Abstract：
:We investigated clinicopathologically pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and the involvement of the primary motor cortex and pyramidal tract, in eight Japanese autopsy cases of amyotrophic lateral sclerosis (ALS) with dementia. Pyramidal signs were observed in seven (88%) of the eight autopsy cases. Hyperreflexia and Babinski sign were evident in seven (88%) and three (38%) patients, respectively, but spasticity was not observed in any of the eight patients. Loss of Betz cells in the primary motor cortex was evident in the seven cases in which this structure was examined. Astrocytosis in the fifth layer of the primary motor cortex was noticed in three cases. In all eight cases, involvement of the pyramidal tract was obvious in the medulla oblongata, but no involvement of the pyramidal tract was found in the midbrain. Involvement of the pyramidal tract in the spinal cord, particularly of large myelinated fibers, was observed in all six cases in which the spinal cord was examined. In ALS with dementia, pyramidal signs were shown to be present more frequently than previously believed, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells and the pyramidal tract in ALS with dementia has not been reported. Our clinicopathological findings may make a contribution to the understanding of the clinicopathological hallmarks of this disorder. Furthermore, we believe that this study will also contribute to the elucidation of the nosological status of ALS with dementia.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Tsuchiya K,Ikeda K,Mimura M,Takahashi M,Miyazaki H,Anno M,Shiotsu H,Akabane H,Niizato K,Uchihara T,Tominaga I,Nakano Idoi
10.1007/s00401-002-0543-7subject
Has Abstractpub_date
2002-09-01 00:00:00pages
249-59issue
3eissn
0001-6322issn
1432-0533journal_volume
104pub_type
杂志文章abstract：:A simple and reproducible method for cryopreservation of brain tissue from patients with Alzheimer's disease is described. Fresh brain slices (1 cm thick) obtained less than 6 h postmortem are placed in sealed plastic bags, sandwiched between 0.3-cm-thick aluminium sheets, and frozen by placing the entire "sandwich" b...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294305
更新日期：1993-01-01 00:00:00
abstract：:We examined whether the Golgi apparatus (GA) is fragmented in nigral neurons in 18 cases with Parkinson's disease (PD) and in 8 control cases. The nigral neurons in cases with PD showed various degrees of Lewy pathology with alpha-synuclein immunohistochemistry, and we divided the neurons into three subtypes according...
journal_title：Acta neuropathologica
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doi：10.1007/s00401-006-0114-4
更新日期：2006-09-01 00:00:00
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 magnesi...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00688211
更新日期：1989-01-01 00:00:00
abstract：:A 65-year-old woman was admitted to our hospital for forgetfulness, depression and eccentric behavior that had been first noticed 2 years prior to admission. She showed memory impairment, perseveration and repeated violent actions, but no limb-kinetic apraxia. She died 12 years after the onset of symptoms. At autopsy,...
journal_title：Acta neuropathologica
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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：:Medulloblastoma (MB) represents approximately 4% of adult brain tumours, and as such is a poorly studied disease. Although many adult MB are treated using paediatric MB protocols, the reported outcomes are inferior to those observed in children. It remains unclear whether biologic differences underlie these clinical o...
