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Trigeminal neuralgia (TN) is an exemplary condition of neuropathic facial pain. However, formally classifying TN as neuropathic pain based on the grading system of the International Association for the Study of Pain is complicated by the requirement of objective signs confirming an underlying lesion or disease of the somatosensory system. The lates...
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Venous compression is a rare cause of trigeminal neuralgia (TN). Vascular decompression of the trigeminal nerve root, as the only etiopathogenetic treatment of trigeminal neuralgia, is a well-known procedure that is extensively used at various neurosurgical clinics. As the number of interventions for TN increases, the absolute number of surgeries f...
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... Trigeminal neuralgia (TN) is a severe neurological disorder characterized by the patient experiencing intense facial pain, often described as "electric shocks" or "stabbing sensations, " which impact the patient's quality of life (Cruccu et al., 2016;Di Stefano et al., 2019;Alwardian et al., 2021;Lambru et al., 2022). However, the exact pathogenesis of the disease is still unclear (Huang and Brown, 2022). ...
This investigation aims to elucidate the novel role of Stromal Interaction Molecule 1 (STIM1) in modulating store-operated calcium entry (SOCE) and its subsequent impact on inflammatory cytokine release in T lymphocytes, thereby advancing our understanding of trigeminal neuralgia (TN) pathogenesis. Employing the Gene Expression Omnibus (GEO) database, we extracted microarray data pertinent to TN to identify differentially expressed genes (DEGs). A subsequent comparison with SOCE-related genes from the Genecards database helped pinpoint potential target genes. The STRING database facilitated protein-protein interaction (PPI) analysis to spotlight STIM1 as a gene of interest in TN. Through histological staining, transmission electron microscopy (TEM), and behavioral assessments, we probed STIM1's pathological effects on TN in rat models. Additionally, we examined STIM1's influence on the SOCE pathway in trigeminal ganglion cells using techniques like calcium content measurement, patch clamp electrophysiology, and STIM1- ORAI1 co-localization studies. Changes in the expression of inflammatory markers (TNF-α, IL-1β, IL-6) in T cells were quantified using Western blot (WB) and enzyme-linked immunosorbent assay (ELISA) in vitro , while immunohistochemistry and flow cytometry were applied in vivo to assess these cytokines and T cell count alterations. Our bioinformatic approach highlighted STIM1's significant overexpression in TN patients, underscoring its pivotal role in TN's etiology and progression. Experimental findings from both in vitro and in vivo studies corroborated STIM1's regulatory influence on the SOCE pathway. Furthermore, STIM1 was shown to mediate SOCE-induced inflammatory cytokine release in T lymphocytes, a critical factor in TN development. Supportive evidence from histological, ultrastructural, and behavioral analyses reinforced the link between STIM1-mediated SOCE and T lymphocyte-driven inflammation in TN pathogenesis. This study presents novel evidence that STIM1 is a key regulator of SOCE and inflammatory cytokine release in T lymphocytes, contributing significantly to the pathogenesis of trigeminal neuralgia. Our findings not only deepen the understanding of TN's molecular underpinnings but also potentially open new avenues for targeted therapeutic strategies.
... Both pain characteristics of secondary and idiopathic TN are the same, therefore further supportive examinations such as MRI are important to determine the main causes of TN. Preoperative neuroimaging, especially MRI, is important to differentiate clinically identified TN (7,8) . Geneidi et al reported that 56% of patients with TN developed a structural abnormality presented in the MRI, of whom 4.44% were found to have CPA tumors and the others were caused by neurovascular contact, traumatic causes, and demyelinating diseases (9) . ...
Purpose: Secondary (TN) caused by an arachnoid cyst in the (CPA) region is a rare finding. Based on the reported literature, there are only 5 cases of secondary trigeminal neuralgia caused by an arachnoid cyst in the cerebellopontine angle region.
Case report: A 27-year-old female presented to our neurosurgery clinic with a 2-year history of brief episodes of paroxysm pain in the left cheek. The pain was described as an electric shock-like pain triggered by simple stimuli. The magnetic resonance imaging (MRI) showed a well-confined cystic lesion in the left CPA, which compresses the left pons and the cisternal segment of the left trigeminal nerve. The patient was managed operatively to fenestrate the cyst and decompress the trigeminal nerve. The histopathological result of the cyst wall was consistent with an arachnoid cyst. Six months after surgery, the patient is in good health condition and symptom-free without medication.
