Abstract:
:Focal cortical dysplasias (FCDs) are increasingly recognized as one of the most common causes of pharmaco-resistant epilepsies. FCDs were recently divided into various clinico-pathological subtypes due to distinct imaging, electrophysiological, and outcome characteristics. In this review, we will overview the international consensus classification of FCDs in light of more recently reported clinical, electrical, imaging and functional observations, and will also address areas of ongoing debate. In addition, we will summarize our current knowledge on pathobiology and epileptogenicity of FCDs as well as its underlying molecular and cellular mechanisms. The clinical (electroencephalographic, imaging, and functional) characteristics of major FCD subtypes and their implications on the presurgical evaluation and surgical management will be discussed in light of studies describing these characteristics and postoperative seizure outcomes in patients with medically intractable focal epilepsy due to histopathologically confirmed FCDs.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Najm IM,Tassi L,Sarnat HB,Holthausen H,Russo GLdoi
10.1007/s00401-014-1304-0subject
Has Abstractpub_date
2014-07-01 00:00:00pages
5-19issue
1eissn
0001-6322issn
1432-0533journal_volume
128pub_type
杂志文章,评审abstract::In spite of several cases reported in the literature, the exact pathogenetic mechanism of neuropathic changes in porphyric neuropathy remains uncertain. Various authors have ascribed the neuropathologic findings to either a dying-back axonal degeneration or segmental demyelination. In recent years, the hypothesis of a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00697191
更新日期:1984-01-01 00:00:00
abstract::To identify antigenic differences between gliomas and normal brain, we have immunohistochemically studied the expression of lymphocyte adhesion molecules (ICAM-1, ICAM-2, ICAM-3, VCAM-1, E-selectin and CD58), epidermal growth factor receptor (EGFR) and extracellular matrix proteins (collagen IV, fibronectin, laminin, ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050696
更新日期:1997-09-01 00:00:00
abstract::N-Methyl-D-aspartate (NMDA) antagonists cause neuronal vacuolation in the posterior cingulate and retrosplenial cortex of the rat. Because the nature of neuronal pathologic changes due to NMDA antagonists may affect the potential clinical use of this class of drugs, we undertook experiments to define the nature and ti...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00386248
更新日期:1994-01-01 00:00:00
abstract::Healthy nodes of Ranvier are crucial for action potential propagation along myelinated axons, both in the central and in the peripheral nervous system. Surprisingly, the node of Ranvier has often been neglected when describing CNS disorders, with most pathologies classified simply as being due to neuronal defects in t...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-014-1305-z
更新日期:2014-08-01 00:00:00
abstract::Surgical specimens of 4 intracranial saccular aneurysms were studied by scanning electron microscopy. The internal surface of the aneurysms showed crater-like defects and cytoplasmic bridges. In some areas the endothelium was preserved, but its longitudinal convolutions were higher and thicker than those found in unch...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691478
更新日期:1978-11-15 00:00:00
abstract::Neurotrophins regulate the proliferation and differentiation of neurons in the central nervous system via a family of specialized receptors, including TrkA, TrkB, and TrkC. As little is known about their expression or potential role in human glial tissues and glial tumors, we undertook an immunohistochemical analysis ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050906
更新日期:1998-10-01 00:00:00
abstract::A human glioma cell line, SA146, was initiated on precoated extracellular matrix from a stereotactic biopsy of a glioblastoma. We report modulation in the expression of glial fibrillary acidic protein (GFAP) by SA146 passed in vitro before or after xenogenic transplantation into nude mice. Immunofluorescence data show...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050722
更新日期:1997-10-01 00:00:00
abstract::Marinesco-Sjögren syndrome (MSS) features cerebellar ataxia, mental retardation, cataracts, and progressive vacuolar myopathy with peculiar myonuclear alterations. Most MSS patients carry homozygous or compound heterozygous SIL1 mutations. SIL1 is a nucleotide exchange factor for the endoplasmic reticulum resident cha...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1224-4
更新日期:2014-05-01 00:00:00
abstract::The morphological effects of two chemically different neuroactive drugs (chlorpromazine and phenobarbital) on vasculogenesis in rat cerebellum were examined to determine the presence of vascular alterations. Therapeutic dosages of both drugs were chronically administered to separate groups of maternal rats beginning o...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692188
更新日期:1982-01-01 00:00:00
abstract::Three cases of spontaneous olfactory neuroblastoma (ONB) in domestic cats were morphologically and immunocytochemically characterized. Diagnostic light microscopic features included Flexner and Homer-Wright rosettes, while ultrastructurally the cells had neuritic processes, intracellular intermediate filaments, and in...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294617
更新日期:1990-01-01 00:00:00
abstract::VM mice were inoculated by intracerebral and intraperitoneal routes with brain homogenates containing the 87V strain of scrapie. The distribution and numbers of plaques were found for the parietal cortex, cingulate cortex, corpus callosum and hippocampus/dentate in coronal sections cut at the level of the thalamus and...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688311
更新日期:1986-01-01 00:00:00
abstract::Progressive supranuclear palsy (PSP) is a 4R-tauopathy predominated by subcortical pathology in neurons, astrocytes, and oligodendroglia associated with various clinical phenotypes. In the present international study, we addressed the question of whether or not sequential distribution patterns can be recognized for PS...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02158-2
