Abstract：
:Cerebrotendinous xanthomatosis (CTX), a rare autosomal-recessive lipid storage disease, has been well characterized clinically and biochemically, and recently also from the molecular biological aspect. However, only a very few publications deal with its neuropathology, and views on its pathogenesis vary. Based on a recently examined case, we propose that central-peripheral distal axonopathy is the major pathogenetic mechanism of nervous system injury in CTX. The latter is characterized by white matter pathology, typically in form of long tract involvement with the more distal parts of the tract more severely affected. Most severely affected are the cerebellar white matter, the optic pathways and the long tracts of the brain stem and spinal cord, particularly the pyramidal tracts, although there is hardly a CNS region which does not display some form of pathology. Lesions are characterized by loss of myelinated fibers and accumulation of lipid products in form of foamy macrophages, clear oil-red-O-positive spaces and crystalline clefts, accompanied by gliosis, occasional axonal spheroids, and in the cerebellum--the most severely affected structure--also by multi-nucleated foreign body giant cells. Demyelination is not seen, and ultrastructurally myelin sheaths are normally structured. Signs of axonal degeneration are also present in the spinal roots. We hypothesize that the basic enzymatic defect in CTX leads to accumulation of metabolites in the brain which may be neurotoxic and may impair the metabolic apparatus of neurons with resultant axonopathy and subsequent nonspecific lipid deposition in the injured tracts.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Soffer D,Benharroch D,Berginer Vdoi
10.1007/BF00294324subject
Has Abstractpub_date
1995-01-01 00:00:00pages
213-20issue
2eissn
0001-6322issn
1432-0533journal_volume
90pub_type
杂志文章abstract：:Using a 3H-labelled virion DNA probe applied to tissue sections, we have previously identified the precise microscopic anatomical location of herpes simplex virus (HSV) during the acute and latent stages of infection of the mouse trigeminal ganglia and central nervous system (CNS). In the present investigation, we com...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00692842
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journal_title：Acta neuropathologica
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journal_title：Acta neuropathologica
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journal_title：Acta neuropathologica
pub_type： 杂志文章
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更新日期：1979-02-15 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00308912
更新日期：1990-01-01 00:00:00
abstract：:Adult white rats were injected daily for 1 month with aluminum chloride and killed. Aluminum was identified, quantitated, and localized in the brains of these rats. The only detectable anomalies were crystals of aluminum and phosphorus in lysosomes and lipofuscin granules in the cytoplasm of neurons. ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00707114
更新日期：1980-01-01 00:00:00
abstract：:The morphology of toxoplasma and its interaction with the cellular elements of the brain were studied in a patient who dies of extensive cerebral toxoplasmosis superimposed on Hodgkin's disease. The cerebral lesions were devoid of inflammatory cellular response and contained numerous organisms mostly in isolated multi...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690360
更新日期：1978-06-30 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00693778
更新日期：1987-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
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更新日期：2000-04-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
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更新日期：1992-01-01 00:00:00
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journal_title：Acta neuropathologica
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更新日期：2011-07-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687004
更新日期：1985-01-01 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
pub_type： 杂志文章
doi：10.1007/BF00690634
更新日期：1982-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690521
更新日期：1979-12-01 00:00:00
abstract：:The distribution of the calcium-binding protein calretinin was investigated by immunohistochemistry in the hippocampus, the subicular areas, and the entorhinal cortex in patients with Alzheimer's disease and in control subjects. By double immunolabelling, the calretinin immunoreactivity was compared to the immunoreact...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294357
更新日期：1994-01-01 00:00:00
abstract：:Motor dysfunction is frequently noted in human immunodeficiency virus type 1 (HIV-1)-infected patients. Until recently, neuropathological changes found in the basal ganglia were advanced as pathogenetic mechanisms. In the present study, further brain structures involved in motor control were analyzed morphometrically....
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050502
更新日期：1996-08-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050866
更新日期：1998-07-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00304638
更新日期：1992-01-01 00:00:00
abstract：:Autopsy findings of a patient, with sialidosis type I phenotype carrying V217M/G243R mutations in the lysosomal sialidase gene and biochemically defined isolated sialidase deficiency, who died of intractable lymphoma at the age of 32 years, are described. Perikaryal expansion of cytoplasm was evident, mostly in motor ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-009-0544-x
更新日期：2010-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010051133
更新日期：2000-04-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
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journal_title：Acta neuropathologica
pub_type： 杂志文章
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更新日期：1978-04-26 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-020-02205-y
更新日期：2020-11-01 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
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journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s004010050604
更新日期：1997-02-01 00:00:00
abstract：:A 50-year-old carpenter died in Western Pennsylvania of rabies on January 4, 1979. He had been hospitalized in an intensive care unit for 28 days. The diagnosis was made postmortem from light and electron microscopic examination of central nervous system tissue. Immunofluorescence studies confirmed the diagnosis later...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687393
更新日期：1980-01-01 00:00:00
abstract：:Using immunohistochemical methods optimized to detect herpes simplex virus type 2 (HSV-2) antigen, paraffin sections from human central nervous system tissues from 31 cases pathologically diagnosed as multiple sclerosis (MS), 34 cases of other neurological diseases, 4 adult cases of HSV encephalitis, and mouse brains ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00686969
更新日期：1988-01-01 00:00:00
abstract：:The dynamics of cell-associated Concanavalin A (Con A) in astrocytes of the newborn rat (RNA), the rat glioma (AC), and the human glioblastoma (GB) were studied in vitro by fluorescence and electron microscopy. Con A receptors on the cell surface were seen usually as a continuous thin layer, and Con A accumulations in...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690804
更新日期：1982-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-015-1462-8
更新日期：2015-09-01 00:00:00
abstract：:Oxidative stress has been implicated in the pathogenesis of a number of diseases including Alzheimer's disease (AD). The oxidative stress hypothesis of AD pathogenesis, in part, is based on beta-amyloid peptide (Abeta)-induced oxidative stress in both in vitro and in vivo studies. Oxidative modification of the protein...
journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-009-0517-0
更新日期：2009-07-01 00:00:00
