First report of Creutzfeldt-Jakob disease occurring in 2 siblings unexplained by PRNP mutation.

abstract：:Neurofilamentous axonal swellings occur in a number of degenerative and toxic disorders of the nervous system. In one of these, experimental intoxication with beta, beta'-iminodiproprionitrile (IDPN), accumulation of neurofilaments has been shown to result from a defect in slow axonal transport. The consequence of thi...

journal_title：Journal of neuropathology and experimental neurology

authors： Clark AW,Griffin JW,Price DL

更新日期：1980-01-01 00:00:00

abstract：:The concept of the presence of mucopolysaccharides forming an interstitial ground substance in central nervous tissues is revived. The biochemical reports of both neutral and acid mucopolysaccharides in both gray and white matter of the normal brain are reviewed. The earlier histochemical reports and the histochemical...

journal_title：Journal of neuropathology and experimental neurology

authors： Feigin I

更新日期：1980-01-01 00:00:00

abstract：:This study establishes the sequence of myelination in a population of autopsied infants from birth through the second postnatal year. Myelination was assessed in 62 precisely defined central nervous system (CNS) sites of 162 infants with diverse diseases who were autopsied from 1972 to 1984 at Children's Hospital, Bos...

journal_title：Journal of neuropathology and experimental neurology

authors： Brody BA,Kinney HC,Kloman AS,Gilles FH

更新日期：1987-05-01 00:00:00

abstract：:Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disorder associated with repetitive traumatic brain injury. Multiple system atrophy (MSA) is a Parkinsonian disorder that can result in repetitive falls with associated head trauma. We hypothesized that patients with neurodegenerative disorders ...

journal_title：Journal of neuropathology and experimental neurology

authors： Koga S,Dickson DW,Bieniek KF

更新日期：2016-10-01 00:00:00

abstract：:Neutral glycolipids and gangliosides were analyzed in 149 astrocytomas (A), 46 oligodendrogliomas (O), and 21 oligoastrocytomas (OA) to determine if specific glycolipids correlate with histologic diagnosis and grade. Positivity for asialoGM1 (GA1) and negativity for paragloboside by immuno-TLC correlated with histolog...

journal_title：Journal of neuropathology and experimental neurology

authors： Yates AJ,Comas T,Scheithauer BW,Burger PC,Pearl DK

更新日期：1999-12-01 00:00:00

abstract：:Histopathologic evaluation of brain tissue derived from surgically treated patients with medically refractory temporal lobe epilepsy (TLE) frequently reveals structural brain lesions in the surgical specimen. While several of the most commonly encountered lesions such as low-grade neoplasms or vascular malformations a...

journal_title：Journal of neuropathology and experimental neurology

authors： Kasper BS,Stefan H,Buchfelder M,Paulus W

更新日期：1999-01-01 00:00:00

abstract：:An A and L system of neutral amino acid transport has been demonstrated previously in cerebral microvessels in vivo and in isolated microvessels in vitro. This report describes the neutral amino acid transport properties of cultured cerebral endothelial cells and investigates the influence of astroglia on the transpor...

journal_title：Journal of neuropathology and experimental neurology

authors： Cancilla PA,DeBault LE

更新日期：1983-03-01 00:00:00

abstract：:In an earlier study we found that a substantial percentage of mice surviving infection with canine distemper virus (CDV) slowly developed a morbid obesity syndrome. In the present study we wished to explore the role of the virus in the development of this syndrome. The distribution of viral antigen(s) in brains of pre...

journal_title：Journal of neuropathology and experimental neurology

authors： Nagashima K,Zabriskie JB,Lyons MJ

更新日期：1992-01-01 00:00:00

abstract：:Calpain, a calcium-activated neutral protease family, has been implicated in the neuropathologic sequelae accompanying various neurological disorders. We have characterized the distribution and time course of calpain activation following brain injury in the rat, using a monoclonal antibody that recognizes calpain-gene...

journal_title：Journal of neuropathology and experimental neurology

authors： Saatman KE,Bozyczko-Coyne D,Marcy V,Siman R,McIntosh TK

更新日期：1996-07-01 00:00:00

abstract：:This report describes a phenotypic differentiation pattern conceived to distinguish invading monocytes from resident microglia in frozen and formalin-fixed human CNS. Phagocytic cells in normal and diseased CNS (multiple sclerosis and encephalitis) were studied immunohistochemically with a panel of antibodies, and phe...

journal_title：Journal of neuropathology and experimental neurology

authors： Ulvestad E,Williams K,Mørk S,Antel J,Nyland H

更新日期：1994-09-01 00:00:00

abstract：:Truncations of tau protein at aspartic acid421 (D421) and glutamic acid391 (E391) residues are associated with neurofibrillary tangles (NFTs) in the brains of Alzheimer disease (AD) patients. Using immunohistochemistry with antibodies to D421- and E391-truncated tau (Tau-C3 and MN423, respectively), we correlated the ...

journal_title：Journal of neuropathology and experimental neurology

authors： Basurto-Islas G,Luna-Muñoz J,Guillozet-Bongaarts AL,Binder LI,Mena R,García-Sierra F

