Frequency, Predictors, and Outcome of Seizures in Patients With Myelomeningocele: Single-Center Retrospective Cohort Study

2021 ◽  
pp. 088307382110531
Author(s):  
Cemal Karakas ◽  
Emin Fidan ◽  
Kapil Arya ◽  
Troy Webber ◽  
Joan B. Cracco

To determine the frequency, predictors, and outcomes of seizures in patients with myelomeningocele, we retrospectively analyzed the data from patients with myelomeningocele followed longitudinally at a single center from 1975 to 2013. We identified a total of 122 patients (61% female). The mean follow-up duration was 11.1 years (minimum-maximum = 0-34.5 years, SD = 8.8, median = 9.1 years). A total of 108 (88.5%) patients had hydrocephalus, and 98 (90.7%) of those patients required a ventriculoperitoneal shunt procedure. Twenty-four (19.7%) patients manifested with seizures, 23 of whom had hydrocephalus. The average age of seizure onset was 4.8 years (median 2 years of age). Falx dysgenesis ( P = .004), lumbar myelomeningocele ( P = .007), and cortical atrophy ( P = .028) were significantly associated with epileptic seizure development. The average seizure-free period at the last follow-up in patients with a history of myelomeningocele and seizures was 8.1 years. We conclude that myelomeningocele patients with seizures have an overall good prognosis with considerable long-term seizure freedom.

Neurosurgery ◽  
2013 ◽  
Vol 73 (3) ◽  
pp. 395-400 ◽  
Author(s):  
Sumeet Vadera ◽  
Lara Jehi ◽  
Jorge Gonzalez-Martinez ◽  
William Bingaman

Abstract BACKGROUND: Patients who continue or begin to have seizures after brain surgery pose significant challenges and often require an invasive electroencephalographic evaluation before reoperation for drug-resistant epilepsy. The safety and seizure-free outcomes associated with subdural grid (SDG) implantation in patients with a prior craniotomy are important for both surgeon and patient to understand before pursuing further surgery. OBJECTIVE: To evaluate the safety of SDG placement and subsequent resective surgery in patients with prior craniotomy and to characterize the seizure outcomes and their predictors after resective epilepsy surgery in this unique cohort. METHODS: We retrospectively reviewed all intractable focal epilepsy patients with a history of craniotomy who underwent SDG insertion between 2000 and 2012 at our institution. A minimum follow-up of 6 months was required. End points analyzed included complications related to each surgery and Engel classification at the last follow-up. RESULTS: The mean age of seizure onset was 15.9 years, and the mean age for the initial surgery was 24.2 years. Only 3 patients began having seizures after the initial surgery. Seven patients (7%) had a complication associated with the SDG placement, and 15 (14%) had a complication after subsequent resection, which was equivalent to the initial procedure. Forty-eight patients (44%) were in Engel class I at the last follow-up. Freedom from seizures was predicted by ictal onset at the edge of the original surgical bed, particularly in patients with lesional epilepsy. CONCLUSION: Surgical intervention with SDG monitoring does not appear to be associated with increased risk of complications in epilepsy patients with a history of prior craniotomy, and rates of freedom from seizures in this challenging group are favorable.


2019 ◽  
Vol 24 (2) ◽  
pp. 200-208
Author(s):  
Ravindra Arya ◽  
Francesco T. Mangano ◽  
Paul S. Horn ◽  
Sabrina K. Kaul ◽  
Serena K. Kaul ◽  
...  

