Darren B. Orbach

Multiple subpial transection for intractable partial epilepsy: an international meta-analysis.

Summary:  Purpose: Because the number and variety of patients at any single facility is not sufficient for clinical or statistical analysis, data from six major epilepsy centers that performed multiple subpial transections (MSTs) for medically intractable epilepsy were collected.

Increased Vertebral Artery Tortuosity Index Is Associated With Adverse Outcomes in Children and Young Adults With Connective Tissue Disorders

Background— Arterial tortuosity is described as a common and distinctive feature of Loeys-Dietz syndrome (LDS), yet reports on arterial tortuosity are based on qualitative observations and none have investigated an association between tortuosity and cardiovascular outcomes in LDS or other connective tissue disorders. Methods and Results— We performed a retrospective analysis of 90 patients ⩽50 years of age with Marfan syndrome, LDS, Ehlers-Danlos syndrome, or nonspecific connective tissue disorder who underwent thoracic contrast-enhanced magnetic resonance angiography. Controls (n=30) underwent magnetic resonance imaging to exclude arrhythmogenic right ventricular dysplasia. Using a volume-rendered angiogram, vertebral arteries were measured along the curvature of the vessel (actual length) and linearly (straight length), and distance factor was calculated: [(actual/straight length−1)×100]. Each subjects maximum distance factor was designated the Vertebral Tortuosity Index (VTI). The VTI was compared among diagnostic groups and among patients with cardiac surgery, dissection, and death. Median age at magnetic resonance imaging was 19.6 years (range 0.2 to 50.1). VTI interrater reliability was excellent (intraclass correlation coefficient =0.987). The VTI was higher in Marfan syndrome (n=57, median 26; interquartile range 10 to 49) and LDS (n=13, median 58; interquartile range 18 to 92) compared with controls (median 4.5; interquartile range 3 to 6; P<0.001 for both). Higher VTI was associated with younger age at surgery even when controlling for root size (adjusted P=0.002). Vertebral tortuosity index ≥50 was associated with earlier age at dissection and death compared with VTI <50 (P=0.001 versus P<0.001). We found no difference in age at surgery, dissection, or death in Marfan syndrome compared with LDS. Conclusion— Arterial tortuosity measured by magnetic resonance angiography is a reproducible marker of adverse cardiovascular outcomes in connective tissue disorders.

A Novel Association between RASA1 Mutations and Spinal Arteriovenous Anomalies.

BACKGROUND AND PURPOSE: CM-AVM is a recently recognized autosomal dominant disorder associated with mutations in RASA1. Arteriovenous lesions have been reported in the brain, limbs, and the face in 18.5% of patients. We report a novel association between RASA1 mutations and spinal arteriovenous anomalies. MATERIALS AND METHODS: In a collaborative study, 5 index patients (2 females, 3 males) with spinal AVMs or AVFs and cutaneous multifocal capillary lesions were investigated for the RASA1 gene mutation. RESULTS: All 5 patients were found to have RASA1 mutation (2 de novo, 3 familial), and all had multifocal capillary malformations at birth. Neurologic deficits developed at ages ranging from infancy to early adulthood. All spinal anomalies (2 AVMs at the conus, 1 AVM at the lumbosacral junction, and 1 cervical and 1 cervicothoracic AVF) were complex, extensive, and fast-flow lesions. All patients required treatment based on the clinical and/or radiologic appearance of the lesions. CONCLUSIONS: To our knowledge, an association of RASA1 mutation and spinal AVM/AVF has not been described. MR imaging screening of patients with characteristic CMs and neurologic symptoms presenting at a young age may be useful in detecting the presence of fast-flow intracranial or intraspinal arteriovenous anomalies before potentially significant neurologic insult has occurred.

Late Seizure Recurrence after Multiple Subpial Transections

Summary: We studied long‐term outcome (range, 28–89 months; mean, 56 months) after multiple subpial transections (MSTs) for medically refractory epilepsy. Forty‐three (79.6%) of 54 patients had a consistent significant reduction in seizure frequency, and 27 (50%) of the 54 were either entirely seizure free or virtually so. However, 10 (18.6%) patients sustained an increase in seizure frequency several years after surgery, after showing initial postoperative improvement. This suggests that late seizure recurrence is a more important problem in cases in which MST has been performed than for pure resections.

Endovascular therapy in children with acute ischemic stroke Review and recommendations

This review provides a summary of the currently available data pertaining to the interventional management of acute ischemic stroke in children. The literature is scarce and is lacking much-needed prospective trials. No study in the literature on the well-established systemic or local thrombolysis trials has included children. Mechanical thrombectomy trials using clot retriever devices have also excluded patients younger than 18 years. The current review is limited to case series of interventional acute ischemic stroke therapy in children and the potential future of endovascular ischemic stroke therapy in this patient population. Recommendations in this review represent the opinion of the authors, based on review of the limited literature covering endovascular acute ischemic stroke therapy in children.

