Wolfram syndrome: a clinicopathologic correlation.

abstract：:Four of seven patients with nemaline myopathy had severe, rapidly progressing symptoms. These four showed an increase in acid phosphatase activity in muscle fibers demonstrated by histochemistry and cathepsin B&L activity by biochemical measurement. On electron microscopy, nemaline bodies, occasionally disorganized my...

journal_title：Acta neuropathologica

authors： Nonaka I,Ishiura S,Arahata K,Ishibashi-Ueda H,Maruyama T,Ii K

更新日期：1989-01-01 00:00:00

abstract：:Immunohistochemical studies of monoamine neurons were performed to evaluate toxic effects of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) on young adult mice and compare them with those of their offspring. Mice, 9-11 weeks old (C57BL/6J), injected subcutaneously with a large dose of MPTP (17 mg/kg per day) duri...

journal_title：Acta neuropathologica

authors： Furune S,Miura K,Watanabe K,Nagao S,Takahashi H,Sakai M,Spatz M,Nagatsu I

更新日期：1989-01-01 00:00:00

abstract：:Conventional genetic approaches and computational strategies have converged on immune-inflammatory pathways as key events in the pathogenesis of late onset sporadic Alzheimer's disease (LOAD). Mutations and/or differential expression of microglial specific receptors such as TREM2, CD33, and CR3 have been associated wi...

journal_title：Acta neuropathologica

authors： Haure-Mirande JV,Audrain M,Fanutza T,Kim SH,Klein WL,Glabe C,Readhead B,Dudley JT,Blitzer RD,Wang M,Zhang B,Schadt EE,Gandy S,Ehrlich ME

更新日期：2017-11-01 00:00:00

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

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

更新日期：2001-11-01 00:00:00

abstract：:The conventional concept of Pick's disease does not distinguish Pick's disease with Pick bodies (Pick body disease, PBD) from Pick's disease without Pick bodies [lobar atrophy without Pick bodies, LA-PB(-)]. Recently, intraneuronal ubiquitin-positive inclusions (ub-inclusions), which are thought to be a hallmark of am...

journal_title：Acta neuropathologica

authors： Ikeda K,Akiyama H,Arai T,Ueno H,Tsuchiya K,Kosaka K

更新日期：2002-07-01 00:00:00

abstract：:In the present work the effect of Piromen is studied on the formation of the glial-mesenchymal scar in the experimentally injuried cortex of hamsters. The uniform effect of the bacterial polysaccharide is suggested in a strong and lasting stimulation of proliferating reactive cells in the traumatic area. The excessive...

journal_title：Acta neuropathologica

authors： von Cramon D

更新日期：1975-01-01 00:00:00

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

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

更新日期：1983-01-01 00:00:00

abstract：:An experiment was designed to examine the course of degeneration, phagocytosis, and regeneration in the central nervous system following surgical deafferentation. The anterior cerebellar vermis was ablated in young male rats. The animals were sacrificed by perfusion at postoperative times ranging from 24 hrs to 6 mont...

journal_title：Acta neuropathologica

authors： Johnson JE Jr

更新日期：1975-12-19 00:00:00

abstract：:Flurothyl-induced status epilepticus was studied by light and electron microscopy (LM, EM) to determine the time course and structural features of neuronal necrosis in the vulnerable brain regions in epilepsy. The cerebral cortex, hippocampus and thalamus were examined after closely spaced recovery periods of up to 1 ...

journal_title：Acta neuropathologica

authors： Ingvar M,Morgan PF,Auer RN

更新日期：1988-01-01 00:00:00

abstract：:Impaired axonal transport of the fast or slow component has been reported in patients with sporadic amyotrophic lateral sclerosis (ALS), animal models for ALS, and familial ALS-linked mutant Cu/Zn superoxide dismutase (SOD1) transgenic mice. However, little is known about the impairment of axonal transport in mutant S...

