Predictors of surgical outcome in focal cortical dysplasia and its subtypes

2021 ◽  
pp. 1-11
Author(s):  
Sita Jayalakshmi ◽  
Sudhindra Vooturi ◽  
Rammohan Vadapalli ◽  
Sailaja Madigubba ◽  
Manas Panigrahi

OBJECTIVE The authors analyzed predictors of surgical outcome in patients with focal cortical dysplasia (FCD) and its ILAE (International League Against Epilepsy) subtypes after noninvasive multimodal evaluation and calculated time to first seizure. METHODS Data of 355 patients with refractory epilepsy, confirmed FCD pathology, and 2–13 years of postsurgical follow-up were analyzed to determine the predictive roles of clinical, EEG, imaging, and surgical factors that influence seizure freedom. RESULTS The mean ± SD age at surgery was 20.26 ± 12.18 years. In total, 142 (40.0%) patients had daily seizures and 90 (25.3%) had multiple seizure types. MRI showed clear-cut FCD in 289 (81.4%) patients. Pathology suggested type I FCD in 27.3% of patients, type II in 28.4%, and type III in 42.8% of patients. At latest follow-up, 72.1% of patients were seizure free and 11.8% were seizure free and not receiving antiepileptic drugs. Among the subtypes, 88.8% of patients with type III, 69.3% with type II, and 50.5% with type I FCD were seizure free. Multiple seizure types, acute postoperative seizures (APOS), and type I FCD were predictors of persistent seizures, whereas type III FCD was the strongest predictor of seizure freedom. Type I FCD was associated with daily seizures, frontal and multilobar distribution, subtle findings on MRI, incomplete resection, and persistent seizures. Type II and III FCD were associated with clear-cut lesion on MRI, regional interictal and ictal EEG onset pattern, focal pattern on ictal SPECT, complete resection, and seizure freedom. Type III FCD was associated with temporal location, whereas type I and II FCD were associated with extratemporal location. Nearly 80% of patients with persistent seizures, mostly those with type I FCD, had their first seizure within 6 months postsurgery. CONCLUSIONS Long-term seizure freedom after surgery can be achieved in more than two-thirds of patients with FCD after noninvasive multimodal evaluation. Multiple seizure types, type I FCD, and APOS were predictors of persistent seizures. Seizures recurred in about 80% of patients within 6 months postsurgery.

