Добавил:
kiopkiopkiop18@yandex.ru t.me/Prokururor I Вовсе не секретарь, но почту проверяю Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз: Предмет: Файл:
Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5526_Библиотеки_им_академика_М_И_Перельмана.pdf
Скачиваний:
0
Добавлен:
31.08.2026
Размер:
29 Мб
Скачать
50 Glossopharyngeal Neuralgia
1. Clinical History and Symptomatology
A detailed patient history is paramount. Hallmark features include:
• Unilateral, paroxysmal, electric shock-like pain
• Distribution in the posterior oropharynx, tonsillar region, base of the tongue, and occasionally the ear
• Pain episodes triggered by swallowing, talking, chewing, yawning, or coughing
• Short duration (seconds to 1 minute), occurring multiple times daily
In the syncopal or vagal variant, patients may also report:
• Bradycardia, dizziness, presyncope, hypotension, or syncope during pain attacks. These autonomic symptoms suggest vagal nerve involvement, which carries a higher risk of misdiagnosis as a cardiac or psychiatric disorder.
2. Physical and Neurological Examination Physical examination is often normal between attacks, with no sensory de-
cits. Provocative maneuvers such as gentle palpation in the tonsillar fossa or posterior pharynx may occasionally reproduce symptoms. Neurological exami­nation helps rule out other cranial nerve pathologies or mass lesions.
3. Cardiac Monitoring In suspected syncopal variants, electrocardiography (ECG) and 24-hour
Holter monitoring are essential. Transient episodes of bradyarrhythmia tempo­rally associated with pain paroxysms provide objective evidence of vagal involvement. In severe cases, additional autonomic testing or referral to cardiol­ogy may be required.
4. Neuroimaging To investigate possible secondary causes—particularly neurovascular com-
pression—high-resolution brain MRI with MR angiography is the imaging modality of choice. Key ndings may include:
481
• Vascular loops (commonly PICA) in contact with the root entry zone of CN IX or X
• Absence of alternative etiologies such as neoplastic lesions, demyelination, or infectious/inammatory changes
Computed tomography (CT) angiography or three-dimensional (3D) con-
structive interference in steady state (CISS) sequences may be helpful in select cases for better visualization of nerve-vessel interfaces.
5. Differential Diagnosis Several other conditions may mimic GPN and should be excluded:
• Trigeminal neuralgia (V2/V3 distribution, no oropharyngeal trigger)
• Eagle syndrome (stylohyoid ligament elongation; may cause oropharyn­geal pain)
• Glossopharyngeal schwannoma or other cerebellopontine angle tumors
• Multiple sclerosis, particularly in younger patients with dem
482
M. Valença

50.7 Treatment

The spontaneous remissions seen in this patient, followed by recurrence, is not unusual in cranial neuralgias [7]. Such patterns may delay diagnosis, especially when autonomic symptoms dominate the presentation. Furthermore, older adults may be more susceptible to cardiovascular consequences of vagal stimulation, increasing the potential severity of this syndrome.
In terms of management, carbamazepine remains the rst-line treatment, as with trigeminal neuralgia. Other agents such as oxcarbazepine, gabapentin, pregabalin, or lamotrigine may also be tried, though evidence is limited. In this case, carbam­azepine provided partial symptom relief, but persistent pain and bradycardia sug­gest the need to consider surgical intervention or microvascular decompression (MVD) [710], especially if quality of life is signicantly impaired or syncopal episodes worsen.
MVD is the preferred surgical treatment for medically refractory GPN with demonstrable vascular compression. Success rates are high, and complications are relatively low in experienced hands. Alternatively, nerve sectioning, radiofrequency ablation, or stereotactic radiosurgery may be considered in selected cases, though with variable outcomes and higher risks of sensory or motor decits [11, 12].
Given the bilateral neurovascular contacts observed in this patient’s MRI, but the unilateral nature of symptoms, conservative management remains justied at this stage. However, progression or increased cardiovascular involvement would war­rant neurosurgical evaluation.