journal_title：Acta neuropathologica

authors： Sasaki S,Warita H,Abe K,Iwata M

更新日期：2005-07-01 00:00:00

abstract：:An unusual case of concurrent MS and anaplastic astrocytoma is presented. MS was diagnosed in a female patient at the age of 22 years. A left side thalamotomy was performed for relief of severe intention tremor at age 28 and at age 32 she received immunosuppressive therapy for 1 year. At the age of 36 after a severe e...

journal_title：Acta neuropathologica

authors： Kalimo H,Frey H,Raine CS,Törmä T,Röyttä M

更新日期：1979-05-15 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：:The quadriceps femoris muscles of experimental allergic myositis, in strain 13 guinea pigs immunised with rabbit myosin B fraction, were subjected to histochemical, immunohistochemical and electron microscopic studies. They demonstrated a variety of degenerative changes of muscle fibres, infiltration of lymphocytes an...

journal_title：Acta neuropathologica

authors： Matsubara S,Takamori M

更新日期：1987-01-01 00:00:00

abstract：:This report presents the largest series of consecutive, neuropathologically confirmed cases of frontotemporal degeneration (FTD). Prior studies have found dementia lacking distinctive histology (DLDH) to be the most common pathology underlying the clinical diagnosis of FTD. In this series of 76 cases, 29 (38%) were fo...

journal_title：Acta neuropathologica

authors： Lipton AM,White CL 3rd,Bigio EH

更新日期：2004-11-01 00:00:00

abstract：:Two young zebra siblings from consecutive pregnancies suffered from neurological disease, characterized by ventral deviation of the neck and tetraparesis which progressed to tetraplegia within a few weeks. On histological examination widespread neuronal degeneration was observed in the ventral horns of the spinal cord...

journal_title：Acta neuropathologica

authors： Higgins RJ,Vandevelde M,Hoff EJ,Jagar JE,Cork LC,Silberman MS

更新日期：1977-01-31 00:00:00

abstract：:It was demonstrated that New Zealand Albino rabbits sensitized to galactocerebroside had high levels of anti-galactocerebroside antibody and of immune complexes. The rabbits was high titers of immune complexes developed demyelination in the peripheral nerves. Lesion were produced in the peripheral nerves of mice by th...

journal_title：Acta neuropathologica

authors： Tsukada N,Koh CS,Yanagisawa N,Taketomi T,Behan PO

更新日期：1985-01-01 00:00:00

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：:We describe the clinical and neuropathological findings of three unrelated autopsy cases of MELAS harboring the A3243G transition in the mitochondrial DNA (mtDNA). Using immunohistochemical techniques, we studied the expression of several subunits of the respiratory chain in various brain regions from the same cases. ...

journal_title：Acta neuropathologica

authors： Sparaco M,Simonati A,Cavallaro T,Bartolomei L,Grauso M,Piscioli F,Morelli L,Rizzuto N

更新日期：2003-09-01 00:00:00

abstract：:Aberrant peripheral nerve fiber bundles, i.e., masses of peripheral nerve fibers without perineural sheath cells, are found in the perivascular spaces of some spinal cords. The 514 spinal cords examined for these structures were separated into two groups; 507 spinal cords without definite necrotic lesions (group 1) an...

journal_title：Acta neuropathologica

authors： Kamiya M,Hashizume Y

更新日期：1989-01-01 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：:A variety of age-related changes occur in the structure of neurons in the cerebral cortex of Wistar-Kyoto and spontaneously hypertensive rats. The most marked alteration associated with increasing age was the deposition of lipofuscin pigment, primarily at the bases of apical dendrites of pyramidal neurons. While no st...

journal_title：Acta neuropathologica

authors： Knox CA,Yates RD,Chen I

更新日期：1980-01-01 00:00:00

abstract：:A model of hypothermic circulatory arrest with recovery has been developed in the newborn dog. Eleven puppies were anesthetized with halothane, paralyzed and artificially ventilated with 70% nitrous oxide -30% oxygen to paO2 > 60 mm Hg, paCO2 = 33-42 mm Hg and pHa = 7.35-7.42. Animals were surface cooled to 20 degrees...