2009 ◽  
Vol 9 (4) ◽  
pp. 100-102 ◽  
Author(s):  
Theodore H. Schwartz

Incomplete Resection of Focal Cortical Dysplasia Is the Main Predictor of Poor Postsurgical Outcome. Krsek P, Maton B, Jayakar P, Dean P, Korman B, Rey G, Dunoyer C, Pacheco-Jacome E, Morrison G, Ragheb J, Vinters HV, Resnick T, Duchowny M. Neurology 2009;72(3):217–223. BACKGROUND: Focal cortical dysplasia (FCD) is recognized as the major cause of focal intractable epilepsy in childhood. Various factors influencing postsurgical seizure outcome in pediatric patients with FCD have been reported. OBJECTIVE: To analyze different variables in relation to seizure outcome in order to identify prognostic factors for selection of pediatric patients with FCD for epilepsy surgery. METHODS: A cohort of 149 patients with histologically confirmed mild malformations of cortical development or FCD with at least 2 years of postoperative follow-up was retrospectively studied; 113 subjects had at least 5 years of postoperative follow-up. Twenty-eight clinical, EEG, MRI, neuropsychological, surgical, and histopathologic parameters were evaluated. RESULTS: The only significant predictor of surgical success was completeness of surgical resection, defined as complete removal of the structural MRI lesion (if present) and the cortical region exhibiting prominent ictal and interictal abnormalities on intracranial EEG. Unfavorable surgical outcomes are mostly caused by overlap of dysplastic and eloquent cortical regions. There were nonsignificant trends toward better outcomes in patients with normal intelligence, after hemispherectomy and with FCD type II. Other factors such as age at seizure onset, duration of epilepsy, seizure frequency, associated pathologies including hippocampal sclerosis, extent of EEG and MRI abnormalities, as well as extent and localization of resections did not influence outcome. Twenty-five percent of patients changed Engel's class of seizure outcome after the second postoperative year. CONCLUSIONS: The ability to define and fully excise the entire region of dysplastic cortex is the most powerful variable influencing outcome in pediatric patients with focal cortical dysplasia. FDG-PET/MRI Coregistration Improves Detection of Cortical Dysplasia in Patients with Epilepsy. Salamon N, Kung J, Shaw SJ, Koo J, Koh S, Wu JY, Lerner JT, Sankar R, Shields WD, Engel J Jr, Fried I, Miyata H, Yong WH, Vinters HV, Mathern GW. Neurology 2008;71(20):1594–1601. OBJECTIVE: Patients with cortical dysplasia (CD) are difficult to treat because the MRI abnormality may be undetectable. This study determined whether fluorodeoxyglucose (FDG)-PET/MRI coregistration enhanced the recognition of CD in epilepsy surgery patients. METHODS: Patients from 2004–2007 in whom FDG-PET/MRI coregistration was a component of the presurgical evaluation were compared with patients from 2000–2003 without this technique. For the 2004–2007 cohort, neuroimaging and clinical variables were compared between patients with mild Palmini type I and severe Palmini type II CD. RESULTS: Compared with the 2000–2003 cohort, from 2004–2007 more CD patients were detected, most had type I CD, and fewer cases required intracranial electrodes. From 2004–2007, 85% of type I CD cases had normal non–University of California, Los Angeles (UCLA) MRI scans. UCLA MRI identified CD in 78% of patients, and 37% of type I CD cases had normal UCLA scans. EEG and neuroimaging findings were concordant in 52% of type I CD patients, compared with 89% of type II CD patients. FDG-PET scans were positive in 71% of CD cases, and type I CD patients had less hypometabolism compared with type II CD patients. Postoperative seizure freedom occurred in 82% of patients, without differences between type I and type II CD cases. CONCLUSIONS: Incorporating fluorodeoxyglucose-PET/MRI coregistration into the multimodality presurgical evaluation enhanced the noninvasive identification and successful surgical treatment of patients with cortical dysplasia (CD), especially for the 33% of patients with nonconcordant findings and those with normal MRI scans from mild type I CD.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Chen Li ◽  
Ao-Fei Liu ◽  
Han-Cheng Qiu ◽  
Xianli Lv ◽  
Ji Zhou ◽  
...  

Abstract Background Treatment of perforator involving aneurysm (piAN) remains a challenge to open and endovascular neurosurgeons. Our aim is to demonstrate a primary outcome of endovascular therapy for piANs with the use of perforator preservation technologies (PPT) based on a new neuro-interventional classification. Methods The piANs were classified into type I: aneurysm really arises from perforating artery, type II: saccular aneurysm involves perforating arteries arising from its neck (IIa) or dome (IIb), and type III: fusiform aneurysm involves perforating artery. Stent protection technology of PPT was applied in type I and III aneurysms, and coil-basket protection technology in type II aneurysms. An immediate outcome of aneurysmal obliteration after treatment was evaluated (satisfactory obliteration: the saccular aneurysm body is densely embolized (I), leaving a gap in the neck (IIa) or dome (IIb) where the perforating artery arising; fusiform aneurysm is repaired and has a smooth inner wall), and successful perforating artery preservation was defined as keeping the good antegrade flow of those perforators on postoperative angiography. The periprocedural complication was closely monitored, and clinical and angiographic follow-ups were performed. Results Six consecutive piANs (2 ruptured and 4 unruptured; 1 type I, 2 type IIa, 2 type IIb, and 1 type III) in 6 patients (aged from 43 to 66 years; 3 males) underwent endovascular therapy between November 2017 and July 2019. The immediate angiography after treatment showed 6 aneurysms obtained satisfactory obliteration, and all of their perforating arteries were successfully preserved. During clinical follow-up of 13–50 months, no ischemic or hemorrhagic event of the brain occurred in the 6 patients, but has one who developed ischemic event in the territory of involving perforators 4 h after operation and completely resolved within 24 h. Follow-up angiography at 3 to 10M showed patency of the parent artery and perforating arteries of treated aneurysms, with no aneurysmal recurrence. Conclusions Our perforator preservation technologies on the basis of the new neuro-interventional classification seem feasible, safe, and effective in protecting involved perforators while occluding aneurysm.


