Trigeminal neuralgia.

Trigeminal neuralgia (TN) is a facial pain disorder characterized by intense and paroxysmal pain that profoundly affects quality of life and presents complex challenges in diagnosis and treatment. TN can be categorized as classical, secondary and idiopathic. Epidemiological studies show variable incidence rates and an increased prevalence in women and in the elderly, with familial cases suggesting genetic factors. The pathophysiology of TN is multifactorial and involves genetic predisposition, anatomical changes, and neurophysiological factors, leading to hyperexcitable neuronal states, central sensitization and widespread neural plasticity changes. Neurovascular compression of the trigeminal root, which undergoes major morphological changes, and focal demyelination of primary trigeminal afferents are key aetiological factors in TN. Structural and functional brain imaging studies in patients with TN demonstrated abnormalities in brain regions responsible for pain modulation and emotional processing of pain. Treatment of TN involves a multifaceted approach that considers patient-specific factors, including the type of TN, with initial pharmacotherapy followed by surgical options if necessary. First-line pharmacological treatments include carbamazepine and oxcarbazepine. Surgical interventions, including microvascular decompression and percutaneous neuroablative procedures, can be considered at an early stage if pharmacotherapy is not sufficient for pain control or has intolerable adverse effects or contraindications.

Motor cortex stimulation for the treatment of trigeminal neuralgia after brainstem infarction: A case report. / 运动皮层电刺激治疗脑干梗死后三叉神经痛1例.

Secondary trigeminal neuralgia after brainstem infarction is rare and rarely reported. A patient with secondary trigeminal neuralgia after brainstem infarction was admitted to the Department of Neurosurgery, Xiangya Hospital, Central South University. The patient was a 44 years old male who underwent motor cortex stimulation treatment after admission. The effect was satisfactory in the first week after surgery, but the effect was not satisfactory after one week. This disease is relatively rare and the choice of clinical treatment still requires long-term observation.

Neuralgia in the occipital region associated with ipsilateral trigeminal herpes zoster: Three case reports.

BACKGROUND: Nerve fibers related to pain and temperature sensation in the trigeminal nerve territory converge with the upper cervical spinal nerves from the level of the lower medulla oblongata to the upper cervical cord. This structure is called the trigemino-cervical complex and may cause referred pain in the territory of the trigeminal or upper cervical spinal nerves. CASE SERIES: Here, we report three cases of paroxysmal neuralgia in the occipital region with mild conjunctivitis or a few reddish spots in the ipsilateral trigeminal nerve territory. The patients exhibited gradual progression of these reddish spots evolving into vesicles over the course of several days, despite the absence of a rash in the occipital region. The patients were diagnosed with trigeminal herpes zoster and subsequently received antiherpetic therapy. Remarkably, the neuralgia in the occipital region showed gradual amelioration or complete resolution before the treatment, with no sequelae reported in the occipital region. DISCUSSION: The trigemino-cervical complex has the potential to cause neuralgia in the occipital region, as referred pain, caused by trigeminal herpes zoster. These cases suggest that, even if conjunctivitis or reddish spots appear to be trivial in the trigeminal nerve territory, trigeminal herpes zoster should be considered when neuralgia occurs in the ipsilateral occipital region.

Long-term outcomes of Gamma Knife radiosurgery in treating glossopharyngeal neuralgia.

