Awake brain mapping paradigms for nondominant hemisphere gliomas.

OBJECTIVE: Traditionally, resection of nondominant hemisphere brain tumors was performed under general anesthesia. An improved understanding of right-lateralized neural networks has led to a paradigm shift in recent decades, where the right or nondominant hemisphere is no longer perceived as "functionally silent." There is an increasing interest in awake brain mapping for nondominant hemisphere resections. The objective of this study was to perform a comprehensive review of the existing brain mapping paradigms for patients with nondominant hemisphere gliomas undergoing awake craniotomies. METHODS: In accordance with PRISMA guidelines, systematic searches of the Medline, Embase, and American Psychological Association PsycInfo databases were undertaken from database inception to July 1, 2023. Studies providing a description of the intraoperative mapping paradigm used to assess cognition during an awake craniotomy for resection of a nondominant hemisphere glioma were included. RESULTS: The search yielded 1084 potentially eligible articles. Thirty-nine unique studies reporting on 788 patients were included in the systematic review. The most frequently tested cognitive domains in patients with nondominant hemisphere tumors were spatial attention/neglect (17/39 studies, 43.6%), speech-motor/language (17/39 studies, 43.6%), and social cognition (9/39 studies, 23.1%). Within the frontal lobe, the highest number of positive mapping sites was identified for speech-motor/language, spatial attention/neglect, dual tasking assessing motor and language function, working memory, and social cognition. Within the parietal lobe, eloquence was most frequently found upon testing spatial attention/neglect, speech-motor/language, and calculation. Within the temporal lobe, the assessment of spatial attention/neglect yielded the highest number of positive mapping sites. CONCLUSIONS: Cognitive testing in the nondominant hemisphere is predominantly focused on evaluating two domains: spatial attention/neglect and the motor aspects of speech/language. Multidisciplinary teams involved in awake brain mapping should consider testing an extended range of functions to minimize the risk of postoperative deficits and provide valuable information about anatomo-functional organization of cognitive networks.

Differential associations between abnormal cardiac left ventricular geometry types and cerebral white matter disease.

OBJECTIVES: Reduced cardiac outflow due to left ventricular hypertrophy has been suggested as a potential risk factor for development of cerebral white matter disease. Our study aimed to examine the correlation between left ventricular geometry and white matter disease volume to establish a clearer understanding of their relationship, as it is currently not well-established. METHODS: Consecutive patients from 2016 to 2021 who were ≥18 years and underwent echocardiography, cardiac MRI, and brain MRI within one year were included. Four categories of left ventricular geometry were defined based on left ventricular mass index and relative wall thickness on echocardiography. White matter disease volume was quantified using an automated algorithm applied to axial T2 FLAIR images and compared across left ventricular geometry categories. RESULTS: We identified 112 patients of which 34.8 % had normal left ventricular geometry, 20.5 % had eccentric hypertrophy, 21.4 % had concentric remodeling, and 23.2 % had concentric hypertrophy. White matter disease volume was highest in patients with concentric hypertrophy and concentric remodeling, compared to eccentric hypertrophy and normal morphology with a trend-P value of 0.028. Patients with higher relative wall thickness had higher white matter disease volume (10.73 ± 10.29 cc vs 5.89 ± 6.46 cc, P = 0.003), compared to those with normal relative wall thickness. CONCLUSION: Our results showed that abnormal left ventricular geometry is associated with higher white matter disease burden, particularly among those with abnormal relative wall thickness. Future studies are needed to explore causative relationships and potential therapeutic options that may mediate the adverse left ventricular remodeling and its effect in slowing white matter disease progression.

Endoscopic trans-eustachian tube approach: identifying the precise landmarks, a novel radiological and anatomical evaluation.

