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BACKGROUND: Trigeminal schwannoma is a rare type of tumor that arises from the Schwann cells of the trigeminal nerve. METHOD: We present a case of a patient with a giant V2 trigeminal schwannoma with painful swelling in the left maxilla. A complete resection using a combined open maxillectomy and endoscopic endonasal approach was performed. CONCLUSION: This case highlights the importance of a multidisciplinary approach to perform a combined open and endoscopic approach for safe resection while preserving adequate speech and swallowing.

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BACKGROUND: 3D-Slicer is an open-source medical image processing and visualization software. In the surgical treatment of trigeminal neuralgia, it is commonly used to predict the responsible vessels. However, there are few reports on the use of 3D-Slicer software to quantitatively measure the bilateral trigeminal nerve volume in patients with primary trigeminal neuralgia (PTN) based on the three-dimensional images. Therefore, this study aims to explore the role of three-dimensional fused images processed by 3D-Slicer in the evaluation of trigeminal nerve atrophy, providing an objective basis for the diagnosis of PTN. METHODS: 57 PTN patients who underwent microvascular decompression (MVD) or percutaneous balloon compression (PBC) surgery in Hebei general hospital between January 2020 and April 2023 were included. Additionally, 30 patients with facial spasms(HFS) were included as a control group. All patients underwent 3D-TOF-MRA and 3D-FIESTA sequence examinations. Comparisons of bilateral trigeminal nerve volumes within and between groups were conducted by performing image fusion using 3D-slicer. RESULTS: The volume of the affected trigeminal nerve in the MVD group (33.96 mm³±12.61 mm³) and PBC group (23.05 mm³±7.71 mm³) was smaller than that of the unaffected trigeminal nerve in the MVD group (39.61 mm³±12.83 mm³) and PBC group (26.14 mm³±6.42 mm³), as well as the average volume of the trigeminal nerve in the control group (40.27 mm³±10.25 mm³) (P<0.05). The differences in bilateral trigeminal ganglion volume (∆V) was significant between the MVD group (∆V=23.59 %±14.32 %) and the control group (∆V=14.64 %±10.00 %) (P<0.05). There was no statistical difference in the trigeminal nerve volume difference between the MVD group (∆V=23.59 %±14.32 %) and the PBC group (∆V=26.52 %±15.00 %) (P>0.05). CONCLUSION: Trigeminal nerve atrophy is correlated with primary trigeminal neuralgia. 3D-slicer software can quantitatively measure trigeminal nerve volume and assist in the diagnosis of primary trigeminal neuralgia based on the difference in bilateral trigeminal nerve volumes. However, trigeminal nerve atrophy is not associated with postoperative pain recurrence in patients.

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BACKGROUND: A schwannoma is a nerve sheath tumor that is formed by Schwann cells. Vestibular schwannomas are thought to account for the majority of intracranial schwannomas. Nonvestibular schwannomas account for about 10%, about half of which are trigeminal schwannomas. Multiple intracranial schwannomas originating from different cranial nerves are extremely rare. METHODS: We describe the clinical case of a 42-year-old female patient with vestibular schwannoma and multiple trigeminal schwannomas. RESULTS: That case shows how multiple trigeminal schwannomas were identified intraoperatively during elective surgery for vestibular schwannoma removal, most of which were resected. No new neurological deficits were observed in the patient. CONCLUSIONS: The presence of multiple intracranial schwannomas is extremely rare in neurosurgical practice and can change the intraoperative strategy and the course of the surgery.

