Trigeminal neuralgia (TN) is a chronic pain disorder affecting the trigeminal nerve, which supplies sensation to the face. Patients experience sudden, severe, and stabbing pain episodes likened to electrical shocks often triggered by light touch, eating, speaking, or similar activities. Even strong or cold wind can provoke an episode. Over time, these episodes of pain become more frequent and debilitating. The disorder is more common in people over the age of 50 and affects more women than men.
“TN is a fairly common neurologic disorder, even though most of the general public has likely never heard of it,” says L. Dade Lunsford, MD, the Lars Leksell & Distinguished Professor of Neurological Surgery at the University of Pittsburgh and Director Emeritus of the Center for Image-Guided Neurosurgery at UPMC. “There are about 10,000 new cases of TN diagnosed in the U.S. each year, and in many cases, an accurate diagnosis takes far too long to the severe detriment of patient health and quality of life.”
There are two recognized types of TN, classified as Primary or Secondary. Primary trigeminal neuralgia is often the result of vascular pulsations leads to a wearing away of the nerve insulation Although the exact causes or mechanisms responsible for intrusion of the blood vessel onto the nerve are often unknown, as we age the brain may gradually sag leading to normal blood vessels touching the nerve. The Secondary form of TN typically results from either a tumor or vascular malformation in the brain impinging upon the nerve and causing symptoms, or in some cases can be the result of underlying multiple sclerosis (MS) affecting the trigeminal nerve.
“Anyone that has ever had TN knows that the pain caused by the disorder is simply off the charts – by orders of magnitude,” says Michael J. Lang, MD, assistant professor of Neurological Surgery and director of Cerebrovascular Neurosurgery at UPMC. “Before effective treatment options for the condition were devised, people often referred to the pain from TN as ‘suicide headaches.’ That’s not a hyperbole. The pain can be that bad, enough for people to have contemplated – or actually – ending their own lives in search of relief. Fortunately, we now have many options for treating and curing the condition depending upon the etiology, but accurate early diagnoses on the condition and be tricky.”
TN often presents significant challenges in diagnosis, particularly among general practitioners, primary care providers, and emergency physicians who are unaware of the condition or have never had a patient with TN. The lack of awareness of the condition and its sometimes complex or confounding presentation can result in delays or misdiagnoses, and even unnecessary treatments, such as root canals or other procedure, leaving patients to endure debilitating pain without relief. As Dr. Lunsford explains, education and awareness are critical for improving the time to diagnosis.
“Physicians, including those in emergency departments, need to understand the nature of trigeminal neuralgia, its initial management, and how to evaluate patients presenting with the symptoms characteristic of the condition,” says Dr. Lunsford.
Patients with TN typically experience sharp, stabbing, intermittent pain on one side of the face, often localized to the cheek or chin. Pain from TN can often be mistaken for or masquerade as dental or craniofacial conditions or other issues. However, unlike tooth pain due to a dental issue, the pain from TN is very specific and can disappear for long periods and then manifest without warning. The excruciating nature of TN pain can make basic activities like eating or speaking almost impossible, though it may temporarily subside for days or weeks. The intermittent nature also can contribute to delays in diagnosis. Over time, these pain-free intervals shorten, and attacks become more frequent and severe, pushing many patients to seek surgical options when medications fail.
Initial treatments for TN often involve medications such as carbamazepine or gabapentin, but their efficacy can often wane over time or is limited by side effects.
“I would go so far as to say that a great initial response to carbamazepine in TN patients is almost a test for the condition,” says Dr. Lunsford.
Surgical options for TN include microvascular decompression, Gamma Knife® radiosurgery, and minimally invasive percutaneous techniques, depending upon the patient’s specific diagnosis or anatomy, particularly in cases of Secondary TN were tumors or vascular malformations are the culprit.
“Trigeminal neuralgia can be effectively treated, but early recognition and targeted interventions are essential to prevent unnecessary suffering,” says Dr. Lunsford.
