Neurovascular Lesions in Pediatric Epilepsy

2019 ◽  
Vol 34 (10) ◽  
pp. 549-555
Author(s):  
J. Kassiri ◽  
T. Rajapakse ◽  
M. Wheatley ◽  
D. B. Sinclair
Keyword(s):  
Clinical Presentation ◽  
Case Series ◽  
Initial Presentation ◽  
Visual Field Defects ◽  
Pediatric Epilepsy ◽  
Seizure Freedom ◽  
Antiepileptic Treatment ◽  
Cavernous Angiomas ◽  
Focal Seizures

Introduction: Neurovascular lesions are rare and understudied in the pediatric population. Their initial presentation can range from seizures to focal neurologic deficits, as well as headaches. The goal of this study was to examine the clinical presentation and natural history of neurovascular lesions in children with epilepsy. Methods: We reviewed all pediatric epilepsy patients with neurovascular lesions diagnosed between 2006 and 2018 at the University of Alberta and the Stollery Children’s Hospital, Edmonton, Canada. Initial clinical presentation and brain imaging, as well as long-term epilepsy and postsurgical outcome, were assessed. Results: Of the 14 patients, 10 patients had an initial presentation of focal seizures with impaired awareness, whereas 2 patients presented with headache, 1 presented with visual field defects as well as chronic headaches, and 1 with decreased level of consciousness. Seven patients had cavernous angiomas, 6 had arteriovenous malformation, and 1 patient had an arteriovenous fistula. Notably, all patients with cavernous angiomas and 4 of 6 patients with arteriovenous malformations presented with seizures. Among 9 of the 14 who underwent neurovascular corrective surgery, all 9 patients required long-term antiepileptic treatment of at least 1 antiepileptic drug for seizure control after the operation. Conclusion: In this novel case series, we describe focal seizures as the initial presentation of pediatric neurovascular lesions. This clinical presentation appears to be independent of the type of neurovascular lesion. Furthermore, unlike our pediatric surgical patients with epilepsy due to other causes, seizure freedom following neurovascular surgery is limited, and patients require long-term antiepileptic treatment.

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

WP1-21 Maximum safe resection of large medial temporal intrinsic tumours: does the outcome justify the risk?

2019 ◽  
Vol 90 (3) ◽  
pp. e7.1-e7
Author(s):  
A Kumaria ◽  
A Paterson ◽  
M Sitaraman ◽  
S Basu
Keyword(s):  
Case Series ◽  
Csf Leak ◽  
Visual Field Defects ◽  
Long Term Outcomes ◽  
Karnofsky Score ◽  
Female Age ◽  
Grade 3 ◽  
Age Range

ObjectivesTo analyse on the long-term outcomes in patients undergoing maximum safe resection (MSR) for large intrinsic temporal tumours.DesignCase seriesSubjectsAll patients undergoing MSR of large medial temporal intrinsic tumours between May 2006 and February 2012 at a tertiary neurosurgical centre with a minimum follow up of 6 years.MethodsRetrospective review of hospital records.ResultsFifty-one patients underwent MSR (28 male, 23 female); age range 20–80 years (mean age 55.3). There was no difference in laterality, although dysphasia was a feature in 32% of left-sided lesions. Presenting features in general included seizures (46%), headaches (27%), hemiparesis (12%) and visual field defects (6%). Surgery was generally well tolerated (median post-operative Karnofsky score 92.5). No patients developed new dysphasia or weakness, but there was transient worsening of existing hemiparesis (n=4) and dysphasia (n=2). Other complications included CSF leak/pseudomeningocoele (n=2), oculomotor palsy (n=1) and wound infection (n=1). Histopathological casemix was GBM (50%), WHO 3 gliomas (14%), WHO 2 gliomas (10%) and metastases (4%). In total, 57% of patients received radiotherapy and 35% received chemotherapy. Survival correlated with pathology; in glioblastoma patients it ranged from 2–19 months (mean 10.4 months). Survival in grade 3 tumours ranged from 10–38 months (mean 24.4 months). 60% of patients with Grade 2 tumours are surviving symptom free with no histological upscale at 8–10 years follow-up. No patient required a second debulking procedure.ConclusionsMSR did not result in survival benefit in glioblastoma. MSR is justified in lesions with pre-operative radiological features of Grade 2 glioma.

scholarly journals Inflammatory myofibroblastic tumours of the lung: a case series

Pneumologia ◽  
2019 ◽  
Vol 68 (2) ◽  
pp. 82-86
Author(s):  
Alexandru Melinte ◽  
Irina Strâmbu ◽  
Diana Leonte ◽  
Mihai Dumitrescu ◽  
Cristian Paleru ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Case Series ◽  
Long Term Results ◽  
Inflammatory Myofibroblastic Tumour ◽  
Rare Group

