Both data of imaging and clinical studies showed evidence that the length and the repetition of major depressive, View largeDownload slide Focal transmantle dysplasia: a specific malformation of cortical development. It is thought that a genetic abnormality in early-progenitor cells forms funnel-shaped lesions of FCD . To the best of our, knowledge, this is the first attempt to identify the etiology of the T1, high signal of the TMS by investigating the imaging and pathologic, findings. The TMS is the main radiologic landmark of FCD type II, but, it is more frequently detected in patients with type IIb than type, IIa and is the only MR imaging feature that can be used to accu-, the TMS is thought to be some combination of gliosis, hypomy-, elination or dysmyelination, neuronal heterotopia, and balloon, though an exact correlation has yet to be verified. In three cases, the MRI diagnosis was other than FCD. Sometimes the hyperintensity is seen extending from the subcortical area to the margin of the ventricle. Wang et al. For transmantle sign this ranged from 19 % to 81 % , and in our series it is 67 %. We selected 25 patients with focal cortical dysplasia with the transmantle sign and divided them into. There was a significant association between the presence of cortical thickening (p = 0.002) and the "transmantle sign" (p < 0.001) and a correct MRI diagnosis of FCD II. There is associated compression atelectasis for the largest empyema(red arrow). The cortical tubers also show MR imaging, findings, known as the radially oriented white matter band, that, fer contrast is a technique for improving image contrast in MR, imaging, based on the difference in magnetic field–induced fre-, quencies between mobile free water protons and macromolecular, cells and calcium deposition may also play a role by causing short-, ening of the water T1, leading to a decrease in the effectiveness of. Nine of the 25 patients had T1-high-signal, significant differences in the age at sei-, and C, though areas of slightly high den-, patients, their densities were lower than, in group A, but in 3 of the 4 patients, the microcalcification was, imperceptible. However, the kindling phenomenon only reflects the concept of vulnerability but omits explaining its mechanisms. The rates of abnormal MRI results and correct MRI diagnoses of FCD II were significantly higher in the IIb subgroup. ), National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan. ), Tokyo Women's Medical University Medical Center East, Tokyo, Japan. ), National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan. Sasaki M; Neurosurgery (N.I., Yuiko K., K.I., Y.T., M.I. This explains the relative high frequency of the imaging features in our series. All 9 patients with a, Approximately 6% (9/141) of this patient series had a T1-high-signal transmantle sign, and all were type IIb. All these characteristics may assist in their earlier diagnosis and improve the predictability of surgical management. vulnerability to stress. Patients who underwent complete resection of MRI and ECoG abnormalities (12 of 13 patients) became seizure free. Pathological subtype IIb was predominantly in frontal lobe and subtype IIa was predominantly seen in temporal. ), Tokyo Women’s Medical University, Medical Center East, Tokyo, Japan; and Department of Neurosurgery (N.I. Dấu hiệu xuyên vỏ (transmantle sign) Đôi khi sự tăng tín hiệu được nhìn thấy kéo dài từ khu vực dưới vỏ não đến rìa của não thất. The primary MRI findings associated with transmantle sign included gray-white junction blurring, appearance of cortical thickening, T2 or FLAIR abnormality, and bottom-of-the-sulcus dysplasia. Jun 7, 2018 - Transmantle sign is only rarely seen in Type I focal cortical dysplasia, and usually implies a Taylor type (Type II) malformation. Correlation of the transmantle sign with FCD histopathological subtypes was highly variable. However, most intriguing was our finding of a microcolumnar arrangement of cortical neurons in layer III. Etiology: disorder of cortical formation MRI: cortical thickening, blurring of grey matter-white matter junction with abnormal architecture of subcortical layer, T2 hyperintense white matter with or without transmantle sign, T2 hyperintense grey matter, abnormal sulcal or … We retrospectively recruited epilepsy patients who had undergone surgical treatment for refractory epilepsy with focal MRI abnormalities and the pathological diagnosis of FCD. Towards in vivo focal cortical dysplasia phenotyping using quantitative MRI, Thin isotropic FLAIR MR images at 1.5T increase the yield of focal cortical dysplasia transmantle sign detection in frontal lobe epilepsy, Utility of Double Inversion Recovery MRI in Pediatric Epilepsy, Clinical characteristics, pathological features and surgical outcomes of focal cortical dysplasia (FCD) type II: Correlation with pathological subtypes, Transmantle sign in focal cortical dysplasia: A unique radiological entity with excellent prognosis for seizure control ; Clinical article, Utility of Magnetization Transfer T1 Imaging in Children with Seizures, Basic Mechanisms of Epileptogenesis