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Open access

Hemifacial spasm caused by multiple vascular attachments due to remote compression effects of a dermoid cyst in the cerebellar hemisphere: illustrative case

Kento Hirayama, Takafumi Tanei, Takenori Kato, Toshinori Hasegawa, Eiji Ito, Yusuke Nishimura, and Ryuta Saito

BACKGROUND

Dermoid cysts located laterally in the posterior fossa are rare. The authors report the case of a dermoid cyst in the cerebellar hemisphere presenting with hemifacial spasm (HFS) caused by multiple vascular attachments due to remote compression effects.

OBSERVATIONS

A 48-year-old man presented with left HFS. Computed tomography showed a mass lesion in the left cerebellar hemisphere with calcification and erosion of skull bone. Magnetic resonance imaging showed no contrast enhancement of the lesion and a dural defect. The lesion compressed the brainstem and cerebellopontine cistern, but no vascular attachments to the facial nerve were seen. Tumor removal and microvascular decompression were performed. The lesion was composed of soft tissue containing oil-like liquid and hairs, and the border of the cerebellar arachnoid was clear. There were multiple vascular attachments to the root exit zone, facial nerve, and brainstem. After displacing these arteries, the intraoperative abnormal muscle response disappeared. Histopathological findings showed stratified squamous epithelium, keratin flakes, calcifications, and hairs. The HFS disappeared completely and has remained absent for 27 months.

LESSONS

The dermoid cyst originating from occipital bone compressed the cerebellar hemisphere, displacing multiple vessels and leading to HFS. Tumor removal and the removal of all vascular factors can completely resolve HFS.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 23 (Dec 2023)

Open access

Radiofrequency thermocoagulation for the treatment of trigeminal neuralgia associated with a focal pontine lesion: illustrative case

Vadym Biloshytsky, Anna Skorokhoda, Inna Buvailo, and Maryna Biloshytska

BACKGROUND

Trigeminal neuralgia (TN) associated with a focal pontine lesion is a rare but challenging condition. The origin of the lesion, which does not fulfill the diagnostic criteria for multiple sclerosis, remains disputable. Pain in such conditions is often refractory to treatment, including microvascular decompression.

OBSERVATIONS

A 36-year-old female presented with a 3.5-year history of shooting pain in the right V2 distribution triggered by talking and chewing. She became less responsive to high-dose carbamazepine over time. Magnetic resonance imaging (MRI) revealed no neurovascular compression but an elongated lesion hyperintense on T2-weighted imaging and T2- fluid-attenuated inversion recovery and hypointense and nonenhancing on T1-magnetization prepared rapid gradient-echo imaging without restricted diffusion, hemorrhage, or supposed malformation along the right pontine trigeminal pathway (PTP). Two other similar lesions were found in the corpus callosum and left thalamus. All lesions were stable compared to MRI data obtained 2 years before. Cerebrospinal fluid contained no oligoclonal bands. Pain attacks ceased with right-sided gasserian radiofrequency thermocoagulation (RFTC), and at the 6-month follow-up, there was no recurrence.

LESSONS

In patients with TN, preoperative neuroimaging should assess for brainstem lesions along the PTP. RFTC can be considered a treatment option in medication-refractory TN associated with a focal pontine lesion.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 23 (Dec 2023)

Open access

Effects of ventro-oral thalamic deep brain stimulation in a patient with musician’s dystonia: illustrative case

Fauve Poncelet, Sara Smeets, Takaomi Taira, Veerle Visser-Vandewalle, Wim Vandenberghe, Jana Peeters, Tine Van Bogaert, and Bart Nuttin

BACKGROUND

Musician’s dystonia is a task-specific focal hand dystonia characterized by involuntary contraction of muscles while playing a musical instrument. Current treatment options are often insufficient.

