Myopathy in Marinesco-Sjögren syndrome links endoplasmic reticulum chaperone dysfunction to nuclear envelope pathology.

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：:Dysferlin is a newly identified sarcolemmal protein related to Miyoshi myopathy and limb-girdle muscular dystrophy. Although its function is still unknown, it is inferred from the presence of C2 domains and a transmembrane domain in its sequence that dysferlin may be expressed or located not only at the sarcolemma but...

journal_title：Acta neuropathologica

authors： Ikezoe K,Furuya H,Ohyagi Y,Osoegawa M,Nishino I,Nonaka I,Kira J

更新日期：2003-06-01 00:00:00

abstract：:Multiple different pathological protein aggregates are frequently seen in human postmortem brains and hence mixed pathology is common. Mixed dementia on the other hand is less frequent and neuropathologically should only be diagnosed if criteria for more than one full blown disease are met. We quantitatively measured ...

journal_title：Acta neuropathologica

authors： Walker L,McAleese KE,Thomas AJ,Johnson M,Martin-Ruiz C,Parker C,Colloby SJ,Jellinger K,Attems J

更新日期：2015-05-01 00:00:00

abstract：:The brain of a 69-year-old man exhibited extensive granulomatous inflammation in the walls of arteries in the leptomeninges, associated with amyloid deposition in the media of the involved arteries. The extracranial arteries exhibited neither granulomatous inflammation nor amyloid deposition in their walls. ...

journal_title：Acta neuropathologica

authors： Shintaku M,Osawa K,Toki J,Maeda R,Nishiyama T

更新日期：1986-01-01 00:00:00

abstract：:Since we reported a case of acute relaxant-steroid myopathy (ARSM) in 1994, we continued histological studies and compared the findings with those in a case of corticosteroid myopathy (CM). It was revealed that (1) dystrophin, spectrin, beta dystroglycan, and sarcoglycans on the cell surface were decreased, (2) regula...

journal_title：Acta neuropathologica

authors： Matsubara S,Kitaguchi T,Isozaki E,Miyamoto K,Hirai S

更新日期：1999-05-01 00:00:00

abstract：:A case of multifocal axonopathy associated with intrathecal methotrexate (IT MTX) and radiation therapy is presented. A 33-year-old woman suffering from meningeal carcinomatosis of breast cancer origin had developed prominent multifocal axonal degeneration in the cerebral white matter after treatment with IT MTX thera...

journal_title：Acta neuropathologica

authors： Shibutani M,Okeda R,Hori A,Schipper H

更新日期：1989-01-01 00:00:00

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

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

abstract：:A series of 50 human primary intracranial tumors were cultivated in vitro in an attempt to establish cell lines with the trypsinization technique. During the in vitro adaptation period, cultures were maintained at high cell density to avoid rapid over-growth by connective tissue. Five lines were established from 5 tum...

journal_title：Acta neuropathologica

authors： Maunoury R

更新日期：1977-07-15 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：:The choroid plexus is an epithelial-endothelial vascular convolute within the ventricular system of the vertebrate brain. It consists of epithelial cells, fenestrated blood vessels, and the stroma, dependent on various physiological or pathological conditions, which may contain fibroblasts, mast cells, macrophages, gr...

journal_title：Acta neuropathologica

authors： Wolburg H,Paulus W

更新日期：2010-01-01 00:00:00

abstract：:Following maternal chicken pox in the 14th week of pregnancy, a male infant was born with low birth weight, muscle wasting and limb contractures, hypotonia and areflexia. A rising titre of varicella-zoster-specific IgM (by enzyme-linked immunoabsorbent assay) confirmed congenital infection, and electromyogram showed w...

journal_title：Acta neuropathologica

authors： Harding B,Baumer JA

更新日期：1988-01-01 00:00:00

abstract：:Several cases of progressive multifocal leukoencephalopathy (PML) have been associated with simian virus 40 (SV40), rather than with JC virus (JCV), the polyomavirus originally isolated from PML tissue. PML has, therefore, been defined as a demyelinating syndrome with possible multiple viral etiologies. Tissues from t...

journal_title：Acta neuropathologica

authors： Stoner GL,Ryschkewitsch CF

更新日期：1998-09-01 00:00:00

abstract：:In order to further evaluate the parameters whereby intracerebral administration of recombinant α-synuclein (αS) induces pathological phenotypes in mice, we conducted a series of studies where αS fibrils were injected into the brains of M83 (A53T) and M47 (E46K) αS transgenic (Tg) mice, and non-transgenic (nTg) mice. ...

journal_title：Acta neuropathologica

authors： Sacino AN,Brooks M,Thomas MA,McKinney AB,McGarvey NH,Rutherford NJ,Ceballos-Diaz C,Robertson J,Golde TE,Giasson BI

更新日期：2014-05-01 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：:Mutations in the fused in sarcoma (FUS) gene are linked to a form of familial amyotrophic lateral sclerosis (ALS), ALS6. The FUS protein is a major component of the ubiquitin-positive neuronal cytoplasmic inclusions in both ALS6 and some rare forms of frontotemporal lobar degeneration (FTLD). The latter are now collec...

journal_title：Acta neuropathologica

authors： Aoki N,Higashi S,Kawakami I,Kobayashi Z,Hosokawa M,Katsuse O,Togo T,Hirayasu Y,Akiyama H