journal_title：Acta neuropathologica
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journal_title：Acta neuropathologica
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doi：10.1007/BF00454906
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journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-010-0719-5
更新日期：2010-09-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-004-0974-4
更新日期：2005-04-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00334513
更新日期：1990-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687584
更新日期：1989-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-002-0575-z
更新日期：2002-10-01 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
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF01273262
更新日期：1978-04-26 00:00:00
abstract：:Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are relentlessly progressive neurodegenerative disorders with overlapping clinical, genetic and pathological features. Cytoplasmic inclusions of fused in sarcoma (FUS) are the hallmark of several forms of FTLD and ALS patients with mutati...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-012-1043-z
更新日期：2013-02-01 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
pub_type： 杂志文章
doi：10.1007/s004010100443
更新日期：2002-02-01 00:00:00
abstract：:The authors describe in biopsies from 6 cases of Werdnig-Hoffmann disease, including 2 of the more benign type, the ultrastructural typical aspects of denervation. They compare their findings with those of other workers. The striking points are the great variation in the diameter of the muscle fibres and the myofibril...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687923
更新日期：1975-01-01 00:00:00
abstract：:Laquinimod (LAQ) is a new oral immunomodulatory compound that reduces relapse rate, brain atrophy and disability progression in multiple sclerosis (MS). LAQ has well-documented effects on inflammation in the periphery, but little is known about its direct activity within the central nervous system (CNS). To elucidate ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-012-1009-1
更新日期：2012-09-01 00:00:00
abstract：:This study reports the histological, ultrastructural, and immunocytochemical characteristics of intracytoplasmic eosinophilic inclusion bodies occurring in various types of neurons of the human central nervous system. By light microscopy, the inclusions were brightly eosinophilic, slightly birefringent, and sharply de...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00308960
更新日期：1989-01-01 00:00:00
abstract：:Peroxidase-labeled lectins were used for detection of specific monosaccharide residues in amyloid plaques in brains of scrapie-infected mice. The lectins tested recognize the following residues: beta-D-galactosyl (Ricinus communis agglutinin 120, RCA-1), alpha-D-galactosyl and alpha-D-galactopyranoside (Bandeirea simp...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00688295
更新日期：1986-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050773
更新日期：1998-01-01 00:00:00
abstract：:Neurofibromas represent one of the hallmarks of neurofibromatosis 1 (NF1) patients. Tumor progression of neurofibromas to malignant peripheral nerve sheath tumors (MPNST) is a frequent and life threatening complication. To learn more about processes involved in malignant transformation, we evaluated differential gene ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-003-0797-8
更新日期：2004-02-01 00:00:00
abstract：:In this report, we describe the clinical, topographical and immunohistochemical characteristics of neurofilament (NF) inclusion formation induced by the intracisternal inoculation of young adult New Zealand white rabbits at 28-day intervals with 100 micrograms AlCl3 over the course of 267 days. The ability to recover ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294460
更新日期：1995-01-01 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
pub_type： 杂志文章,评审
doi：10.1007/s00401-019-02000-4
更新日期：2019-08-01 00:00:00
abstract：:Emerging evidence has demonstrated the neuroprotection of estrogen in Alzheimer's disease (AD). The hippocampus, an important target of estrogen action, is severely affected in the Alzheimer process. The aim of present study was to detect the distribution of estrogen receptor-alpha (ER-alpha) and the relationship betw...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-003-0720-3
更新日期：2003-09-01 00:00:00
abstract：:The incidence of CNS lymphoma has increased significantly in the past 30 years, primarily in the elderly and immunocompromised. While T-cell lymphomas comprise 15-20% of systemic lymphomas, they comprise less than 4% of primary CNS lymphomas, suggesting that they may be under-recognized compared to their systemic coun...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-007-0338-y
更新日期：2008-03-01 00:00:00
abstract：:Frontotemporal lobar degeneration is the term used to describe the non-Alzheimer clinical syndromes of frontotemporal dementia, semantic dementia and progressive non-fluent aphasia, regardless of the underlying neuropathological features. Considerable progress has been made in recent years in our understanding of the ...
journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-007-0241-6
更新日期：2007-07-01 00:00:00
abstract：:In a previous study the occurrence of nerve fibre degeneration with a distribution as in dying-back neuropathies was described in young rats subjected to severe protein deprivation (Oldfors 1981). In this study the ultrastructural appearance of the degeneration of the nerve fibres at different levels of the longitudin...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00688871
更新日期：1982-01-01 00:00:00
abstract：:Mineralization in the wall of central nervous system blood vessels is sporadically encountered in aged horses and cattle as in man, generally as an age-related change. This phenomenon has not to date been located in the meninges in dogs or cats. The present study reports a retrospective histological examination of 50 ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-002-0641-6
更新日期：2003-03-01 00:00:00
abstract：:We have previously reported a pathological investigation of peripheral neuropathy in a horse with knuckling. This report describes details of the muscle and peripheral nerve lesions in two additional cases of light horse yearlings with knuckling. The skeletal muscles showed neurogenic atrophy characterized by scattere...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050916
更新日期：1998-10-01 00:00:00