in Pediatric Cortical Dysplasia, Imaging Spectrum of Cortical Dysplasia in Children, Comparison of MRI features and surgical outcome among the subtypes of focal cortical dysplasia, Focal cortical dysplasia type IIa and IIb: MRI aspects in 118 cases proven by histopathology, Dépressions récidivantes : neurotoxicité des épisodes et prévention des récurrences, Loss of endophilin-B1 exacerbates Alzheimer's disease pathology. All 9 patients with a T1-high-signal transmantle sign were diagnosed as type IIb (group A). Morimoto E; Department of Pediatrics (Y.O. The transmantle sign was more clearly identified at 3T than 1.5T (mean visualization score: 1.72 vs. 0.56; p = 0.002). ... Radiology 1997;203(2):553–559. Despite characteristic radiographic features, focal cortical dysplasia can be subtle on magnetic resonance imaging. The transmantle sign was usually a focal finding, typically confined to 1 or several gyri with well-circumscribed epileptic tissue. In a subset of FCDs, a transmantle sign is observed on imaging that focally spans the entire cerebral mantle from the ventricle to the cortical surface. Focal cortical dysplasia (FCD) type II is a major cause of drug-resistant epilepsy. Results: Results: The transmantle sign was usually a focal finding, typically confined to 1 or several gyri with well-circumscribed epileptic tissue. By introducing 3D thin-slice isotropic FLAIR, false-negative reports can be reduced without reference for higher MR field structural scanning or other modalities, and more FLE patients can benefit from epilepsy surgery candidacy. spect to epileptic seizures following epilepsy surgery. However, in the present study, there were few cases with sparse calcifications, in the pathologic findings, and the association between high den-, sity on CT and calcifications was weak. ), National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan. Second, the internal structure of the balloon cell has not been, clarified. Patients who underwent complete resection of MRI and ECoG abnormalities (12 of 13 patients) became seizure free. Detecting focal abnormalities in MRI examinations of children with epilepsy can be a challenging task given the frequently subtle appearance of cortical dysplasia, mesial temporal sclerosis and similar lesions. Outcomes after surgery for FCD are highly variable, and prognosticators of seizure freedom are unclear. However, presurgical identification of MRI abnormalities in FCD patients remains difficult, and there are no highly sensitive imaging parameters available that can reliably differentiate among FCD subtypes. sification system and diagnostic implications for MRI. Focal cortical dysplasia (FCD) is the most common pathological diagnosis in patients who have undergone surgical treatment for intractable neocortical epilepsy. Images of a 27-year-old male with refractory occipital lobe epilepsy. II: correlation with pathological subtypes. All statistical analyses were per-, The details of the clinical demographics and radiologic and, pathologic findings of the 25 patients with FCD with the TMS are, FLAIR images obtained at 13 months of age demonstrate focal hyperintensity in the white matter, of the right frontal lobe, representing the TMS (, summarized in the On-line Table. and IIb: MRI aspects in 118 cases proven by histopathology. First, the sample size of T1-, high-signal FCD cases was small (9 patients). , Photomicrograph shows many balloon cells, ). Radiology description. Transmantle sign in focal cortical dysplasia: a unique radiological entity with excellent prognosis for seizure control. Radiology: Volume 274: Number 2—February 2015 n radiology.rsna.org 501 Published online before print 10.1148/radiol.14140773 Content codes: Radiology 2015; 274:500–507 Abbreviations: FCD = focal cortical dysplasia FCD2 = type 2 FCD PBS = power button sign 3D = three-dimensional Author contributions: Guarantors of integrity of entire study, C.M., F.C., S.L., J.F.M., C.O. Access scientific knowledge from anywhere. In addition other conditions such as vascular malformations, certain phakomatoses, encephaloceles, or infections can be present. To explain this spontaneous aggravation, we have been mainly focusing on the Kindling hypothesis. When compared with 114 FCD patients without the transmantle sign, patients with the transmantle sign showed significantly improved seizure-free outcomes after complete resections (p = 0.04). Although the transmantle sign detection yield is high by routine imaging protocols for epilepsy at 3T, most centers around the world have access to 1.5T MR technology and FLE patients often receive negative imaging reports. Quantitative MRI offers the possibility to probe tissue biophysical properties in vivo and may bridge the gap between radiological assessment and ex-vivo histology. The mean transmantle sign thickness by thick images was 12.3mm, by thin images was 8.9mm, and in the patients undetected by thick FLAIR was 3.5mm. There were no balloon cells in group C (data not shown). Received February 3, 2019; accepted after revision March 27. Our study showed that patients with FCD III have poor surgical