更新日期：2012-09-01 00:00:00

abstract：:Human immune deficiency virus (HIV) disease may be associated, neuropathologically, with significant neuronal loss and clinically with a severe dementia. However, the significance of neuronal loss in the development of dementia has not been established. In this study we have undertaken a stereological determination of...

journal_title：Acta neuropathologica

authors： Everall IP,Glass JD,McArthur J,Spargo E,Lantos P

更新日期：1994-01-01 00:00:00

abstract：:Widespread damage to axons in the white matter of the brain is a well-recognised consequence of non-missile head injury. This diffuse axonal injury is characterised by a gradual swelling of the axon associated with an accumulation of cellular organelles and proteins. We have investigated the relationship between the s...

journal_title：Acta neuropathologica

authors： Wilkinson AE,Bridges LR,Sivaloganathan S

更新日期：1999-08-01 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：: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：:In this immunocytochemical study, the brains of nine squirrel monkeys (Saimiri sciureus), ranging from 8 to 27 years of age, were examined for the presence and distribution of beta/A4 amyloid, a 4-kilodalton peptide. In aged squirrel monkeys, amyloid is associated primarily with intracerebral and meningeal capillaries...

journal_title：Acta neuropathologica

authors： Walker LC,Masters C,Beyreuther K,Price DL

更新日期：1990-01-01 00:00:00

abstract：:We studied the microscopic features of 137 cases of human cerebral infarct. In each case, the age of the lesion was determined by measuring the time elapsed between initial clinical presentation and date of surgery or death. Multiple microscopic variables were analyzed on hematoxylin and eosin-stained sections. There ...

journal_title：Acta neuropathologica

authors： Mena H,Cadavid D,Rushing EJ

更新日期：2004-12-01 00:00:00

abstract：:The pro-inflammatory and blood-brain barrier (BBB) effects of intratumoral (IT) injection of human recombinant tumor necrosis factor-alpha (rTNF-alpha) were studied in the Fischer rat RT-2 glioma model. Animals received a single stereotaxic injection of either 6 x 10(4) U rTNF-alpha or excipient (vehicle) into the cen...

journal_title：Acta neuropathologica

authors： Wright JL,Merchant RE

更新日期：1997-01-01 00:00:00

abstract：:The formation of oedema in peripheral nerves was studied in rats at intervals varying from 6 h to 14 days after transection of the right sciatic nerve. Samples were removed proximal and distal to the injury, and the degree of oedema was determined by a microgravimetric method and by measurements of the water content a...

journal_title：Acta neuropathologica

authors： Pettersson CA,Tengvar C

更新日期：1986-01-01 00:00:00

abstract：:Zitter mutant rats exhibit abnormal metabolism of superoxide species and demonstrate progressive degeneration of dopamine (DA) neurons in the substantia nigra (SN). Furthermore, long-term intake of vitamin E, an effective free radical scavenger, prevents the loss of DA neurons caused by free radicals. However, it is u...

journal_title：Acta neuropathologica

authors： Nakadate K,Noda T,Sakakibara S,Kumamoto K,Matsuura T,Joyce JN,Ueda S

更新日期：2006-07-01 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：:Granule-containing vacuoles in the cytoplasm of hippocampal neurons are a neuropathological feature of Alzheimer's disease. Granulovacuolar degeneration (GVD) is not disease-specific and can be observed in other neurodegenerative disorders and even in the brains of non-demented elderly people. However, several studies...

journal_title：Acta neuropathologica

authors： Köhler C

更新日期：2016-09-01 00:00:00

abstract：:We studied the muscle biopsy from an asymptomatic patient with high serum creatine kinase values. Subsarcolemmal and intermyofibrillar granular inclusions were seen at the light microscopy level. Ultrastructural observation showed clusters of cylindrical spirals (CS). CS are nonspecific, morphological finding, so far ...

journal_title：Acta neuropathologica

authors： Rapuzzi S,Prelle A,Moggio M,Rigoletto C,Ciscato P,Comi G,Francesca F,Scarlato G

更新日期：1995-01-01 00:00:00

abstract：:A histochemical study is reported of regional differences of the lipid storage in a case of Niemann-Pick disease (NPD) type C. Besides tissues known to be affected (reticulo-endothelium, hepatocytes, nervous system), storage was demonstrated in adrenal cortical spongiocytes, sweat glands, renal glomerular and tubular ...

journal_title：Acta neuropathologica

authors： Elleder M,Jirásek A,Smíd F

更新日期：1975-12-19 00:00:00

abstract：:Mouse embryos were infected with murine cytomegalovirus (MCMV) by injecting the virus into the cerebral ventricles at the late gestation. After deliveries, offspring were fed by the mothers until 4 weeks. Cystic brain lesions, regarded as porencephaly or paraventricular cysts, were observed in about 20% of the MCMV-in...

journal_title：Acta neuropathologica

authors： Tsutsui Y,Kashiwai A,Kawamura N,Kadota C,Nagahama M

更新日期：1991-01-01 00:00:00

abstract：:We described the first two unrelated Polish families with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). In the morphological examination with light microscopy, two kinds of changes were observed: (1). panarteritis nodosa-like changes with eosinophilic fibrinoid n...

journal_title：Acta neuropathologica

authors： Rafalowska J,Fidziańska A,Dziewulska D,Podlecka A,Szpak GM,Kwieciński H

更新日期：2003-12-01 00:00:00