OBSERVATIONS

We present the effects of ventro-oral thalamic deep brain stimulation in a patient with musician’s dystonia. The patient was a 67-year-old pianist with musician’s dystonia who underwent deep brain stimulation with the ventralis oralis anterior and posterior nuclei of the thalamus as targets. The Tubiana and Chamagne rating scale was used to evaluate the effects of stimulation. The outcome was evaluated independently by four clinicians in a blinded manner at 3 months postoperatively. There was a distinct reduction of symptoms during stimulation. At 15 months postoperatively, the beneficial effect remained. No lasting side effects were observed.

LESSONS

Further studies are warranted to evaluate the safety and long-term efficacy of this treatment modality.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 18 (Oct 2023)

Open access

Pterygoid venous plexus anastomosis in trigeminal percutaneous glycerol rhizotomy: illustrative case

Kevin Cordeiro, Jason Kim, Niall Buckley, Mark Kraemer, Conrad Pun, and Daniel Resnick

BACKGROUND

Percutaneous glycerol rhizotomy (PGR) is a common, effective, and relatively safe treatment for trigeminal neuralgia that is refractory to medical management. Anastomotic skull base venous anatomy should be considered when delivering percutaneous agents.

OBSERVATIONS

The authors report an anastomotic connection, not previously described in the literature, between the bilateral pterygoid venous plexuses upon air injection during PGR with computed tomography (CT) guidance for trigeminal neuralgia. Pertinent microsurgical and skull base venous anatomy is reviewed.

LESSONS

Anastomoses between the pterygoid venous plexuses present a potential passage for materials used during PGR to reach unintended contralateral neurovascular structures. The use of CT guidance may identify this anastomotic connection and decrease the likelihood of an aberrant flow of materials used during the PGR.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 18 (Oct 2023)

Open access

Utilization of three-dimensional fusion images with high-resolution computed tomography angiography for preoperative evaluation of microvascular decompression: patient series

Takamitsu Iwata, Koichi Hosomi, Naoki Tani, Hui Ming Khoo, Satoru Oshino, and Haruhiko Kishima

BACKGROUND

High-resolution computed tomography (CT), outfitted with a 0.25-mm detector, has superior capability for identifying microscopic anatomical structures compared to conventional CT. This study describes the use of high-resolution computed tomography angiography (CTA) for preoperative microvascular decompression (MVD) assessment and explores the potential effectiveness of three-dimensional (3D) image fusion with magnetic resonance imaging (MRI) by comparing it with traditional imaging methods.

OBSERVATIONS

Four patients who had undergone preoperative high-resolution CTA and MRI for MVD at Osaka University Hospital between December 2020 and March 2022 were included in this study. The 3D-reconstructed images and intraoperative findings were compared. One patient underwent conventional CTA, thus allowing for a comparison between high-resolution and conventional CTA in terms of radiation exposure and vascular delineation. Preoperative simulations reflected the intraoperative findings for all cases; small vessel compression of the nerve was identified preoperatively in two cases.

LESSONS

Compared with conventional CTA, high-resolution CTA showed superior vascular delineation with no significant change in radiation exposure. The use of high-resolution CTA with reconstructed 3D fusion images can help to simulate prior MVD. Knowing the location of the nerves and blood vessels can perioperatively guide neurosurgeons.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 9 (Aug 2023)

Open access

Abnormal magnetic resonance imaging signal in the burr hole and the identification of the intracranial infection of a deep brain stimulation device: illustrative case

Ryota Sasaki, Hidehiro Hirabayashi, and Ichiro Nakagawa

BACKGROUND

Device infection is a critical postoperative complication in deep brain stimulation (DBS). However, intracranial infections are rare and lack specific findings, which lead to a challenging diagnosis.