2020 ◽  
Vol 102-B (12) ◽  
pp. 1620-1628
Author(s):  
Alexander Klug ◽  
Angela Nagy ◽  
Yves Gramlich ◽  
Reinhard Hoffmann

Aims To evaluate the outcomes of terrible triad injuries (TTIs) in mid-term follow-up and determine whether surgical treatment of the radial head influences clinical and radiological outcomes. Methods Follow-up assessment of 88 patients with TTI (48 women, 40 men; mean age 57 years (18 to 82)) was performed after a mean of 4.5 years (2.0 to 9.4). The Mayo Elbow Performance Score (MEPS), Oxford Elbow Score (OES), and Disabilities of the Arm, Shoulder and Hand (DASH) score were evaluated. Radiographs of all patients were analyzed. Fracture types included 13 Mason type I, 16 type II, and 59 type III. Surgical treatment consisted of open reduction and internal fixation (ORIF) in all type II and reconstructable type III fractures, while radial head arthroplasty (RHA) was performed if reconstruction was not possible. Results At follow-up the mean MEPS was 87.1 (20 to 100); mean OES, 36.9 (6 to 48); and mean DASH score, 18.6 (0 to 90). Mean movement was 118° (30° to 150°) for extension to flexion and 162° (90° to 180°) for pronation to supination. The overall reoperation rate was 24%, with nine ORIF, ten RHA, and two patients without treatment to the radial head needing surgical revision. When treated with RHA, Mason type III fractures exhibited significantly inferior outcomes. Suboptimal results were also identified in patients with degenerative or heterotopic changes on their latest radiograph. In contrast, more favourable outcomes were detected in patients with successful radial head reconstruction after Mason type III fractures. Conclusion Using a standardized protocol, sufficient elbow stability and good outcomes can be achieved in most TTIs. Although some bias in treatment allocation, with more severe injuries assigned to RHA, cannot be completely omitted, treatment of radial head fractures may have an independent effect on outcome, as patients subjected to RHA showed significantly inferior results compared to those subjected to reconstruction, in terms of elbow function, incidence of arthrosis, and postoperative complications. As RHA showed no apparent advantage in Mason type III injuries between the two treatment groups, we recommend reconstruction, providing stable fixation can be achieved. Cite this article: Bone Joint J 2020;102-B(12):1620–1628.


2019 ◽  
Vol 101-B (12) ◽  
pp. 1512-1519 ◽  
Author(s):  
Alexander Klug ◽  
Felix Konrad ◽  
Yves Gramlich ◽  
Reinhard Hoffmann ◽  
Kay Schmidt-Horlohé

Aims The aim of this study was to evaluate the outcome of Monteggia-like lesions at midterm follow-up and to determine whether the surgical treatment of the radial head influences the clinical and radiological results. Patients and Methods A total of 78 patients with a Monteggia-like lesion, including 44 women and 34 men with a mean age of 54.7 years (19 to 80), were available for assessment after a mean 4.6 years (2 to 9.2). The outcome was assessed using the Mayo Elbow Performance Score (MEPS), Oxford Elbow Score (OES), Mayo Modified Wrist Score (MMWS), and The Disabilities of the Arm, Shoulder and Hand (DASH) score. Radiographs were analyzed for all patients. A total of 12 Mason type I, 16 type II, and 36 type III fractures were included. Surgical treatment consisted of screw fixation for all type II and reconstructable type III fractures, while radial head arthroplasty (RHA) or excision was performed if reconstruction was not possible. Results The mean MEPS was 88.9 (40 to 100), mean OES was 40.1 (25 to 48), mean MMWS was 88.1 (50 to 100), mean DASH score was 14.7 (0 to 60.2), and mean movement was 114° (sd 27) in extension/flexion and 155° (sd 37) in pronation/supination. Mason III fractures, particularly those with an associated coronoid fracture treated with RHA, had a significantly poorer outcome. Suboptimal results were also identified in patients who had degenerative changes or heterotopic ossification on their latest radiograph. In contrast, all patients with successful radial head reconstruction or excision had a good outcome. Conclusion Good outcomes can be achieved in Monteggia-like lesions with Mason II and III fractures, when reconstruction is possible. Otherwise, RHA is a reliable option with satisfactory outcomes, especially in patients with ligamenteous instability. Whether the radial head should be excised remains debatable, although good results were achieved in patients with ligamentous stability and in those with complications after RHA. Cite this article: Bone Joint J 2019;101-B:1512–1519


Epilepsia ◽  
2012 ◽  
Vol 53 (2) ◽  
pp. 349-358 ◽  
Author(s):  
Francine Chassoux ◽  
Elisabeth Landré ◽  
Charles Mellerio ◽  
Baris Turak ◽  
Michael W. Mann ◽  
...  