PURPOSE: The endoscopic trans-eustachian approach (ETETA) is a less invasive approach to the infratemporal fossa (ITF), providing superior exposure compared to traditional transcranial approaches. The anatomy of the pharyngotympanic (eustachian) tube and adjacent neurovascular structures is complex and requires in-depth knowledge to safely perform this approach. We present a cadaveric and radiological assessment of critical anatomic considerations for ETETA. METHODS: Six adult cadaveric heads were dissected alongside examination of 50 paranasal sinus CT scans. Key anatomic relationships of the pharyngotympanic tube and adjacent structures were qualitatively and quantitatively evaluated. Descriptive statistics were performed for quantitative data. RESULTS: Anatomical and radiological measurements showed lateralization of the pharyngotympanic tube allows access to the ITF. The pharyngotympanic tube has bony and cartilaginous parts with the junction formed by the sphenoid spine and foramen spinosum. The bony part and tendon of the tensor tympani muscle were located at the posterior genu of the internal carotid artery. The anterior and inferior wall of the carotid canal was located between the horizontal segment of the internal carotid artery and petrous segment of the cartilaginous pharyngotympanic tube. CONCLUSION: The combination of preoperative radiographic assessment and anatomical correlation demonstrates a safe and effective approach to ETETA, which allowed satisfactory visualization of ITF. The morphological evaluation showed that the lateralization of the pharyngotympanic tube and related structures allowed a surgical corridor to reach the ITF. Endoscopic surgery through the pharyngotympanic tube is challenging, and in-depth understanding of the key anatomic relationships is critical for performing this approach.

Personalizing Deep Brain Stimulation Using Advanced Imaging Sequences.

OBJECTIVE: With a growing appreciation for interindividual anatomical variability and patient-specific brain connectivity, advanced imaging sequences offer the opportunity to directly visualize anatomical targets for deep brain stimulation (DBS). The lack of quantitative evidence demonstrating their clinical utility, however, has hindered their broad implementation in clinical practice. METHODS: Using fast gray matter acquisition T1 inversion recovery (FGATIR) sequences, the present study identified a thalamic hypointensity that holds promise as a visual marker in DBS. To validate the clinical utility of the identified hypointensity, we retrospectively analyzed 65 patients (26 female, mean age = 69.1 ± 12.7 years) who underwent DBS in the treatment of essential tremor. We characterized its neuroanatomical substrates and evaluated the hypointensity's ability to predict clinical outcome using stimulation volume modeling and voxelwise mapping. Finally, we determined whether the hypointensity marker could predict symptom improvement on a patient-specific level. RESULTS: Anatomical characterization suggested that the identified hypointensity constituted the terminal part of the dentatorubrothalamic tract. Overlap between DBS stimulation volumes and the hypointensity in standard space significantly correlated with tremor improvement (R2  = 0.16, p = 0.017) and distance to hotspots previously reported in the literature (R2  = 0.49, p = 7.9e-4). In contrast, the amount of variance explained by other anatomical atlas structures was reduced. When accounting for interindividual neuroanatomical variability, the predictive power of the hypointensity increased further (R2  = 0.37, p = 0.002). INTERPRETATION: Our findings introduce and validate a novel imaging-based marker attainable from FGATIR sequences that has the potential to personalize and inform targeting and programming in DBS for essential tremor. ANN NEUROL 2022;91:613-628.

Incidence and risk factors associated with seizures in cerebral amyloid angiopathy.

BACKGROUND AND PURPOSE: Cerebral amyloid angiopathy (CAA) is a common cause of intracranial hemorrhage (ICH), which is a risk factor for seizures. The incidence and risk factors of seizures associated with a heterogeneous cohort of CAA patients have not been studied. METHODS: We conducted a retrospective study of patients with CAA treated at Mayo Clinic Florida between 1 January 2015 and 1 January 2021. CAA was defined using the modified Boston criteria version 2.0. We analyzed electrophysiological and clinical features, and comorbidities including lobar ICH, nontraumatic cortical/convexity subarachnoid hemorrhage (cSAH), superficial siderosis, and inflammation (CAA with inflammation [CAA-ri]). Cognition and mortality were secondary outcomes. Univariate and multivariate analyses were performed to determine risk of seizures relative to clinical presentation. RESULTS: Two hundred eighty-four patients with CAA were identified, with median follow-up of 35.7 months (interquartile range = 13.5-61.3 months). Fifty-six patients (19.7%) had seizures; in 21 (37.5%) patients, seizures were the index feature leading to CAA diagnosis. Seizures were more frequent in females (p = 0.032) and patients with lobar ICH (p = 0.002), cSAH (p = 0.030), superficial siderosis (p < 0.001), and CAA-ri (p = 0.005), and less common in patients with microhemorrhage (p = 0.006). After controlling for age and sex, lobar ICH (odds ratio [OR] = 2.1, 95% confidence interval [CI] = 1.1-4.2), CAA-ri (OR = 3.8, 95% CI = 1.4-10.3), and superficial siderosis (OR = 3.7, 95% CI = 1.9-7.0) were independently associated with higher odds of incident seizures. CONCLUSIONS: Seizures are common in patients with CAA and are independently associated with lobar ICH, CAA-ri, and superficial siderosis. Our results may be applied to optimize clinical monitoring and management for patients with CAA.