Conclusion: Arachnoid cyst in the CPA region is one of the rare causes of secondary TN. Preoperative imaging with MRI is important to provide better results to differentiate the pathology. Surgical treatment to fenestrate the arachnoid cyst and decompress the trigeminal nerve have a good result and can improve the patient's quality of life.
... [11] Affecting one or more divisions of the trigeminal nerve, TN may be triggered by innocuous sensory stimuli such as teeth brushing or touching the face. [7,19] e excruciating pain may significantly decrease one's quality of life. [33] TN is classified as (1) classical due to morphologic changes in the trigeminal nerve root from vascular compression, (2) secondary due to an identifiable underlying neurologic disease (tumor at the cerebellopontine angle or multiple sclerosis), or (3) idiopathic. ...
... [33] TN is classified as (1) classical due to morphologic changes in the trigeminal nerve root from vascular compression, (2) secondary due to an identifiable underlying neurologic disease (tumor at the cerebellopontine angle or multiple sclerosis), or (3) idiopathic. [7,10,19] e pathophysiology involves demyelination of primary sensory trigeminal afferents in the root entry zone (REZ) caused by an enlarged blood vessel (usually the cerebellar artery) pressing against the trigeminal nerve. [19,22] e incidence rates of TN in the United States range between 5.9 and 12.6/100,000 individuals with a lifetime prevalence of 0.7/1000 people. ...
Background: Frameless image-guided radiosurgery (IGRS) is an effective and noninvasive method of treating patients who are unresponsive to medical management for trigeminal neuralgia (TN). This study evaluated the use of frameless IGRS to treat patients with medically refractory TN.
Methods: We performed a retrospective review of records of 116 patients diagnosed with TN who underwent frameless IGRS using a linear accelerator (LINAC) over a 10-year period (March 2012- February 2023). All patients had failed medical management for TN. Facial pain was graded using the Barrow Neurological Institute (BNI) scoring system. Each patient received a BNI score prior to frameless IGRS and following treatment. Failure was defined as a BNI score IV-V at last follow-up and/or undergoing a salvage procedure following IGRS.
Results: All patients had a BNI score of either IV or V prior to the frameless IGRS. The mean follow-up duration for all 116 patients following IGRS was 44.1 months. Most patients (81 [69.8%]) had not undergone surgery (microvascular decompression [MVD] or rhizotomy) or SRS for TN prior to frameless IGRS. A total of 41 (35.3%) patients underwent a salvage procedure (MVD, rhizotomy, or an additional IGRS) following frameless IGRS. The mean duration between the initial frameless IGRS and salvage procedure was 20.1 months. At last follow-up, a total of 110 (94.8%) patients had a BNI score of I-III. No complications were reported after the frameless IGRS. The BNI score at last follow-up was lower compared to the initial BNI for patients regardless of prior intervention (p<0.001). Patients who failed IGRS had a higher BNI score at last follow-up compared to those who did not fail IGRS (2.8 vs 2.5, p=0.05). Patients with pain relief had a shorter follow-up compared to those with pain refractory to SRS (38.0 vs. 55.1, p=0.005).
Conclusions: In this large cohort of patients with medically refractory TN, frameless IGRS resulted in durable pain control in the majority of patients without any toxicities.
... However, these drugs often have limited efficacy over time and come with several side effects. In cases of classical TN, microvascular decompression (MVD) surgery can provide rapid symptom relief but carries risks of serious complications, including brainstem infarction, hearing loss on the operated side, cerebrospinal fluid leakage, or facial numbness [4]. Percutaneous ablative techniques, which target the gasserian ganglion, are effective but pose a high risk of adverse effects like dysesthesias, corneal keratitis, or facial numbness [5]. ...
... Microvascular decompression (MVD) is considered the first option to treat refractory classical trigeminal neuralgia (TN) [3]. However, when imaging cannot demonstrate neurovascular conflict or in cases of idiopathic or secondary TN lesioning techniques can be helpful. ...
... The corneal reflex must be tested through the entire procedure to ensure that corneal hypoesthesia is avoided Fig. 6 Decision algorithm for surgical treatment of TN. Classical means by definition with a significant probability that the TN is due to an NVC [3] Content courtesy of Springer Nature, terms of use apply. Rights reserved. ...