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Magnetic resonance-diffusion tensor imaging (MR-DTI) has been used in the microvascular decompression and gamma knife radiosurgery in trigeminal neuralgia (TN) patients; however, use of percutaneous stereotactic radiofrequency rhizotomy (PSR) to target an abnormal trigeminal ganglion (ab-TG) is unreported. Fractional anisotropy (FA), mean and radial diffusivity (MD and RD, respectively), and axial diffusivity (AD) of the trigeminal nerve (CNV) were measured in 20 TN patients and 40 healthy control participants immediately post PSR, at 6-months, and at 1 year. Longitudinal alteration of the diffusivity metrics and any correlation with treatment effects, or prognoses, were analyzed. In the TN group, either low FA (value < 0.30) or a decreased range compared to the adjacent FA (dFA) > 17% defined an ab-TG. Two-to-three days post PSR, all 15 patients reported decreased pain scores with increased FA at the ab-TG (P < 0.001), but decreased MD and RD (P < 0.01 each). Treatment remained effective in 10 of 14 patients (71.4%) and 8 of 12 patients (66.7%) at the 6-month and 1-year follow-ups, respectively. In patients with ab-TGs, there was a significant difference in treatment outcomes between patients with low FA values (9 of 10; 90%) and patients with dFA (2 of 5; 40%) (P < 0.05). MR-DTI with diffusivity metrics correlated microstructural CNV abnormalities with PSR outcomes. Of all the diffusivity metrics, FA could be considered a novel objective quantitative indicator of treatment effects and a potential indicator of PSR effectiveness in TN patients.

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BACKGROUND: Although trigeminal nerve involvement is a characteristic of multiple sclerosis (MS), its prevalence across studies varies greatly due to MRI resolution and cohort selection bias. The mechanism behind the site specificity of trigeminal nerve injury is still unclear. We aim to determine the prevalence of trigeminal nerve involvement in patients with MS in a consecutive 7T brain MRI cohort. METHODS: This observational cohort originates from an ongoing China National Registry of Neuro-Inflammatory Diseases. Inclusion criteria were the following: age 18 years or older, diagnosis of MS according to the 2017 McDonald criteria and no clinical relapse within the preceding 3 months. Each participant underwent 7T MAGNETOM Terra scanner (Siemens, Erlangen, Germany), using a 32-channel phased array coil at Beijing Tiantan Hospital. T1-weighted magnetisation-prepared rapid acquisition gradient echoes, fluid-attenuated inversion recovery (FLAIR) and fluid and white matter suppression images were used to identify lesions. FLAIR* and T2* weighted images were used to identify central vein sign (CVS) within the trigeminal lesions. RESULTS: 120 patients underwent 7T MRI scans between December 2021 and May 2023. 19/120 (15.8%) patients had a total of 45 trigeminal lesions, of which 11/19 (57.9%) were bilateral. The linear lesions extended along the trigeminal nerve, from the root entry zone (REZ) (57.8%, 26/45) to the pontine-medullary nucleus (42.2%, 19/45). 26.9% (7/26) of the lesions in REZ showed a typical central venous sign. CONCLUSION: In this 7T MRI cohort, the prevalence of trigeminal nerve involvement was 15.8%. Characteristic CVS was detected in 26.9% of lesions in REZ. This suggests an inflammatory demyelination mechanism of trigeminal nerve involvement in MS.

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Trigeminal neuralgia is a heterogeneous disorder with likely multifactorial and complex etiology; however, trigeminal nerve demyelination and injury are observed in almost all patients with trigeminal neuralgia. The current management strategies for trigeminal neuralgia primarily involve anticonvulsants and surgical interventions, neither of which directly address demyelination, the pathological hallmark of trigeminal neuralgia, and treatments targeting demyelination are not available. Demyelination of the trigeminal nerve has been historically considered a secondary effect of vascular compression, and as a result, trigeminal neuralgia is not recognized nor treated as a primary demyelinating disorder. In this article, we review the evolution of our understanding of trigeminal neuralgia and provide evidence to propose its potential categorization, at least in some cases, as a primary demyelinating disease by discussing its course and similarities to multiple sclerosis, the most prevalent central nervous system demyelinating disorder. This proposed categorization may provide a basis in investigating novel treatment modalities beyond the current medical and surgical interventions, emphasizing the need for further research into demyelination of the trigeminal sensory pathway in trigeminal neuralgia. PERSPECTIVE: This article proposes trigeminal neuralgia as a demyelinating disease, supported by histological, clinical, and radiological evidence. Such categorization offers a plausible explanation for controversies surrounding trigeminal neuralgia. This perspective holds potential for future research and developing therapeutics targeting demyelination in the condition.