Synthetic Inversion Image Generation using MP2RAGE T1 Mapping for Surgical Targeting in Deep Brain Stimulation and Lesioning.

BACKGROUND: Advances in MRI technology have increased interest in direct targeting for deep brain stimulation (DBS). Various imaging sequences have been shown to provide increased contrast of numerous common DBS targets, such as T1-weighted, Fast Gray Matter Acquisition T1 Inversion Recovery (FGATIR), gray matter nulled, and Edge-Enhancing Gradient Echo (EDGE); however, the continual increase in the number of necessary sequences has led to an increase in imaging time, which is undesirable. Additionally, carefully timed inversion pulses can often lead to less-than-ideal contrast in some subjects, particularly in ultra-high field MRI, where B1+ field inhomogeneity can lead to substantial contrast variation. OBJECTIVES: This study proposes using 3D MP2RAGE-based T1 maps to retrospectively synthesize images of any desired inversion time, including T1-weighted, FGATIR, and EDGE contrasts, to visualize specific DBS targets at both 3T and 7T. METHOD: First, a systematic sequence optimization framework was applied to optimize MP2RAGE T1 mapping sequence parameters for the purpose of DBS planning. Next, we show that synthetic inversion-time images can be generated through a mathematical transformation of the T1 maps. The sequence was then applied to patients undergoing preoperative planning for DBS at 3T and 7T to generate synthetic contrasts used in surgical planning. RESULTS: We show that synthetic image contrasts can be generated across a full range of inversion times at 3T and 7T, including commonly used sequences for DBS targeting, such as T1-weighted, FGATIR, and EDGE. Acquisition through a single sequence shortens scan time compared to acquiring the sequences independently without affecting image quality or contrast. CONCLUSIONS: The generation of synthetic images for DBS targeting allows faster acquisition of many key sequences, as well as the ability to optimize contrast properties post-acquisition to account for the variable B1+ effects present in ultra-high field MRI. The proposed approach has the potential to reduce imaging time and improve the accuracy of DBS targeting at 1.5T, 3T, and 7T.

Current advances in the surgical treatment of glossopharyngeal neuralgia.

Glossopharyngeal neuralgia (GPN) is a neurological condition characterized by paroxysmal, stabbing-like pain along the distribution of the glossopharyngeal nerve that lasts from a couple of seconds to minutes. Pharmacological treatment with anticonvulsants is the first line of treatment; however, about 25% of patients remain symptomatic and require surgical intervention, which is usually done via microvascular decompression (MVD) with or without rhizotomy. More recently, the use of stereotactic radiosurgery (SRS) has been utilized as an alternative treatment method to relieve patient symptoms by causing nerve ablation. We conducted a systematic review to analyze whether MVD without rhizotomy is an equally effective treatment for GPN as MVD with the use of concurrent rhizotomy. Moreover, we sought to explore if SRS, a minimally invasive alternative surgical option, achieves comparable outcomes. We included retrospective studies and case reports in our search. We consulted PubMed and Medline, including articles from the year 2000 onwards. A total of 36 articles were included for review. Of all included patients with glossopharyngeal neuralgia, the most common offending artery compressing the glossopharyngeal nerve was the posterior inferior cerebellar artery (PICA). MVD alone was successful achieving pain relief immediately postoperatively in about 85% of patients, and also long term in 65-90% of patients. The most common complication found on MVD surgery was found to be transient hoarseness and transient dysphagia. Rhizotomy alone shows an instant pain relief in 85-100% of the patients, but rate of long-term pain relief was lower compared to MVD. The most common adverse effects observed after a rhizotomy were dysphagia and dysesthesia along the distribution of the glossopharyngeal nerve. SRS had promising results in pain reduction when using 75 Gy radiation or higher; however, long-term rates of pain relief were lower. MVD, rhizotomy, and SRS are effective methods to treat GPN as they help achieve instant pain relief and the decrease use of medication. Patients with MVD alone presented with less adverse effects than the group that underwent MVD plus rhizotomy. Although SRS may be a viable alternative treatment for GPN, further studies must be done to evaluate long-term treatment efficacy.

Flow diversion using the Pipeline embolization device for intracranial and extracranial pseudoaneurysms: a systematic review and meta-analysis of the literature.