Based on a personal experience of 4200 surgeries, radiofrequency thermocoagulation is useful lesional treatment for those trigeminal neuralgias (TNs) not amenable to microvascular decompression (idiopathic or secondary TNs). Introduced through the foramen ovale, behind the trigemnial ganglion in the triangular plexus, the needle is navigated by radiology and neurophysiological testing to target the retrogasserian fibers corresponding to the trigger zone. Heating to 55–75 °C can achieve hypoesthesia without anaesthesia dolorosa if properly controlled. Depth of anaesthesia varies dynamically sedation for cannulation and lesioning, and awareness during neurophysiologic navigation. Proper technique ensures long-lasting results in more than 75% of patients.
Supplementary Information
The online version contains supplementary material available at 10.1007/s00701-024-06074-2.
... These new medications have advanced pharmacokinetics in comparison to the conventional antiepileptic medications in terms of longer half-lives, allowing twice-daily dosing, reduced drug interactions and a general lack of hepatic enzyme induction, making poly-therapy and other aspects of treatment easier. 5,6 Carbamazepine is the current drug of choice for trigeminal neuralgia, results in the stabilization of hyperexcited neuronal membranes and the inhibition of repetitive firing. The typical starting dose is 100 to 200 mg twice daily. ...
... A slightly higher score was recorded for patients belonging to group B (7.7±1.0) as compared to participants of group A (7.1±1.2), with a significant difference (p=0.029). At first follow up, 1-month after the initiation of therapy, the mean pain score in both the groups decreased where in group A the pain score decreased to 5 The baseline pain score before starting the procedure was measured by using VAS log for both the groups. When the pain scores at 1-month follow-up were compared for both the groups, no significant difference was found. ...
Background: Despite various pharmacological drugs used for the treatment of Trigeminal Neuralgia (TN), the clinical efficacy of carbamazepine with oxcarbazepine in terms of pain relief particularly in Pakistani population is poorly studied. This study aimed to compare the efficacy of carbamazepine with oxcarbazepine for treating Trigeminal Neuralgia. Objective: To compare oxcarbazepine with carbamazepine in the management of trigeminal neuralgia in terms of mean pain. Study Design: Quasi-Experimental study. Settings: Fauji Foundation Hospital, Rawalpindi Pakistan. Duration: 06 months i.e. 30th Apr 2018 to 30th Oct 2018. Methods: This single-centre, prospective clinical study was conducted on 70 patients diagnosed with TN. Patients were divided into two groups; Group A: Control group (n = 35), in which patients were given Carbamazepine 200mg twice a day upto1800mg and Group B: an Experimental group (n = 35), in which the patients receive Oxcarbazepine (300mg BD daily up-to 1800mg). The clinical parameters were evaluated by a self-reported questionnaire. Pain level was assessed at 1st and 2nd month follow-up using a Visual Analog Scale. Results: The mean postoperative pain was measured for both groups. Although the mean pain score at 1st follow-up month was similar for Group A (5.2±1.0) and B (4.9±1.1), p=0.328, higher clinical efficacy was observed in 2nd follow-up month for Group B– Oxcarbazepine (3.1±0.8) when compared with Group A– Carbamazepine (5.14±0.8) in terms of mean pain score that was statistically significant p<0.001. Conclusion: This study concludes that in term of pain relief, Oxcarbazepine appears to be a more effective as compared to Carbamazepine in the treatment of TN.
... The incidence of TN is 2 to 5 patients per 100,000 people (3)(4)(5)(6). TN is evaluated in three etiological categories: 1) Idiopathic TN develops without any recognized cause; 2) classic TN occurs as a result of vascular compression of the trigeminal nerve root; and 3) secondary TN occurs due to a secondary cause such as multiple sclerosis or cerebellopontine angle tumor (7). ...
... The pain is commonly characterized as electric-shock-like, stabbing, shooting in quality, and can be triggered by innocuous stimuli such as light touch or vibration. 1,2 In the majority of cases, the etiology of TN is attributed to neurovascular compression of the trigeminal nerve at the root entry zone (REZ), caused by adjacent blood vessels, resulting in demyelination and hyperexcitability of the affected nerve fibers. Additionally, other potential causative factors encompass structural anomalies such as tumors or arteriovenous malformations, as well as demyelinating diseases like multiple sclerosis. ...