2017 ◽  
Vol 24 (2) ◽  
pp. 5-10
Author(s):  
M. V Girkalo ◽  
I. A Norkin

Purpose. To study the short-term results of revision knee arthroplasty performed using metaphyseal sleeves. Patients and methods. During the period from 2013 to 2015 the total number of 40 patients was operated on. Type I femoral defects (by AORI classification) were diagnosed in 11 (27.5%), type II - in 26 (65.0%), type III - in 3 (7.5%) cases. Type I tibial defects were diagnosed in 2 (5.0%), type IIa - in 24 (65.0%), type IIb - in 11 (27.0%) and type III - in 3 (7.54%) cases. Metaphyseal femoral sleeves were used in 8 and metaphyseal tibial sleeve - in 40 patients. Results. Follow up period made up from 12 to 43 (mean 32.8) months. Excellent and good results by KSS scale were achieved in 30 (75.0%) of patients, by functional KSS scale - in 24 (60.0%) patients. Mean point by Knee Society Total Knee Arthroplasty Roentgenographic Evaluation scale in patients with femoral and tibial metaphyseal sleeves made up 2.35 versus 0.375 that confirmed the absence of progressive bone resorption. No one case of aseptic instability of the revision implant was observed. Repeated surgical intervention due to reinfection and knee joint contracture with pain syndrome was performed in 2 (5.0%) patients. Conclusion. The obtained data enable to recommend the use of metaphyseal sleeves for revision knee arthroplasty in patients with tibial and femoral defects of types II and III by AORI classification.


2021 ◽  
Vol 26 (1) ◽  
Author(s):  
Patrick Pflüger ◽  
Michael Zyskowski ◽  
Michael Müller ◽  
Chlodwig Kirchhoff ◽  
Peter Biberthaler ◽  
...  

Abstract Background Metatarsal fractures are common skeletal injuries of the lower extremity in adults. The majority involves the proximal fifth metatarsal bone. In the current literature, there still exists controversy regarding treatment recommendations for the different fracture entities. Methods All patients suffering from single fractures to the proximal fifth metatarsal bone between 2003 and 2015 were enrolled in this retrospective analysis. Only patients with a minimum follow-up of 12 months were included. The fractures were classified according to Lawrence and Botte (L&B). Data were collected via patient registry, radiographs and a standardized questionnaire (Foot and Ankle Outcome Score = FOAS). For outcome analysis, the nonparametric Mann–Whitney U test was performed and Spearman’s rank correlation coefficient calculated. Results In total, the functional outcomes of 103 patients suffering from fractures to the proximal fifth metatarsal bone were analyzed. L&B type I fractures (n = 13) had a FAOS score of 91 ± 23, L&B type II (n = 67) presented a score of 91 ± 15 and L&B type III (n = 23) a score of 93 ± 11. Surgically treated patients with an L&B type II fracture had no statistically significant better functional outcome in comparison to conservative management (p = 0.89). Operatively treated L&B type III fractures tended to have a better functional score (p = 0.16). The follow-up time was 58 (min: 15; max: 164) months. Conclusions Overall, the functional outcome following fractures to the proximal fifth metatarsal bone is satisfactory. Conservatively treated L&B type II fractures showed an equivalent functional outcome compared to surgical management. Patients with an L&B type III fracture mainly were treated surgically, but difference in FAOS score did not reach level of significance.


2021 ◽  
Author(s):  
Dulcie Lai ◽  
Meethila Gade ◽  
Edward Yang ◽  
Hyun Yong Koh ◽  
Nicole M. Walley ◽  
...  