OBJECTIVE: Pseudoaneurysms (PSAs) are complex vascular lesions. Flow diversion has been proposed as an alternative treatment to parent artery occlusion that preserves laminar flow. The authors of the present study investigated the safety and short-term (< 1 year) and long-term (≥ 1 year) aneurysm occlusion rates following the treatment of intracranial and extracranial PSAs using the Pipeline embolization device (PED). METHODS: An electronic database search for full-text English-language articles in Ovid MEDLINE and Epub Ahead of Print, Ovid Embase, Ovid Cochrane Central Register of Controlled Trials, Ovid Cochrane Database of Systematic Reviews, and Scopus was conducted following the PRISMA guidelines. Studies of any design including at least 4 patients with intracranial or extracranial PSAs treated using a PED were included in this analysis. The primary outcome of interest was the rate of peri- and postprocedural complications. Secondarily, the authors analyzed the incidence of complete aneurysm occlusion. RESULTS: A total of 90 patients with 96 PSAs across 9 studies were included. The mean age was 38.2 (SD 15.14) years, and 37.8% of the patients were women. The mean PSA size was 4.9 mm. Most PSAs were unruptured, and the most common etiology was trauma (n = 32, 35.5%), followed by spontaneous formation (n = 21, 23.3%) and iatrogenic injury (n = 19, 21.1%). Among the 51 (53.1%) intracranial and 45 (46.9%) extracranial PSAs were 19 (19.8%) dissecting PSAs. Sixty-six (77.6%) PSAs were in the internal carotid artery and 10 (11.8%) in the vertebral artery. Thirty-three (34.4%) PSAs were treated with ≥ 2 devices, and 8 (8.3%) underwent adjunctive coiling. The mean clinical and angiographic follow-up durations were 10.7 and 12.9 months, respectively. The short-term (< 1 year) and long-term (≥ 1 year) complete occlusion rates were 79% (95% CI 66%-88%, p = 0.82) and 84% (95% CI 70%-92%, p = 0.95), respectively. Complication rates were 8% for iatrogenic dissection (95% CI 3%-16%, p = 0.94), 10% for silent thromboembolism (95% CI 5%-21%, p = 0.77), and 12% for symptomatic thromboembolism (95% CI 6%-23%, p = 0.48). No treatment-related hemorrhage was observed. The overall mortality rate at the last follow-up was 14%. CONCLUSIONS: The complete occlusion rate for PSAs treated with the PED was high and increased over time. Although postprocedural complications and mortality were not insignificant, flow diversion represents a reasonably safe option for managing these complex lesions.

Greater burden of white matter lesions and silent infarcts ipsilateral to carotid stenosis.

OBJECTIVES: Carotid stenosis may cause silent cerebrovascular disease (CVD) through atheroembolism and hypoperfusion. If so, revascularization may slow progression of silent CVD. We aimed to compare the presence and severity of silent CVD to the degree of carotid bifurcation stenosis by cerebral hemisphere. MATERIALS AND METHODS: Patients age ≥40 years with carotid stenosis >50% by carotid ultrasound who underwent MRI brain from 2011-2015 at Mayo Clinic were included. Severity of carotid stenosis was classified by carotid duplex ultrasound as 50-69% (moderate), 70-99% (severe), or occluded. White matter lesion (WML) volume was quantified using an automated deep-learning algorithm applied to axial T2 FLAIR images. Differences in WML volume and prevalent silent infarcts were compared across hemispheres and severity of carotid stenosis. RESULTS: Of the 183 patients, mean age was 71±10 years, and 39.3% were female. Moderate stenosis was present in 35.5%, severe stenosis in 46.5% and occlusion in 18.0%. Patients with carotid stenosis had greater WML volume ipsilateral to the side of carotid stenosis than the contralateral side (mean difference, 0.42±0.21cc, p=0.046). Higher degrees of stenosis were associated with greater hemispheric difference in WML volume (moderate vs. severe; 0.16±0.27cc vs 0.74±0.31cc, p=0.009). Prevalence of silent infarct was 23.5% and was greater on the side of carotid stenosis than the contralateral side (hemispheric difference 8.8%±3.2%, p=0.006). Higher degrees of stenosis were associated with higher burden of silent infarcts (moderate vs severe, 10.8% vs 31.8%; p=0.002). CONCLUSIONS: WML and silent infarcts were greater on the side of severe carotid stenosis.