Glossopharyngeal neuralgia (GPN) is an unusual disorder causing severe, brief pain episodes in the areas supplied by the glossopharyngeal nerve. Initial treatment involves medications like carbamazepine, but if these are ineffective or cause side effects, interventional pain management techniques or surgery may be considered. Gamma Knife radiosurgery is becoming popular in managing GPN due to its lower risk of complications than surgical interventions like microvascular decompression or rhizotomy. In this retrospective case series, we examined the outcomes of Gamma Knife radiosurgery in eight patients with GPN. The decision to utilize Gamma Knife radiosurgery was made following specific criteria, including failed surgical interventions, patient preference against surgery, or contraindications to surgical procedures. Patients were administered radiation doses within the range of 80 to 90 Gy, targeting either the cisternal glossopharyngeal nerve or glossopharyngeal meatus of the jugular foramen. Evaluations were conducted before the Gamma Knife radiosurgery; at 3, 6, and 12 months after Gamma Knife radiosurgery; and annually thereafter. Pain severity was assessed using the modified Barrow Neurological Institute scale grades, with patients achieving grade I-IIIa considered to have a good treatment outcome and grade IV-V to have a poor treatment outcome. Pain control and absence of radiosurgery-related complications were primary endpoints. The median age of the patients was 46.5 years, varying from 8 to 72 years. The median duration of pain was 32 months (range, 12-120 months). All patients, except one, were on polydrug therapy. All cases exhibited preoperative grade V pain. The median follow-up duration after Gamma Knife radiosurgery was 54.5 months, varying from 14 to 90 months. The overall clinical assessments revealed a gradual neurological improvement, particularly within the first 8.5 weeks (range, 1-12 weeks). The immediate outcomes at 3 months revealed that all patients (8/8, 100%) experienced pain relief, with 25% (2/8) achieving a medication-free status (Grade I). Three patients (37%) experienced a recurrence during the follow-up and were managed with repeat Gamma Knife radiosurgery (n = 2) and radiofrequency rhizotomy (n = 1). At the last follow-up, 88% (7/8) of patients had pain relief (Grades I-IIIa), with three (37%) achieving a medication-free status (Grade I). No adverse events or neurological complications occurred. The patient who underwent radiofrequency rhizotomy continued to experience inadequately controlled pain despite medication (Grade IV). Gamma Knife radiosurgery is a non-invasive, efficacious treatment option for idiopathic GPN, offering short- and long-term relief without permanent complications.

A prospective cohort study on perioperative percutaneous balloon compression for trigeminal neuralgia: safety and efficacy analysis.

With the recent emergence of percutaneous balloon compression (PBC) as a promising treatment for trigeminal neuralgia (TN), there is a growing need for research on its safety and efficacy. This study was designed to evaluate the safety and efficacy of PBC in the treatment of TN patients during the perioperative period. This study involved a total of 400 TN patients who were selected and treated with PBC at our institution. The clinical data and short-term outcomes were analyzed based on sex, initial PBC treatment for TN, and subsequent PBC treatment for recurrent TN after previous PBC or microvascular decompression (MVD) or radiofrequency thermocoagulation (RFT). No statistically significant difference was found when comparing postoperative pain relief between male and female patients with TN. Nevertheless, female patients were found to be more vulnerable than male patients to abnormal facial sensations (P = 0.001), diplopia (P = 0.015), postoperative headache (P = 0.012), and hyposmia (P = 0.029). Additionally, it was observed that there was no substantial difference in the postoperative pain relief rate between the first-time PBC group and PBC for recurrent TN patients postoperatively following procedures such as PBC, MVD, and RFT. In conclusion, this study has shown that PBC treatment is effective in managing TN in both males and females, regardless of whether the treatment was administered as a primary intervention or following prior surgical procedures such as PBC, MVD, or RFT. Nonetheless, it is noted that the risk of postoperative complications appears to be higher in female patients compared to male patients.

Unusual Atrophic Nervus Intermedius in a Patient with Refractory Nervus Intermedius Neuralgia and History of Ipsilateral Sudden-Onset Central Facial Palsy and Sensorineural Hearing Loss: Cadaveric-Clinical Images with Surgical Video.

Nervus intermedius (NI) arises from the superior salivary nucleus, solitary nucleus, and trigeminal tract. It leaves the pons as 1 to 5 roots and travels between the facial and vestibulocochlear nerves before merging with the facial nerve within the internal auditory canal. The mastoid segment of the facial nerve then gives rise to a sensory branch that supplies the posteroinferior wall of the external auditory meatus and inferior pina. This complex pathway renders the nerve susceptible to various pathologies, leading to NI neuralgia. Here, the authors present an unusual intraoperative finding of an atrophic NI in a patient with refractory NI neuralgia and a history of ipsilateral sudden-onset central facial palsy and microvascular decompression for trigeminal neuralgia. The patient underwent NI sectioning via the previous retrosigmoid window and achieved partial ear pain improvement. The gross size of the NI is compared with a cadaveric specimen through stepwise dissection. This case highlights the potential significance of subtle central ischemic events and subsequent atrophy of NI in the pathogenesis of NI neuralgia, as well as the ongoing need to investigate the therapeutic efficacy of nerve sectioning.

Surgical management of nervus intermedius neuralgia: A report of 4 cases and literature review.