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OBJECTIVE: High-resolution magnetic resonance imaging (MRI) of the trigeminal nerve is indispensable for workup of trigeminal neuralgia (TN) before microvascular decompression; however, the evaluation is often subjective and prone to variability. We aim to develop and assess sequential thresholding-based automated reconstruction of the trigeminal nerve (STAR-TN) as an algorithm for segmenting the trigeminal nerve and contacting structures that will allow for a structured method for assessing neurovascular conflict. METHODS: A total of 42 patients with TN who underwent high-resolution MRI before microvascular decompression in 2022 were included in our study. Segmentation of the trigeminal nerve and contacting structures was performed on preoperative MRI scans using STAR-TN. The segmentations were then evaluated for neurovascular conflict and compared to the preoperative radiology and operative notes. Geometric features, including the area of contact and distance to conflict, were extracted. RESULTS: Of the 42 patients, 32 (76.2%) were found to show neurovascular conflict based solely on their STAR-TN segmentations and 10 (23.8%) were found to not show neurovascular conflict. Compared with the intraoperative findings, this resulted in a sensitivity of 78.0% and specificity of 100%. In contrast, assessments of neurovascular conflict by radiologists using only 2-dimensional MRI views had a sensitivity of 68.3% and specificity of 100%. Of the 32 patients with neurovascular conflict, 29 (90.9%) had conflict within the root entry zone. Overall, the patients had a median area of contact of 10.66 mm2. CONCLUSIONS: STAR-TN allows for 3-dimensional visualization and identification of neurovascular conflict with improved sensitivity compared with neuroradiologist assessments from MRI slices.

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INTRODUCTION: The most frequent pathology of the fifth cranial nerve is trigeminal neuralgia (TN), characterized by unilateral orofacial pain, of a paroxysmal nature, with distribution in one or more divisions of the trigeminal nerve. The main objective of this work is to demonstrate the efficacy and safety of neurovascular decompressive surgery (NVD). METHODS: Retrospective analytical study, patients operated on for NVD by TN (n: 155), from January 2006 - 2022, using a retrosigmoid approach and clinicalradiological follow-up, whose intraoperative recording was in 3D system (n: 42). RESULTS: 83.3% (n=35) presented classic NT and 16.7% (n = 7) idiopathic. The right side prevailed with 59.5% (n = 25) and paroxysmal pain with 81% (n=34) of representation in the entire series. Compression of arterial origin represented 76.2% (n: 32) of the cases, the superior cerebellar artery represented the first cause of neurovascular compression (NVC) in 52.4% (n: 22) in the exit zone of the trigeminal nerve. The validity of the magnetic resonance imaging (MRI) protocol selected for this series was analyzed; evidencing a high sensitivity of MRI with 97%, and a specificity of 86%. CONCLUSION: The sensitivity and specificity of highdefinition MRI studies and with special protocols for the evaluation of TN, as gold standard paraclinical, were presented. The DNV offered safe results, a high percentage of the patients were described as successful. The 3D recording allowed retrospective analysis with a vision identical to that of the surgeon during the intraoperative period, the type of NVC.