Optimization of fast gray matter acquisition T1 inversion recovery (FGATIR) on 7T MRI for deep brain stimulation targeting.

Deep brain stimulation (DBS) is an increasingly utilized treatment for multiple neurological disorders. Continued improvements in DBS outcome are, in part, related to increasing ability to directly visualize stimulation targets by MRI. However, it is challenging to image DBS targets with conventional MRI techniques due to limited contrast. Fast Gray Matter Acquisition T1 Inversion Recovery (FGATIR) is a commonly used MRI sequence that improves visualization of several key DBS targets by suppressing white matter (WM) signal to better reveal deep-brain gray matter (GM) structures. Due to increased signal level at high field strength, application of FGATIR on 7T MRI may allow higher spatial resolution and better DBS targeting accuracy. However, successful utilization of FGATIR requires meticulous sequence optimization involving multiple parameters to maximize GM signal while suppressing WM. This is further complicated by the transmit RF field (B1+) inhomogeneity on 7T, which can cause severe contrast degradation. In this work, we introduce a systematic approach to optimize FGATIR and to improve visualization of thalamic DBS targets on 7T. FGATIR optimization is cast into a constrained optimization problem whose objective function and constraints are designed to maximize the GM-WM contrast-to-noise ratio (CNR) while accounting for B1+ inhomogeneity. This approach allows a systematic search for optimal parameters across the multi-dimensional parametric space while limiting the negative effect of B1+ variation. Bloch equation simulations were performed to solve the proposed optimization problem and to compare the sequence derived from this method against the sequence optimized without considering B1+ inhomogeneity. The results showed that this approach can improve GM-WM CNR in the presence of B1+ inhomogeneity, especially in some high relative B1+ areas where several key thalamic DBS targets are located. Additionally, in vivo images were acquired on a clinical 7T MRI to further validate this approach. Severe contrast degradation in the thalamus was observed when B1+ effect was not considered in sequence optimization, while the proposed approach yielded improved image contrast in the thalamus with key DBS targets well-defined. These results demonstrated that the proposed method allowed optimization of FGATIR on 7T to better visualize thalamic DBS targets, which may lead to improved DBS targeting accuracy as well as treatment outcome.

MR Intracranial Venography at 7 T.

Online supplemental material is available for this article.

Mesenchymal stem cell therapy for focal epilepsy: A systematic review of preclinical models and clinical studies.

Drug-resistant epilepsy (DRE) is characterized by recurrent seizures despite appropriate treatment with antiseizure medication (ASM). Due to their regenerative and immunomodulatory potential, therapies with biologics such as mesenchymal stem cells (MSCs) offer a potential therapeutic benefit for structural causes of epilepsy, such as hippocampal sclerosis. In this article, we report a systematic review of the literature evaluating the preclinical and clinical studies of MSCs for DRE. Medline, Ovid EMBASE, Scopus, and the Cochrane Databases were searched electronically from their dates of inception to November 2021 using the following keywords: (("mesenchymal") AND ("stem cell")) AND (("epilepsy") OR ("convulsion") OR ("seizures")). This review followed Preferred Reporting Items for Systemic Reviews and Meta-Analyses (PRISMA) guidelines. The initial query identified 488 studies representing 323 unique manuscripts. After application of selection criteria, 15 studies were included in this systematic review; 11 were preclinical studies and 4 were clinical studies. All preclinical studies were performed in rodents and all clinical studies were phase 1 trials. Thus far, therapy with MSCs appears to be safe for use in humans, as no severe adverse events related directly to the therapy were reported. Furthermore, MSC therapy appears to provide a statistically significant clinical benefit by reducing the seizure burden of patients, reducing the electrophysiological biomarkers of epilepsy, and improving their comorbidities, such as depression and anxiety. In addition, animal studies reveal that the therapy exerts its effect by reducing aberrant mossy fiber sprouting (reduce excitatory pathways) and increasing γ-aminobutyric acid (GABA)ergic interneurons (increase inhibitory pathways). Both preclinical and clinical studies have shown MSC therapy to be safe and preliminary effective, thus warranting further studies to investigate its therapeutic potential.

The early infiltrative phase of GBM hypothesis: are molecular glioblastomas histological glioblastomas in the making? A preliminary multicenter study.

