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Open access

Stereoelectroencephalography in the setting of a previously implanted responsive neural stimulation device: illustrative case

Dorian M Kusyk, Nicholas Blaney, Timothy Quezada, and Alexander C Whiting

BACKGROUND

Responsive neural stimulation (RNS) is a relatively novel procedure for drug-resistant epilepsy, which involves implantation of a device into the skull and brain. As more devices are implanted, there may be an increasing need to perform intracranial electrocorticography in implant patients with persistent seizures. Given the device location, imaging difficulties with implanted devices, and other technical hurdles, stereoelectroencephalography (SEEG) could be especially challenging. The authors describe the first reported SEEG investigation in a patient with an RNS device, highlighting the technical challenges and clinical data ascertained.

OBSERVATIONS

A 41-year-old male with drug-resistant epilepsy presented several years after a local surgeon had placed an RNS device with two electrodes in the bilateral parieto-occipital lobes. Because of inadequate seizure control, the patient was offered a repeat SEEG investigation to characterize his epilepsy better. Although more technically challenging than a traditional SEEG implantation, the SEEG investigation was successfully performed, which led to a confirmation of bilateral hippocampal seizure onset. The patient underwent repositioning of his RNS leads with a significant decrease in his seizure frequency.

LESSONS

Concurrent implantation of SEEG electrodes in a functioning RNS device can be safely performed and can augment our understanding of a patient’s seizures.

Open access

A new strategy for treating drug-resistant focal aware seizures: thalamic specific nuclei deep brain stimulation. Illustrative case

Osvaldo Vilela-Filho, Hélio F. Silva-Filho, Lissa C. Goulart, Paulo C. Ragazzo, and Francisco M. Arruda

BACKGROUND

Focal aware seizures (FASs) are relatively common and frequently pharmaco-resistant. If the seizure onset zone (SOZ) is in eloquent cortical areas, making resective surgery risky and inadvisable, deep brain stimulation (DBS) of the anterior nucleus of the thalamus, which is efficacious in less than half of the cases, has been the main alternative. New targets should be searched to address this deficiency. The present study aims to determine if DBS of different thalamic specific nuclei can modulate the abnormal electrical activity of the SOZ located in their respective cortical projection areas. Herein, the authors present the first patient in an ongoing trial.

OBSERVATIONS

A 60-year-old female patient presented with 25-year history of pharmaco-resistant focal aware visual seizures frequently evolving to focal impaired awareness seizures. The SOZ was in the right occipital lobe (positron emission tomography-computed tomography/video electroencephalography). Magnetic resonance imaging was normal. She underwent ipsilateral lateral geniculate nucleus (LGN) DBS procedure. After a 24-month follow-up, seizure frequency decreased by 97%, improving quality of life and daily functioning without complications.

LESSONS

This is the first time the LGN has been targeted in humans. The results support the hypothesis that led to this study. This strategy represents a paradigm shift in the way of treating pharmaco-resistant FASs not amenable to resective surgery.

Open access

Radiofrequency ablation during stereoelectroencephalography: from diagnostic tool to therapeutic intervention. Illustrative case

Demitre Serletis, Juan Bulacio, Justin Bingaman, Elham Abushanab, Stephen P. Harasimchuk, Richard Rammo, Silvia Neme-Mercante, and William Bingaman

BACKGROUND

Radiofrequency thermocoagulation (RFTC) during intracranial stereoelectroencephalography (sEEG) was first described as a safe technique for creating lesions of epileptic foci in 2004. Since that time, the method has been applied as a diagnostic and/or palliative intervention. Although widely practiced in European epilepsy surgical programs, the technique has not been popularized in the United States given the lack of Food and Drug Administration (FDA)–approved technologies permitting safe usage of in situ sEEG electrodes for this purpose.

OBSERVATIONS

The authors present a case report of a young female patient with refractory left neocortical temporal lobe epilepsy undergoing sEEG electrode implantation, who underwent sEEG-guided RFTC via a stereotactic temperature-sensing pallidotomy probe. Although used as a diagnostic step in her workup, the patient has remained seizure-free for nearly 18 months.

LESSONS

The use of in situ sEEG electrodes for RFTC remains limited in the United States. In this context, this case highlights a safe alternative and temporizing approach to performing diagnostic sEEG-guided RFTC, using a temperature-sensing pallidotomy probe to create small, precise stereotactic lesions. The authors caution careful consideration of this technique as a temporary work-around solution while also highlighting the rising need for new FDA-approved technologies for safe RFTC through in situ temperature-sensing sEEG electrodes.

