Abstract:
:A patient with mixed gonadal dysgenesis showed glove and stocking-type sensory impairment and slowing of motor and sensory nerve conduction. Sural nerve biopsy revealed minifascicular formation with decreased density of myelinated fibers. As far as we are aware, this is the first report of polyneuropathy with minifascicular formation in 46XY mixed gonadal dysgenesis.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Umehara F,Yamaguchi N,Kodama D,Takenaga S,Kiwaki T,Sonoda Y,Arimura Y,Yamada H,Arimura K,Osame Mdoi
10.1007/s004010051085subject
Has Abstractpub_date
1999-09-01 00:00:00pages
309-12issue
3eissn
0001-6322issn
1432-0533journal_volume
98pub_type
杂志文章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::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::A description is given of three cases of ganglioneuromas, which originated from ganglia of the sympathetic chain of hamsters. Other reports of such tumors in this species are reviewed and the literature relative to hamster ganglioneuromas presented. In addition, the possible genesis of origin from adult ganglion cells...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00696794
更新日期:1975-10-01 00:00:00
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::Several missense mutations within exons 1, 2, 4 and 5 of the gene for Cu/Zn-binding superoxide dismutase (SOD1) have been discovered to be involved in the development of chromosome 21q-linked familial amyotrophic lateral sclerosis (FALS). We describe here an autopsied patient with FALS, in whom we have recently identi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294513
更新日期:1994-01-01 00:00:00
abstract::Spinal cord-notochord relationship was analyzed histologically and immunohistochemically in normal human conceptuses between the 4-8 developmental weeks and in a 8-week embryo with double spinal cord. In the early 4-week embryo, the gradual closure of the neural tube along the cranio-caudal body axis was paralleled by...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00228587
更新日期:1993-01-01 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::Two cases of Central neurocytoma arising in the lateral ventricles are presented. Both patients had well-circumscribed masses in the right lateral ventricle causing hydrocephalus. The tumors were composed of small round cells forming Homer Wright rosettes against a fine fibrillary background. In one patient surgical r...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687981
更新日期:1986-01-01 00:00:00
abstract::To clarify the significance of the constituents of canine senile plaques (SPs) or cerebrovascular amyloid deposits, paraffin and cryostat sections of canine brains were examined by immunohistochemistry using antibodies against cathepsin B (CB), cathepsin D (CD), cystatin C (CC), alpha-1-antichymotrypsin (ACT), heat sh...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050615
更新日期:1997-03-01 00:00:00
abstract::A quantitative study was performed on spinal cord lesions in seven patients with X-linked recessive spinal and bulbar muscular atrophy. The myelinated fiber density of the lateral corticospinal tracts at the T7 cord level was well preserved for both large and small myelinated fibers. On the other hand, neurons in the ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050575
更新日期:1997-01-01 00:00:00
abstract::Accumulation of mitochondrial DNA (mtDNA) deletions has been proposed to be responsible for the presence of respiratory-deficient neurons in several CNS diseases. Deletions are thought to originate from double-strand breaks due to attack of reactive oxygen species (ROS) of putative inflammatory origin. In epileptogene...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-016-1561-1
更新日期:2016-08-01 00:00:00
abstract::Although it is known that deletions or mutations of the SMN1 gene on chromosome 5 cause decreased levels of the SMN protein in subjects with proximal autosomal recessive spinal muscular atrophy (SMA), the exact sequence of pathological events leading to selective motoneuron cell death is not fully understood yet. In t...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-008-0411-1
更新日期:2008-09-01 00:00:00
abstract::Alpha-synuclein (alphaS) and ubiquitin (Ub) are shared constituents of glial cytoplasmic inclusions (GCIs) and Lewy bodies (LBs), both composed of fibrillary structures. Staining profiles of GCIs were investigated with triple immunofluorescence involving immunostaining for alphaS and Ub, both amplified with catalyzed ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-1066-9
更新日期:2005-10-01 00:00:00
abstract::Neurons of Area 11 in the fronto-orbital cortex of 18 unselected AIDS brains are analyzed by means of stereology. Neurological abnormalities including dementing symptoms were described in eight patients. Neuropathology diagnosed human immunodeficiency virus (HIV)-specific changes in four, and diffuse poliodystrophy in...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294228
更新日期:1990-01-01 00:00:00
abstract::Circumscribed pink areas 0.3-1.0 cm in diameter seen in deeper parts of otherwise well-fixed brains are usually ascribed to inadequate fixation. Twenty-three patients with pink areas in their fixed brains had evidence of inflammation in at least one organ. Blood vessels in the pink areas contained bacteria. Postmortem...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687020
更新日期:1985-01-01 00:00:00
abstract::Equine grass sickness (EGS) is an acquired disease of unknown aetiology affecting horses kept at grass. The disease is characterised by postganglionic sympathetic and parasympathetic neuronal pathology and is categorised as a dysautonomia. This study undertook a systematic examination of brain stem cranial nerve nucle...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000289
更新日期:2001-08-01 00:00:00
abstract::Apoptosis of neurons and glial cells has been shown to occur in the brain of patients with the acquired immune deficiency syndrome (AIDS) and was postulated as contributing to brain atrophy and white matter damage in these patients. Since apoptotic events may be induced by the Fas-Fas ligand (FasL) system, we analyzed...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051094
更新日期:1999-10-01 00:00:00
abstract::Three female cats, littermates born from clinically normal parents, were examined at 8 to 10 weeks of age because of a slowly progressive posterior ataxia. Another cat from a previous litter from the same parents suffered from similar neurological symptoms. Histopathological examination of the nervous tissues of these...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293957
更新日期:1991-01-01 00:00:00
abstract::The ultrastructure of the curly fibers was examined by the transmission and immunoelectron microscopy as well as by the rapid-freeze, deep-etch and replica method. The curly fibers consisted mainly of paired helical filaments (PHF) in the neuropils, both pre- and post-synaptic. On the deep-etch replicas, PHF in the ne...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00334498
更新日期:1990-01-01 00:00:00
abstract::In a study designed to identify the neuropathological features typical of chronic inflammatory demyelinating polyneuropathy (CIDP), we reviewed the sural nerve biopsy findings in 105 patients with this disorder. The patients' mean age at biopsy was 49 years. In 65% of patients the disease had a progressive and in 35% ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050941
更新日期:1998-12-01 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::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::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::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 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::Inflammatory processes have been implicated in the formation of senile plaques in the cerebral cortex of patients with dementia of the Alzheimer type (DAT), since several inflammation-induced proteins are present within these plaques. The relation between inflammatory components and other amyloid beta protein (A beta)...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050474
更新日期:1996-01-01 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::The formation of oedema in peripheral nerves was studied in rats at intervals varying from 6 h to 14 days after transection of the right sciatic nerve. Samples were removed proximal and distal to the injury, and the degree of oedema was determined by a microgravimetric method and by measurements of the water content a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686069
更新日期:1986-01-01 00:00:00
abstract::We have developed a functional vegetative model by an 18-min clamping of the ascending aorta combined with a bypass formation between the aorta to right atrium and the aorta to femoral vein. Complete global brain ischemia (CGBI) induced for 18 min with this model provided the following distinct advantages: cardiopulmo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294614
更新日期:1990-01-01 00:00:00
abstract::An experiment was designed to examine the course of degeneration, phagocytosis, and regeneration in the central nervous system following surgical deafferentation. The anterior cerebellar vermis was ablated in young male rats. The animals were sacrificed by perfusion at postoperative times ranging from 24 hrs to 6 mont...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688396
更新日期:1975-12-19 00:00:00