UPMC’s Experience and Pioneering Treatments for TN
UPMC has long been recognized as a leader in the management of trigeminal neuralgia. Building on the foundational work of former department chair, Peter J. Jannetta, MD, who pioneered the development and use of microvascular decompression to treat TN, UPMC offers a range of advanced treatment options tailored to the specific circumstances of each patient.
Microvascular decompression relieves nerve compression caused by blood vessels and is considered the gold standard for many cases of TN. Gamma Knife radiosurgery, introduced at UPMC and the University of Pittsburgh and pioneered in the United States by Dr. Lunsford in the mid-1980s uses focused radiation in a minimally invasive approach to disrupt pain pathways in the nerve. Indeed, Dr. Lunsford has cared more than 3,000 patients with TN during his career, either with the Gamma Knife or percutaneous procedures.
For rare causes of TN, like dural arteriovenous fistulas, endovascular approaches provide minimally invasive solutions.
“Endovascular methods allow us to treat complex neurovascular conditions that would otherwise require more invasive and higher-risk open surgical approaches,” says Dr. Lang. “Techniques such as glycerol rhizotomy or balloon compression are also used in select cases to disrupt nerve pain pathways.”
The multidisciplinary structure of UPMC’s Department of Neurosurgery ensures that patients benefit from the collective expertise of specialists in functional neurosurgery, neurovascular imaging, and interventional techniques. This collaborative approach is central to UPMC’s ability to handle complex cases and function as a national referral center for trigeminal neuralgia – and many other conditions affecting the brain and central nervous system.
Endovascular Treatment of Trigeminal Neuralgia Secondary to a Dural Arteriovenous Fistula: A Case Study
A recent case of trigeminal neuralgia treated by Dr. Lang and Dr. Lunsford from 2024 illustrates both the challenges patients can face getting the right diagnosis, but also the clinical prowess and collaborative nature of UPMC’s neurological surgery service in finding the optimal treatment approach for every individual.
Patient History
During the winter of 2022, a 58-year old western Pennsylvania man experienced a slip and fall on the ice at his farm, injuring his shoulder but also hitting the back of his head on the ground. The patient’s shoulder injury was minor, and he recovered well. No issues from the blow to the back of the head were noted at the time and the patient forgot about the incident.
In early 2023, the patient began experiencing numbness in his tongue and on the right side of the roof of the mouth but had no associated pain. The patient also indicated that over time it felt as if his tongue were covered in blisters.
Early evaluations by a dentist and a primary care physician revealed no abnormalities, and initial diagnoses ranged from dental issues to possible autoimmune conditions. Over the following year, the patient’s symptoms progressed to severe facial pain, accompanied by difficulty eating and significant weight loss. He experienced swelling on the right side of his body, beginning with the hand and progressing to the leg, foot, arm and shoulder.
Multiple misdiagnoses, including tooth-related issues and nerve problems, led to several dental interventions, none of which provided relief.
As the facial pain intensified and the episodes of pain increased in frequency and duration, consultations with specialists included evaluations by an ENT physician, a rheumatologist, and an endodontist. Despite these efforts, the cause of the patient’s condition remained undiagnosed.
In mid-March 2024, the patient visited the emergency department at UPMC Passavant due to his pain and deteriorating physical status. A CT scan of the patient’s brain proved inconclusive. The patient was then referred to a neurologist for consultation and discharged from the ED.
The patient was seen in a follow-up telemedicine appointment with a UPMC neurologist who made a diagnosis of trigeminal neuralgia. The patient was prescribed carbamazepine for the pain and was given instructions for obtaining a follow-up MRI scan.
While waiting for the MRI scan, the patient’s dose of carbamazepine was initially 100mg which was subsequently titrated several times to 1,000 mg but its mild initial efficacy completely waned.
