Browse

You are looking at 1 - 10 of 10 items for :

  • Refine by Access: all x
Clear All
Open access

Myasthenia gravis in a pediatric patient with Lennox-Gastaut syndrome following responsive neurostimulation device implantation: illustrative case

David A Zuckerman, Cameron P Beaudreault, Carrie R Muh, Patricia E McGoldrick, and Steven M Wolf

BACKGROUND

Myasthenia gravis (MG) is an autoimmune disorder in which the postsynaptic acetylcholine receptor of the neuromuscular junction is destroyed by autoantibodies. The authors report a case of MG in a pediatric patient who also suffered from Lennox-Gastaut syndrome (LGS) and is one of a limited number of pediatric patients who have undergone placement of a responsive neurostimulation (RNS) device (NeuroPace).

OBSERVATIONS

A 17-year-old female underwent placement of an RNS device for drug-resistant epilepsy in the setting of LGS. Five months after device placement, the patient began experiencing intermittent slurred speech, fatigue, and muscle weakness. Initially, the symptoms were attributed to increased seizure activity and/or medication side effects. However, despite changing medications and RNS settings, no improvements occurred. Her antiacetylcholine receptor antibodies measured 62.50 nmol/L, consistent with a diagnosis of MG. The patient was then prescribed pyridostigmine and underwent a thymectomy, which alleviated most of her symptoms.

LESSONS

The authors share the cautionary tale of a case of MG in a pediatric patient who was treated with RNS for intractable epilepsy associated with LGS. Although slurred speech, fatigue, muscle weakness, and other symptoms might stem from increased seizure activity and/or medication side effects, they could also be due to MG development.

Open access

Tandem pediatric neurosurgery: treatment of synostosis and intractable epilepsy. Illustrative case

Ogechukwu Ariwodo, Douglas R Nordli III, Nathan J Ranalli, and Alexandra D Beier

BACKGROUND

Hemispherectomy is a surgical procedure reserved for hemispheric intractable epilepsy. Sagittal craniosynostosis is a congenital disorder treated with open or endoscope-assisted approaches for synostosis correction. These procedures are not commonly performed in the same setting.

OBSERVATIONS

In this report, the authors present a 6-month-old female with sagittal craniosynostosis, hemimegalencephaly, and intractable epilepsy who underwent a left hemispherotomy with open sagittal synostosis correction followed by cranial molding orthosis therapy.

LESSONS

The report highlights the technical nuances of the procedure, but also discusses the possible genetic disorder responsible for both conditions, megalencephaly-capillary malformation syndrome.

Open access

Severe hyperglycorrhachia and status epilepticus after endoscopic aqueductoplasty: illustrative case

Anand A. Dharia, Ahmad Masri, Jay F. Rilinger, and Christian B. Kaufman

BACKGROUND

While hypoglycorrhachia is observed and managed frequently, there are few reports in the literature of clinically significant hyperglycorrhachia after neurosurgery. Understanding the effects and management of severe hyperglycorrhachia is important to the neurosurgeon and neurocritical care teams who care for patients in these rare scenarios.

OBSERVATIONS

The authors present the case of a 3-month-old male with congenital hydrocephalus who faced profound hyperglycorrhachia and status epilepticus after an endoscopic aqueductoplasty using an irrigant composed of lactated Ringer’s solution with dextrose 5% in water. A multidisciplinary approach was developed to monitor and treat the patient’s seizures and cerebrospinal fluid (CSF) osmolytes.

LESSONS

This case provides several learning opportunities for understanding CSF physiology, pathogenesis of common brain injuries related to osmotic shifts and inflammatory states, as well as clinical management of hyperglycorrhachia. It also reiterates the significance of meticulous intraoperative assessment to avoid preventable medical errors.

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

Cronobacter brain abscess and refractory epilepsy in a newborn: role of epilepsy surgery. Illustrative case

Meredith Yang, John Tsiang, Melissa A. LoPresti, and Sandi Lam

BACKGROUND

Neonatal meningitis due to Cronobacter is associated with powdered infant formula. Prompt recognition of this rare but aggressive infection is critical.

OBSERVATIONS

The authors report a unique case of neonatal Cronobacter meningoencephalitis complicated by brain abscess and status epilepticus, requiring surgical intervention in a preterm 4-week-old male and related to contaminated powdered infant formula. They discuss the medical and surgical management in this patient, as well as the role of epilepsy surgery in acute drug-resistant epilepsy. This is paired with a literature review examining Cronobacter infections in infants to provide a summative review of the existing literature.

LESSONS

Cronobacter contamination in powdered infant formula and breast pumps is rare but can cause life-threatening infections. When evaluating patients with Cronobacter central nervous system infections, serial neuroimaging, infection control, and prompt surgical management are essential. Future studies are needed regarding the role of epilepsy surgery in the acute infectious period.

Open access

Seizure freedom after laser amygdalohippocampotomy guided by bilateral responsive neurostimulation in pediatric epilepsy: illustrative case

Belinda Shao, Bryan Zheng, David D. Liu, Matthew N. Anderson, Konstantina Svokos, Luca Bartolini, and Wael F. Asaad

BACKGROUND

For patients with difficult-to-lateralize temporal lobe epilepsy, the use of chronic recordings as a diagnostic tool to inform subsequent surgical therapy is an emerging paradigm that has been reported in adults but not in children.