... The current classification of trigeminal neuralgia is divided into idiopathic (no cause can be identified), classic (due to vascular compression of one or more trigeminal roots), and secondary (due to neurological disease (MS lesion) or tumor compression of the angle of the cerebellar bridge). Multiple sclerosis is the most common cause of secondary trigeminal neuralgia, as suggested by the findings of a 20-fold increased risk of trigeminal neuralgia in patients with MS [53,54]. For a long time, it was assumed that the main cause of trigeminal fiber demyelination in trigeminal neuralgia was vascular compression in the area before the nerve enters the pons, and trigeminal neuralgia in MS is associated with demyelinating plaques in the area immediately after entering the pons [55,56]. ...
Multiple sclerosis is a chronic infammatory disease that afects the central nervous system and can cause various types of
pain including ongoing extremity pain, Lhermitte’s phenomenon, trigeminal neuralgia, and mixed pain. Neuropathic pain is
a major concern for individuals with multiple sclerosis as it is directly linked to myelin damage in the central nervous system
and the management of neuropathic pain in multiple sclerosis is challenging as the options available have limited efcacy and
can cause unpleasant side efects. The literature search was conducted across two databases, PubMed, and Google Scholar.
Eligible studies included clinical trials, observational studies, meta-analyses, systematic reviews, and narrative reviews.
The objective of this article is to provide an overview of literature on pharmacological and non-pharmacological strategies
employed in the management of neuropathic pain in multiple sclerosis. Pharmacological options include cannabinoids,
muscle relaxants (tizanidine, baclofen, dantrolene), anticonvulsants (benzodiazepines, gabapentin, phenytoin, carbamazepine,
lamotrigine), antidepressants (duloxetine, venlafaxine, tricyclic antidepressants), opioids (naltrexone), and botulinum toxin
variants, which have evidence from various clinical trials. Non-pharmacological approaches for trigeminal neuralgia may
include neurosurgical methods. Non-invasive methods, physical therapy, and psychotherapy (cognitive behavioral therapy,
acceptance and commitment therapy and mindfulness-based stress reduction) may be recommended for patients with
neuropathic pain in multiple sclerosis. The choice of treatment depends on the severity and type of pain as well as other
factors, such as patient preferences and comorbidities. There is a pressing need for healthcare professionals and researchers
to prioritize the development of better strategies for managing multiple sclerosis-induced neuropathic pain.
... The differential diagnosis includes classical trigeminal neuralgia (caused by vascular compression of the nerve root or demyelination), 9 postherpetic neuralgia, deafferentation, or neuropathic pain (secondary to trauma). 10 In this case, traumatic trigeminal neuropathic pain is the most likely explanation for the patient's symptoms. ...
Evaluating patients with a traumatic spinal cord injury can be complicated by other injuries. In this case, a 24-year-old woman injured by a needlefish presented with combined motor and sensory defects, cranial nerve deficits, and a blunt vascular injury. This case highlights the importance of neurologic and vascular localizations and an understanding of spinal cord injuries involving various ascending and descending tracts. Appreciation of these anatomical considerations through this case illustrates the diagnostic approach to neurologic evaluation. While we present a traumatic etiology for multiple neurologic syndromes, this case gives readers an opportunity to develop a comprehensive differential diagnosis and tailor investigations for other relevant etiologies. Readers walking through this stepwise process will ultimately arrive at several distinct but related diagnoses. Section 1 A 24-year-old woman was night surfing in rural Mexico when she was struck in the right side of her neck by a needlefish. She was taken to the nearest clinic where her puncture wound was sutured. Her friends held her in cervical-spine precautions while a taxi took them through the jungle to a hospital. On arrival to the hospital, physical examination revealed a Glasgow Coma Scale of 15, 4/5 strength in her right triceps and mild weakness with right wrist extension, finger abduction, finger adduction corresponding to the right C6-T1 myotomes, and severe right lower extremity weakness. Deep tendon reflexes were 2 throughout, with negative Hoffmann and Babinski signs. She had decreased sensation to pinprick on the left face, upper extremity, and lower extremity and decreased vibratory sense and absent proprioception of the right hemibody. Questions for Consideration: 1. What spinal tracts are responsible for the modes of sensory information affected and where do they decussate? 2.