Abstract Inflammatory myofibroblastic tumours represent a rare group of lesions reported in various organs but with an unclear aetiology. Although so far they are considered benign, some cases of recurrence and invasive behaviour have been noted. We report three consecutive cases of tumours of the lung, with heterogeneous clinical presentation. They were all treated by means of surgery, with good long-term results. Diagnosing an inflammatory myofibroblastic tumour relies mainly on an experienced pathologist.

scholarly journals Rosai-Dorfman disease of cranial and spinal origin - A case series

2020 ◽  
Vol 11 ◽  
pp. 298
Author(s):  
Saleh Salah Safi ◽  
Khaled Murshed ◽  
Arshad Ali ◽  
Surjith Vattoth ◽  
Abdulrazzaq Haider ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Inflammatory Diseases ◽  
Management Strategies ◽  
Case Series ◽  
Current Management ◽  
Rosai Dorfman Disease ◽  
Lymph Node Enlargement ◽  
Long Term Follow Up

Background: Rosai-Dorfman disease (RDD) is an idiopathic nonneoplastic lymphadenopathy disorder which is characterized by lymph node enlargement, but it may also presents primarily involving a variety of extranodal sites, including central nerves system and craniospinal axis. This study reports five cases of craniospinal RDD, with review of epidemiology, clinical presentation, imaging, and histopathological features with current management strategies. Case Description: Five cases of RDD are diagnosed at Hamad General Hospital, Qatar, during 2013–2018. Two cases had dural-based cranial lesions with overlying cranial involvement while three cases were having extradural thoracic spine lesions. All cases underwent surgical intervention and confirmed by histopathology. Conclusion: Craniospinal RDD is a rare clinical presentation and poses significant diagnostic challenges preoperatively due to its similarity with other neoplastic or inflammatory diseases. Surgical option to remove compressive neural pathology provides a good clinical outcome with no recurrence in long-term follow-up.

scholarly journals Relative contribution of individual versus combined functional imaging studies in predicting seizure freedom in pediatric epilepsy surgery: an area under the curve analysis

2020 ◽  
Vol 48 (4) ◽  
pp. E13
Author(s):  
Pongkiat Kankirawatana ◽  
Ismail S. Mohamed ◽  
Jason Lauer ◽  
Inmaculada Aban ◽  
Hyunmi Kim ◽  
...  
Keyword(s):  
Functional Imaging ◽  
Surgical Outcomes ◽  
Partial Epilepsy ◽  
Curve Analysis ◽  
Imaging Modalities ◽  
Pediatric Epilepsy ◽  
Seizure Freedom ◽  
Relative Contribution ◽  
Refractory Partial Epilepsy

OBJECTIVEThe goal of this study was to evaluate the predictive value and relative contribution of noninvasive presurgical functional imaging modalities based on the authors’ institutional experience in pursuing seizure-free surgical outcomes in children with medically refractory epilepsy.METHODSThis was a retrospective, single-institution, observational cohort study of pediatric patients who underwent evaluation and surgical treatment for medically refractory partial epilepsy between December 2003 and June 2016. During this interval, 108 children with medically refractory partial epilepsy underwent evaluation for localization and resective epilepsy surgery. Different noninvasive functional imaging modalities, including ictal SPECT, FDG-PET, and magnetoencephalography–magnetic source imaging, were utilized to augment a standardized paradigm (electroencephalography/semiology, MRI, and neuropsychology findings) for localization. Outcomes were evaluated at a minimum of 2 years (mean 7.5 years) utilizing area under the receiver operating characteristic curve analysis. Localizing modalities and other clinical covariates were examined in relation to long-term surgical outcomes.RESULTSThere was variation in the contribution of each test, and no single presurgical workup modality could singularly and reliably predict a seizure-free outcome. However, concordance of presurgical modalities yielded a high predictive value. No difference in long-term outcomes between inconclusive (normal or diffusely abnormal) and abnormal focal MRI results were found. Long-term survival analyses revealed a statistically significant association between seizure freedom and patients with focal ictal EEG, early surgical intervention, and no history of generalized convulsions.CONCLUSIONSComprehensive preoperative evaluation utilizing multiple noninvasive functional imaging modalities is not redundant and can improve pediatric epilepsy surgical outcomes.