The Use of Onyx for Embolization of Central Nervous System Arteriovenous Lesions in Pediatric Patients

BACKGROUND AND PURPOSE: The safety and efficacy of Onyx for the embolization of central nervous system (CNS) arteriovenous (AV) lesions have been widely reported in adults. However, data describing the use of this agent in children are limited. This study presents our experience with Onyx in the treatment of CNS AV lesions in pediatric patients. MATERIALS AND METHODS: We retrospectively analyzed clinical and imaging records of 15 pediatric patients who underwent 36 transarterial embolizations by using Onyx for CNS AV lesions, from March 2007 through April 2009 at our institution. Underlying pathologies included brain AV malformations (AVMs) (n = 7), vein of Galen malformations (n = 4), dural AV fistulas (n = 2), and spinal AVMs (n = 2). For 7 procedures in very high-flow lesions, detachable coils were deployed before Onyx embolization, whereas in 29 procedures, Onyx was the sole embolic agent. The efficacy of embolization was judged by the residuum of AV shunting within the target region. RESULTS: Embolization was complete in 2 patients, nearly complete in 9 patients, and partial (and ongoing) in 4 patients. Following staged embolization, 7 patients underwent surgical resection without significant blood loss and with good functional outcome in all cases. Clinically silent non-target embolization was encountered in 2 of 36 procedures. After 3 of the 36 embolizations, patients developed transient neurologic symptoms, all of which resolved to baseline within 24 hours. There were no non-neurologic adverse events. There was no imaging evidence of infarct or hemorrhage. CONCLUSIONS: Onyx embolization of pediatric CNS AV lesions can be an efficacious treatment technique, with extremely low associated morbidity.

Surgical treatment of multifocal epilepsy involving eloquent cortex.

Summary:  Purpose: This report describes our long‐term follow‐up for combined resective surgery and multiple subpial transections (MSTs) in patients with refractory epilepsy involving eloquent and noneloquent cortex in multiple lobes. Multiple independent seizure foci made these patients poor candidates for conventional surgery.

Safety and Clinical Efficacy of Onyx for Embolization of Extracranial Head and Neck Vascular Anomalies

BACKGROUND AND PURPOSE: Onyx was developed for embolization of central nervous system AVMs but is increasingly used extracranially because of its unique physical properties. We review our experience and results with the use of Onyx for the treatment of fast-flow extracranial vascular lesions. MATERIALS AND METHODS: We retrospectively analyzed clinical and imaging records of 22 patients who underwent 71 extracranial embolizations from March 2007 through January 2010. The diagnoses were the following: cervicofacial AVM (n = 18), traumatic fistula (n = 3), and vessel laceration (n = 1). In 62 of 71 procedures (87%), Onyx was the sole embolic agent; it was delivered transarterially in 67/71 and percutaneously in 4/71 procedures. Clinical goals included amelioration of pain and control of bleeding. The clinical efficacy of embolization was judged by symptom control, and adverse events were assessed by clinical examination and history, both postembolization and 4 weeks postprocedure. RESULTS: Cessation of acute bleeding was achieved in 13/14 cases, with 1 case of immediate recurrent massive epistaxis prompting reintubation and further embolization. Control of subacute bleeding episodes and pain was achieved for all patients. Following staged embolization, 7 patients underwent surgical resection without significant blood loss. Surgeons reported high satisfaction with the intraoperative handling properties of Onyx. Transient swelling, local tenderness, or numbness was encountered after 7 procedures. There were no stuck catheters, vessel dissections, or vessel ruptures and no skin discoloration. CONCLUSIONS: Staged Onyx embolization was clinically efficacious in managing extracranial fast-flow vascular malformations and lesions, with low associated morbidity.

Psychogenic, Nonepileptic seizures associated with video-EEG-verified sleep

Summary:  Purpose: Nonepileptic seizures (NES) are expressions of a psychiatric disease state, usually conversion disorder, that mimic epileptic seizures (ES) but are not associated with the neurophysiologic changes of epilepsy. Conversion has not been demonstrated to emerge from the sleeping state. Emergence out of sleep is usually considered a virtual exclusion criterion for NES, signifying the presence instead of ES. We sought to test this hypothesis.

Management of Arteriovenous Malformations

This article describes the clinical features, diagnosis, and management of arteriovenous malformation, capillary malformation-arteriovenous malformation, and PTEN-associated vascular anomaly.