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 of 54, followed by frozen gait, dysarthria and bradykinesia and he was diagnosed with Parkinson's disease. He gradually developed spastic paresis and died of respiratory failure 6 years after onset of the illness. Neuropathological examinations showed prominent degeneration in the striatonigral and pallidoluysian systems in addition to the neuronal loss and microvacuolation in the second to third layers of the frontal and temporal cortex, the involvement of the upper and lower motor neuron systems and the presence of ubiquitinated neuronal inclusions. To our knowledge, five cases of motor neuron disease (MND) combined with pallido-nigro-luysian atrophy (PNLA) have been reported previously, but the present case is the first report of MND-D combined with the degeneration of the striatonigral and pallidoluysian systems. Such an association may represent more than a coincidental occurrence, and it suggests that MND-D is not simply a disease of the motor neuron system but a multisystem degeneration.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Sudo S,Fukutani Y,Matsubara R,Sasaki K,Shiozawa M,Wada Y,Naiki H,Isaki Kdoi
10.1007/s00401-001-0482-8subject
Has Abstractpub_date
2002-05-01 00:00:00pages
521-5issue
5eissn
0001-6322issn
1432-0533journal_volume
103pub_type
杂志文章abstract：:Ubiquitin-immunoreactive (ub-ir) neuronal cytoplasmic inclusions are characteristically found in the extramotor cortex in patients with motor neuron disease and dementia (MND-dementia) and a subset of patients with frontotemporal dementia without motor symptoms (FTD-MND type). Recently, ub-ir neuronal intranuclear inc...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-003-0678-1
更新日期：2003-06-01 00:00:00
abstract：:Data on the relationship between Parkinson's disease (PD) and stroke have been conflicting, some studies showing a reduced risk of stroke during life, and others indicating an increased risk of stroke-related death. Consecutive cases (n=617) of autopsy-proven idiopathic PD (Lewy body disease of the brain stem type) an...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-003-0676-3
更新日期：2003-05-01 00:00:00
abstract：:A massive expansion of a GGGGCC repeat upstream of the C9orf72 coding region is the most common known cause of amyotrophic lateral sclerosis and frontotemporal dementia. Despite its intronic localization and lack of a canonical start codon, both strands are translated into aggregating dipeptide repeat (DPR) proteins: ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-015-1450-z
更新日期：2015-10-01 00:00:00
abstract：:We have studied microtubule-associated protein 2 (MAP2) expression in anterior horn neurons in the cervical and lumbar spinal cords of 19 cases of adult-onset sporadic amyotrophic lateral sclerosis (ALS) using immunohistochemistry. Specimens from 7 patients without neurological disease served as controls. MAP2 express...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050950
更新日期：1999-01-01 00:00:00
abstract：:Detailed neuropathologic and immunohistologic analysis of a case of serologically and polymerase chain reaction-confirmed human immunodeficiency virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is reported in a 73-year-old North American black woman. In addition to the usual neuropath...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00304136
更新日期：1993-01-01 00:00:00
abstract：:A complex neuropathological study of two cases of Niemann-Pick disease (NPD) type C (NPDC) revealed some novel features in the chemical pathology of the neuronal storage. Lipid histochemistry showed the presence of a lipid which met the criteria of a neuronal glycosphingolipid. Sphingomyelin (SM) was not detected in t...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690966
更新日期：1985-01-01 00:00:00
abstract：:Progressive myopathy developed in two women who consumed ipecac syrup containing emetine hydrochloride to induce vomiting as part of their anorexia nervosa. Muscle biopsy specimens were characterized by severe disruption of the sarcomeres. The ultrastructural spectrum extended from "Z-band streaming" to the formation ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00686202
更新日期：1988-01-01 00:00:00
abstract：:Unusual inclusions with some of the features of "reducing bodies" were encountered in the skeletal muscle biopsy of a 2.5-year-old boy with childhood-onset acid maltase deficiency. The biopsy revealed a vacuolar myopathy with lysosomal storage of glycogen and eosinophilic refractile inclusions in myofibers, which appe...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00304641
更新日期：1992-01-01 00:00:00
abstract：:We described the first two unrelated Polish families with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). In the morphological examination with light microscopy, two kinds of changes were observed: (1). panarteritis nodosa-like changes with eosinophilic fibrinoid n...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-003-0764-4
更新日期：2003-12-01 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
pub_type： 杂志文章
doi：10.1007/s00401-008-0425-8
更新日期：2008-11-01 00:00:00
abstract：:The pathological changes in the nucleus basalis of Meynert (nbM) in 10 autopsied cases with Pick's disease were studied in comparison with 15 age-matched controls. Both the number and density of nerve cells and the degree of fibrillary gliosis were examined at the anterior, intermediate, and posterior divisions of the...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687402
更新日期：1989-01-01 00:00:00
abstract：:The calcium-activated protease calpain cleaves a variety of biologically important proteins and serves, therefore, as a key regulator of many cellular functions. Activation of both main isoforms, calpain 1 and calpain 2, was demonstrated previously in Alzheimer's disease. In this report, antibodies specifically recogn...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-002-0528-6
更新日期：2002-07-01 00:00:00
abstract：:Essential mixed cryoglobulinemia, which can cause hypersensitivity vasculitis, was observed in five patients with peripheral neuropathy. Three cases presented with multifocal neuropathies and two cases with symmetrical polyneuropathy. One had cryoglobulinemia with IgM monoclonal gammopathy IgG polyclonal gammopathy, a...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00686206