Open access

Focal drug-resistant temporal lobe epilepsy associated with an ipsilateral anterior choroidal artery aneurysm: illustrative case

H. Westley Phillips, Shivani D. Rangwala, Joanna Papadakis, David J. Segar, Melissa Tsuboyama, Anna L. R. Pinto, Joseph P. Harmon, Sulpicio G. Soriano, Carlos J. Munoz, Joseph R. Madsen, Alfred P. See, and Scellig S. Stone

BACKGROUND

The occurrence of both an intracranial aneurysm and epilepsy, especially drug-resistant epilepsy (DRE), is rare. Although the overall incidence of aneurysms associated with DRE is unclear, it is thought to be particularly infrequent in the pediatric population. Surgical ligation of the offending aneurysm has been reported in conjunction with resolving seizure activity, although few cases have cited a combined approach of aneurysm ligation and resection of an epileptogenic focus.

OBSERVATIONS

We present the case of a 14-year-old female patient with drug-resistant temporal lobe epilepsy and an ipsilateral supraclinoid internal carotid artery aneurysm. Seizure semiology, electroencephalography monitoring, and magnetic resonance imaging all indicated a left temporal epileptogenic focus, in addition to an incidental aneurysm. The authors recommended a combined surgery involving resection of the temporal lesion and surgical clip ligation of the aneurysm. Near-total resection and successful ligation were achieved, and the patient has remained seizure free since surgery at 1 year postoperatively.

LESSONS

In patients with focal DRE and an adjacent intracranial aneurysm, a combined surgical approach involving both resection and surgical ligation can be used. Several surgical timing and neuroanesthetic considerations should be made to ensure the overall safety and efficacy of this procedure.

Open access

Infraclavicular de novo placement of a responsive neurostimulator for a patient with eloquent glioma-associated epilepsy: illustrative case

Ahmad R. Masri, Bailey R. Yekzaman, Bradley J. Estes, Christopher S. Park, Patrick Landazuri, and Michael Kinsman

BACKGROUND

The authors present a 50-year-old female with high-grade glioma involving the motor cortex as the cause of her drug-resistant epilepsy (DRE). Responsive neurostimulation (RNS) was chosen for epilepsy treatment. Due to concerns regarding the generator impeding the regular imaging surveillance required for treatment and monitoring of her glioma, surgeons placed the internal pulse generator (IPG) within an infraclavicular chest pocket.

OBSERVATIONS

Implantation of the RNS device and IPG within the infraclavicular pocket was uneventful. However, both subdural and depth electrodes were used and connected to the IPG, and subdural electrodes are considerably shorter than depth electrodes (37 vs 44 cm). The shorter strip leads presumably generated significant tension, leading to fracture of the leads. Therefore, surgery was repeated using only depth electrodes for more length and less tension. The device has good-quality electrocorticography signals that continue to be used for device programming. The seizure burden was reduced, and quality of life improved for the patient.

LESSONS

The RNS system with infraclavicular IPG placement reduced the seizure burden and improved the quality of life of a patient with glioma-associated epilepsy. Surgeons may consider the infraclavicular location as an alternative site for implantation for RNS candidates who require recurrent intracranial magnetic resonance imaging.

Open access

Vascular anomaly, lipoma, and polymicrogyria associated with schizencephaly: developmental and diagnostic insights. Illustrative case

Kevin K. Kumar, Angus Toland, Nancy Fischbein, Martha Morrell, Jeremy J. Heit, Donald E. Born, and Gary K. Steinberg

BACKGROUND

Schizencephaly is an uncommon central nervous system malformation. Intracranial lipomas are also rare, accounting for approximately 0.1% of brain “tumors.” They are believed to be derived from a persistent meninx primitiva, a neural crest–derived mesenchyme that develops into the dura and leptomeninges.

OBSERVATIONS

The authors present a case of heterotopic adipose tissue and a nonshunting arterial vascular malformation arising within a schizencephalic cleft in a 22-year-old male. Imaging showed right frontal gray matter abnormality and an associated suspected arteriovenous malformation with evidence of hemorrhage. Brain magnetic resonance imaging revealed right frontal polymicrogyria lining an open-lip schizencephaly, periventricular heterotopic gray matter, fat within the schizencephalic cleft, and gradient echo hypointensity concerning for prior hemorrhage. Histological assessment demonstrated mature adipose tissue with large-bore, thick-walled, irregular arteries. Mural calcifications and subendothelial cushions suggesting nonlaminar blood flow were observed. There were no arterialized veins or direct transitions from the arteries to veins. Hemosiderin deposition was scant, and hemorrhage was not present. The final diagnosis was consistent with ectopic mature adipose tissue and arteries with meningocerebral cicatrix.