50.8 Conclusion

Glossopharyngeal neuralgia is a rare but debilitating cranial neuropathy that may occasionally present with autonomic symptoms such as bradycardia or syncope— the so-called syncopal variant. In such cases, a comprehensive evaluation including cardiac monitoring and high-resolution neuroimaging is essential for accurate diagnosis.
This case underscores the importance of considering GPN in elderly patients with paroxysmal oropharyngeal pain and unexplained bradycardia. The nding of neurovascular contact on Angio-MRI supports a secondary etiology and strengthens the indication for surgical treatment in refractory cases.
Although carbamazepine remains the rst-line therapy, partial response and recurrence over time may necessitate escalation of care. Clinicians should remain vigilant for potentially life-threatening autonomic manifestations in this under­recognized syndrome. Early diagnosis and appropriate treatment can signicantly improve patient outcomes and quality of life.
50 Glossopharyngeal Neuralgia
483

References

1. Khan M, Nishi SE, Hassan SN, Islam MA, Gan SH. Trigeminal neuralgia, glossopharyn­geal neuralgia, and myofascial pain dysfunction syndrome: an update. Pain Res Manag. 2017;2017:7438326. https://doi.org/10.1155/2017/7438326.
2. Allam AK, Larkin MB, Sharma H, Viswanathan A.Trigeminal and glossopharyngeal neural­gia. Neurol Clin. 2024;42(2):585–98. https://doi.org/10.1016/j.ncl.2023.12.011.
3. Shah RJ, Padalia D.Glossopharyngeal Neuralgia. In: StatPearls [Internet]. Treasure Island: StatPearls Publishing; 2023.
4. Han A, Montgomery C, Zamora A, Winder E, Kaye A, Carroll C, Aquino A, Kakazu J, Kaye AD.Glossopharyngeal neuralgia: epidemiology, risk factors, pathophysiology, differ­ential diagnosis, and treatment options. Health Psychol Res. 2022;10(5):36042. https://doi.
org/10.52965/001c.36042.
5. Singh PM, Chaturvedi A, Kaushik S.An uncommonly common: glossopharyngeal neuralgia. Ann Indian Acad Neurol. 2013;16(1):1–8.
6. Zakrzewska JM, Linskey ME.Glossopharyngeal neuralgia. In: Handbook of clinical neurol­ogy, vol. 139. Elsevier; 2016. p.215–27.
7. Zhao H, Zhang X, Zhu J, Tang YD, Li ST.Microvascular decompression for glossopharyngeal neuralgia: long-term follow-up. World Neurosurg. 2017;102:151–6. https://doi.org/10.1016/j.
wneu.2017.02.106.
8. Franzini A, Messina G, Franzini A, Marchetti M, Ferroli P, Fariselli L, Broggi G.Treatments of glossopharyngeal neuralgia: towards standard procedures. Neurol Sci. 2017;38(Suppl 1):51–5.
https://doi.org/10.1007/s10072- 017- 2909- 6.
9. Kuzucu P, Türkmen T, Ülkü G, Yaman ME, Aykol Ş. Glossopharyngeal neuralgia as ini­tial symptom in combined hyperactive dysfunction syndrome: case report. Br J Neurosurg. 2023;37(3):309–12. https://doi.org/10.1080/02688697.2020.1817317.
10. Revuelta-Gutiérrez R, Piñon-Jiménez F, Contreras-Vázquez OR, Vales-Hidalgo LO, Martinez­Anda JJ.Glossopharyngeal and vagoglossopharyngeal neuralgia: long-term surgical outcomes in a single institution. Surg Neurol Int. 2023;14:267. https://doi.org/10.25259/SNI_518_2023.
11. Wu Z, Liu J, Fang Q, Yang Y, Fan Y.Safety and efcacy of radiofrequency treatment for glos­sopharyngeal neuralgia: a systematic review and single-arm meta-analysis. Neurosurg Rev. 2025;48(1):341.
12. Zhang J, Li Z, Wang N, Zhang P, Li Y, Chen Y.Efcacy and safety of stereotactic radiosurgery for idiopathic glossopharyngeal neuralgia: a systematic review and meta-analysis. Clin Neurol Neurosurg. 2025;252:108878. https://doi.org/10.1016/j.clineuro.2025.108878.
https://doi.org/10.1007/s10143- 025- 03490- 5.
Chapter 51
Nervus Intermedius Neuralgia
RaizaBorges