Introducción: La afección más frecuente del V nervio craneal es la neuralgia del trigémino (NT), se caracteriza por dolor orofacial unilateral, paroxístico, distribuyéndose en una o más divisiones del nervio trigémino. El objetivo de presente trabajo es demostrar la eficacia y seguridad de la cirugía descompresiva neurovascular (DNV). Métodos: Estudio analítico retrospectivo, pacientes intervenidos para DNV por NT (n: 155), desde enero de 2006 - 2022, mediante un abordaje retrosigmoideo y seguimiento clínico-radiológico, cuyo registro intraoperatorio fue en sistema 3D (n: 42). Resultados: 83.3% (n=35) presentó NT clásica y 16.7% (n = 7) idiopática. La mayoría con afectación del lado derecho 59.5% (n = 25) y el dolor paroxístico con 81% (n = 34). La compresión de origen arterial representó 76.2% (n: 32) de los casos, la arteria cerebelosa superior representó la primera causa de compresión neurovascular (CNV) en un 52.4% (n:22) en la zona de salida del nervio trigémino. Se analizó la validez del protocolo de resonancia magnética (RM) seleccionado para esta serie; evidenciando una alta sensibilidad de la RM con 97%, y una especificidad del 86%. Conclusión: Se demostró la sensibilidad y especificidad de los estudios de resonancia magnética (RM) de alta definición y con protocolos especiales para la evaluación de la NT, como paraclínico estándar de oro. La DNV ofreció resultados seguros, un alto porcentaje de los pacientes se calificaron como exitosos. El registro 3D permitió analizar en retrospectiva con una visión idéntica a la del cirujano durante el intraoperatorio, el tipo de CNV.

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Background: Trigeminal neuralgia (TN) is a debilitating disorder that presents with sudden onset of severe, unilateral, paroxysmal, and lancinating pain usually lasting for few seconds to few minutes. Aims and Objectives: The main aim of our study was to correlate the prepontine cisternal space thickness, with a severity of neurovascular compression (NVC) and percentage reduction of pain (patient outcome). Materials and Methods: Ours is an observational prospective study of 40 patients presenting with TN for magnetic resonance imaging in our department. Patients were followed up on medical treatment and their pain severity evaluated on their follow-up visit. Patients were divided into two groups based on prepontine cisternal space (Group A: ≤4 mm, Group B: >4 mm) and into three groups based on the percentage reduction of pain, Group 1 (0-35%), Group 2 (36-70%), and Group 3 consisted of patients with pain reduction of more than 70%. Ipsilateral prepontine cisternal space thickness was correlated with grade of NVC and percentage reduction of pain. Results: Mean percentage of pain reduction in group A and group B was 34.12 and 60.68%, respectively. Approximately 23.80% of grade1 NVC were seen in group A and 76.20% in group B, while 80% of grade 3 NVC were seen in group A and only 20% were seen in group B. Conclusion: There was poor response to medical treatment, in patients with narrowed prepontine cisternal space thickness with an inverse relationship between the grade of NVC and cisternal space thickness.

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PURPOSE: We aimed to describe the association between trigeminal nerve (TN) dose and toxicity and determine a threshold value that leads to TN toxicity in patients with parotid tumors treated with adjuvant conventional fractionated radiation therapy. METHODS AND MATERIALS: Eighteen patients who underwent adjuvant radiotherapy (RT) between 2013 and 2018 were included in this retrospective study. TN and its branches were outlined subsequently on the planning CT scans. The doses received by TN were obtained based on the dose-volume histogram. The dose and toxicity relationship was investigated over the total prescribed dose. RT-related toxicity was graded according to Common Terminology Criteria for Adverse Events V4.0 (CTCAEv4.0). RESULTS: The median follow-up was 29.5 months. After RT, 61% of patients had Grade I-II late TN toxicity divided into Grade I in 4 (22%) and Grade II in 7 (39%) patients. TN injury symptoms were as follows: loss of sensation in the chin area in 3, difficulty in jaw movements in 3, and paresthesia in 5 patients. The total RT dose (p = 0.001), Dmax (p = 0.001), PTV-TN Dmax (p = 0.001), D1cc (p = 0.004), D0.5cc (p = 0.001), and D0.1cc (p = 0.01) had a significant effect on TN toxicity. Cut-off values leading to toxicity were determined as 66, 65.5, 65.25, 63.6, and 62.7 Gy for Dmax, PTV-TN Dmax, D0.1cc, D 0.5cc, and D1cc, respectively. CONCLUSIONS: Radiation-induced TN injury in head and neck cancer patients may further be investigated in clinically prospective trials by virtue of high toxicity rates with current RT doses in our retrospectively designed dosimetric study in parotid tumors.