Children With Trisomy 21 and Lennox-Gastaut Syndrome With Predominant Myoclonic Seizures

2021 ◽  
pp. 088307382110260
Author(s):  
Matthew Macdonald ◽  
Jacqueline Crawford ◽  
Anita N. Datta
Keyword(s):  
Trisomy 21 ◽  
Case Series ◽  
Severe Form ◽  
Seizure Type ◽  
Pediatric Epilepsy ◽  
Retrospective Case ◽  
Spike Wave Discharges ◽  
Long Term Prognosis ◽  
Atypical Absence

Introduction: Lennox-Gastaut syndrome is a severe form of pediatric epilepsy that is classically defined by a triad of drug-resistant seizures, including atonic, tonic, and atypical absence seizures; slow spike-and-wave discharges and paroxysmal fast activity on electroencephalography (EEG); and cognitive and behavioral dysfunction. In the vast majority, Lennox-Gastaut syndrome develops in patients with an identified etiology, including genetic or structural brain abnormalities. Long-term prognosis is generally poor with progressive intellectual deterioration and persistent seizures. At present, there are few reported cases of Lennox-Gastaut syndrome and trisomy 21 in the literature. To further delineate the spectrum of epilepsy in trisomy 21, we reviewed children with trisomy 21 and Lennox-Gastaut syndrome at one center over 28 years. Methods: This is a retrospective case series. At our institution, all EEG results are entered into a database, which was queried for patients with trisomy 21 from 1992 to 2019. Pertinent electroclinical data was obtained from medical records. Results: Of 63 patients with trisomy 21 and epilepsy, 6 (10%) had Lennox-Gastaut syndrome and were included in the study. Four of the 6 patients were male and 5 of 6 had neuroimaging, which was normal. Follow-up ranged from 3 to 20 years. Notably, 5 of 6 had predominant myoclonic seizures throughout the course of their epilepsy, associated with generalized spike-wave discharges, <100 milliseconds. Conclusion: We observed myoclonic seizures to be a predominant seizure type in patients with trisomy 21, suggestive that trisomy 21 patients may have a unique pattern of Lennox-Gastaut syndrome.

scholarly journals P.104 Children with Trisomy 21 and Lennox-Gastaut Syndrome with predominant myoclonic seizures

2021 ◽  
Vol 48 (s3) ◽  
pp. S49-S49
Author(s):  
MJ MacDonald ◽  
J Crawford ◽  
A Datta
Keyword(s):  
Trisomy 21 ◽  
Case Series ◽  
Severe Form ◽  
Seizure Type ◽  
Pediatric Epilepsy ◽  
Retrospective Case ◽  
Spike Wave Discharges ◽  
Long Term Prognosis ◽  
Neuro Imaging

Background: Lennox-Gastaut syndrome (LGS) is a severe form of pediatric epilepsy that is classically defined by a triad of drug-resistant seizures, characteristic EEG patterns, and intellectual disability. Long-term prognosis is generally poor with progressive intellectual deterioration and persistent seizures. At present, there are few reported cases of LGS and Trisomy 21 (T21) in the literature. To further delineate the spectrum of epilepsy in T21, we reviewed children with T21 and LGS at one center over 28 years. Methods: This is a retrospective case series. At our institution, all EEG results are entered into a database, which was queried for patients with T21 from 1992-2019. Pertinent electro-clinical data was obtained from medical records. Results: 63 patients with T21 and epilepsy, 6 (10%) had LGS and were included in the study. Four of the six patients were male and 5/6, had neuro-imaging, which was normal. Follow-up ranged from 3-20 years. Notably, 5/6 had predominant myoclonic seizures throughout the course of their epilepsy, associated with generalized spike-wave discharges. Conclusions: Myoclonic seizures appear to be a predominant seizure type in patients with T21, suggestive that T21 patients may have a unique pattern of LGS.

scholarly journals P.103 Midline Spikes and Intractable Seizures in Pediatric Epilepsy

2021 ◽  
Vol 48 (s3) ◽  
pp. S49-S49
Author(s):  
K Luan ◽  
J Mailo ◽  
N Liu ◽  
J Kassiri ◽  
DB Sinclair
Keyword(s):  
Focal Cortical Dysplasia ◽  
Intractable Epilepsy ◽  
Pediatric Epilepsy ◽  
Seizure Freedom ◽  
Epileptogenic Zone ◽  
Seizure Onset ◽  
Focal Seizures ◽  
Intractable Seizures ◽  
Epileptic Discharges ◽  
Common Etiology