OBJECTIVEThere is emerging data that adults with temporal lobe epilepsy (TLE) without a discrete lesion on brain MRI have surgical outcomes comparable to those with hippocampal sclerosis (HS). However, pediatric TLE is different from its adult counterpart. In this study, the authors investigated if the presence of a potentially epileptogenic lesion on presurgical brain MRI influences the long-term seizure outcomes after pediatric temporal lobectomy.METHODSChildren who underwent temporal lobectomy between 2007 and 2015 and had at least 1 year of seizure outcomes data were identified. These were classified into lesional and MRI-negative groups based on whether an epilepsy-protocol brain MRI showed a lesion sufficiently specific to guide surgical decisions. These patients were also categorized into pure TLE and temporal plus epilepsies based on the neurophysiological localization of the seizure-onset zone. Seizure outcomes at each follow-up visit were incorporated into a repeated-measures generalized linear mixed model (GLMM) with MRI status as a grouping variable. Clinical variables were incorporated into GLMM as covariates.RESULTSOne hundred nine patients (44 females) were included, aged 5 to 21 years, and were classified as lesional (73%), MRI negative (27%), pure TLE (56%), and temporal plus (44%). After a mean follow-up of 3.2 years (range 1.2–8.8 years), 66% of the patients were seizure free for ≥ 1 year at last follow-up. GLMM analysis revealed that lesional patients were more likely to be seizure free over the long term compared to MRI-negative patients for the overall cohort (OR 2.58, p < 0.0001) and for temporal plus epilepsies (OR 1.85, p = 0.0052). The effect of MRI lesion was not significant for pure TLE (OR 2.64, p = 0.0635). Concordance of ictal electroencephalography (OR 3.46, p < 0.0001), magnetoencephalography (OR 4.26, p < 0.0001), and later age of seizure onset (OR 1.05, p = 0.0091) were associated with a higher likelihood of seizure freedom. The most common histological findings included cortical dysplasia types 1B and 2A, HS (40% with dual pathology), and tuberous sclerosis.CONCLUSIONSA lesion on presurgical brain MRI is an important determinant of long-term seizure freedom after pediatric temporal lobectomy. Pediatric TLE is heterogeneous regarding etiologies and organization of seizure-onset zones with many patients qualifying for temporal plus nosology. The presence of an MRI lesion determined seizure outcomes in patients with temporal plus epilepsies. However, pure TLE had comparable surgical seizure outcomes for lesional and MRI-negative groups.


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Gemma Patella ◽  
Alessandro Comi ◽  
Giuseppe Coppolino ◽  
Nicolino Comi ◽  
Giorgio Fuiano ◽  
...  

Abstract Background and Aims Steroid-dependent nephrotic syndrome (SDNS) may require a prolonged multi-drug therapy with risk of drug toxicity and renal failure. Rituximab (RTX) treatment has been found to be helpful in reducing the steroid dosage and the need for immunosuppressants (ISs), but little data are currently available regarding very long-term outcomes in adults. We herein describe a long-term, single-center experience of RTX use in a large series of adults with SDNS. Method We studied 23 adult patients with SDNS (mean age 54.2±17.1 y; 65% male; BMI 28.5±4.7), mostly consequent to membranous (47.8%) or focal glomerulonephritis (30.2 %) who were eligible to start a RTX regimen. Before entering the RTX protocol, proteinuria and eGFR were 7.06±3.87 g/24h and 65.9±28.2 ml/min/1.73 m2, respectively; albumin and CD19/CD20 ratio were 2.9±0.9 g/L and 0.99±0.01 respectively; the mean number of ISs was 2.39±0.89 and the mean annual rate of relapses was 2.2±0.9. Results Patients were followed over a mean follow-up of 64 months (range: 12-144). After RTX (mean dose: 1202.1±372.4 mg) the rate of relapses was virtually nullified (p&lt;0.001). eGFR remained roughly stable (62.1±19.8 ml/min/1.73 m2, p=NS), while proteinuria, albumin, CD19/CD20 and BMI all significantly improved (p ranging from 0.01 to 0.001). The mean number of additional ISs was also reduced (0.44±0.12; p&lt;0.001) and RTX enabled discontinuation of steroids in 13/23 (56.5%) patients. No major adverse events related to therapy were recorded. Conclusion Findings from this large case-series with a remarkable very long follow-up reinforce the role of RTX as an efficient and safe weapon to improve outcomes in adult patients suffering from SDNS.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2338-2338
Author(s):  
Lena Coïc ◽  
Suzanne Verlhac ◽  
Emmanuelle Lesprit ◽  
Emmanuelle Fleurence ◽  
Francoise Bernaudin