BACKGROUND: Nervus intermedius neuralgia (NIN) is characterized by paroxysmal episodes of sharp, lancinating pain in the deep ear. Unfortunately, only a few studies exist in the literature on this pain syndrome, its pathology and postoperative outcomes. METHOD: We conducted a retrospective review of four cases diagnosed with NIN who underwent a neurosurgical intervention at our center from January 2015 to January 2023. Detailed information on their MRI examinations, intraoperative findings and other clinical presentations were obtained, and the glossopharyngeal and vagus nerves were isolated for immunohistochemistry examination. RESULTS: A total of 4 NIN patients who underwent a microsurgical intervention at our institution were included in this report. The NI was sectioned in all patients and 3 of them underwent a microvascular decompression. Of these 4 patients, 1 had a concomitant trigeminal neuralgia (TN), and 1 a concomitant glossopharyngeal neuralgia (GPN). Three patients underwent treatment for TN and 2 for GPN. Follow-up assessments ranged from 8 to 99 months. Three patients reported complete pain relief immediately after the surgery until last follow-up, while in the remaining patient the preoperative pain gradually resolved over the 3 month period. Immunohistochemistry revealed that a greater amount of CD4+ and CD8+ T cells had infiltrated the glossopharyngeal versus vagus nerve. CONCLUSIONS: NIN is an extremely rare condition showing a high degree of overlap with TN/GPN. An in depth neurosurgical intervention is effective to completely relieve NIN pain, without any serious complications. It appears that T cells may play regulatory role in the pathophysiology of CN neuralgia.

[Microvascular Decompression for Trigeminal Neuralgia Due to Venous Compression].

In microvascular decompression surgery for trigeminal neuralgia, the veins are essential as an anatomical frame for the microsurgical approach and as an offending vessel to compress the trigeminal nerve. Thorough arachnoid dissection of the superior petrosal vein and its tributaries provides surgical corridors to the trigeminal nerve root and enables the mobilization of the bridging, brainstem, and deep cerebellar veins. It is necessary to protect the trigeminal nerve by coagulating and cutting the offending vein. We reviewed the clinical features of trigeminal neuralgia caused by venous decompression and its outcomes after microvascular decompression. Among patients with trigeminal neuralgia, 4%-14% have sole venous compression. Atypical or type 2 trigeminal neuralgia may occur in 60%-80% of cases of sole venous compression. Three-dimensional MR cisternography and CT venography can help in detecting the offending vein. The transverse pontine vein is the common offending vein. The surgical cure and recurrence rates of trigeminal neuralgia with venous compression are 64%-75% and 23%, respectively. Sole venous compression is a unique form of trigeminal neuralgia. Its clinical characteristics differ from those of trigeminal neuralgia caused by arterial compression. Surgical procedures to resolve venous compression include nuances in safely handling venous structures.

[Microvascular Decompression for Trigeminal Neuralgia Caused by Trigeminocerebellar Artery].

The trigeminocerebellar artery(TCA)is a unique branch of the basilar artery. The TCA was first described in detail by Markovic et al. in 1996. The incidence of TCA was 6.9%-13.3% in previous cadaveric studies. The TCA branches from the distal part of the basilar artery, courses very close to the trigeminal nerve root entry zone, and occasionally twists or encircles the nerve root. A close relationship between the TCA and trigeminal nerve can cause trigeminal neuralgia(TN). This characteristic course of TCA requires adjuvant decompression techniques performed by the operators. In the microvascular decompression for TN caused by the TCA, operators should pay attention to the following: 1)sufficient arachnoid dissection around the TCA, 2)combined transposition and interposition technique, 3)decompression of perforators and vessels penetrating the nerve, and 4)recognition of the existence of the TCA.

Corticotropin-releasing hormone neurons control trigeminal neuralgia-induced anxiodepression via a hippocampus-to-prefrontal circuit.