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PURPOSES: Transcriptomics-based analysis of key transcriptional molecules in the pathogenesis of trigeminal neuropathic pain was conducted to screen key molecules in the pathogenesis of trigeminal neuralgia. METHODS: Rat trigeminal nerve pathological pain model, namely chronic constriction injury of distal infraorbital nerve (IoN-CCI), was constructed and animal behaviors postsurgery were observed and analyzed. Trigeminal ganglia were collected for RNA-seq transcriptomics analysis. StringTie was used to annotate and quantify genome expression. DESeq2 was applied to compare between groups with P value less than 0.05 and fold change greater than 2 times and less than 0.5 times to screen differential genes, and display them with volcano graphs and cluster graphs. ClusterProfiler software was used to perform GO function enrichment analysis of differential genes. RESULTS: On the fifth postoperative day (POD5), the rat's face-grooming behavior increased to a peak; on the seventh postoperative day (POD7), the von-frey value dropped to the lowest value, indicating that the mechanical pain threshold of rats was significantly decreased. RNA-seq analysis of IoN-CCI rat ganglia found that the significantly up-regulated signaling pathways included B cell receptor signaling pathway, cell adhesion, complement and coagulation cascade pathways; significantly down-regulated pathways were related to systemic lupus erythematosus. Multiple genes among Cacna1s, Cox8b, My1, Ckm, Mylpf, Myoz1, Tnnc2 were involved in mediating the occurrence of trigeminal neuralgia. CONCLUSIONS: B cell receptor signaling pathway, cell adhesion, complement and coagulation cascade pathways, neuroimmune pathways are closely related to the occurrence of trigeminal neuralgia. The interaction of multiple genes among Cacna1s, Cox8b, My11, Ckm, Mylpf, Myoz1, Tnnc2 leads to the occurrence of trigeminal neuralgia.

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Trigeminal neuralgia (TN) is a syndrome consisting of episodic neuropathic facial pain. Although the precise symptoms vary across individuals, TN is typically described as lancinating electrical shocks triggered by sensory stimuli (light touch, talking, eating, and brushing teeth) that improve with antiepileptic medication (especially carbamazepine), remit spontaneously for weeks to months (pain-free intervals), and do not involve any changes in baseline sensation. The etiology of TN has not been definitively established, but many cases are associated with compression of the trigeminal nerve by a blood vessel at the trigeminal root entry zone adjacent to the brainstem. Patients who do not respond to medical management and who are not candidates for microvascular decompression often benefit from focal therapeutic injury to the trigeminal nerve at some point along its course. Many lesions have been described, including peripheral neurectomies that target distal branches of the trigeminal nerve, rhizotomies of the Gasserian ganglion of the nerve within Meckel's cave, radiosurgery of the trigeminal nerve at its root entry zone, partial sensory rhizotomy at the root entry zone, tractotomy of the spinal nucleus of the trigeminal nerve, and DREZotomy of the trigeminal nucleus caudalis, Though the latter two interventions are seldom done for TN and more commonly performed for trigeminal neuropathic pain. This article reviews the relevant anatomy and lesioning procedures for the treatment of trigeminal neuralgia.