Background: Epileptic discharges localized to the midline vertex are rare. However, they have been associated with intractable seizures and severe long-term consequences in the developing brain. Our study aimed to understand the etiology of pediatric midline seizures and define post-surgical seizure outcomes. Methods: We reviewed charts, electroencephalography (EEG), and neuroimaging studies of ten pediatric patients with epileptic discharges localized to the midline vertex in the Comprehensive Epilepsy Program. The seizures were classified according to the International League Against Epilepsy criteria, patient age, sex, neuroimaging results, seizure etiology and outcomes were obtained. Results: Age of seizure onset was within the first 10 years of life in 90% of patients, with focal seizures being the most prevalent. Focal cortical dysplasia (FCD) was the most common etiology present in 50% of patients. These children had normal neuroimaging studies and intractable epilepsy. However, seizure freedom was achieved following surgical resection of the epileptogenic zone. Conclusions: We demonstrated that patients with midline epileptic discharges are associated with intractable focal seizures and early seizure onset. Despite normal neuroimaging reports, FCD was the most common pathology. Thus our study suggests early localization and resection of the epileptogenic zone may be beneficial for achieving seizure freedom in children with this electroclinical syndrome.

scholarly journals Cavernous angiomas: An uncontrolled clinical study of 87 surgically treated patients

Medicina ◽  
2009 ◽  
Vol 45 (1) ◽  
pp. 21 ◽  
Author(s):  
Giedrimantas Bernotas ◽  
Daiva Rastenytė ◽  
Vytenis Deltuva ◽  
Algis Matukevičius ◽  
Vanda Jaškevičienė ◽  
...  
Keyword(s):  
Clinical Study ◽  
Clinical Presentation ◽  
Postoperative Outcome ◽  
Epileptic Seizures ◽  
Neurological Status ◽  
Initial Presentation ◽  
Tertiary Referral Center ◽  
Cavernous Angiomas ◽  
Common Clinical Presentation ◽  
The Mean

Background. Cerebral cavernous angiomas remain as one of the most negotiable and controversial topics in neurological and neurosurgical practice. We present statistical evaluation of initial presentation, preoperative neurological findings, surgical complications, and outcome of surgically treated patients with intracerebral cavernous angiomas. Methods. During 1997 to 2004, 87 patients (41 men, 46 women) with intracerebral cavernous angiomas underwent surgical treatment in the tertiary referral center, and these were included into the present uncontrolled clinical study. The mean age of the patients at the time of operation was 42.4 years, and the mean duration of illness was 120.5 days. All patients underwent preoperative magnetic resonance imaging, and pre- and postoperative clinical examination. The clinical course was documented using the Karnofski performance scale. A simplified version of Engel’s classification of the outcome of the patients with chronic seizures was applied. Results. The most common clinical presentation of cavernous angiomas was seizures, significant part of which was chronic. No association was found between lesion location by cerebral lobes and clinical presentation by seizures. No significant differences in the likelihood of presenting with hemorrhage between supratentorial and subtentorial lesions were detected. In addition, no associations were found between size of cavernoma and the initial presentation of cavernoma by an extralesional hemorrhage or chronic seizures. In the group of patients presenting with headache, sporadic seizures, or intracerebral hemorrhage, good postoperative outcome was achieved in 83% of the patients. Of the patients who underwent operation for seizure control, significant seizure reduction or elimination after surgery was observed in 79% of the patients. Conclusions. Microsurgical removal of cavernous angiomas and surrounding hemosiderin plate tends to significant reduction or elimination of epileptic seizures and improved postoperative neurological status.

scholarly journals Recurrent Hypocalcemic Tetany as the Initial Presentation in Some Persons with Hyperthyroidism: A Case Series

2021 ◽  
pp. 1-4
Author(s):  
Yakubu Lawal ◽  
Umar Faruk Dahuwa ◽  
Ibrahim Abdullahi ◽  
Felicia Ehusani Anumah
Keyword(s):  
Bone Mineral Density ◽  
Vitamin D ◽  
Calcium Metabolism ◽  
Clinical Presentation ◽  
Case Series ◽  
Initial Presentation ◽  
Calcium Balance ◽  
Mineral Density ◽  
Resource Poor ◽  
Laboratory Investigations

This is a case series of recurrent hypocalcemic tetany as the initial presentation in some cases of hyperthyroidism. Literature review has revealed more reports of hypercalcemia than hypocalcemia as a feature of hyperthyroidism. The recurrence of hypocalcemic tetany in the 3 cases reported ceased following the treatment of hyperthyroidism. The aim of this case series is to alert clinicians to the fact that hypocalcemic tetany can be the initial clinical presentation of hyperthyroidism. It also highlights the resilience of managing endocrine patients in resource-poor settings with the aid of high clinical acumen and fewer-than-expected laboratory investigations. Further research is needed to investigate our hypothesis that disordered calcium metabolism in thyrotoxicosis may manifest in successive phases of hypercalcemia-normocalcemia-hypocalcemia. The rate at which individual thyrotoxic patient traverses these phases may be directly proportional to the background vitamin D deficiency, negative calcium balance, and bone mineral density.

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