Abstract Abnormal TCD defined as high mean maximum velocities &gt; 200 cm/sec are highly predictive of stroke risk and justify long term transfusion program. Outcome and risk factors of conditional TCD defined as velocities 170–200 cm/sec remains to be described. Patients and methods Since 1992, 371 pediatric SCD patients (303 SS, 44 SC, 18 Sß+, 6 Sß0) were systematically explored once a year by TCD. The newborn screened cohort (n=174) had the first TCD exploration between 12 and 18 months of age. TCD was performed with a real-time imaging unit, using a 2 MHz sector transducer with color Doppler capabilities. Biological data were assessed at baseline, after the age of 1.5 years and remotely of transfusion or VOC. We report the characteristics and the outcome in patients (n=43) with an history of conditional TCD defined by mean maximum velocities ranging between 170 and 200 cm/s in the ACM, the ACA or the ICA. Results: The mean follow-up of TCD monitoring was 5,5 years (0 – 11,8 y). All patients with an history of conditional doppler were SS/Sb0 (n=43). Mean (SD) age of patients at the time of their first conditional TCD was 4.3 years (2.2) whereas in our series the mean age at abnormal TCD (&gt; 200 cm/sec) occurrence was 6.6 years (3.2). Comparison of basal parameters showed highly significant differences between patients with conditional TCD and those with normal TCD: Hb 7g4 vs 8g5 (p&lt;0.001), MCV 82.8 vs 79 (p=0.047). We also had found such differences between patients with normal and those with abnormal TCD (Hb and MCV p&lt; 0.001). Two patients were lost of follow-up. Two patients died during a trip to Africa. Conditional TCD became abnormal in 11/43 patients and justified transfusion program. Mean (SD) conversion delay was 1.8 (2.0) years (range 0.5–7y). No stroke occurred. 16 patients required a treatment intensification for other indications (frequent VOC/ACS, splenic sequestrations): 6 were transplanted and 10 received HU or TP. Significant risk factors (Pearson) of conversion to abnormal were the age at time of conditional TCD occurrence &lt; 3 y (p&lt;0.001), baseline Hb &lt; 7g/dl (p=0.02) and MCV &gt; 80 (p=0.04). MRI/MRA was performed in 31/43 patients and showed ischemic lesions in 5 of them at the mean (SD) age of 7.1 y (1.8) (range 4.5–8.9): no significant difference was observed in the occurrence of lesions between the 2 groups. Conclusions This study confirms the importance of age as predictive factor of conditional to abnormal TCD conversion with a risk of 64% when first conditional TCD occured before the age of 3 years. TCD has to be frequently controled during the 5 first years of life.


2009 ◽  
Vol 27 (2) ◽  
pp. E6 ◽  
Author(s):  
Dmitry Ruban ◽  
Richard W. Byrne ◽  
Andres Kanner ◽  
Michael Smith ◽  
Elizabeth J. Cochran ◽  
...  

Object The authors undertook a study to review the clinical features and outcome in patients who underwent surgery for intractable chronic epilepsy caused by temporal lobe tumors. Methods The Rush Surgical Epilepsy Database was queried to identify patients with chronic intractable epilepsy who underwent resection of temporal lobe tumors between 1981 and 2005 at Rush University Medical Center. Medical records were reviewed for age of the patient at seizure onset, delay to referral for surgery, seizure frequency and characteristics, preoperative MR imaging results, extent of resection, pathological diagnosis, complications, duration of follow-up period, and seizure improvement. Results Thirty-eight patients were identified, all with low-grade tumors. Gangliogliomas were the most common (36.8%), followed in descending order by dysembryoplastic neuroepithelial tumors (26.3%) and low-grade diffuse astrocytoma (10.5%). The mean duration between seizure onset and surgery was 15.4 years. Complex partial seizures were the most common presenting symptom. Detailed operative data were available for 28 patients; of these, 89.3% underwent complete resection of the amygdala, and 82.1% underwent partial or complete resection of hippocampus, in addition to lesionectomy. The mean follow-up duration was 7.7 years (range 1.0–23.1 years), with 78.9% of patients having seizure status that improved to Engel Class I, 15.8% to Engel Class II, and 5.3% to Engel Class III. Permanent complications were noted in 2.6% of patients. Conclusions The authors' examination of the long-term follow-up data in patients with temporal lobe tumors causing chronic intractable epilepsy demonstrated excellent results in seizure improvement after surgery.


2021 ◽  
Vol 12 ◽  
Author(s):  
Anil Tanburoglu ◽  
Cagatay Andic

Background and Purpose: Blood Blister-like aneurysms (BBAs) of the internal carotid artery (ICA) are rare entities of cerebral aneurysms. FD use in acutely ruptured aneurysms, timing of treatment and antiplatelet regimen are main debate topics in clinical practice when the treatment decision is flow diversion. The aim of this study is to report the safety and efficacy of a single-center FD treatment for ruptured BBAs in the early phase of SAH using the SAPT regimen.Material and Method: This study involved a retrospective analysis of a prospectively collected database. Records of patients admitted to our clinic and treated by endovascular route on ruptured BBA between January 2013 and December 2020 were reviewed. Ruptured supraclinoid ICA BBAs treated with FD devices with SAPT within 48 h from ictus of SAH are included. BBA of atypical anatomic locations, other endovascular techniques performed, and delayed admissions (&gt;48 h) were excluded from the study. Demographic, clinical and angiographic features of patients and aneurysms, FD types and numbers, periprocedural complications, immediate and follow-up angiographic and clinical outcomes were recorded.Results: A total of six patients with ruptured BBAs treated via FDs within 48 h and used SAPT were included in the study. The mean age was 41.6 years (range from 34 to 45 years), and four of six patients were female. All patients were treated within 48 h after ictus, and the mean treatment day was 1.33 days. One patient received ticagrelor, and five patients received prasugrel as SAPT for one year after treatment. No procedure-related death and rebleeding were recorded. One (16.7 %) treatment responsive procedure-related complication occurred (transient ischemia). Overall good outcome rate was 83.3%. One patient died due to pneumonia. The immediate control angiograms showed complete occlusions of BBAs in one patient (16.6%). The complete occlusion rate was 100 % for five survivors at the control angiogram. The median follow-up was 49.5 months.Conclusion: This single-center experience suggests that early treatment (&lt;2 days) within SAH of ruptured BBAs with FDs using SAPT is safe and effective in terms of clinical and radiological long-term outcomes.