OBSERVATIONS

The authors reported the case of a 15-year-old girl with pharmacoresistant temporal lobe epilepsy who was found to have bitemporal epilepsy during a stereoelectroencephalography (sEEG) admission. She underwent placement of a responsive neurostimulator system with bilateral hippocampal depth electrodes. However, over many months, her responsive neurostimulation (RNS) recordings revealed that her typical, chronic seizures were right-sided only. This finding led to a subsequent right-sided laser amygdalohippocampotomy, resulting in seizure freedom.

LESSONS

In this case, RNS chronic recording provided real-world data that enabled more precise seizure localization than inpatient sEEG data, informing surgical decision-making that led to seizure freedom. The use of RNS chronic recordings as a diagnostic adjunct to seizure localization procedures and laser ablation therapies in children is an area with potential for future study.

Open access

Presurgical language mapping in bilingual children using transcranial magnetic stimulation: illustrative case

Savannah K. Gibbs, Stephen Fulton, Basanagoud Mudigoudar, Frederick A. Boop, and Shalini Narayana

BACKGROUND

Presurgical mapping of eloquent cortex in young patients undergoing neurosurgery is critical but presents challenges unique to the pediatric population, including motion artifact, noncompliance, and sedation requirements. Furthermore, as bilingualism in children increases, functional mapping of more than one language is becoming increasingly critical. Transcranial magnetic stimulation (TMS), a noninvasive brain stimulation technique, is well suited to evaluate language areas in children since it does not require the patient to remain still during mapping.

OBSERVATIONS

A 13-year-old bilingual male with glioblastoma multiforme involving the left parietal lobe and deep occipital white matter underwent preoperative language mapping using magnetic resonance imaging-guided TMS. Language-specific cortices were successfully identified in both hemispheres. TMS findings aided in discussing with the family the risks of postsurgical deficits of tumor resection; postoperatively, the patient had intact bilingual speech and was referred for chemotherapy and radiation.

LESSONS

The authors’ findings add to the evolving case for preoperative dual language mapping in bilingual neurosurgical candidates. The authors illustrate the feasibility and utility of TMS as a noninvasive functional mapping tool in this child. TMS is safe, effective, and can be used for preoperative mapping of language cortex in bilingual children to aid in surgical planning and discussion with families.

Open access

Surgical strategy for refractory epilepsy secondary to porencephaly: ictal SPECT may obviate the need for intracranial electroencephalography. Patient series

Naoki Ichikawa, Naotaka Usui, Akihiko Kondo, Takayasu Tottori, Tokito Yamaguchi, Hirowo Omatsu, Takayoshi Koike, Hiroko Ikeda, Katsumi Imai, and Yukitoshi Takahashi

BACKGROUND

Surgical treatment of intractable epilepsy caused by porencephaly can be difficult because of poorly localizing or lateralizing electroclinical findings. The authors aimed to determine whether noninvasive evaluations are sufficient in these patients.

OBSERVATIONS

Eleven patients were included in this study. The porencephalic cyst was in the left middle cerebral artery (MCA) area in 9 patients, the left posterior cerebral artery area in 1 patient, and the bilateral MCA area in 1 patient. Interictal electroencephalography (EEG) revealed multiregional, bilateral, interictal epileptiform discharges in 5 of 11 patients. In 6 of 10 patients whose seizures were recorded, the ictal EEG was nonlateralizing. Nine patients underwent ictal single-photon emission computed tomography (SPECT), which revealed lateralized hyperperfusion in 8 of 9 cases. Fluorodeoxyglucose positron emission tomography (FDG-PET) was useful for identifying the functional deficit zone. No patient had intracranial EEG. The procedure performed was hemispherotomy in 7 patients, posterior quadrant disconnection in 3 patients, and occipital disconnection in 1 patient. A favorable seizure outcome was achieved in 10 of 11 patients without the onset of new neurological deficits.

LESSONS

Ictal SPECT was useful for confirming the side of seizure origin when electroclinical findings were inconclusive. Thorough noninvasive evaluations, including FDG-PET and ictal SPECT, enabled curative surgery without intracranial EEG. Seizure and functional outcomes were favorable.

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.

Open access

Removal of malformation in cerebral proliferative angiopathy: illustrative case

Gwang Yoon Choi, Hyuk Jai Choi, Jin Pyeong Jeon, Jin Seo Yang, Suk-Hyung Kang, and Yong-Jun Cho

BACKGROUND

Cerebral proliferative angiopathy (CPA) is a rare vascular disorder distinct from arteriovenous malformation. Because of the disorder’s rarity, there is still a controversy on the most promising treatment method for CPA. However, several meta-analysis articles suggest indirect vascularization such as encephalo-duro-arterio-synangiosis as an effective way of treating symptoms that are medically uncontrolled.

OBSERVATIONS

The authors describe a case of an 11-year-old boy with this disease, who had epilepsy that was intractable despite conservative management. The patient recovered from his symptoms after the vascular malformation was surgically removed. This is the first reported case of surgical removal in CPA.

LESSONS

Although further investigation on the best treatment for CPA is needed, the authors believe surgical intervention may also be an effective treatment modality when a patient presents with persisting symptoms.