Abstract：
:In chronic granulomatous disease (CGD) enzyme-deficient neutrophils and mononuclear cells lack the respiratory burst required for biocidal activity. Recurrent infections lead to granulomas in various organs but brain lesions are rare. In the present case, a 23-year-old male with numerous infections since early childhood died of overwhelming pulmonary aspergillosis. He first began to experience neurological deficits at the age of 17. Computerized tomography and magnetic resonance imaging revealed fleeting white matter lesions that were interpreted as multiple sclerosis (MS). At post mortem, three types of brain lesions were found: (1) Pigmented macrophages in perivascular spaces and the leptomeninges similar to those reported previously. They contained fine, golden-brown, lipofuscin-like material whose chemical composition included a sulfur peak by X-ray analysis. (2) Focal, well-demarcated, "burnt out" white matter lesions with loss of both myelin and axons and intense sclerosis. (3) Diffuse areas of mild pallor in the centrum ovale which spared the U fibers. The pigmented macrophages are characteristic of those seen in the periphery in CGD. The origin of the discrete, destructive white matter lesions is unclear. They may have resulted from: (i) earlier activity by CGD macrophages; (ii) previous infections due to sepsis or embolism; or (iii) possibly post-infectious encephalomyelitis. The more diffuse, mild, white matter lesions are attributed to edema. Evidence for MS, progressive multifocal leukoencephalopathy, or human immunodeficiency virus encephalitis was lacking. This case is presented to alert us to look more carefully for brain lesions in CGD, characterize them and to help determine their cause.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Hadfield MG,Ghatak NR,Laine FJ,Myer EC,Massie FS,Kramer WMdoi
10.1007/BF00293469subject
Has Abstractpub_date
1991-01-01 00:00:00pages
467-70issue
4eissn
0001-6322issn
1432-0533journal_volume
81pub_type
杂志文章abstract：:Accumulation of the tau protein in fibrillar intracellular aggregates is a defining feature of multiple neurodegenerative diseases collectively referred to as tauopathies. Despite intensive study of tau, there is limited information on the formation and clearance dynamics of tau inclusions. Using rAAV vectors to media...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-021-02264-9
更新日期：2021-01-26 00:00:00
abstract：:Both protein kinases and phosphoprotein phosphatases are important components of signal transduction systems in cells. Recent studies in Alzheimer's disease (AD) have shown abnormal protein phosphorylation in the cortex suggesting an alteration in these enzymes. In the present study, an antibody against CD45 was used ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294425
更新日期：1991-01-01 00:00:00
abstract：:Microglia are long-living resident immune cells of the brain, which secure a stable chemical and physical microenvironment necessary for the proper functioning of the central nervous system (CNS). These highly dynamic cells continuously scan their environment for pathogens and possess the ability to react to damage-in...
journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-015-1524-y
更新日期：2016-03-01 00:00:00
abstract：:Onion bulbs are concentric lamellar structures formed by Schwann or perineurial cells, which may be seen in several generalized or localized diseases of the peripheral nerve. There is debate regarding the pathogenesis of localized tumefactions displaying these microscopic structures. We report the fifth case, to our k...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/pl00007408
更新日期：2000-01-01 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：:Human adenovirus type 12 (Ad 12) was inoculated intraperitoneally, intrapleurally, intramuscularly or subcutaneously into newborn rodents. Tumors developed preferentially in the peritoneal cavities in 93.9% of the hamsters and 82.6% of the mice, but none in rats; in contrast to the high incidence of brain tumors in ra...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687575
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
pub_type： 杂志文章
doi：10.1007/BF00685251
更新日期：1984-01-01 00:00:00
abstract：:In comparison to the levels in age and gender-matched controls, reduced levels of pathological amyloid-β protein in cerebrospinal fluid routinely precede the onset of Alzheimer's disease-related symptoms by several years, whereas elevated soluble abnormal tau fractions (phosphorylated tau, total tau protein) in cerebr...
journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-013-1139-0
更新日期：2013-11-01 00:00:00
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
更新日期：1987-01-01 00:00:00
abstract：:Rats were given daily injections of 0.2 or 0.5 g/kg chlorophenoxyisobutyrate (clofibrate) for 9 to 46 days. Lower leg muscles were studied with light and electron microscopy. Daily treatment with 0.5 g/kg of the drug for 26--46 days caused myopathic changes. ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00703319
更新日期：1977-08-16 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
pub_type： 杂志文章
doi：10.1007/BF00684532
更新日期：1977-01-31 00:00:00
abstract：:Endoneurial cell response and type of nerve fibre damage were studied after perineural injections of 7% phenol-aqua and pure glycerol. Our previous studies have shown that phenol and glycerol induce different types of nerve fibre degeneration after intraneural injections: phenol dissolves axons and Schwann cells insid...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-004-0896-1
更新日期：2004-10-01 00:00:00
abstract：:A 9-year-old male patient developed a germ cell tumor in the right basal ganglia which secreted beta-human chorionic gonadotropin (beta-HCG) and caused precocious puberty. Histology and immunohistochemical staining for placental alkaline phosphatase (PLAP), alpha-fetoprotein (alpha-FP), and beta-HCG showed a mixed pop...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687797
更新日期：1988-01-01 00:00:00
abstract：:A limited cortical resection including the rolandic fissure and the pre- and postcentral cortical regions was carried out in a patient suffering from epilepsia partialis continua resistant to antiepileptic drugs. The histological examination revealed several foci of very large neurons distributed with no laminar organ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00299415
更新日期：1992-01-01 00:00:00
abstract：:A 48-year-old man with dural arteriovenous malformation (AVM) is reported. Radiologically, the dural AVM was demonstrated mainly in the region of the left transverse sinus. Postmortem examination revealed dural AVM involving the bilateral transverse, superior sagittal and straight sinuses. In addition, numerous malfor...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00307635
更新日期：1990-01-01 00:00:00
abstract：:With increasing longevity, the number of older schizophrenic patients is growing. Previous criteria used the age of symptom onset to differentiate between the late manifestations of early-onset schizophrenia and late-onset schizophreniform disorders. Current DSM-IV or ICD 10 nomenclatures do not differentiate between ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-001-0468-6
更新日期：2002-04-01 00:00:00
abstract：:Immunohistochemical analysis of inflammatory cell density and infiltrate subpopulations in 42 meningiomas was performed. Evaluation of infiltrating cell density was carried out by cell counting. Meningothelial and fibroblastic meningiomas contained an average of 3% mononuclear cells; the few lymphocytes were localized...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294381
更新日期：1989-01-01 00:00:00
abstract：:Focal cerebral ischemia was induced by occlusion of the right middle cerebral artery in hypoglycemic, normoglycemic, as well as in acute and chronic diabetic rats. The brain damage was studied after 4 days. The volume of infarction was decreased in hypoglycemia (29 +/- 19 mm3 (mean +/- SD) versus 58 +/- 35 mm3, P less...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00693778
更新日期：1987-01-01 00:00:00
abstract：:The mechanisms underlying neurodegenerative diseases are the outcome of pathological alterations of evolutionary conserved molecular and cellular cascades. For this reason, Drosophila and C. elegans serve as useful model systems to study various aspects of neurodegenerative diseases. Here, we introduce the advantageou...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-010-0689-7
更新日期：2010-08-01 00:00:00
abstract：:Inappropriate apoptosis has been implicated in the mechanism of neuronal death in Huntington's disease (HD). In this study, we report the expression of apoptotic markers in HD caudate nucleus (grades 1-4) and compare this with controls without neurological disease. Terminal transferase-mediated biotinylated-UTP nick e...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-004-0957-5
更新日期：2005-03-01 00:00:00
abstract：:In the cytoplasm of Schwann cells of a sural nerve biopsy from a 21-year-old female patient with chronic neuropathy we noted numerous unique, usually double membrane-bound, osmiophilic, granular or globular inclusions, approximately 30-600 microm in diameter. Some of these membrane-bound vesicular or tubular structure...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010051104
更新日期：1999-10-01 00:00:00
abstract：:Sporadic Creutzfeldt-Jakob disease (sCJD) is a transmissible brain proteinopathy. Five main clinicopathological subtypes (sCJD-MM(V)1, -MM(V)2C, -MV2K, -VV1, and -VV2) are currently distinguished. Histopathological evidence suggests that the localisation of prion aggregates and spongiform lesions varies among subtypes...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-020-02168-0
更新日期：2020-08-01 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
pub_type： 杂志文章
doi：10.1007/BF00687789
更新日期：1988-01-01 00:00:00
abstract：:Pediatric glioblastomas (GBM) including diffuse intrinsic pontine gliomas (DIPG) are devastating brain tumors with no effective therapy. Here, we investigated clinical and biological impacts of histone H3.3 mutations. Forty-two DIPGs were tested for H3.3 mutations. Wild-type versus mutated (K27M-H3.3) subgroups were c...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-012-0998-0
更新日期：2012-09-01 00:00:00
abstract：:A 1-year-old male chinchilla with a 2-week history of conjunctivitis suffered subsequently from neurological signs comprising seizures, disorientation, recumbency and apathy. After 3 weeks of progressive central nervous disease the animal was killed in view of the poor prognosis. A non-suppurative meningitis and polio...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-002-0597-6
更新日期：2002-12-01 00:00:00
abstract：:Fifteen cases of Down syndrome between age 25-59 years were examined neuropathologically. A variety of histological methods were used to identify plaques and neurofibrillary tangles (NFT). All cases had some plaques or NFT, but their density was generally not high before the age of 40 years. Plaques and NFT tended to ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687256
更新日期：1989-01-01 00:00:00
abstract：:Localization of organ-specific brain antigens in the central nervous system of the rat has been studied by means of indirect immunofluorescence. Rabbit antiserum against homogenate of rat brain, previously absorbed with normal serum and homogenates of rat organs (kidney, liver, spleen), reacted with the water-soluble ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00703316
更新日期：1977-08-16 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：:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and a phenotypically similar recessive condition (CARASIL) have emerged as important genetic model diseases for studying the molecular pathomechanisms of cerebral small vessel disease (SVD). CADASIL, the most frequent ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-018-1853-8
更新日期：2018-07-01 00:00:00
abstract：:This article highlights the features that connect prion diseases with other cerebral amyloidoses and how these relate to neurodegeneration, with focus on tau phosphorylation. It also discusses similarities between prion disease and Alzheimer's disease: mechanisms of amyloid formation, neurotoxicity, pathways involved ...
journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-010-0691-0
更新日期：2011-01-01 00:00:00