F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 165
Author(s):  
Supawan Surukrattanaskul ◽  
Pukkapol Suvannachart ◽  
Sunee Chansangpetch ◽  
Anita Manassakorn ◽  
Visanee Tantisevi ◽  
...  

Purpose: To evaluate the clinical characteristics and treatment outcomes of patients with childhood glaucoma. Methods: We retrospectively reviewed the data of patients with childhood glaucoma who visited the glaucoma clinics at the Queen Sirikit National Institute of Child Health and the King Chulalongkorn Memorial Hospital between January 2008 and January 2018. The diagnosis was based on the Childhood Glaucoma Research Network classification. We recorded their clinical characteristics and requirement of any glaucoma interventions. Results: A total of 691 eyes from 423 patients were included in this study. The patients predominantly comprised boys. The average follow-up duration was 71.3±63.8 months. The mean age at presentation was 3.9±4.4 years. Most patients presented with a high initial intraocular pressure (IOP) of 28.5±11.2 mmHg. Glaucoma associated with non-acquired ocular anomalies (22.9%) was the most common subtype, followed by primary congenital glaucoma (20.8%). We recorded a family history of glaucoma in 6.4% of patients. Most patients had bilateral glaucoma (63.4%) and required at least one intervention (51.5%). The average IOP at the latest follow-up visit was 19.1±10.8 mmHg. All glaucoma types had significantly lower IOP, compared to that at their baselines (all p<0.001). Moreover, most patients had an unfavourable visual acuity (49.5%) at their latest visit. Conclusions: Secondary glaucoma associated with non-acquired ocular anomalies is the most common subtype of glaucoma. All subtypes, including primary glaucoma, were sporadic. The majority of patients had unfavourable visual outcomes. These real-world findings are fundamental to acquire a better understanding of childhood glaucoma.


2020 ◽  
Vol 24 (6) ◽  
pp. 606-611
Author(s):  
Y. Li ◽  
Z. Jia ◽  
S. Li ◽  
Y. Huang ◽  
X. Yuan ◽  
...  

OBJECTIVE: To assess factors associated with long-term haemoptysis recurrence after transarterial embolisation (TAE) for haemoptysis due to bronchiectasis.METHODS: Patients with haemoptysis due to bronchiectasis who underwent TAE between May 2010 and May 2019 were included in this retrospective study. Long-term haemoptysis recurrence was defined as the expectoration of >10 mL/day of fresh blood (for at least 1 day) 1 month after TAE. Univariate and multivariate analyses were performed to identify risk factors for long-term haemoptysis recurrence after TAE.RESULTS: A total of 197 patients (108 women; mean age, 61.0 ± 12.2 years) were included in the study. TAE was performed successfully in all patients. Side effects occurred in 43 (21.8%) patients, and all patients recovered uneventfully. During 37.6 ± 11.6 months of follow-up, long-term haemoptysis recurrence occurred in 41 (20.8%) patients; the mean interval between the TAE and haemoptysis recurrence was 21.4 ± 16.3 months. Long-term haemoptysis recurrence after TAE was associated with a history of haemoptysis (OR 3.483, 95% CI 1.373–8.836; P = 0.009).CONCLUSIONS: Approximately one fifth patients with bronchiectasis had long-term haemoptysis recurrence after TAE. Risk factor for long-term haemoptysis recurrence after TAE was a history of haemoptysis.