outcome. This study investigates the optimization of transmantle detection yield at 1.5T by introducing a 3D thin-slice isotropic FLAIR sequence in the epilepsy imaging protocol. Đây được gọi là dấu hiệu xuyên vỏ (transmantle sign). Transmantle sign. inspection of neurosurgically resected specimens revealed dysplastic neurons with/without balloon cells in only 7 patients. The. Images of a 27-year-old male with refractory occipital lobe epilepsy. Conclusions: The 2D thick-slice FLAIR detected a transmantle sign in seven (35.0%) patients. The purpose of our study was to investigate the surgical outcome in FCD patients with identifiable MRI abnormalities and to evaluate the prognostic role of the various MRI features and the characteristics of FCD pathology. Developmental venous anomaly (DVA), also known as cerebral venous angioma, is a congenital malformation of veins which drain normal brain.They were thought to be rare before cross-sectional imaging but are now recognized as being the most common cerebral vascular malformation, accounting for ~55% of all such lesions.. A DVA is characterized by the caput medusae sign of veins draining … Future research linking quantitative MRI to FCD histological properties should improve clinical protocols, allow better characterisation of lesions in vivo and tailored surgical planning to the individual. However, in some cases, it shows T1 high signal. The transmantle sign was usually a focal finding, typically confined to 1 or several gyri with well-circumscribed epileptic tissue. Sato N; Neurosurgery (N.I., Yuiko K., K.I., Y.T., M.I. We, therefore speculate the following: 1) The density of the balloon cells, may be associated with the T1 high signal, and 2) our findings could. Although histological abnormalities occurring during postnatal maturation of the brain challenge any neuropathological classification in this group of young patients, we propose that these findings are classified according to FCD type I. Advances in neuroimaging have proven effective in early identification of the more severe lesions and timely surgical removal to treat epilepsy. No significant difference in the cell numbers is shown by both stains. In order to gain insight into the possible correlations between FCD II pathological pattern and different clinical characteristics (including clinical information, imaging characteristics and surgical outcomes), different clinicopathological characteristics in two types of FCD II were analyzed (especially in FCD IIb). Ikegaya N; From the Department of Radiology (Yukio K., Y. Shigemoto, E.M., F.S., N.S. of Science (KAKENHI grant No. This is called the transmantle sign. The multicenter study of epilepsy surgery: recruitment, Cortical tubers without other stigmata of tuber-, Magnetization transfer contrast (MTC) and. We report about a cohort of 25 pediatric patients (mean age 8.1+/-4.8 years) with severe drug-resistant early onset focal epilepsies (mean duration 2.1+/-0.4 years), mental/psychomotor retardation, and multilobar epileptogenesis. Shigemoto Y; Child Neurology (Y.O., M.S.). only 1 study has reported such atypical signal intensity, no study has examined the etiology of these signal abnormali-, ties. In regard to this field of application, specific requirements apply. This transmantle sign is almost exclusively found in FCD type II. Patients who underwent complete resection of MRI and ECoG abnormalities (12 of 13 patients) became seizure free. This review will cover histological, genetic and radiological features of FCD following the ILAE classification and will explain how quantitative voxel- and surface-based techniques can characterise these features. Cortical dysplasia (CD) is a neurodevelopmental disorder due to aberrant cell proliferation and differentiation. We divided the patients into, 3 groups based on their pathologic FCD subtype and the T1, signal of the TMS. The transmantle sign was significantly more frequent in the IIb subgroup (p = 0.003). This could be because neuronal circuits display morphological and functional signs of dysmaturity. These microcolumns can be statistically defined as vertical lining of more than eight neurons (two times standard deviation of cell countings obtained from controls). ), National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan. For transmantle sign this ranged from 19 % to 81 % , and in our series it is 67 %. Correlation of the transmantle sign with FCD histopathological subtypes was highly variable. We failed to find a prognostic value of specific MRI abnormalities of prognostic value in terms of post-epilepsy surgery outcome in FCD patients. Twenty FLE patients underwent diagnostic imaging for epilepsy with typical 2D thick-slice (3.0mm) coronal FLAIR sequences and a 3D thin-slice (1.0mm) isotropic FLAIR sequences at 1.5T, and transmantle sign detection yields and thickness measurements were derived. of the International League Against Epilepsy (ILAE). 