PURPOSE: The presence of necrosis or microvascular proliferation was previously the hallmark for glioblastoma (GBM) diagnosis. The 2021 WHO classification now considers IDH-wildtype diffuse astrocytic tumors without the histological features of glioblastoma (that would have otherwise been classified as grade 2 or 3) as molecular GBM (molGBM) if they harbor any of the following molecular abnormalities: TERT promoter mutation, EGFR amplification, or chromosomal + 7/-10 copy changes. We hypothesize that these tumors are early histological GBM and will eventually develop the classic histological features. METHODS: Medical records from 65 consecutive patients diagnosed with molGBM at three tertiary-care centers from our institution were retrospectively reviewed from November 2017-October 2021. Only patients who underwent reoperation for tumor recurrence and whose tissue at initial diagnosis and recurrence was available were included in this study. The detailed clinical, histopathological, and radiographic scenarios are presented. RESULTS: Five patients were included in our final cohort. Three (60%) patients underwent reoperation for recurrence in the primary site and 2 (40%) underwent reoperation for distal recurrence. Microvascular proliferation and pseudopalisading necrosis were absent at initial diagnosis but present at recurrence in 4 (80%) patients. Radiographically, all tumors showed contrast enhancement, however none of them showed the classic radiographic features of GBM at initial diagnosis. CONCLUSIONS: In this manuscript we present preliminary data for a hypothesis that molGBMs are early histological GBMs diagnosed early in their natural history of disease and will eventually develop necrosis and microvascular proliferation. Further correlative studies are needed in support of this hypothesis.

The survival outcomes of molecular glioblastoma IDH-wildtype: a multicenter study.

PURPOSE: Histological diagnosis of glioblastoma (GBM) was determined by the presence of necrosis or microvascular proliferation (histGBM). The 2021 WHO classification now considers IDH-wildtype diffuse astrocytic tumors without the histological features of glioblastoma (that would have otherwise been classified as grade 2 or 3) as molecular GBM (molGBM, WHO grade 4) if they harbor any of the following molecular abnormalities: TERT promoter mutation, EGFR amplification, or chromosomal + 7/- 10 copy changes. The objective of this study was to explore and compare the survival outcomes between histGBM and molGBM. METHODS: Medical records for patients diagnosed with GBM at the three tertiary care academic centers of our institution from November 2017 to October 2021. Only patients who underwent adjuvant chemoradiation were included. Patients without molecular feature testing or with an IDH mutation were excluded. Univariable and multivariable analyses were performed to evaluate progression-free (PFS) and overall- survival (OS). RESULTS: 708 consecutive patients were included; 643 with histGBM and 65 with molGBM. Median PFS was 8 months (histGBM) and 13 months (molGBM) (p = 0.0237) and median OS was 21 months (histGBM) versus 26 months (molGBM) (p = 0.435). Multivariable analysis on the molGBM sub-group showed a worse PFS if there was contrast enhancement on MRI (HR 6.224 [CI 95% 2.187-17.714], p < 0.001) and a superior PFS on patients with MGMT methylation (HR 0.026 [CI 95% 0.065-0.655], p = 0.007). CONCLUSIONS: molGBM has a similar OS but significantly longer PFS when compared to histGBM. The presence of contrast enhancement and MGMT methylation seem to affect the clinical behavior of this subset of tumors.

Neuroradiology in Transgender Care: Facial Feminization, Laryngeal Surgery, and Beyond.

Transgender individuals experience incongruence between their gender identity and the sex assigned to them at birth. This incongruence can cause many transgender individuals to experience distressing physical and mental discord, a diagnosis known as gender dysphoria. Craniofacial structures have distinct anthropometric characteristics that affect perceived masculinity and femininity. The face, neck, and voice are highly exposed anatomic areas that have recognizable gender-specific characteristics that may hinder a transgender individual's successful social integration and public acceptance. Reconstructive facial and laryngeal procedures are among the surgical options transgender persons may elect to undergo to better align their physical appearance with their gender identity. These include feminization surgeries such as facial feminization and reduction chondrolaryngoplasty, as well as masculinizing facial and laryngeal surgeries. Maxillofacial CT is frequently used in the preoperative evaluation of patients before facial feminization surgery (FFS). Several CT measurements guide surgeons to the optimal correction required in FFS to achieve appropriate aesthetic planes. Mapping important craniofacial landmarks to avoid untoward surgical complications is crucial. Transgender patients may encounter other neurologic complications that require neuroimaging evaluation. For example, gender-affirming hormone therapy (eg, estrogen and testosterone) may increase the risk of stroke or may influence growth of various hormone-sensitive tumors such as pituitary adenomas. Radiologists may interpret imaging examinations in transgender patients for routine care or for evaluation before and after facial and laryngeal surgeries and must be aware of the role of neuroimaging in the care of this population. An invited commentary by Callen is available online. The online slide presentation from the RSNA Annual Meeting is available for this article. ©RSNA, 2022.

