Polyneuropathy with minifascicle formation in a patient with 46XY mixed gonadal dysgenesis.

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 Neuropathol

journal_title

Acta neuropathologica

authors

Umehara F,Yamaguchi N,Kodama D,Takenaga S,Kiwaki T,Sonoda Y,Arimura Y,Yamada H,Arimura K,Osame M

doi

10.1007/s004010051085

subject

Has Abstract

pub_date

1999-09-01 00:00:00

pages

309-12

issue

3

eissn

0001-6322

issn

1432-0533

journal_volume

98

pub_type

杂志文章
  • Decreased estrogen receptor-alpha expression in hippocampal neurons in relation to hyperphosphorylated tau in Alzheimer patients.

    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

    authors: Hu XY,Qin S,Lu YP,Ravid R,Swaab DF,Zhou JN

    更新日期:2003-09-01 00:00:00

  • Spontaneous human herpes virus type 1 infection in a chinchilla (Chinchilla lanigera f. dom.).

    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

    authors: Wohlsein P,Thiele A,Fehr M,Haas L,Henneicke K,Petzold DR,Baumgärtner W

    更新日期:2002-12-01 00:00:00

  • Ganglioneuromas in hamsters.

    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

    authors: Rustia M,Cardesa A

    更新日期:1975-10-01 00:00:00

  • Photodynamic studies reveal rapid formation and appreciable turnover of tau inclusions.

    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

    authors: Croft CL,Goodwin MS,Ryu DH,Lessard CB,Tejeda G,Marrero M,Vause AR,Paterno G,Cruz PE,Lewis J,Giasson BI,Golde TE

    更新日期:2021-01-26 00:00:00

  • Familial amyotrophic lateral sclerosis with a mutation in the Cu/Zn superoxide dismutase gene.

    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

    authors: Takahashi H,Makifuchi T,Nakano R,Sato S,Inuzuka T,Sakimura K,Mishina M,Honma Y,Tsuji S,Ikuta F

    更新日期:1994-01-01 00:00:00

  • Spinal cord-notochord relationship in normal human embryos and in a human embryo with double spinal cord.

    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

    authors: Saraga-Babić M,Stefanović V,Wartiovaara J,Lehtonen E

    更新日期:1993-01-01 00:00:00

  • Intermitochondrial septate structures in dystrophic axons.

    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

    authors: Ramos PL,Wisniewski K,Jervis GA,Wisniewski HM

    更新日期:1980-01-01 00:00:00

  • Central neurocytoma--a rare benign intraventricular tumor.

    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

    authors: Townsend JJ,Seaman JP

    更新日期:1986-01-01 00:00:00

  • Immunohistochemical study of constituents other than beta-protein in canine senile plaques and cerebral amyloid angiopathy.

    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

    authors: Uchida K,Kuroki K,Yoshino T,Yamaguchi R,Tateyama S

    更新日期:1997-03-01 00:00:00

  • The lateral corticospinal tract and spinal ventral horn in X-linked recessive spinal and bulbar muscular atrophy: a quantitative study.

    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

    authors: Terao S,Sobue G,Li M,Hashizume Y,Tanaka F,Mitsuma T

    更新日期:1997-01-01 00:00:00

  • Neuropathological signs of inflammation correlate with mitochondrial DNA deletions in mesial temporal lobe epilepsy.

    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

    authors: Volmering E,Niehusmann P,Peeva V,Grote A,Zsurka G,Altmüller J,Nürnberg P,Becker AJ,Schoch S,Elger CE,Kunz WS

    更新日期:2016-08-01 00:00:00

  • Pathogenesis of proximal autosomal recessive spinal muscular atrophy.

    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

    authors: Simic G

    更新日期:2008-09-01 00:00:00

  • Progressive accumulation of ubiquitin and disappearance of alpha-synuclein epitope in multiple system atrophy-associated glial cytoplasmic inclusions: triple fluorescence study combined with Gallyas-Braak method.

    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

    authors: Sakamoto M,Uchihara T,Nakamura A,Mizutani T,Mizusawa H

    更新日期:2005-10-01 00:00:00

  • Loss of neurons in the frontal cortex in AIDS brains.