Post-zygotically acquired genetic variants, or somatic variants, that arise during cortical development have emerged as important causes of focal epilepsies, particularly those due to malformations of cortical development. Pathogenic somatic variants have been identified in many genes within the PI3K-AKT3-mTOR-signaling pathway in individuals with hemimegalencephaly and focal cortical dysplasia (type II), and more recently in SLC35A2 in individuals with focal cortical dysplasia (type I) or non-dysplastic epileptic cortex. Given the expanding role of somatic variants across different brain malformations, we sought to delineate the landscape of somatic variants in a large cohort of patients who underwent epilepsy surgery with hemimegalencephaly or focal cortical dysplasia. We evaluated samples from 123 children with hemimegalencephaly (n=16), focal cortical dysplasia type I and related phenotypes (n=48), focal cortical dysplasia type II (n=44), or focal cortical dysplasia type III (n=15) classified using imaging and pathological findings. We performed high-depth exome sequencing in brain tissue-derived DNA from each case and identified somatic single nucleotide, indel, and large copy number variants. In 75% of individuals with hemimegalencephaly and 29% with focal cortical dysplasia type II, we identified pathogenic variants in PI3K-AKT-mTOR pathway genes. Four of 48 cases with focal cortical dysplasia type I (8%) had a likely pathogenic variant in SLC35A2. While no other gene had multiple disease-causing somatic variants across the focal cortical dysplasia type I cohort, four individuals in this group had a single pathogenic or likely pathogenic somatic variant in CASK, KRAS, NF1, and NIPBL, genes associated with neurodevelopmental disorders. No rare pathogenic or likely pathogenic somatic variants in any neurological disease genes like those identified in the focal cortical dysplasia type I cohort were found in 63 neurologically normal controls (P = 0.017), suggesting a role for these novel variants. We also identified a somatic loss-of-function variant in the known epilepsy gene, PCDH19, present in a very small number of alleles in the dysplastic tissue from a female patient with focal cortical dysplasia IIIa with hippocampal sclerosis. In contrast to focal cortical dysplasia type II, neither focal cortical dysplasia type I nor III had somatic variants in genes that converge on a unifying biological pathway, suggesting greater genetic heterogeneity compared to type II. Importantly, we demonstrate that FCD types I, II, and III, are associated with somatic gene variants across a broad range of genes, many associated with epilepsy in clinical syndromes caused by germline variants, as well as including some not previously associated with radiographically evident cortical brain malformations.


2021 ◽  
Author(s):  
Horst Urbach ◽  
Elias Kellner ◽  
Nico Kremers ◽  
Ingmar Blümcke ◽  
Theo Demerath

AbstractFocal cortical dysplasia (FCD) are histopathologically categorized in ILAE type I to III. Mild malformations of cortical development (mMCD) including those with oligodendroglial hyperplasia (MOGHE) are to be integrated into this classification yet. Only FCD type II have distinctive MRI and molecular genetics alterations so far. Subtle FCD including FCD type II located in the depth of a sulcus are often overlooked requiring the use of dedicated sequences (MP2RAGE, FLAWS, EDGE) and/or voxel (VBM)- or surface-based (SBM) postprocessing. The added value of 7 Tesla MRI has to be proven yet.


2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Changbin Wang ◽  
Zudong Yin ◽  
Xinyi Zhang ◽  
Xiumin Zhao

Purpose. To analyze the characteristics of hyperdense lesions on brain CT conducted immediately after arterial revascularization (AR) in patients with acute ischemic stroke (AIS), track the outcome of those lesions and investigate their clinical significance. Materials and Methods. 97 AIS patients were enrolled in our study. Among them, 52 patients showed hyperdense lesions and were divided into three categories: type I, type II and type III according to the morphologic characteristics of hyperdense lesions. All patients underwent several follow-up CT/MR examinations to visualize the outcomes of the lesions. Results. Among the 52 patients, 22 showed contrast extravasation, 23 displayed contrast extravasation combined with hemorrhagic transformation (HT) and 7 confirmed symptomatic intracranial hemorrhage (SICH) in follow-up CT/MR. Among the without hyperdense lesions group, only 7 converted to hemorrhage, and no SICH occurred. All type I lesions showed contrast extravasation; 23 type II lesions turned to hemorrhage, 2 revealed SICH and 6 were pure contrast extravasation; all of the type III developed into SICH. Conclusion. Hyperdense lesions on non-enhanced brain CT obtained immediately after arterial revascularization (AR) exhibited varying features. Type I indicated a pure contrast extravasation. Type II and type III hyperdense lesions suggested higher incidence of HT, the presence of type III lesions indicated an ominous outcome.


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