CNS dysplasia in dysencephalia splanchnocystica (Gruber's syndrome). A case report.

abstract：:This study concerns an autopsy case of motor neuron disease with dementia (MND-D) that exhibited unusual clinical and neuropathological findings. The patient was a Japanese man without any relevant family history who was 60 years old at the time of death. His clinical manifestation included character change at the age...

journal_title：Acta neuropathologica

authors： Sudo S,Fukutani Y,Matsubara R,Sasaki K,Shiozawa M,Wada Y,Naiki H,Isaki K

更新日期：2002-05-01 00:00:00

abstract：:Large cell/anaplastic (LC/A) medulloblastoma (MB) is a recently recognized variant of medulloblastoma known to be associated with an advanced stage and a poor prognosis. Although Eberhart et al. suggested histopathologic grading of medulloblastoma in 2002, no consensus has been reached in terms of determining the crit...

journal_title：Acta neuropathologica

authors： Min HS,Lee YJ,Park K,Cho BK,Park SH

更新日期：2006-07-01 00:00:00

abstract：:A 24-year-old woman was found comatose after 2 days of cephalalgia and vomiting. An immediate diagnosis of carbon monoxyde poisoning was disclaimed when blood carbon monoxyde was found to be 1.75 ml/100. A diagnosis of acute intracranial hypertension led to trephination with ventricular punction and brain biopsy on th...

journal_title：Acta neuropathologica

authors： Foncin JF,Le Beau J

更新日期：1978-08-07 00:00:00

abstract：:In 2011, genome-wide association studies implicated a polymorphism near CD33 as a genetic risk factor for Alzheimer's disease. This finding sparked interest in this member of the sialic acid-binding immunoglobulin-type lectin family which is linked to innate immunity. Subsequent studies found that CD33 is expressed in...

journal_title：Acta neuropathologica

authors： Estus S,Shaw BC,Devanney N,Katsumata Y,Press EE,Fardo DW

更新日期：2019-08-01 00:00:00

abstract：:"Diffuse neurofibrillary tangles with calcification" (DNTC) is a rare form of slowly progressive dementia characterized by temporal or fronto-temporal atrophy with neuronal loss and astrocytosis, neurofibrillary tangles and Fahr-type calcification, but no senile plaques in the cerebral cortex. In patients with DNTC, w...

journal_title：Acta neuropathologica

authors： Terada S,Ishizu H,Tanabe Y,Takehisa Y,Haraguchi T,Hamaya K,Nose S,Sudo K,Kuroda S

更新日期：2001-12-01 00:00:00

abstract：:This report illustrates a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) masquerading as neurofibromatosis due to multifocal enlargements of spinal nerve roots. The patient initially complained of intermittent numbness of the hands and leg weakness at age 62. Nerve conduction velocities were ...

journal_title：Acta neuropathologica

authors： Pytel P,Rezania K,Soliven B,Frank J,Wollmann R

更新日期：2003-02-01 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：: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：:Histopathological, immunocytochemical, and electron microscopical investigations were carried out in a man with a protracted history of spastic paraparesis, adrenal insufficiency and hypogonadism. Pathological findings were identical with those of the few previously reported cases of adrenomyeloneuropathy (AMN) includ...

journal_title：Acta neuropathologica

authors： Probst A,Ulrich J,Heitz PU,Herschkowitz N

更新日期：1980-01-01 00:00:00

abstract：:Progression of neuritic dystrophy is a histological hallmark of Alzheimer's disease (AD) in addition to amyloid deposition and neurofibrillary tangle formation. Dystrophic neurites (DNs) are abnormal neurites, and are closely associated with amyloid deposits. To clarify the process of DN formation, we immunohistochemi...

journal_title：Acta neuropathologica

authors： Noda-Saita K,Terai K,Iwai A,Tsukamoto M,Shitaka Y,Kawabata S,Okada M,Yamaguchi T

更新日期：2004-11-01 00:00:00

abstract：:Macroautophagy is a dynamic process whereby cytoplasmic components are initially sequestered within autophagosomes. Recent studies have shown that the autophagosome membrane can selectively recognize ubiquitinated proteins and organelles through interaction with adapter proteins such as p62 and NBR1. Both proteins are...

journal_title：Acta neuropathologica

authors： Odagiri S,Tanji K,Mori F,Kakita A,Takahashi H,Wakabayashi K

更新日期：2012-08-01 00:00:00

abstract：:The hemizygote of the macular mutant mice, which is clinically and neuropathologically considered to be a model of Menkes kinky hair disease (MKHD), were injected intraperitoneally four times with 10, 20, 20 and 30 micrograms of cupric chloride on days 4, 6, 8 and 10 after birth, respectively. Their cerebral and cereb...

journal_title：Acta neuropathologica

authors： Yamano T,Shimada M,Onaga A,Kawasaki H,Iwane S,Ono K,Nishimura M

更新日期：1988-01-01 00:00:00

abstract：:This report presents the ultrastructural nerve study of a patient with sensorimotor neuropathy by a shoe glue containing an association of n-hexane and methylethylketone. Giant axons, distended by microfilamentous proliferation typical of such cases of neuropathy, were found in significant amounts. An unusual histolog...

journal_title：Acta neuropathologica

authors： Vallat JM,Leboutet MJ,Loubet A,Piva C,Dumas M

更新日期：1981-01-01 00:00:00

abstract：:Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive...