Anxiety and depression are frequently observed in patients suffering from trigeminal neuralgia (TN), but neural circuits and mechanisms underlying this association are poorly understood. Here, we identified a dedicated neural circuit from the ventral hippocampus (vHPC) to the medial prefrontal cortex (mPFC) that mediates TN-related anxiodepression. We found that TN caused an increase in excitatory synaptic transmission from vHPCCaMK2A neurons to mPFC inhibitory neurons marked by the expression of corticotropin-releasing hormone (CRH). Activation of CRH+ neurons subsequently led to feed-forward inhibition of layer V pyramidal neurons in the mPFC via activation of the CRH receptor 1 (CRHR1). Inhibition of the vHPCCaMK2A-mPFCCRH circuit ameliorated TN-induced anxiodepression, whereas activating this pathway sufficiently produced anxiodepressive-like behaviors. Thus, our studies identified a neural pathway driving pain-related anxiodepression and a molecular target for treating pain-related psychiatric disorders.

Fully Endoscopic Microvascular Decompression for Trigeminal Neuralgia Caused by Vertebrobasilar Artery: A Case Series Review: 2-Dimensional Operative Video.

BACKGROUND AND OBJECTIVE: Microvascular decompression (MVD) is the most definitive and preferred surgical treatment for trigeminal neuralgia (TN). Treatment of TN caused by the vertebrobasilar artery (VBA) has been reported to be challenging and less satisfactory in complications and recurrence. Endoscopy has been implemented to provide a comprehensive view of neurovascular conflicts and minimize brain tissue stretch injury while exploring the trigeminal nerve. However, there are few retrospective studies on the treatment of TN caused by VBA by fully endoscopic microvascular decompression (E-MVD). This article aimed to illustrate the safety and efficacy of E-MVD for TN caused by the VBA. METHODS: Clinical data for 26 patients with TN caused by the VBA who underwent E-MVD from 2019 to 2022 were retrospectively analyzed. The characteristics of vertebrobasilar-associated TN were summarized. The safety and efficacy of E-MVD for vertebrobasilar-associated TN were estimated based on the analysis of intraoperative manipulation, postoperative symptom relief, and complications. RESULTS: Intraoperatively, the vertebrobasilar artery was regarded as a direct offending vessel in all 26 patients with TN, the vertebral artery in 18 (69.23%) and the basilar artery in 10 (38.46%). In addition to the vertebrobasilar artery, other vessels involved included the superior cerebellar artery in 12 patients, anterior inferior cerebellar artery in 9, posterior inferior cerebellar artery in 1, and veins in 4. All patients underwent E-MVD, and TN was entirely resolved in 26 (100%) patients immediately postoperatively. During the follow-up period of 12-45 months, no recurrence or serious complications were found. There were no serious postoperative complications, such as cerebellar swelling, intracranial hemorrhage, or death. CONCLUSION: E-MVD for vertebrobasilar-associated TN is effective and safe.

Management outcomes of peripontine arteriovenous malformation patients presenting with trigeminal neuralgia.

OBJECTIVE: Trigeminal neuralgia as the presenting symptom of brain arteriovenous malformation (bAVM) has been rarely reported. Treatment of reported cases has been skewed toward surgery for these scarce, deeply located bAVMs. Here, the authors report their management and outcomes of bAVM patients presenting with ipsilateral trigeminal neuralgia (TN) at their institution. METHODS: This is a retrospective cohort study. The authors' institutional bAVM database was queried for non-hereditary hemorrhagic telangiectasia bAVMs in pontine, cistern, brainstem, trigeminal nerve, or tentorial locations. Patients with complete data were included in a search for trigeminal neuralgia or "facial pain" as the presenting symptom with TN being on the same side as the bAVM. Demographics, TN and bAVM characteristics, management strategies, and outcomes of bAVM and TN management were analyzed. RESULTS: Fifty-seven peripontine bAVMs were identified; 8 (14.0%) of these bAVMs were discovered because of ipsilateral TN, including 4 patients (50%) with facial pain in the V2 distribution. Five patients (62.5%) were treated with carbamazepine as the initial medical therapy, 2 (25%) underwent multiple rhizotomies, and 1 (12.5%) underwent microvascular decompression. None of the patients with TN-associated bAVMs presented with hemorrhage, compared with 25 patients (51%) with bAVMs that were not associated with TN (p < 0.01). TN-associated bAVMs were overall smaller than non-TN-associated bAVMs, but the difference was not statistically significant (1.71 cm vs 2.22 cm, p = 0.117), and the Spetzler-Martin grades were similar. Six patients (75%) underwent radiosurgery to the bAVM (mean dose 1800 cGy, mean target volume 0.563 cm3) and had complete resolution of TN symptoms (100%). The mean time from radiosurgery to TN resolution was 193 (range 21-360) days, and 83.3% of treated TN-associated bAVMs were obliterated via radiosurgery. Two patients (12.5%) were recommended for conservative management, with one undergoing subsequent rhizotomies and another patient died of hemorrhage during follow-up. CONCLUSIONS: TN-associated bAVM is a rare condition with limited evidence for management guidance. Radiosurgery can be safe and effective in achieving durable TN control in patients with TN-associated bAVMs. Despite their deep location and unruptured presentation, obliteration can reach 83.3% with radiosurgery.

