Abstract:
:Long-term epidemiological studies indicate that environmental factors play a causative role in high-incidence amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) in the western Pacific. An increased risk for disease is acquired in youth and remains for life. The low concentrations of calcium and magnesium and high levels of aluminum in the soil and drinking water, along with the relative isolation of these populations, constitute an unusual environmental feature common to all three high-incidence foci. Studies of mineral deposition in brain tissue of Guamanian ALS and PD patients, as well as of neurologically normal Guamanians with neurofibrillary degeneration, demonstrate accumulations of calcium, aluminum and silicon in neurofibrillary tangle-bearing neurons. In an attempt to duplicate the low calcium and high aluminum and manganese in soil and drinking water in these foci, we maintained juvenile cynomolgus monkeys for 41 to 46 months on a low-calcium diet with or without supplemental aluminum and manganese. Experimental animals exhibited mild calcium and aluminum deposition and degenerative changes, compatible with those of early ALS and PD, in motor neurons of the spinal cord, brain stem, substantia nigra and cerebrum. Neuropathological findings included chromatolysis, aberrant perikaryal accumulation of phosphorylated neurofilament, neurofibrillary tangles, axonal spheroids, and basophilic and hyaline-like inclusions consisting of abnormal cytoskeletal elements by electron microscopy. The magnitude and extent of these lesions far exceeded those found in normal aged monkeys.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Garruto RM,Shankar SK,Yanagihara R,Salazar AM,Amyx HL,Gajdusek DCdoi
10.1007/BF00688211subject
Has Abstractpub_date
1989-01-01 00:00:00pages
210-9issue
2eissn
0001-6322issn
1432-0533journal_volume
78pub_type
杂志文章abstract::A 79-year-old woman had a 10-year history of dementia, initially presenting as non-fluent aphasia. Magnetic resonance imaging showed frontal atrophy (left greater than right) and hyperintense foci within white matter. Neuropathologically, there was severe frontal atrophy due to cortical neuronal loss with spongy chang...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0719-9
更新日期:2003-08-01 00:00:00
abstract::The essential role of the cellular prion protein (PrPC) in prion disorders such as Creutzfeldt-Jakob disease is well documented. Moreover, evidence is accumulating that PrPC may act as a receptor for protein aggregates and transduce neurotoxic signals in more common neurodegenerative disorders, such as Alzheimer's dis...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-017-1790-y
更新日期:2018-02-01 00:00:00
abstract::New pericapillary inclusion bodies were found in 17 cases of sporadic amyotrophic lateral sclerosis (ALS). The inclusion bodies consisted of paracrystalline arrays with 5-7 nm electron-dense subunits, were discernible with the light microscope and had the staining properties of protein. They were surrounded by capilla...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687970
更新日期:1986-01-01 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
pub_type: 杂志文章
doi:10.1007/BF00689595
更新日期:1988-01-01 00:00:00
abstract::We present here an unusual case of papillary neuroepithelial tumor of the pineal region. The patient was a 29-year-old female who presented with headaches. A computed tomography scan revealed a tumorous lesion at the pineal region and hydrocephalus. The resected tumor was composed of columnar and cuboidal cells showin...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0898-z
更新日期:2004-10-01 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
pub_type: 杂志文章
doi:10.1007/BF00685010
更新日期:1978-08-07 00:00:00
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
doi:10.1007/s004010050875
更新日期:1998-08-01 00:00:00
abstract::The morphological effects of two chemically different neuroactive drugs (chlorpromazine and phenobarbital) on vasculogenesis in rat cerebellum were examined to determine the presence of vascular alterations. Therapeutic dosages of both drugs were chronically administered to separate groups of maternal rats beginning o...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692188
更新日期:1982-01-01 00:00:00
abstract::Numbers of dystrophic neurites, seen with the electron microscope, in CA1 of the hippocampus of either C3H mice infected with 22C or 79A strains of scrapie, or LM mice infected with strain ME7 were greater than in age-matched control mice. Vacuolation, seen by light microscopy in CA1 of the hippocampus of mice infecte...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688263
更新日期:1987-01-01 00:00:00
abstract::Axonal polyglucosan bodies in myelinated axons in the ventral posterolateral nucleus of the human thalamus (VPL) are described. These axonal inclusions were distributed exclusively in the dorsolateral part of the caudal VPL, and their arrangement may be associated with fibres originating from the gracile nucleus. They...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688332
更新日期:1987-01-01 00:00:00
abstract::Flow cytofluorometric analysis was used to determine the distribution of the DNA content in cells from selected areas of normal human brain and in benign and malignant brain tumors. Propidium iodide was employed as DNA fluorochrome and the analysis was carried out on a suspension of single cells. Normal, nonstimulated...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684802
更新日期:1979-04-12 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease with no effective treatments. Numerous RNA-binding proteins (RBPs) have been shown to be altered in ALS, with mutations in 11 RBPs causing familial forms of the disease, and 6 more RBPs showing abnormal expression/distribution in ALS albeit...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-017-1785-8