OBSERVATIONS

A 59-year-old female with generalized dystonia underwent bilateral globus pallidus internus and subthalamic nucleus (STN) DBS device implantation. One year earlier, a left STN-DBS extension wire disconnection was observed and replaced. The patient presented to our department because of tenderness along the extension wire that had persisted for 1 month. Magnetic resonance imaging (MRI) of the head indicated abnormal signals around the lead of the left STN and burr hole. Intraoperatively, the authors observed pus and infected granulation tissue in the burr holes. After device removal, antibiotics were administered, and the patient successfully progressed without complications. Moreover, the abnormal MRI signal disappeared.

LESSONS

A characteristic abnormal MRI signal within the burr hole in DBS may suggest early infection even in the absence of other inflammatory findings. Clinicians should ensure that MRI is not limited to intracranial findings but extends beyond the extracranial space.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 8 (Aug 2023)

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.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 8 (Aug 2023)

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.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 7 (Aug 2023)

Open access

Deep brain stimulation in a pediatric dystonia patient with cochlear implants and mitochondrial disorder: novel application of a frameless stereotactic system and navigating the anesthesia choice and neurosurgical complexities. Illustrative case

Audrey Grossen, Helen H. Shi, Mallory Schenk, Amber Stocco, Justin Ramsey, Suneet Sahgal, Andrew K. Conner, and Virendra R. Desai

BACKGROUND

This report presents a case of medically refractory dystonia in a pediatric patient successfully treated with bilateral subthalamic nucleus (STN) deep brain stimulation (DBS) while under general anesthesia by using microelectrode recordings (MERs) with intraoperative computed tomography (CT).

OBSERVATIONS

The patient was an 18-year-old female with primary dystonia secondary to mitochondrial Leigh syndrome. Her past medical history was significant for complex partial epilepsy and hearing loss treated with cochlear implants. Her cochlear implants precluded anatomical targeting via magnetic resonance imaging. Additionally, the patient could not tolerate awake surgery with MER. The decision was made to proceed with bilateral STN DBS with intraoperative CT with the patient under general anesthesia. The patient’s cochlear implants made standard frame placement difficult, so navigation was performed with the Nexframe system. Recordings were obtained with the patient under general anesthesia with ketamine, dexmedetomidine, and remifentanil. At the 3- and 6-month follow-ups, the patient demonstrated marked improvement in dystonia without neurological complications.

LESSONS

This is the first case of dystonia secondary to Leigh syndrome treated with DBS. Additionally, the authors describe the novel use of the Nexframe for DBS lead placement in a pediatric patient. This demonstrates that STN DBS with the use of MER and intraoperative CT can be a safe and effective method of treating dystonia in certain pediatric patients.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 6 (Aug 2023)

Open access

Deep brain stimulation in an adolescent with hypomyelination with atrophy of the basal ganglia and cerebellum due to a TUBB4A mutation: illustrative case

Jennifer A. MacLean, Jaya Nataraj, Joffre Olaya, Mark A. Liker, and Terence D. Sanger

BACKGROUND

Hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC) is a rare genetic disease due to a TUBB4A mutation, with motor features including dystonia. Deep brain stimulation (DBS) can be used to treat dystonia in pediatric populations, although the response is highly variable and preferential toward specific etiologies.

OBSERVATIONS

A single pediatric subject with H-ABC received DBS using a staged procedure involving temporary depth electrode placement, identification of optimal stimulation targets, and permanent electrode implantation. After surgery, the patient significantly improved on both the Burke-Fahn-Marsden Dystonia Rating Scale and the Barry-Albright Dystonia Scale. The patient’s response suggests that DBS can have potential benefit in H-ABC.

LESSONS

TUBB4A mutations are associated with a variety of clinical phenotypes, and there is a lack of clearly identified targets for DBS, with this case being the second reported instance of DBS in this condition. The staged procedure with temporary depth electrode testing is recommended to identify optimal stimulation targets. The response seen in this patient implies that such a staged procedure may provide benefit in other conditions where DBS targets are currently unknown, including rare genetic or metabolic conditions associated with movement disorders.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 2 (Jul 2023)