    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

    authors: Ketzler S,Weis S,Haug H,Budka H

    更新日期:1990-01-01 00:00:00

  • "Pink spots" in formalin-fixed brains--poor fixation or bacterial byproduct?

    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

    authors: Hedley-Whyte ET

    更新日期:1985-01-01 00:00:00

  • Central neuropathology of equine grass sickness.

    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

    authors: Hahn CN,Mayhew IG,de Lahunta A

    更新日期:2001-08-01 00:00:00

  • Upregulated expression of Fas and Fas ligand in brain through the spectrum of HIV-1 infection.

    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

    authors: Elovaara I,Sabri F,Gray F,Alafuzoff I,Chiodi F

    更新日期:1999-10-01 00:00:00

  • Peripheral and central distal axonopathy of suspected inherited origin in Birman cats.

    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

    authors: Moreau PM,Vallat JM,Hugon J,Leboutet MJ,Vandevelde M

    更新日期:1991-01-01 00:00:00

  • Curly fibers are tau-positive strands in the pre- and post-synaptic neurites, consisting of paired helical filaments: observations by the freeze-etch and replica method.

    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

    authors: Ohtsubo K,Izumiyama N,Kuzuhara S,Mori H,Shimada H

    更新日期:1990-01-01 00:00:00

  • Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP).

    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

    authors: Rizzuto N,Morbin M,Cavallaro T,Ferrari S,Fallahi M,Galiazzo Rizzuto S

    更新日期:1998-12-01 00:00:00

  • Differentially expressed genes in neurofibromatosis 1-associated neurofibromas and malignant peripheral nerve sheath tumors.

    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

    authors: Holtkamp N,Mautner VF,Friedrich RE,Harder A,Hartmann C,Theallier-Janko A,Hoffmann KT,von Deimling A

    更新日期:2004-02-01 00:00:00

  • Neurofibrillary accumulation in the zebra (Equus burchelli).

    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

    authors: Higgins RJ,Vandevelde M,Hoff EJ,Jagar JE,Cork LC,Silberman MS

    更新日期:1977-01-31 00:00:00

  • The nature and timing of excitotoxic neuronal necrosis in the cerebral cortex, hippocampus and thalamus due to flurothyl-induced status epilepticus.

    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

    authors: Ingvar M,Morgan PF,Auer RN

    更新日期:1988-01-01 00:00:00

  • Expression of the c-erbB-2-encoded oncoprotein and progesterone receptor in human meningiomas.

    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

    authors: Schlegel J,Ullrich B,Stumm G,Gass P,Harwerth IM,Hynes NE,Kiessling M

    更新日期:1993-01-01 00:00:00

  • Oligodendrocyte survival in Wallerian degeneration.

    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

    authors: Ludwin SK

    更新日期:1990-01-01 00:00:00

  • Differential expression of intercellular adhesion molecule-1 (ICAM-1) in the A beta-containing lesions in brains of patients with dementia of the Alzheimer type.

    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

    authors: Verbeek MM,Otte-Höller I,Wesseling P,Ruiter DJ,de Waal RM

    更新日期:1996-01-01 00:00:00

  • The function of the cellular prion protein in health and disease.

    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

    authors: Watts JC,Bourkas MEC,Arshad H

    更新日期:2018-02-01 00:00:00

  • Formation of oedema in transected rat sciatic nerve. A microgravimetric study.

    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

    authors: Pettersson CA,Tengvar C

    更新日期:1986-01-01 00:00:00

  • Histological changes of neuronal damage in vegetative dogs induced by 18 minutes of complete global brain ischemia: two-phase damage of Purkinje cells and hippocampal CA1 pyramidal cells.

    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

    authors: Sato M,Hashimoto H,Kosaka F

    更新日期:1990-01-01 00:00:00

  • A fine structural study of degenerative-regenerative pathology in the surgically deafferentated lateral vestibular nucleus of the rat.

    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

    authors: Johnson JE Jr

    更新日期:1975-12-19 00:00:00