LESSONS

This example of a complex maldevelopment of derivatives of the meninx primitiva in association with cortical maldevelopment highlights the unique challenges from both a radiological and histological perspective during diagnostic workup.

Open access

Dorsal medullary cavernous hemangioma presenting as obstinate hiccups and its surgical treatment: illustrative case

Sumirini Puppala, Abhijit Acharya, Atmaranjan Dash, and Surjyaprakash S. Choudhury

BACKGROUND

Hiccups are characterized by involuntary, intermittent, repetitive, myoclonic, and spasmodic contractions of the diaphragm. Hiccups are termed “intractable” when they last for over 1 month.

OBSERVATIONS

A rare case of intractable hiccups due to an uncommon location of cavernous hemangioma in the dorsal medulla is illustrated. With respect to the management, surgical excision was performed, and postsurgical complete recovery was witnessed, which has been reported only in six cases worldwide to date.

LESSONS

A mechanism of the hiccups reflex arc is discussed in detail with special reference to the need for equal emphasis on evaluating central nervous system causes and peripheral etiologies for pertinent hiccups.

Open access

Magnetic resonance imaging–guided laser interstitial thermal therapy for complete corpus callosotomy: technique and 1-year outcomes. Patient series

Benjamin J. Best, Irene Kim, and Sean M. Lew

BACKGROUND

Magnetic resonance imaging (MRI)-guided stereotactic laser interstitial thermal therapy (LITT) is a minimally invasive technique that has been described for the treatment of certain forms of epilepsy through partial or complete callosotomy, with few cases describing single-stage complete LITT callosotomy. The authors aimed to demonstrate this technique’s feasibility and efficacy through description of the technique and 1-year outcomes in 3 cases of single-stage complete LITT callosotomy in patients with anatomically normal corpa callosa (CCs).

OBSERVATIONS

The patients were aged 14–27 years and experienced atonic seizures. Completeness of callosotomy was determined from MRI scans obtained >3 months after LITT procedures. The estimated ablations of the CC were 94%, 89%, and 100%, respectively. The second patient had a catheter breach the lateral ventricle, resulting in the lowest estimated percentage of ablation in this series (89%), with minimal atonic seizure reduction. The first patient had significant reduction in atonic seizure frequency, and the third patient had complete resolution of atonic seizures. None of the patients experienced any long-term complications. Intensive care length of stay was 1 night for each patient, and total length of stay was between 2 and 7 nights. Postoperative follow-up was between 14 and 18 months.

LESSONS

Complete laser callosotomy is achievable and is a safe alternative to microsurgical or endoscopic approaches.

Open access

The utility of diffusion tractography for speech preservation in laser ablation of the dominant insula: illustrative case

Timothy J. Kaufmann, Vance T. Lehman, Lily C. Wong-Kisiel, Panagiotis Kerezoudis, and Kai J. Miller

BACKGROUND

Open surgical treatment of insular epilepsy holds particular risk of injury to middle cerebral artery branches, the operculum (through retraction), and adjacent language-related white matter tracts in the language-dominant hemisphere. Magnetic resonance imaging (MRI)-guided laser interstitial thermal therapy (LITT) is a surgical alternative that allows precise lesioning with potentially less operative risk. The authors presented the case of a 13-year-old girl with intractable, MRI-negative, left (dominant hemisphere) insular epilepsy that was treated with LITT. Diffusion tensor imaging (DTI) tractography was used to aid full posterior insular lesioning in the region of stereo electroencephalography–determined seizure onset while avoiding thermal injury to the language-related superior longitudinal fasciculus (SLF)/arcuate fasciculus (AF) and inferior fronto-occipital fasciculus (IFOF).

OBSERVATIONS

DTI tractography was used successfully in planning insular LITT and facilitated a robust insular ablation with sharp margins at the interfaces with the SLF/AF and IFOF. These tracts were spared, and no neurological deficits were induced through LITT.

LESSONS

Although it is technically demanding and has important limitations that must be understood, clinically available DTI tractography adds precision and confidence to insular laser ablation when used to protect important language-related white matter tracts.