Comparative connectivity correlates of dystonic and essential tremor deep brain stimulation.

The pathophysiology of dystonic tremor and essential tremor remains partially understood. In patients with medication-refractory dystonic tremor or essential tremor, deep brain stimulation (DBS) targeting the thalamus or posterior subthalamic area has evolved into a promising treatment option. However, the optimal DBS targets for these disorders remains unknown. This retrospective study explored the optimal targets for DBS in essential tremor and dystonic tremor using a combination of volumes of tissue activated estimation and functional and structural connectivity analyses. We included 20 patients with dystonic tremor who underwent unilateral thalamic DBS, along with a matched cohort of 20 patients with essential tremor DBS. Tremor severity was assessed preoperatively and approximately 6 months after DBS implantation using the Fahn-Tolosa-Marin Tremor Rating Scale. The tremor-suppressing effects of DBS were estimated using the percentage improvement in the unilateral tremor-rating scale score contralateral to the side of implantation. The optimal stimulation region, based on the cluster centre of gravity for peak contralateral motor score improvement, for essential tremor was located in the ventral intermediate nucleus region and for dystonic tremor in the ventralis oralis posterior nucleus region along the ventral intermediate nucleus/ventralis oralis posterior nucleus border (4 mm anterior and 3 mm superior to that for essential tremor). Both disorders showed similar functional connectivity patterns: a positive correlation between tremor improvement and involvement of the primary sensorimotor, secondary motor and associative prefrontal regions. Tremor improvement, however, was tightly correlated with the primary sensorimotor regions in essential tremor, whereas in dystonic tremor, the correlation was tighter with the premotor and prefrontal regions. The dentato-rubro-thalamic tract, comprising the decussating and non-decussating fibres, significantly correlated with tremor improvement in both dystonic and essential tremor. In contrast, the pallidothalamic tracts, which primarily project to the ventralis oralis posterior nucleus region, significantly correlated with tremor improvement only in dystonic tremor. Our findings support the hypothesis that the pathophysiology underpinning dystonic tremor involves both the cerebello-thalamo-cortical network and the basal ganglia-thalamo-cortical network. Further our data suggest that the pathophysiology of essential tremor is primarily attributable to the abnormalities within the cerebello-thalamo-cortical network. We conclude that the ventral intermediate nucleus/ventralis oralis posterior nucleus border and ventral intermediate nucleus region may be a reasonable DBS target for patients with medication-refractory dystonic tremor and essential tremor, respectively. Uncovering the pathophysiology of these disorders may in the future aid in further improving DBS outcomes.

Examining the role of the uncinate fasciculus in proper noun naming: awake brain tumor resections and stereo EEG targeted electrical stimulation multiple case study.

While there is strong evidence from lesion and functional imaging studies implicating the left anterior temporal pole (LTP) in naming unique entities, less is known about white matter tracts in category-specific naming. We present evidence that implicates the uncinate fasciculus (UF) in proper noun naming. First, we describe two patients with left LTP gliomas who developed category specific worsening in proper noun naming in real time during awake surgery when the UF was surgically involved . We then describe a third case involving targeted electrical stimulation of the UF using stereo-electroencephalography (sEEG) that resulted in category specific naming disturbance for proper nouns..

Motor Cortex Stimulation for Pain: A Narrative Review of Indications, Techniques, and Outcomes.