51.1 Introduction

Intermediate nerve neuralgia, also known as Ramsay Hunt syndrome, is named after Ramsay Hunt, who in 1908 identied the correlation between herpes zoster, the intermediate nerve, and geniculate neuralgia. Additionally, he described the “Ramsay Hunt zone,” whose distribution corresponded to the innervation of the intermediate nerve, initially considered a branch of the facial nerve [1, 2]. This nerve has parasympathetic functions, contributing to the innervation of the lacrimal and salivary glands, in addition to transmitting sensory information from the tongue, nasal cavity, and auricular concha [3, 4].
Pain attributed to a lesion or disease of the nervus intermedius is a rare condition characterized by episodes of intense pain in the deep auricular region, which may radiate to the parieto-occipital area. In addition to the characteristic pain, associated symptoms may include dysgeusia, reduced lacrimation, and hyperacusis [5]. A lit­erature review conducted by Robblee (2021) found that 2.6% of patients with inter­mediate nerve neuralgia reported hyperacusis, while 7.9% experienced reduced lacrimation [6].
Intermediate nerve neuralgia presents a diagnostic challenge due to its heteroge­neous clinical presentation and the complex overlap of external ear innervation, which involves the trigeminal nerve (auriculotemporal branch), the facial nerve (intermediate nerve), the glossopharyngeal nerve, the vagus nerve, and upper cervi­cal branches. In addition to being frequently mistaken for other cranial neuralgias, its differential diagnosis includes various otolaryngological conditions, such as oti­tis and temporomandibular joint dysfunction [7, 8].
R. Borges (*) Federal University of Pernambuco, Recife, Brazil
Switzerland AG 2026 D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_51
485© The Author(s), under exclusive license to Springer Nature
486
Fig. 51.1 Classication of painful syndromes of the nervus intermedius according to the International Classication of Headache Disorders, third edition (ICHD-3), with subdivisions into nervus intermedius neuralgia and painful nervus intermedius neuropathy
R. Borges
Previously, intermediate nerve neuralgia was considered a single entity. However, the International Classication of Headache Disorders, third edition (ICHD-3) revised this terminology, adopting the designation “Pain attributed to a lesion or disease of the nervus intermedius” [4]. This term has since been subdivided into distinct categories, allowing for a more precise characterization of the different eti­ologies and clinical presentations (Fig.51.1).
According to the third edition of the International Classication of Headache Disorders, nervus intermedius neuralgia (NIN) is dened by brief paroxysms of pain felt deeply in the auditory canal, sometimes radiating to the parieto-occipital region (Panel 51.1).
51 Nervus Intermedius Neuralgia
Panel 51.1 Diagnostic criteria of nervus intermedius neuralgia
according to the ICHD-3
ICHD-3
Description: A rare disorder characterized by brief paroxysms of pain felt deeply in the auditory canal, sometimes radiating to the parieto-occipital region. In the vast majority of cases, vascular compression is found at opera­tion, occasionally with a thickened arachnoidea, but it may develop without apparent cause or as a complication of herpes zoster or, very rarely, multiple sclerosis or tumor. It is provoked by stimulation of a trigger area in the poste­rior wall of the auditory canal and/or periauricular region.
Criteria
A. Paroxysmal attacks of unilateral pain in the distribution of nervus inter-
medius1 and fullling criterion B
B. Pain has all of the following characteristics:
1. Lasting from a few seconds to minutes
2. Severe in intensity
3. Shooting, stabbing or sharp in quality
4. Precipitated by stimulation of a trigger area in the posterior wall of the
auditory canal and/or periauricular region
C. Not better accounted for by another ICHD-3 diagnosis.
487

51.2 Case Presentation

A 58-year-old woman presented approximately 1 year ago with an episode of peripheral facial palsy on the left side. There was no report of blister formation or erythema in the ear. About 15days after the event, she developed deep pain in the left ear, experiencing dozens of daily paroxysms, rated 9 to 10 on the pain scale. The pain could be triggered by stimulation of a periauricular trigger zone. In addition to the intense pain in the inner ear region, she also reported continuous pain in the left hemiface, consistent with the distribution of the trigeminal nerve.
Her medical, family, and social history was unremarkable. Magnetic resonance
imaging (MRI) did not reveal any neurovascular conict. After several weeks of treatment with pregabalin (75mg, twice daily), followed by the addition of dulox­etine (60mg/day), there was a signicant reduction in pain, leading to an improve­ment in quality of life.
488
R. Borges

51.3 Clinical Characteristics

The clinical evaluation begins with the characterization of pain, its location, and possible triggering factors. The physical examination should include inspection of the ear and auditory canal to rule out external otitis, foreign bodies, or visible lesions, as well as palpation of the mastoid and periauricular regions to assess for local inammatory signs. The trigger test for intermediate nerve neuralgia should be performed by stimulating the posterior wall of the auditory canal and the periauricu­lar region to check for pain reproduction. The evaluation of cranial nerves is essen­tial, as decits in the facial (VII), vestibulocochlear (VIII), or glossopharyngeal (IX) nerves may indicate associated pathologies such as pontocerebellar angle tumors or multiple neurovascular compressions. The Schirmer test can be used to assess lacrimal dysfunction, while taste evaluation may reveal alterations in taste perception and help localize the lesion.