After the patient’s MRI scan, but before it could be evaluated by the consulting neurologist, the patient’s family, in a search for answers about trigeminal neuralgia came across Dr. Lunsford’s information online and his history and perspectives on treating the condition. The patient’s family emailed Dr. Lunsford directly, explaining the situation and providing some background about their loved one’s condition, hoping to get some kind of response and perhaps guidance. Since the patient’s history and MRI scan was in the UPMC system, Dr. Lunsford was able to review the imaging remotely during the weekend while catching up on administrative work. His review of the MRI results was met with an immediate recognition of the urgency of the patient’s condition and the presence of a rare dural arteriovenous fistula (DAVF) as the likely cause.
That same day, Dr. Lunsford emailed and then called the patient and his family, explained the situation, and arranged for the patient to be admitted to UPMC Presbyterian that night. He discussed the case with Dr. Lang who agreed that in this particular instance, the patient’s best option for treatment was an endovascular approach to dealing with the likely DAVF and that he and his team would handle the procedure the following day. During the ER admission the patient received an intravenous load of a phenytoin to begin to reestablish pain control.
Treating a Complex DAVF and Secondary TN
Dr. Lang’s approach to treating the patient’s DAVF was executed using advanced endovascular techniques. He began with a diagnostic angiogram, which revealed an abnormal arteriovenous connection on the left side near the entrance of the trigeminal nerve into the brainstem.
“The fistula was located at the sigmoid sinus, a key venous channel at the base of the skull,” says Dr. Lang. “It was causing elevated venous outflow obstruction and resultant venous compression of the opposite sided trigeminal nerve. Interestingly, the actual fistula location highlights the complexity of this condition and the need for advanced imaging to pinpoint the exact cause.”
Dr. Lang’s treatment approach required accessing the DAVF in reverse, through the venous system rather than through the patient’s arteries. This approach was chosen due to the location and anatomy of the fistula at the sigmoid sinus, where direct arterial access posed significant risks of collateral damage and inefficiency in targeting the lesion.
The procedure began with venous access through the patient’s jugular vein. From there, Dr. Lang advanced microcatheters through the dural venous sinuses, navigating upstream to reach the site of the DAVF. This pathway required precision, as the venous system’s anatomy is highly variable and the risk of inadvertently disrupting normal venous drainage is high.
“Working through the veins is more technically challenging,” says Dr. Lang “But in this case, it was the safest and most effective way to reach the fistula without compromising nearby structures.”
This reverse approach allowed Dr. Lang to position the catheters directly at the fistula for the embolization process. Using real-time imaging guidance, he deployed platinum coils to slow blood flow and initiate clot formation during the first stage of treatment.
“Because of our patient’s anatomy and the nature of the fistula, navigating to the area with our catheters and instruments took longer than expected,” says Dr. Lang. “So as to avoid undue amounts of radiation exposure and other complications, we decided to stage the intervention over two sessions.”
In the second procedure, Dr. Lang injected Onyx™, which is a liquid embolic agent (ethyl vinyl alcohol copolymer), through a microcatheter placed during the first session with the patient. Dr. Lang injected the Onyx through the pre-positioned microcatheter in a controlled manner, allowing it to intercalate with the coils and progressively fill the fistula. The agent's dual-phase behavior — remaining liquid in the core while crystallizing at the edges — enabled precise deployment, minimizing the risk of complications, and preserving normal venous drainage.
“Onyx crystallizes gradually, giving us the ability to precisely control its deployment within the vessels we want to occlude,” says Dr. Lang.
The technical challenge of this case was enhanced by the need to avoid blocking normal venous outflow or creating dangerous alterations in blood flow dynamics. Dr. Lang monitored the patient’s blood flow dynamics during the injection to ensure that the Onyx did not inadvertently enter critical venous pathways or adjacent vascular structures. This required detailed knowledge of the patient’s unique venous anatomy and constant adjustments during the procedure to achieve complete occlusion.
Despite these complexities, the fistula was successfully obliterated, as confirmed by post-procedure angiography.
“This was a highly satisfying outcome, not only because we cured the fistula, but also because we avoided the need for open surgery,” says Dr. Lang.