更新日期:2018-02-01 00:00:00
abstract::The present study investigated the expression of c-erbB-2 in 59 meningiomas, including different histological subtypes and anaplastic variants, by immunocytochemistry and molecular biological techniques. Immunohistochemistry using the monoclonal antibody FWP-51 directed against c-erbB-2-encoded oncoprotein gp185 demon...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00228582
更新日期:1993-01-01 00:00:00
abstract::The cholinergic basal forebrain is divided into four subregions (Ch1-4), and cholinergic neuronal loss in the nucleus basalis of Meynert (Ch4) has been correlated with cognitive impairments in both Alzheimer's disease (AD) and dementia with Lewy bodies (DLB). However, the Ch1-2 regions, which provide the major choline...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-0004-1
更新日期:2006-02-01 00:00:00
abstract::Marinesco-Sjögren syndrome (MSS) features cerebellar ataxia, mental retardation, cataracts, and progressive vacuolar myopathy with peculiar myonuclear alterations. Most MSS patients carry homozygous or compound heterozygous SIL1 mutations. SIL1 is a nucleotide exchange factor for the endoplasmic reticulum resident cha...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1224-4
更新日期:2014-05-01 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
pub_type: 杂志文章
doi:10.1007/s00401-003-0686-1
更新日期:2003-06-01 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
pub_type: 杂志文章
doi:10.1007/BF00686094
更新日期:1986-01-01 00:00:00
abstract::We report the case of an 11-year-old male who developed subacute diffuse polyradiculoneuropathy, associated with digestive symptoms and Epstein-Barr virus infection. Parental consanguinity was present. The laboratory findings including bone marrow smear were consistent with hemophagocytic lymphohistiocytosis (HLH). El...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0897-0
更新日期:2004-10-01 00:00:00
abstract::The thoracic sympathetic preganglionic outflow is important in the maintenance of postural normotension in man. Normative data on the intermediolateral column (ILC) neuron cell bodies is lacking. Counts and measurements on the right ILC cytons have been performed on the T6, T7 and T8 segments of 12 spinal cords of man...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688573
更新日期:1977-09-26 00:00:00
abstract::Prions cause scrapie and Creutzfeldt-Jakob disease (CJD); these infectious pathogens are composed largely, if not entirely, of protein molecules. No prion-specific polynucleotide has been identified. Purified preparations of scrapie prions contain high titers (greater than or equal to 10(9.5) ID50/ml), one protein (Pr...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/BF00687261
更新日期:1987-01-01 00:00:00
abstract::The peripheral immune system plays a critical role in aging and in the response to brain injury. Emerging data suggest inflammatory responses are exacerbated in older animals following ischemic stroke; however, our understanding of these age-related changes is poor. In this work, we demonstrate marked differences in t...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-018-1859-2
更新日期:2018-07-01 00:00:00
abstract::Charcot-Marie-Tooth disease type 1A (CMT1A) is a common hereditary demyelinating neuropathy caused by a duplication of the gene for the myelin protein PMP22, resulting in overexpression of PMP22 in young patients. Although genetically well defined, the pathogenesis of the hereditary demyelinating neuropathy CMT1A is s...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050712
更新日期:1997-10-01 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
pub_type: 杂志文章
doi:10.1007/BF00691868
更新日期:1983-01-01 00:00:00
abstract::The authors present the case of a hypertensive patient with a thrombosis of a saccular microaneurysm (SMA) and underlying cerebral (pontine) lacunae. This SMA lay at the junction between the main vessel and two small feeding arteries penetrating into the necrotic territory. Microhemorrhages were observed in and outsid...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688313
更新日期:1986-01-01 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
pub_type: 杂志文章
doi:10.1007/BF00690850
更新日期:1979-05-15 00:00:00
abstract::The very existence of astroblastoma has been a question of considerable controversy, although there appears now to be sufficient documentation to establish it as a tenable entity. Due to the rarity of this tumor, little information exists in the literature as to its natural history, efficacy of therapy and its patholo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00689508
更新日期:1986-01-01 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
pub_type: 杂志文章
doi:10.1007/BF00294437
更新日期:1991-01-01 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
pub_type: 杂志文章
doi:10.1007/BF00690990
更新日期:1981-01-01 00:00:00
abstract::Cavernous malformations (CVMs) and arteriovenous malformations (AVMs) were immunostained for three smooth muscle cell (SMC)-specific protein markers (smooth muscle alpha-actin, SM1 and SM2). Smooth muscle alpha-actin, a widely used marker of SMCs, is reportedly one of the earliest proteins expressed during differentia...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100362
更新日期:2001-09-01 00:00:00
abstract::Pontocerebellar hypoplasia type 2 (PCH-2; MIM 277470), an autosomal recessive neurodegeneration with fetal onset, was studied in six autopsies with ages at death ranging between 1 and 22 years. Three patients were distantly related. A case of olivopontocerebellar hypoplasia (OPCH; MIM 225753) was studied for compariso...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0263-0
更新日期:2007-10-01 00:00:00