51.4 Diagnosis

For an accurate assessment, a detailed anamnesis must be complemented by a thor­ough neurological examination, which is essential for detecting possible alterations in taste, lacrimation, and salivation, as well as for evaluating the involvement of other cranial nerves. This approach is crucial for differentiating between intermedi­ate nerve neuralgia and other neurological conditions.
The diagnosis of intermediate nerve neuralgia is predominantly clinical, but neu-
roimaging is necessary for its subclassication. Brain magnetic resonance imaging (MRI) with angiographic sequences is the exam of choice for investigating associ­ated structural causes. The FIESTA (Fast Imaging Employing Steady-State Acquisition) sequence is benecial, as it provides a detailed visualization of the cranial nerves in the brainstem and facilitates the identication of possible neuro­vascular conicts. The main nding in classic intermediate nerve neuralgia is neu­rovascular compression of the nerve, while MRI also plays a crucial role in detecting demyelinating lesions suggestive of multiple sclerosis and in identifying tumors such as schwannomas and meningiomas in cases of secondary neuralgia [9, 10].

51.5 Treatment

The initial management of intermediate nerve neuralgia generally involves the use of medications for neuropathic pain control, with a particular emphasis on anticon­vulsants and antidepressants [11]. Among the anticonvulsants, the most commonly used include carbamazepine, oxcarbazepine, gabapentin, topiramate, phenytoin,
51 Nervus Intermedius Neuralgia
489
and lamotrigine, due to their ability to modulate neuronal excitability. Carbamazepine and oxcarbazepine are widely cited in the literature for their efcacy in trigeminal neuralgia. Although studies on intermediate nerve neuralgia are limited, it is believed that they may offer similar benets, making them the most frequently reported drugs for treating this condition [6, 12].
Tricyclic antidepressants, such as amitriptyline and nortriptyline, as well as dual-
action antidepressants, are often prescribed due to their ability to inhibit serotonin and norepinephrine reuptake, thereby modulating pain transmission in the central nervous system. When pharmacological treatment does not provide adequate relief, minimally invasive interventions may be considered. Among these approaches, intermediate nerve block with local anesthetics and corticosteroids may offer pain relief, although evidence for sustained benet remains limited [6, 13].
Another option described in the literature is the use of botulinum toxin type A,
which has been extensively studied for the treatment of trigeminal neuralgia. However, to date, no clinical studies are proving its efcacy in intermediate nerve neuralgia, with only isolated case reports suggesting a possible benet as an inter­mediate step before surgical intervention [13].
The surgical treatment of nervus intermedius neuralgia (NIN) is widely described
in the literature and is indicated for cases refractory to pharmacological therapy and minimally invasive approaches. Microvascular decompression is the most com­monly performed procedure, especially when there is evidence of neurovascular conict involving the root of the nervus intermedius, with high success rates. This procedure aims to relieve vascular compression on the nerve, reducing irritation and, consequently, pain episodes [1416].
In cases where microvascular decompression is not feasible or does not result in
signicant clinical improvement, more aggressive neurosurgical options may be considered, such as nerve sectioning, rhizotomy, and tractotomy. These procedures aim to interrupt the transmission of pain signals and are reserved only for highly refractory cases, due to the risk of permanent sensory decits [14, 17].

51.6 Conclusion

Intermediate nerve neuralgia is a rare and challenging condition that is often under­diagnosed due to its symptomatic overlap with other cranial neuralgias. Early rec­ognition of this syndrome is crucial for effective management, enabling the implementation of therapeutic strategies that alleviate pain and enhance patients’ quality of life.
This chapter explored the pathophysiology, clinical presentation, diagnostic
approach, and treatment options for intermediate nerve neuralgia, emphasizing the importance of detailed evaluation and advanced neuroimaging in identifying poten­tial underlying causes.
490
R. Borges
Despite advancements in understanding intermediate nerve neuralgia, the avail-
able literature on this condition remains limited, highlighting the need for more robust studies to enhance knowledge of its mechanisms and validate therapeutic strategies.