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Many anatomical variants can potentially contribute to trigeminal neuralgia. These include compression by the superior cerebellar artery and less commonly, bony compression near the trigeminal cave. Herein, we report the gross and histological findings of a cadaveric specimen found to have a bony roof of the trigeminal cave. During the routine dissection of a male cadaver, an unusual finding of the skull base was observed. Palpation over the porus trigeminus identified a completely ossified roof. The bony spicule was 1.22 cm long and 0.76 mm wide. The trigeminal nerve was noted to have an indented region just below its contact with the ossified roof of the porus trigeminus. No frank nerve degeneration was noted with histological analysis. Normal mature bone tissue was noted surrounded by a sheath of dura mater. Future radiographic research is needed to better elucidate if ossification of the roof of the trigeminal cave is related to clinical symptoms of trigeminal neuralgia (TN). However, physicians should be cognizant of radiographic ossification of the trigeminal cave as a potential cause of TN.

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Trigeminal neuroma (TN) is a benign neoplasm arising from trigeminal nerve sheath. The tumor can grow from any part of the nerve from the root in posterior cranial fossa to peripheral extracranial branches. Symptoms of trigeminal neuroma are variable and usually depend on location and dimensions of tumor. We present a review devoted to the problem of TN. Surgery was the only possible option in patients with TN for a long time. However, radiotherapy became one of the options and sometimes alternative to surgical treatment since the late 1980s. Besides active management of patients with TN, follow-up with regular radiographic control of small asymptomatic tumors also seems to be reasonable. When evaluating treatment outcomes, physicians consider quality of life and return to previous work and activity in addition to resection quality, neurological impairment, relapse-free period and tumor growth control. However, assessment of these indicators after certain treatment is rare. Thus, it is difficult to determine treatment strategy with maximum ratio of effectiveness and quality of life. Therefore, optimization of TN treatment is currently an urgent problem that requires further study.

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A solitary pontine lesion (SPL) is a single brainstem lesion on the trigeminal nerve pathway without any other central nervous system lesion. This research aimed to investigate the demographic and clinical features of nonpainful TNO patients with SPL and identify the most frequently affected anatomical areas using lesion mapping techniques. Demographic and clinical features were retrospectively reviewed from the patients' charts. Brain lesions were mapped using MRIcroGL software. The study included 6 patients (three females and three males) with an SPL. The median age of the patients was 57 (range: 46-68) years. Cranial MRI displayed lesions in the dorsolateral pons and the cerebellar peduncle. The lesion mapping revealed that the lesions were on the trigeminal nerve pathway. SPL is an uncommon cause of TNO. Nonpainful SPL patients have demographic, clinical, and radiological features similar to those of painful SPL patients. The lesion mapping showed that the same brainstem areas are affected in painful and nonpainful SPL patients.

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Anatomical variations can occasionally result in unexpected findings on physical examination. Here, we report two cases of seemingly unique connections between V2 and V3 parts of the trigeminal nerve. In these two cadaveric specimens, at the foramen ovale, small neural connections, confirmed with histology, were identified joining V2 to specifically, the motor root of V3. The findings of these two cadaveric specimens and the potential clinical ramifications are discussed.

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A 74-year-old woman who presented initially with trigeminal neuralgia of the left forehead and scalp was later found to have a poorly differentiated squamous cell carcinoma (SCC) with large-nerve perineural and intraneural invasion of the left supraorbital nerve. Negative histopathologic margins were achieved in three stages of permanent fixed tissue en face processing and the final defect was repaired with a large rotation flap. Approximately one month after repair, the patient presented with new-onset diplopia and was found to have a complete left cranial nerve VI palsy suspicious for continued disease spread. MRI confirmed perineural spread along the ophthalmic branch of the trigeminal nerve through the superior orbital fissure into the cavernous sinus. She was subsequently treated with radiation therapy (66Gy in 33 fractions). The involvement of two distinct cranial nerves by perineural invasion is uncommon and has mostly been described involving branches of the trigeminal and facial nerves. This case highlights the rare presentation of perineural invasion involving both the trigeminal nerve and the abducens nerve. Anatomically, this clinical presentation can be explained by the retrograde perineural spread along the ophthalmic branch of the trigeminal nerve through the supraorbital fossa into the cavernous sinus where these two nerves are in close proximity.

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