30.6C, D). Most commonly encountered pathologies include mesial temporal lobe sclerosis (that can be uni- or bilateral and may occur as a “dual” pathology in conjunction with other epileptogenic lesions), malformations of cortical development (including disorders of neuronal proliferation, migration or organization) and epileptogenic tumors (such as gangliogliomas, dysembryoblastic neuroepithelial tumors). One of the most robust signs of FCD2 is the transmantle sign, which spreads along the axis of the abnormal sulcus and runs perpendicular to the wall of the lateral ventricle along the path of migrating neuroblasts. that depressive episodes could be neurotoxic per se. cortical dysplasia: a unique radiological entity with excellent prog-, outcomes of patients with refractory magnetic resonance imaging-, of MR sequences to detect structural brain lesions in tuberous scle-. mild cortical dyslamination to more severe forms. Prev : 1; 2; 3; Continue > Next Case > Case courtesy of Assoc Prof Craig Hacking rID: 39056. mechanisms in resected cortical dysplasia. The T1 high signal in the TMS may thus be regarded as, reported patients with FCD who had shown T1, hyperintensity during the first year of their lives, but it was diffi-, cult to identify them by the process of myelination. According to the current ILAE classification system, thology of FCD is classified into 3 types: Type I has isolated cor-, tical dyslamination, type II has dysmorphic neurons with or with-, out balloon cells in addition to cortical dyslamination, and type. hama City University, Yokohama, Kangawa, Japan. Methods: © 2015 The Authors. Objective: Sometimes the hyperintensity is seen extending from the subcortical area to the margin of the ventricle. However, the exact mechanisms of epileptogenesis are not well understood. Thirteen patients underwent resective surgery and 1 underwent multiple subpial transections with vagus nerve stimulator placement. In addition, there was no T1 high signal in the other types, of FCD. This review examines possible mechanisms based on anatomical and electrophysiological studies. Neurology (A.S.), Mito Kyodo General Hospital, University of Tsukuba, Tsukuba, Ibaraki, Japan; Department of Pediatrics (Y.O. This work was funded by a grant from the Japan Society for the Promotion of. Patients with FCD type III had a lower chance for achieving seizure freedom (7/15) than in patients with isolated FCD (FCD types I and II) (40/54, p=0.044). Đây được gọi là dấu hiệu xuyên vỏ (transmantle sign). In contrast, drugs that counteract depolarizing actions of GABA or drugs that inhibit the mammalian target of rapamycin (mTOR) pathway could be more effective. Cortical thickness and blurring of gray-white matter junction were more common in isolated FCD than in FCD type III, but most MRI features failed to differentiate between FCD types I and II, and only the transmantle sign was specific for FCD type II. vulnerability to stress. J Neurosurg. Published by the British Institute of Radiology. Our observations support a concept compatible with regional loss of high-order brain organization. ), Na-, tional Center Hospital, National Center of Neurology and Psychiatry, Kodaira, To-. This neurotoxicity hypothesis of major depressive disorder, apart from allowing a different way of communicating with our patients and to facilitate their compliance, strengthen the necessity to prevent recurrent depressive episodes. We evaluated the clinical, radiologic, and pathologic findings, including the number of balloon cells and dysmorphic neurons and the severity of gliosis or calcifications and, T1-high-signal transmantle sign were diagnosed as type IIb (group A). Among MRI abnormalities, the transmantle sign is a very useful evidence, frequently related with FCD type IIb. An award-winning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, GI, cardiac and musculoskeletal diseases containing hundreds of lectures, quizzes, hand-out notes, interactive material, most commons lists and pictorial differential diagnoses Funnel-shaped tapering of the subcortical signal abnormality toward the ventricle (transmantle sign) is more commonly associated with FCD type IIb (balloon cell subtype) . All enrolled pa-, tients were diagnosed on the basis of the, histologic classification system proposed, identified by the following definition: “a, subcortical zone tapering toward the lat-, eral ventricle was the characteristic finding, institutional review board at the National, ing sex, age at operation, age at the onset, For statistical analysis, ILAE outcomes 1. 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Series it is thought that a genetic abnormality in early-progenitor cells forms funnel-shaped lesions of FCD in infants gliosis! Indicated a high likelihood of a 27-year-old male with refractory occipital lobe epilepsy to help work! Of lesions but is unable to accurately predict the histopathological features neuronal glial! Despite characteristic radiographic features, focal cortical dysplasia is commonly recognized in pediatric CD or arteriovenous malformations, and of..., aging signal intensity, no study has reported such atypical signal intensity of the (... Unique radiological entity with excellent prognosis for seizure control outcomes and prognostic significance of more.