The Sagittal Angle of the Trigeminal Nerve at the Porus Trigeminus is a Radiologic Predictor of Surgical Outcome in Microvascular Decompression for Classical Trigeminal Neuralgia.

BACKGROUND AND OBJECTIVES: Classical trigeminal neuralgia (cTN) is a painful disease. Microvascular decompression (MVD) provides immediate and durable relief in many patients. A variety of positive and negative prognostic biomarkers for MVD have been identified. The sagittal angle of the trigeminal nerve at the porus trigeminus (SATNaPT) is an MRI biomarker that can identify a subset of patients with cTN whose trigeminal nerve anatomy is different from normal controls. The purpose of this case-control study was to determine whether an abnormally hyperacute SATNaPT is a negative prognostic biomarker in patients with cTN undergoing MVD. METHODS: Preoperative MRIs from 300 patients with cTN who underwent MVD were analyzed to identify patients with a hyperacute SATNaPT (defined as less than 3 SDs below the mean). The rate of surgical success (pain-free after at least 12 months) was compared between patients with a hyperacute SATNaPT and all other patients. RESULTS: Patients without a hyperacute SATNaPT had an 82% likelihood of surgical success, whereas patients with a hyperacute SATNaPT had a 58% likelihood of surgical success ( P < .05). Patients with a hyperacute SATNaPT who also had no evidence of vascular compression on preoperative MRI had an even lower likelihood of success (29%, P < .05). CONCLUSION: In patients with cTN being considered for MVD, a hyperacute SATNaPT is a negative prognostic biomarker that predicts a higher likelihood of surgical failure. Patients with a hyperacute SATNaPT, particularly those without MRI evidence of vascular compression, may benefit from other surgical treatments or a modification of MVD to adequately address the underlying cause of cTN.

Trigeminal Neuralgia as a Primary Demyelinating Disease: Potential Multimodal Evidence and Remaining Controversies.

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.

A rare case of vestibular schwannoma manifesting as trigeminal neuralgia.

BACKGROUND: In this case report, the authors reviewed a rare case of a vestibular schwannoma manifesting as trigeminal neuralgia (TN). Intracranial tumors can have a variety of orofacial pain symptoms. Among benign cerebellopontine angle tumors, vestibular schwannoma is the most common cause of a TN-like manifestation. Although the most common symptoms of a vestibular schwannoma are hearing loss and vestibulopathy, the unique feature of this case was the manifestation of symptoms consistent with TN. CASE DESCRIPTION: The patient had right-sided episodic facial pain that was short in duration and severe in intensity. The initial differential diagnoses included short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing and TN. As part of the routine evaluation, the patient was referred for brain magnetic resonance imaging, which revealed a right-sided vestibular schwannoma. The patient was prescribed 200 mg of gabapentin 3 times daily and was referred to neurosurgery for excision of the schwannoma. Surgical excision resulted in complete resolution of pain. PRACTICAL IMPLICATIONS: This case illustrates the importance of interdisciplinary treatment and how it can lead to an optimal outcome for a patient with complex orofacial pain symptoms.

Sixth cranial nerve palsy and trigeminal neuropathic pain due to a space-occupying lesion.

Various neuropathies of the cranil nerves can accompany trigeminal neuropathic pain attributed to space-occupying lesions. In this case report, the patient presented with persistent intraoral pain and numbness on the right side of the face. Cranial nerve examination revealed dysfunctional eye movements, diplopia, and mechanical hyposensitivity in the mandibular region. The patient was diagnosed with neuropathy due to intracranial lesions and referred to the Department of Neurosurgery and Otorhinolaryngology. The patient was suspected of having malignant lymphoma and is currently undergoing neurosurgical intervention. This article discusses the importance of the examination of the cranial nerve for patients with persistent pain in the trigeminal nerve distribution.