更新日期:2020-08-01 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) is a lethal disease characterized by motor neuron degeneration and associated with aggregation of nuclear RNA-binding proteins (RBPs), including FUS. How FUS aggregation and neurodegeneration are prevented in healthy motor neurons remain critically unanswered questions. Here, we use...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-019-01998-x
更新日期:2019-07-01 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::An immunoperoxidase technique was used to locate synaptophysin (protein p38), a major integral membrane glycoprotein of synaptic vesicles, in the rat brain. In addition to a diffuse distribution of nerve terminal stainings for synaptophysin appearing as numerous small puncta, the large-sized cells with spindled or pol...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00304146
更新日期:1993-01-01 00:00:00
abstract::The suprascapular nerve from 14 horses, which had no clinical evidence of spinatus muscle atrophy, were obtained to determine whether the nerve was sub-clinically compressed at the scapular edge. The nerves were divided into three portions, proximal and distal to the scapular edge and as it reflected around it. In nin...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688338
更新日期:1987-01-01 00:00:00
abstract::Pediatric ependymomas are highly recurrent tumors resistant to conventional chemotherapy. Telomerase, a ribonucleoprotein critical in permitting limitless replication, has been found to be critically important for the maintenance of tumor-initiating cells (TICs). These TICs are chemoresistant, repopulate the tumor fro...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-014-1327-6
更新日期:2014-12-01 00:00:00
abstract::With an incidence of approximately 350 in 100,000, stroke is the third leading cause of death and a major cause of disability in industrialized countries. At present, although progress has been made in understanding the molecular pathways that lead to ischemic cell death, the current clinical treatments remain poorly ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-009-0496-1
更新日期:2009-05-01 00:00:00
abstract::The relationship between diabetes and the size, density and area of the ventromedial hypothalamic nucleus (VMH) was studied in the genetically diabetic Chinese hamster. Matched diabetic and non-diabetic control chinese hamsters were perfused, the hypothalamus collected, sectioned and stained for light microscopy. The ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691183
更新日期:1982-01-01 00:00:00
abstract::Abnormal TDP-43 aggregation is a prominent feature in the neuropathology of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. Mutations in TARDBP, the gene encoding TDP-43, cause some cases of ALS. The normal function of TDP-43 remains incompletely understood. To better understand TDP-43 biolo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0659-0
更新日期:2010-04-01 00:00:00
abstract::Missense mutations of the V600E type constitute the vast majority of tumor-associated somatic alterations in the v-RAF murine sarcoma viral oncogene homolog B1 (BRAF) gene. Initially described in melanoma, colon and papillary thyroid carcinoma, these alterations have also been observed in primary nervous system tumors...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-011-0802-6
更新日期:2011-03-01 00:00:00
abstract::This report illustrates a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) masquerading as neurofibromatosis due to multifocal enlargements of spinal nerve roots. The patient initially complained of intermittent numbness of the hands and leg weakness at age 62. Nerve conduction velocities were ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0616-7
更新日期:2003-02-01 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::Recent studies of genetic abnormalities in pediatric low-grade gliomas (LGGs) have focused on activation of the ERK/MAPK pathway by KIAA1549-BRAF gene fusions in the majority of pilocytic astrocytomas (PAs) and by rare mutations in elements of the pathway across histopathologically diverse LGGs. This study reports tha...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0763-1
更新日期:2010-12-01 00:00:00
abstract::A monoclonal antibody, raised against extracts from Alzheimer brain, that recognizes a phosphorylated epitope in high molecular weight neurofilament proteins and tau proteins also immunostains Alzheimer neurofibrillary tangles, neurites in senile plaques and granulovacuolar degeneration. This result suggest that granu...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686619
更新日期:1987-01-01 00:00:00
abstract::The differential diagnosis of Creutzfeldt-Jakob disease (CJD) from other, sometimes treatable, neurological disorders is challenging, owing to the wide phenotypic heterogeneity of the disease. Real-time quaking-induced prion conversion (RT-QuIC) is a novel ultrasensitive in vitro assay, which, at variance with surroga...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-017-1683-0
更新日期:2017-04-01 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::Ectopic neurons have been detected in the hippocampus of postnatal hooded rats aged 5-24 days. These rats were exposed to methylazoxymethanol acetate (MAMac) during foetal development by injecting the mother rats with this neurotoxin. At birth, the hippocampus of rats exposed to MAMac showed a normal cytoarchitecture;...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688698
更新日期:1977-10-10 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::Dysferlin is a newly identified sarcolemmal protein related to Miyoshi myopathy and limb-girdle muscular dystrophy. Although its function is still unknown, it is inferred from the presence of C2 domains and a transmembrane domain in its sequence that dysferlin may be expressed or located not only at the sarcolemma but...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0686-1
更新日期:2003-06-01 00:00:00