2014 ◽  
Vol 121 (Suppl_2) ◽  
pp. 143-149 ◽  
Author(s):  
Shibin Sun ◽  
Ali Liu

ObjectThe aim of this study was to evaluate long-term clinical outcomes after Gamma Knife surgery (GKS) for patients with neurofibromatosis Type 2 (NF2) and the role of GKS in the management of NF2.MethodsFrom December 1994 through December 2008, a total of 46 patients (21 male, 25 female) with NF2 underwent GKS and follow-up evaluation for at least 5 years at the Gamma Knife Center of the Beijing Neurosurgical Institute. GKS was performed using the Leksell Gamma Knife Models B and C. The mean age of the patients was 30 years (range 13–59 years). A family history of NF2 was found for 9 (20%) patients. The NF2 phenotype was thought to be Wishart for 20 (44%) and Feiling-Gardner for 26 (56%) patients. Among these 46 patients, GKS was performed to treat 195 tumors (73 vestibular schwannomas and 122 other tumors including other schwannomas and meningiomas). For vestibular schwannomas, the mean volume was 5.1 cm3 (median 3.6 cm3, range 0.3–27.3 cm3), the mean margin dose was 12.9 Gy (range 10–14 Gy), and the mean maximum dose was 27.3 Gy (range 16.2–40 Gy). For other tumors, the mean volume was 1.7 cm3 (range 0.3–5.5 cm3), the mean margin dose was 13.3 Gy (range 11–14 Gy), and the mean maximum dose was 26.0 Gy (range 18.0–30.4 Gy). The median duration of follow-up was 109 months (range 8–195 months).ResultsFor the 73 vestibular schwannomas that underwent GKS, the latest follow-up MR images demonstrated regression of 30 (41%) tumors, stable size for 31 (43%) tumors, and enlargement of 12 (16%) tumors. The total rate of tumor control for bilateral vestibular schwannomas in patients with NF2 was 84%. Of the 122 other types of tumors that underwent GKS, 103 (85%) showed no tumor enlargement. The rate of serviceable hearing preservation after GKS was 31.9% (15/47). The actuarial rates for hearing preservation at 3 years, 5 years, 10 years, and 15 years were 98%, 93%, 44%, and 17%, respectively. Of the 46 patients, 22 (48%) became completely bilaterally deaf, 17 (37%) retained unilateral serviceable hearing, and 7 (15%) retained bilateral serviceable hearing. The mean history of the disease course was 12 years (range 5–38 years).ConclusionsGKS was confirmed to provide long-term local tumor control for small- to medium-sized vestibular schwannomas and other types of tumors, although vestibular schwannomas in patients with NF2 responded less well than did unilateral sporadic vestibular schwannomas. Phenotype is the most strongly predictive factor of final outcome after GKS for patients with NF2. The risk for loss of hearing is high, whereas the risk for other cranial nerve complications is low.


2019 ◽  
Vol 2019 ◽  
pp. 1-5 ◽  
Author(s):  
Masaya Iwamuro ◽  
Yuki Aoyama ◽  
Seiyuu Suzuki ◽  
Sayo Kobayashi ◽  
Tatsuya Toyokawa ◽  
...  

Background. Clinical characteristics and prognosis of patients with a solitary Peutz-Jeghers polyp (PJP) have not been fully investigated. Methods. Solitary PJP was diagnosed when a single hamartomatous lesion was identified in the gastrointestinal tract of patients without mucocutaneous pigmentation or a family history of Peutz-Jeghers syndrome. We retrospectively reviewed 51 patients (32 men and 19 women) with a solitary PJP and analyzed the sex, age at diagnosis, endoscopic features, and outcomes in this patient group. The STK11/LKB1 germline mutation was not investigated in any of the patients. Results. The mean age of the 51 patients was 66.1 years. The polyp was found in the duodenum (N=10), jejunum (N=2), cecum (N=2), transverse colon (N=5), sigmoid colon (N=21), or rectum (N=11). Most of the polyps presented as a pedunculated lesion (N=40), followed by semipedunculated (N=9) and sessile (N=2) morphologies. The mean size of a solitary PJP was 15.6 mm (range: 5 to 33 mm). During a mean endoscopic follow-up period of 4.5 years (range: 0.1 to 16.1 years), no recurrence was identified. Eighteen of the enrolled patients had a history of cancer or concomitant cancer. Five patients died due to non-gastrointestinal-related causes. No additional cancer or death directly related to solitary PJP was observed. Conclusions. Solitary PJPs did not recur in this study. Although examination of the entire gastrointestinal tract using esophagogastroduodenoscopy, enteroscopy, and colonoscopy is desirable to exclude Peutz-Jeghers syndrome, follow-up endoscopy after endoscopic polyp resection may be unnecessary, once the diagnosis of a solitary PJP is made.


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