更新日期：2008-05-01 00:00:00

abstract：:The microtubule-associated phosphoprotein, tau, is an integral component of paired helical filaments in Alzheimer neurofibrillary tangles (NFT). The mechanism of NFT formation is unknown but aberrant phosphorylation of tau may be contributory. Calcium/calmodulin-dependent protein kinase type II (CaM kinase II), the mo...

journal_title：Journal of neuropathology and experimental neurology

authors： McKee AC,Kosik KS,Kennedy MB,Kowall NW

更新日期：1990-01-01 00:00:00

abstract：:A primary cerebellar rhabdomyosarcoma (RMS) in a six and a half year old boy is reported. Microscopy of the surgical material revealed lobules of closely packed cells with a high mitotic rate, pleomorphic hyperchromatic nuclei and scant cytoplasm. At their periphery, the lobules merged with rounded cells with similar ...

journal_title：Journal of neuropathology and experimental neurology

authors： Hinton DR,Halliday WC

更新日期：1984-07-01 00:00:00

abstract：:Intracranial hemangiopericytic meningiomas (HM) from seven patients were examined by immunostaining, transmission electron microscopy (TEM), scanning electron microscopy (SEM) and cell culture. Positive staining for Factor VIII-related antigen was restricted to capillary endothelial cells. There was no reaction with a...

journal_title：Journal of neuropathology and experimental neurology

authors： Nakamura M,Inoue HK,Ono N,Kunimine H,Tamada J

更新日期：1987-01-01 00:00:00

abstract：:The changes of nitric oxide synthase (NOS) activity and expression in experimental diabetic neuropathy have not been examined. Increases in ganglia NOS might be similar to those that follow axotomy, whereas declines in endothelial NOS (eNOS) and immunological NOS (iNOS) might explain dysfunction of microvessels or mac...

journal_title：Journal of neuropathology and experimental neurology

authors： Zochodne DW,Verge VM,Cheng C,Höke A,Jolley C,Thomsen K,Rubin I,Lauritzen M

更新日期：2000-09-01 00:00:00

abstract：:The role of axonal transport in the pathogenesis of the axonal swellings which develop at the severed ends of transected axons was studied by electron microscopic (EM) autoradiography. Proteins carried by fast anterograde transport in rat sciatic nerves were labeled with [3H]-leucine or [3H]-fucose; [3H]-leucine, [3H]...

journal_title：Journal of neuropathology and experimental neurology

authors： Griffin JW,Price DL,Engel WK,Drachman DB

更新日期：1977-03-01 00:00:00

abstract：:After experimental nerve injuries that extensively disrupt axons, such as chronic constriction injury, immune cells invade the nerve, related dorsal root ganglia (DRGs), and spinal cord, leading to hyperexcitability, raised sensitivity, and pain. Entrapment neuropathies, such as carpal tunnel syndrome, involve minimal...

journal_title：Journal of neuropathology and experimental neurology

authors： Schmid AB,Coppieters MW,Ruitenberg MJ,McLachlan EM

更新日期：2013-07-01 00:00:00

abstract：:Aberrant axonal reorganization and altered distribution of neurotransmitter receptor subtypes have been proposed as major pathogenic mechanisms for hippocampal hyperexcitability in chronic temporal lobe epilepsies (TLE). Recent data point to excitatory class I metabotropic glutamate receptors (mGluR1 and mGluR5) as in...

journal_title：Journal of neuropathology and experimental neurology

authors： Blümcke I,Becker AJ,Klein C,Scheiwe C,Lie AA,Beck H,Waha A,Friedl MG,Kuhn R,Emson P,Elger C,Wiestler OD

更新日期：2000-01-01 00:00:00

abstract：:Wallerian degeneration of the CNS is accompanied by axonal dystrophy or swelling. To understand the mechanisms by which swellings arise, we studied their spatiotemporal dynamics, ultrastructure, composition, and the conditions that affect their formation in vivo and ex vivo. In contrast to peripheral nerve axons, lesi...

journal_title：Journal of neuropathology and experimental neurology

authors： Beirowski B,Nógrádi A,Babetto E,Garcia-Alias G,Coleman MP

更新日期：2010-05-01 00:00:00

abstract：:Infection with human immunodeficiency virus (HIV) clade C is the most common HIV infection worldwide, yet its impact on the nervous system remains largely unknown. Autopsy studies from regions affected by this virus are scarce, and HIV dementia has only rarely been reported from these countries. Most patients who deve...

journal_title：Journal of neuropathology and experimental neurology

authors： Mahadevan A,Shankar SK,Satishchandra P,Ranga U,Chickabasaviah YT,Santosh V,Vasanthapuram R,Pardo CA,Nath A,Zink MC

更新日期：2007-09-01 00:00:00

abstract：:G-protein coupled receptor kinases (GRKs) constitute a serine/threonine kinase family playing a major role in agonist-induced phosphorylation and desensitization of G-protein coupled receptors. Recently, GRK2 and GRK5 have been demonstrated to phosphorylate alpha-synuclein (Ser129) and other synuclein isoforms. We stu...