journal_title：Acta neuropathologica

authors： Kobayashi A,Arima K,Ogawa M,Murata M,Fukuda T,Kitamoto T

更新日期：2008-11-01 00:00:00

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：: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：: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：: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：:A murine model of Tay-Sachs disease, the prototype of the GM2 gangliosidoses, was produced through the targeted disruption of the Hexa gene encoding the subunit of alpha-hexosaminidase A. The mice were completely devoid of beta-hexosaminidase A activity and accumulated GM2 ganglioside in the CNS in an age-dependent ma...

journal_title：Acta neuropathologica

authors： Taniike M,Yamanaka S,Proia RL,Langaman C,Bone-Turrentine T,Suzuki K

更新日期：1995-01-01 00:00:00

abstract：:A case of 14-year-old girl is reported in whom an alveolar rhabdomyosarcoma occurred in the soft tissues of the left forearm 4 years prior to death. Despite extensive surgery as well as chemotherapy and radiotherapy the tumor recurred locally and produced extensive metastases including a metastasis to the brain. Cereb...

journal_title：Acta neuropathologica

authors： Kleinert R,Beham A,Rosanelli G

更新日期：1985-01-01 00:00:00

abstract：:Histochemical and electron microscopic studies were performed in an attempt to clarify the muscle pathology in an 18-year-old man with Fabry disease, showing proximal limb muscle atrophy, and his 52-year-old mother, who is a Fabry carrier with hypertrophic cardiomyopathy. Despite the relatively mild myopathic changes ...

journal_title：Acta neuropathologica

authors： Uchino M,Uyama E,Kawano H,Hokamaki J,Kugiyama K,Murakami Y,Yasue H,Ando M

更新日期：1995-01-01 00:00:00

abstract：:Two distinct species of amyloid beta protein (A beta) with different carboxyl termini, A beta 40 and A beta 42(43), are deposited in plaques in the brains of patients with Alzheimer's disease and Down's syndrome. The relationship between these two forms of A beta and microglial cells was investigated in 16 subjects wi...

journal_title：Acta neuropathologica

authors： Mann DM,Iwatsubo T,Fukumoto H,Ihara Y,Odaka A,Suzuki N

更新日期：1995-01-01 00:00:00

abstract：:This study was designed to compare the degree of reactive astrogliosis occurring around a puncture wound in the brain of normal rats and at different intervals after a similar puncture wound in rats with a portocaval anastomosis. The gliosis was evaluated by the number of astrocytes, the thickness of their processes a...

journal_title：Acta neuropathologica

authors： Ma KC,Chang ZH,Shih H,Zhu JH,Wu JY

更新日期：1991-01-01 00:00:00

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

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

更新日期：1987-01-01 00:00:00

abstract：:Lysolecithin has been used in many studies to induce demyelination in peripheral nerves. In the present investigation lysolecithin (lysophosphatidyl choline) was injected into rat sciatic nerves at a dose of 2-3 microns of a 10 mg/ml solution in order to study the effects of this lipid on cellular elements other than ...

journal_title：Acta neuropathologica

authors： Mitchell J,Caren CA

更新日期：1982-01-01 00:00:00

abstract：:With an incidence of approximately 350 in 100,000, stroke is the third leading cause of death and a major cause of disability in industrialized countries. At present, although progress has been made in understanding the molecular pathways that lead to ischemic cell death, the current clinical treatments remain poorly ...

journal_title：Acta neuropathologica

authors： Kriz J,Lalancette-Hébert M

更新日期：2009-05-01 00:00:00

abstract：:We report a 69-year-old female with cerebral and cerebellar symptomatology of 15-month duration. At autopsy, both panencephalopathic Creutzfeldt-Jakob and plaque-predominant Alzheimer diseases were found. Plaque amyloid was exclusively of the beta/A4 type, but abundant abnormal protease-resistant protein was identifie...

journal_title：Acta neuropathologica

authors： Powers JM,Liu Y,Hair LS,Kascsack RJ,Lewis LD,Levy LA

更新日期：1991-01-01 00:00:00

abstract：:Isocitrate dehydrogenase 1 (IDH1) mutations are frequent in astrocytomas, oligoastrocytomas and oligodendrogliomas. We previously reported the generation of a mutation-specific antibody that specifically detects R132H mutated IDH1 protein (clone H09). Here, we investigate the feasibility of H09 immunohistochemistry to...

journal_title：Acta neuropathologica

authors： Capper D,Reuss D,Schittenhelm J,Hartmann C,Bremer J,Sahm F,Harter PN,Jeibmann A,von Deimling A

更新日期：2011-02-01 00:00:00

abstract：:We present the neuropathological findings in a female patient with clinically definite multiple sclerosis (MS), who at autopsy had multifocal amyloid deposits in the white matter of the brain without other signs of amyloidosis. The patient had relapsing/remitting MS between the ages of 26 and 45, and during her last 1...

journal_title：Acta neuropathologica

authors： Nennesmo I,Bogdanovic N,Petrén AL,Fredrikson S

更新日期：1997-02-01 00:00:00

abstract：:Alpha-synuclein is known to play an important role in several neurodegenerative diseases. Moreover, it is expressed in central nervous system neuronal tumors, and another member of the synuclein family, gamma-synuclein, is overexpressed in breast and ovarian carcinomas. However, the expression of alpha-synuclein has n...

journal_title：Acta neuropathologica

authors： Mori F,Inenaga C,Yoshimoto M,Umezu H,Tanaka R,Takahashi H,Wakabayashi K

更新日期：2002-02-01 00:00:00