References

1. Tubbs RS, Steck DT, Mortazavi MM, Cohen-Gadol AA. The nervus intermedius: a review of its anatomy, function, pathology, and role in neurosurgery. World Neurosurg. 2013;79:763–7.
2. Aleri A, Strauss C, Prell J, Peschke E.History of the nervus intermedius of Wrisberg. Ann Anat-Anatomischer Anzeiger. 2010;192:139–44.
3. Rhoton AL Jr, Kobayashi S, Hollinshead WH. Nervus intermedius. J Neurosurg. 1968;29(6):609–18. https://doi.org/10.3171/jns.1968.29.6.0609.
4. Jordan DR. The nervus intermedius. Arch Ophthalmol. 1993;111(12):1691–2. https://doi.
org/10.1001/archopht.1993.01090120115031.
5. Head Classication Committee of the International Headache Society (IHS). The International Classication of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013;33:629–808.
6. Robblee J.A pain in the ear: two case reports of nervus intermedius neuralgia and nar­rative review. Headache. 2021;61(3):414–21. https://doi.org/10.1111/head.14066. Epub 2021 Feb 15.
7. DeLange JM, Garza I, Robertson CE.Clinical reasoning: a 50-year-old woman with deep stab­bing ear pain. Neurology. 2014;83:e152–7.
8. Harrison E, Cronin M.Otalgia. Aust Fam Physician. 2016;45:493–7.
9. Oh JH, Chung JH, Min HJ, Cho SH, Park CW, Lee SH.Clinical application of 3D-FIESTA image in patients with unilateral inner ear symptom. Korean J Audiol. 2013;17(3):111–7.
https://doi.org/10.7874/kja.2013.17.3.111.
10. Martínez ER, Gredilla Molinero J, Arevalo N, Carretero M, Azpeitia Armán FJ.Study of lower cranial nerves by MRI Fiesta sequence. Madrid: European Congress of Radiology; 2012.
https://doi.org/10.1594/ecr2012/C- 0566.
11. McQuay HJ, Carroll D, Jadad AR, Wiffen P, Moore A.Anticonvulsant drugs for management of pain: a systemic review. BMJ. 1995;311:1047–52.
12. Bendtsen L, Zakrzewska JM, Heinskou TB, Hodaie M, Leal PRL, Nurmikko T, Obermann M, Cruccu G, Maarbjerg S.Advances in diagnosis, classication, pathophysiology, and man­agement of trigeminal neuralgia. Lancet Neurol. 2020;19(9):784–96. https://doi.org/10.1016/
S1474- 4422(20)30233- 7.
13. Erdocia-Goñi A, Hernando de la Bárcena I.The use of botulinum toxin type a in nervus inter­medius neuralgia. Neurología (English Edition). 2020;35(8):618–9.
14. Peris-Celda M, Oushy S, Perry A, Graffeo CS, Carlstrom LP, Zimmerman RS, Meyer FB, Pollock BE, Link MJ.Nervus intermedius and the surgical management of geniculate neural­gia. J Neurosurg. 2019;131(2):343–51. https://doi.org/10.3171/2018.3.JNS172920.
15. Saers SJF, Han KS, de Ru JA.Microvascular decompression may be an effective treatment for nervus intermedius neuralgia. J Laryngol Otol. 2011;125:520–2.
16. Lovely TJ, Jannetta PJ. Surgical management of geniculate neural gia. Am J Otol. 1997;18:512–7.
17. Holste KG, Hardaway FA, Raslan AM, Burchiel KJ.Pain-free and pain-controlled survival after sectioning the nervus intermedius in nervus intermedius neuralgia: a single-institution review. J Neurosurg. 2018;131:352–9.
Chapter 52
Occipital Neuralgia
IdaFortini

52.1 Introduction

Occipital neuralgia (ON) was rst described in 1821 by Benito Lentijo and Martinez Ramos. It is a condition marked by neuropathic pain affecting the distribution of the occipital nerves, including the greater occipital nerve (GON), lesser occipital nerve (LON), and third occipital nerve (TON), either singly or in combination. Although relatively rare, ON predominantly affects women and is considered an uncommon cause of head and neck pain [1].
ON is a relatively rare condition, with an estimated prevalence of approximately 3 per 100.000 people [2]. In a Dutch population, ON comprised 8.3% of facial pain cases. The total incidence was 3.2 per 100,000 people, with a mean age of diagnosis of 54.1years [3]. In a headache outpatient clinic, ON was diagnosed in 1.2% of patients [4].
This condition predominantly affects women, as evidenced by studies showing a higher female prevalence among patients diagnosed with ON [4, 5].
Several risk factors can inuence the development of ON, with a higher preva­lence observed in women, as indicated by research. This condition is prevalent in individuals with multiple sclerosis, where demyelinating lesions in the upper cervi­cal spinal cord, especially at the C2–3 vertebral level, can lead to ON [5]. The patients tend to present with unilateral and episodic symptoms, with associated sen­sory loss. The response to treatment may vary depending on the clinical phenotype of multiple sclerosis [5]. While rare in children, ON can occur with trauma [6].
I. Fortini (*) University of São Paulo, São Paulo, Brazil
Switzerland AG 2026 D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_52
491© The Author(s), under exclusive license to Springer Nature