journal_title：Journal of neuropathology and experimental neurology

authors： Takahashi M,Uchikado H,Caprotti D,Weidenheim KM,Dickson DW,Ksiezak-Reding H,Pasinetti GM

更新日期：2006-12-01 00:00:00

abstract：:The effect of experimental obstructive jaundice on the oxidative status of brain tissues in rats was examined. Twenty-four male Wistar rats were divided into 4 groups: Group I was the control, group II was the sham operated, and groups III and IV were bile duct ligated and killed on the 5th and the 10th day, respectiv...

journal_title：Journal of neuropathology and experimental neurology

authors： Chroni E,Patsoukis N,Karageorgos N,Konstantinou D,Georgiou C

更新日期：2006-02-01 00:00:00

abstract：:Clinical and neuropathological staging of Alzheimer disease (AD) neurodegeneration and neuronal loss dynamics is the baseline for identification of treatment targets and timing. The aim of this study of 14 brain regions in 25 subjects diagnosed with AD and 13 age-matched control subjects was to establish the pattern o...

journal_title：Journal of neuropathology and experimental neurology

authors： Wegiel J,Flory M,Kuchna I,Nowicki K,Ma SY,Wegiel J,Badmaev E,Leon M,Wisniewski T,Reisberg B

更新日期：2021-01-01 00:00:00

abstract：:Cerebral microbleeds (CMB) are a common MRI finding, representing underlying cerebral microhemorrhages (CMH). The etiology of CMB and microhemorrhages is obscure. We conducted a pathological investigation of CMH, combining standard and immunohistological analyses of postmortem human brains. We analyzed 5 brain regions...

journal_title：Journal of neuropathology and experimental neurology

authors： Wadi LC,Grigoryan MM,Kim RC,Fang C,Kim J,Corrada MM,Paganini-Hill A,Fisher MJ

更新日期：2020-10-01 00:00:00

abstract：:Lhermitte-Duclos disease (LDD) is a rare cerebellar tumor associated with Cowden disease (CD) and germline mutations in the PTEN gene. To further define these relationships, we reviewed clinical and pathologic findings in 31 LDD cases and analyzed the status of the PTEN pathway in 11 of them. We hypothesized that the ...

journal_title：Journal of neuropathology and experimental neurology

authors： Abel TW,Baker SJ,Fraser MM,Tihan T,Nelson JS,Yachnis AT,Bouffard JP,Mena H,Burger PC,Eberhart CG

更新日期：2005-04-01 00:00:00

abstract：:The triplication of the DYRK1A gene encoding proline-directed serine/threonine kinase and located in the critical region of Down syndrome (DS) has been implicated in cognitive deficits and intellectual disability of individuals with DS. We investigated the effect of abnormal levels of this kinase on the cytoskeleton i...

journal_title：Journal of neuropathology and experimental neurology

authors： Dowjat K,Adayev T,Kaczmarski W,Wegiel J,Hwang YW

更新日期：2012-12-01 00:00:00

abstract：:Little is known about the molecular features of desmoplastic infantile ganglioglioma (DIG) and desmoplastic infantile astrocytoma (DIA). We performed a genome-wide DNA copy number analysis in combination with a multiplex ligation-dependent probe amplification-based analysis of copy number changes of candidate genes in...

journal_title：Journal of neuropathology and experimental neurology

authors： Gessi M,Zur Mühlen A,Hammes J,Waha A,Denkhaus D,Pietsch T

更新日期：2013-09-01 00:00:00

abstract：:Protein aggregate myopathies, including myofibrillar myopathies and sporadic inclusion body myositis (sIBM), are characterized by abnormal protein aggregates composed of various muscular and ectopic proteins. Previous studies have shown the crucial role ofdysregulated transcription factors such as neuron-restrictive s...

journal_title：Journal of neuropathology and experimental neurology

authors： Olivé M,Janué A,Moreno D,Gámez J,Torrejón-Escribano B,Ferrer I

更新日期：2009-03-01 00:00:00

abstract：:Multiple sclerosis (MS) is an inflammatory demyelinating disorder of the CNS. A recent study identified 4 patterns of demyelination in active MS lesions. The characteristics of pattern II lesions suggested a primary inflammatory mechanism of myelin injury, while pattern III lesions showed features consistent with dyin...

journal_title：Journal of neuropathology and experimental neurology

authors： Mahad DJ,Trebst C,Kivisäkk P,Staugaitis SM,Tucky B,Wei T,Lucchinetti CF,Lassmann H,Ransohoff RM

更新日期：2004-03-01 00:00:00

abstract：:Normal human frontal lobe white matter obtained at autopsy was used to determine the extent of in situ post-mortem degradation of myelin basic protein (BP). Effects of the following two factors were studied: 1) time interval between death and autopsy, and 2) freezing and thawing the tissue. Quantitative extraction of...

journal_title：Journal of neuropathology and experimental neurology

authors： Ansari KA,Rand A,Hendrickson H,Bentley MD

更新日期：1976-03-01 00:00:00