更新日期：1988-01-01 00:00:00
abstract：:Mutations of isocitrate dehydrogenase 1 (IDH1) gene are most common in glioma, arguably preceding all known genetic alterations during tumor development. IDH1 mutations nearly invariably target the enzymatic active site Arg132, giving rise to the predominant IDH1R132H. Cells harboring IDH1 R132H -heterozygous mutation...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-017-1800-0
更新日期：2018-02-01 00:00:00
abstract：:The spinal cord of 20 patients with amyotrophic lateral sclerosis (ALS) and 5 patients with lower motor neuron disease (LMND) were investigated immunohistochemically using anti-human excitatory amino acid transporter 1 (EAAT1) and EAAT2 antibodies which are the astrocytic transporters. The purpose of the study was to ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004019900159
更新日期：2000-08-01 00:00:00
abstract：:X-linked adrenoleukodystrophy (X-ALD) is a rare neurometabolic disease characterized by the accumulation of very long chain fatty acids (VLCFAs) due to a loss of function of the peroxisomal transporter ABCD1. Here, using in vivo and in vitro models, we demonstrate that autophagic flux was impaired due to elevated mamm...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-014-1378-8
更新日期：2015-03-01 00:00:00
abstract：:Cerebellar torpedoes, unique fusiform swellings of Purkinje cell axons within the granular layer, have been known to occur sparsely associated with diffuse cerebellar changes. This report describes, in three human autopsy cases with focal necrotic lesions in the cerebellar white matter, torpedoes which were essentiall...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00311388
更新日期：1992-01-01 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
pub_type： 杂志文章
doi：10.1007/s00401-004-0907-2
更新日期：2004-11-01 00:00:00
abstract：:Cystic necrosis in the cerebellar white matter was found in three premature infants. The necrosis was characteristically localized in the center of the white matter of the superficial cerebellar folia, sparing the overlying cortex. The patients were aged between 28 and 34 gestational weeks, and had a clinical history ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00315013
更新日期：1995-01-01 00:00:00
abstract：:Eleven atypical teratoid/rhabdoid tumors (AT/RT) and 121 primitive neuroectodermal tumors/medulloblastomas (PNET/MB) were included in this study for evaluation of the histopathological features of AT/RT and comparison between AT/RT and PNET/MB. Histopathological studies of AT/RT showed that in addition to the commonly...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010051149
更新日期：2000-05-01 00:00:00
abstract：:Intermitochondrial septate structures were found in the dystrophic axons of two cases of infantile neuroaxonal dystrophy. Septate structures were previously seen in some tumors (glioblastomas and Schwannomas) and several organs of vertebrates and invertebrates, but never in human central nervous system (CNS). The stru...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00688007
更新日期：1980-01-01 00:00:00
abstract：:A morphometric study of the basal nucleus of Meynert (bnM) has been performed in a 70-year-old man with a 4-year history of pathologically confirmed progressive supranuclear palsy (PSP). An important neuronal loss (52%) was demonstrated in the bnM. This finding has not been previously documented with morphometric meth...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00697397
更新日期：1983-01-01 00:00:00
abstract：:Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrP(Sc), and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenot...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-009-0585-1
更新日期：2009-11-01 00:00:00
abstract：:This report describes three brothers belonging to a consanguineous family suffering from a progressive neurological disorder associated with L-2-hydroxyglutaric aciduria. Clinically this disorder is characterized by childhood onset, pyramidal signs, cerebellar and pseudobulbar syndromes and epilepsy. Pathological exam...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00310381
更新日期：1994-01-01 00:00:00
abstract：:The evolvement of amyloid beta (Abeta) deposition in the frontal cerebral cortex of 24 patients of increasing age with Dutch-type hereditary cerebral hemorrhage with amyloidosis (HCHWA-D) was studied using end-specific monoclonal antibodies to Abetax-42 (Abeta42) or Abetax-40 (Abeta40) and markers for degenerating neu...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010051143
更新日期：2000-04-01 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：:The long-term survival of oligodendrocytes in the absence of axons in adult animals was studied following Wallerian degeneration of the optic nerves of adult rats for periods up to 22 months. In contrast to the findings in development and in young animals, large numbers of oligodendrocytes survived during this time pe...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00308922
更新日期：1990-01-01 00:00:00
abstract：:An in vivo method for positively staining dead neurons was developed and compared with an in vitro staining method using acid fuchsin. Neurons previously killed by intracerebral injections of kainic acid were selectively stained by trypan blue within 15 min of its injection in vivo into the central nervous system of r...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687254
更新日期：1989-01-01 00:00:00
abstract：:Mouse embryos were infected with murine cytomegalovirus (MCMV) by injecting the virus into the cerebral ventricles at the late gestation. After deliveries, offspring were fed by the mothers until 4 weeks. Cystic brain lesions, regarded as porencephaly or paraventricular cysts, were observed in about 20% of the MCMV-in...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00293377
更新日期：1991-01-01 00:00:00
abstract：:Histomorphological and histochemical variability was studied in muscle specimens from 30 patients with congenital muscular dystrophy (CMD). We found involvement of the central nervous system in 8 patients (Fukuyama CMD, F-CMD), involvement of the brain and the eyes in 5 patients (muscle, eye and brain disease, MEB-D) ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00369452
更新日期：1993-01-01 00:00:00