journal_title：Acta neuropathologica

authors： Mujsce DJ,Towfighi J,Yager JY,Vannucci RC

更新日期：1993-01-01 00:00:00

abstract：:The finding of novel fibroblastic onion bulb-like structures in peripheral nerves is reported for the first time in avian riboflavin deficiency. Day old broiler meat chickens were fed a riboflavin deficient diet (1.8 mg/kg) and were killed on postnatal days 6, 11, 16, 21 and 31, whereas control chickens were fed a con...

journal_title：Acta neuropathologica

authors： Cai Z,Blumbergs PC,Finnie JW,Manavis J,Thompson PD

更新日期：2007-08-01 00:00:00

abstract：:Thirty-three rectus superior extraocular muscles from 23 autopsy cases, all over 60 years, were examined. Eosinophilic inclusions (Hirano bodies) were observed in all the muscles; they were stained deep red by Masson's trichrome stain and were positive for protein stain. They consisted of a collection of filamentous s...

journal_title：Acta neuropathologica

authors： Tomonaga M

更新日期：1983-01-01 00:00:00

abstract：:Abuse of the club drugs Methamphetamine (Meth) and Ecstasy (MDMA) is an international problem. The seriousness of this problem is the result of what appears to be programmed cell death (PCD) occurring within the brain following their use. This follow up study focused on determining which cell types, neurons and/or gli...

journal_title：Acta neuropathologica

authors： Warren MW,Larner SF,Kobeissy FH,Brezing CA,Jeung JA,Hayes RL,Gold MS,Wang KK

更新日期：2007-09-01 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：:A desmoplastic primary cerebral neuroblastoma originating in the frontal lobe of a boy who died at the age of 6 years contained dense core vesicles within the cytoplasm of neoplastic cells as evidence of neuronal differentiation. Sarcomatous transformation had occurred at the time of recurrence. At autopsy, he also ha...

journal_title：Acta neuropathologica

authors： Goldammer D,Goebel HH

更新日期：1980-01-01 00:00:00

abstract：:Foetal rat brain reaggregate cultures have been employed to investigate the morphological changes associated with the neurotoxic action of ethylcholine mustard aziridinium (ECMA). In a companion study we provided evidence for apparent selective cholinergic neurotoxicity. Exposure of 9-day-old cultures to 12.5 microM E...

journal_title：Acta neuropathologica

authors： Jones HB,Pillar AM,Prince AK

更新日期：1993-01-01 00:00:00

abstract：:Mutations in the gene coding for Sequestosome 1 (SQSTM1) have been genetically associated with amyotrophic lateral sclerosis (ALS) and Paget disease of bone. In the present study, we analyzed the SQSTM1 coding sequence for mutations in an extended cohort of 1,808 patients with frontotemporal lobar degeneration (FTLD),...

journal_title：Acta neuropathologica

authors： van der Zee J,Van Langenhove T,Kovacs GG,Dillen L,Deschamps W,Engelborghs S,Matěj R,Vandenbulcke M,Sieben A,Dermaut B,Smets K,Van Damme P,Merlin C,Laureys A,Van Den Broeck M,Mattheijssens M,Peeters K,Benussi L,Binetti

更新日期：2014-09-01 00:00:00

abstract：:The presence and distribution of human immunodeficiency virus (HIV) were examined in the CNS of two children with severe HIV encephalitis and myelitis. Using polymerase chain reaction-mediated DNA amplification and subsequent Southern analysis, proviral HIV gag sequences were identified in brain tissue of both patient...

journal_title：Acta neuropathologica

authors： Brüstle O,Spiegel H,Leib SL,Finn T,Stein H,Kleihues P,Wiestler OD

更新日期：1992-01-01 00:00:00