell lines by Northern blot analysis of cellular RNAs using a cloned mouse GFAP cDNA p...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Dewhurst S,Stevenson M,McComb RD,Volsky DJ

    更新日期:1987-01-01 00:00:00

  • Differentiation of classic medulloblastoma into metastatic large cell medulloblastoma with focal rhabdoid differentiation in the absence of posterior fossa recurrence.

    abstract::A case of classic medulloblastoma that metastasized, despite the absence of local recurrence, to extraneural sites 7 years after treatment is reported. The metastases were, in contrast to the primary tumor, of large cell type and displayed abortive myogenic and, in one site, also rhabdoid differentiation. The primary ...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Donner LR

    更新日期:2005-05-01 00:00:00

  • Cognitive dysfunction in patients with amyotrophic lateral sclerosis is associated with spherical or crescent-shaped ubiquitinated intraneuronal inclusions in the parahippocampal gyrus and amygdala, but not in the neostriatum.

    abstract::Skeins or skein-like inclusions, one of the two types of ubiquitinated intraneuronal inclusions in amyotrophic lateral sclerosis (ALS), in the neostriatum are not specific to the disease, but it has not yet been determined whether the other, spherical or crescent-shaped inclusions (SCI) are pathognomonic. To clarify t...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Kawashima T,Doh-ura K,Kikuchi H,Iwaki T

    更新日期:2001-11-01 00:00:00

  • Spinal and cranial motor nerve roots in amyotrophic lateral sclerosis and X-linked recessive bulbospinal muscular atrophy: morphometric and teased-fiber study.

    abstract::Amyotrophic lateral sclerosis (ALS) and adult onset X-linked recessive bulbospinal muscular atrophy (SPMA), constituting the category of adult onset form of motor neuron disease, were analyzed on motor nerve roots. The results of morphometric analysis on ventral spinal roots (VSR) of all spinal segments from ALS and S...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Sobue G,Matsuoka Y,Mukai E,Takayanagi T,Sobue I,Hashizume Y

    更新日期:1981-01-01 00:00:00

  • Doublecortin immunoreactivity in giant cells of tuberous sclerosis and focal cortical dysplasia.

    abstract::Cerebral cortical lesions of tuberous sclerosis (TSC) and focal cortical dysplasia (FCD) show disturbances in laminar architecture and cellular differentiation. We immunohistochemically studied the expression of doublecortin, a fetal neuronal protein that regulates neuronal migration, in the surgical specimens of five...

    journal_title:Acta neuropathologica

    pub_type: 杂志文章


    authors: Mizuguchi M,Yamanouchi H,Becker LE,Itoh M,Takashima S

    更新日期:2002-10-01 00:00:00