BACKGROUND: Motor cortex stimulation (MCS) was introduced in 1985 and has been tested extensively for different types of peripheral and central neuropathic pain syndromes (eg, central poststroke pain, phantom limb pain, trigeminal neuropathic pain, migraines, etc). The motor cortex can be stimulated through different routes, including subdural, epidural, and transcranial. OBJECTIVES: In this review, we discuss the current uses, surgical techniques, localization techniques, stimulation parameters, and clinical outcomes of patients who underwent chronic MCS for treatment-resistant pain syndromes. MATERIALS AND METHODS: A broad literature search was conducted through PubMed to include all articles focusing on MCS for pain relief (keywords: subdural, epidural, repetitive transcranial magnetic stimulation, transcranial direct current stimulation, motor cortex stimulation, pain). LITERATURE REVIEW: Epidural MCS was the most widely used technique and had varying response rates across studies. Long-term efficacy was limited, and pain relief tended to decrease over time. Subdural MCS using similar stimulation parameters demonstrated similar efficacy to epidural stimulation and less invasive methods, such as repetitive transcranial magnetic stimulation (rTMS), which have been shown to provide adequate pain relief. rTMS and certain medications (ketamine and morphine) have been shown to predict the long-term response to epidural MCS. Complications tend to be rare, the most reported being seizures during subdural or epidural stimulation or hardware infection. CONCLUSIONS: Scientific evidence supports the use of MCS for treatment of refractory neuropathic pain syndromes. Further studies are warranted to elucidate the specific indications and stimulation protocols that are most amenable to the different types of MCS.

Treatment of CSF1R-Related Leukoencephalopathy: Breaking New Ground.

BACKGROUND: Colony-stimulating factor-1 receptor (CSF1R)-related leukoencephalopathy is a rapidly progressive neurodegenerative disease for which there is currently no cure. Hematopoietic stem cell transplantation (HSCT) has been proposed as a disease-modifying treatment. OBJECTIVE: The objective of this study was to determine the effect of HSCT on disease progression. METHODS: We collected all available clinical data from a cohort of 7 patients with CSF1R-related leukoencephalopathy who underwent HSCT at our institutions. Clinical data included detailed neurological examination by a board-certified neurologist, serial cognitive screens, formal neuropsychological evaluations, and serial brain magnetic resonance imaging (MRI). RESULTS: Our patients had an average disease duration of 27.6 months at the time of transplant, and we have 87 months of total posttransplant follow-up time (median, 11; range, 2-27). One patient died in the periprocedural period. The remaining patients showed a variable response to treatment, with 6 of 7 patients trending toward stabilization on motor examination, cognitive scores, and/or MRI abnormalities, especially with white matter lesion burden. CONCLUSIONS: This is the largest series of patients with CSF1R-related leukoencephalopathy receiving HSCT. We conclude that HSCT can stabilize the disease in some patients. Variability in patient responsiveness suggests that measures of disease heterogeneity and severity need to be considered when evaluating a patient's candidacy for transplant. HSCT appears to be the first disease-modifying therapy for CSF1R-related leukoencephalopathy. This milestone may serve as a foothold toward better understanding the disease's pathomechanism, thus providing new opportunities for better disease-specific therapies. © 2021 International Parkinson and Movement Disorder Society.

Pallidal Connectivity Profiling of Stimulation-Induced Dyskinesia in Parkinson's Disease.

OBJECTIVES: The aim of this study is to identify anatomical regions related to stimulation-induced dyskinesia (SID) after pallidal deep brain stimulation (DBS) in Parkinson's disease (PD) patients and to analyze connectivity associated with SID. METHODS: This retrospective study analyzed the clinical and imaging data of PD patients who experienced SID during the monopolar review after pallidal DBS. We analyzed structural and functional connectivity using normative connectivity data with the volume of tissue activated (VTA) modeling. Each contact was assigned to either that producing SID (SID VTA) or that without SID (non-SID VTA). Structural and functional connectivity was compared between SID and non-SID VTAs. "Optimized VTAs" were also estimated using the DBS settings at 6 months after implantation. RESULTS: Of the 68 consecutive PD patients who underwent pallidal implantation, 20 patients (29%) experienced SID. SID VTAs were located more dorsally and anteriorly compared with non-SID and optimized VTAs and were primarily in the dorsal globus pallidus internus (GPi) and dorsal globus pallidus externus (GPe). SID VTAs showed significantly higher structural connectivity than non-SID VTAs to the associative cortex and supplementary motor area/premotor cortex (P < 0.0001). Simultaneously, non-SID VTAs showed greater connectivity to the primary sensory cortex, cerebellum, subthalamic nucleus, and motor thalamus (all P < 0.0004). Functional connectivity analysis showed significant differences between SID and non-SID VTAs in multiple regions, including the primary motor, premotor, and prefrontal cortices and cerebellum. CONCLUSION: SID VTAs were primarily in the dorsal GPi/GPe. The connectivity difference between the motor-related cortices and subcortical regions may explain the presence and absence of SID. © 2020 International Parkinson and Movement Disorder Society.