scholarly journals Concurrent Glossopharyngeal Neuralgia and Hemi-Laryngopharyngeal Spasm (HeLPS): A Case Report and a Review of the Literature

Neurosurgery ◽  
2019 ◽  
Vol 87 (5) ◽  
pp. E573-E577
Author(s):  
C Michael Honey ◽  
Marie T Krüger ◽  
Alan R Rheaume ◽  
Josue M Avecillas-Chasin ◽  
Murray D Morrison ◽  
...  

Abstract BACKGROUND AND IMPORTANCE Hemi-laryngopharyngeal spasm (HeLPS) has been recently described but is not yet widely recognized. Patients describe intermittent coughing and choking and can be cured following microvascular decompression of their Xth cranial nerve. This case report and literature review highlight that HeLPS can co-occur with glossopharyngeal neuralgia (GN) and has been previously described (but not recognized) in the neurosurgical literature. CLINICAL PRESENTATION A patient with GN and additional symptoms compatible with HeLPS is presented. The patient reported left-sided, intermittent, swallow-induced, severe electrical pain radiating from her ear to her throat (GN). She also reported intermittent severe coughing, throat contractions causing a sense of suffocation, and dysphonia (HeLPS). All her symptoms resolved following a left microvascular decompression of a loop of the posterior inferior cerebellar artery that was pulsating against both the IXth and Xth cranial nerves. A review of the senior author's database revealed another patient with this combination of symptoms. An international literature review found 27 patients have been previously described with symptoms of GN and the additional (but not recognized at the time) symptoms of HeLPS. CONCLUSION This review highlights that patients with symptoms compatible with HeLPS have been reported since 1926 in at least 4 languages. This additional evidence supports the growing recognition that HeLPS is another neurovascular compression syndrome. Patients with HeLPS continue to be misdiagnosed as conversion disorder. The increased recognition of this new medical condition will require neurosurgical treatment and should alleviate the suffering of these patients.

2020 ◽  
Vol 55 (6) ◽  
pp. 439-443
Author(s):  
Georgios Zenonos ◽  
Michael M. McDowell ◽  
Hussam Abou-Al-Shaar ◽  
Kenan Alkhalili ◽  
Paul A. Gardner

<b><i>Background:</i></b> Classic geniculate neuralgia (GN) is a rare condition characterized by lancinating pain centered in the ear and not involving the throat. To the best of our knowledge, no case of pediatric GN has been reported in the English literature. <b><i>Case Presentation:</i></b> We present the first reported case of successfully treated GN in a child via an endoscopic approach. The patient was a 9-year-old boy who presented with a 1-year history of lancinating right ear pain. Neuroleptics resulted in a short-lived improvement in symptoms, but with significant side effects. Extensive evaluation by multiple specialties did not reveal a cause for his pain. Imaging disclosed a tortuous loop of the right posterior inferior cerebellar artery abutting cranial nerves IX and X but no other abnormalities. The patient underwent an endoscopic microvascular decompression of cranial nerves IX and X, and sectioning of the nervus intermedius through a right retromastoid craniotomy. Postoperatively, the patient reported complete resolution of his symptoms that persisted at 3 months of follow-up. At the 5-year follow-up, the patient maintained pain relief and was developing normally. <b><i>Conclusion:</i></b> GN can affect the pediatric population. In carefully selected patients with consistent clinical and radiographic presentation, sectioning of the nervus intermedius and microvascular decompression of the lower cranial nerves can be an effective treatment.


2020 ◽  
Vol 8 (2) ◽  
pp. 104-113
Author(s):  
Dezhong Liu

Background:Neurovascular compression syndromes including trigeminal neuralgia (TN) and hemifacial spasm (HFS) are caused by neurovascular conflicts at the root entry zone of the corresponding cranial nerves in the posterior fossa. Microvascular decompression (MVD) is the best choice for the treatment of TN and HFS. An accurate delineation of the responsible vessel could decrease the rate of possible operative complications such as nerve paresis.Methods:In this study, three-dimensional fast imaging employing steady-state acquisition (3D-FIESTA) and three-dimensional time-of-flight magnetic resonance angiography (3D-TOF MRA) were performed on 113 patients with TN or HFS. The imaging data were compared to the intraoperative findings and the accuracy of the data was calculated among the different responsible blood vessels and disease types. The accuracy of the data among different genders, disease durations, disease sides, and disease types was also calculated to identify the target patients for the preoperative diagnostic approach with 3D-FIESTA combined with 3D-TOF MRA.Results:The accuracy of detection with the imaging was above 75% in cases with single-vessel compression. Among these, the accuracy of the preoperative imaging result was the highest when the lesions were in the superior cerebellar artery (SCA; 91.1%). In cases of multiple-vessel compression, however, the coincidence between the preoperative and intraoperative results was only 30.0%. In most of the cases of TN, the responsible blood vessels were in the SCA, and the accuracy in the SCA reached 94.9%. In HFS patients, the responsible blood vessels were in the anterior inferior cerebellar artery (AICA) and posterior inferior cerebellar artery (PICA), and the accuracy was 86.8% and 90.0%, respectively. The differences in the accuracy of the data among different genders, disease durations, disease sides, and disease types were not statistically significant.Conclusion:This study verified the clinical instructional value of 3D-FIESTA combined with 3D-TOF MRA in MVD, and showed that this preoperative examination is reliable for all genders, disease durations, disease sides, and disease types.


2019 ◽  
Vol 130 (6) ◽  
pp. 1865-1869 ◽  
Author(s):  
Christopher R. Honey ◽  
Murray D. Morrison ◽  
Manraj K. S. Heran ◽  
Baljinder S. Dhaliwal

Inducible laryngeal obstruction has been described under at least 40 different monikers, including vocal cord dysfunction, paroxysmal vocal fold motion, and irritable larynx. The etiology of this condition is believed to be laryngeal hyperactivity in response to psychological issues or acid reflux. Most patients are treated with some combination of proton pump inhibitors, speech therapy, and psychotherapy. However, a small cohort of patients remains refractory to all medical interventions. The authors describe a novel condition, hemi-laryngopharyngeal spasm (HELPS), which can cause severe episodic stridor leading to unconsciousness in association with cough. The first recognized and surgically cured patient with HELPS was reported in an earlier issue of this journal. Three additional patients have been followed up for at least a year postoperatively, and their cases are reported here.Each patient presented with a similar pattern of episodic coughing and choking that increased in frequency, severity, and duration over years. The episodes eventually occurred while sleeping and could cause severe stridor with loss of consciousness. All three patients were initially misdiagnosed with a psychiatric illness and subjected to multiple intubations and one tracheostomy. Unilateral botulinum toxin injections in the vocal fold eased the severity of the throat contractions but not the cough. Magnetic resonance imaging showed a looping posterior inferior cerebellar artery juxtaposed to a vagus nerve in each case. Microvascular decompression (MVD) of that vessel relieved all symptoms.The introduction of this new medical condition may help a small cohort of patients with inducible laryngeal obstructions that have not responded to the current standard treatments. Patients are asymptomatic between episodes of progressively severe coughing and choking with stridor that may lead to intubation. Severe anxiety about the unpredictable symptoms is expected and may contribute to a psychiatric misdiagnosis. Microvascular decompression for HELPS is more difficult than that for trigeminal neuralgia because the involved nerve is more susceptible to manipulation. Ultimately, the final proof that HELPS is a real and distinct syndrome will require its recognition and successful treatment by colleagues around the world.


Neurosurgery ◽  
2006 ◽  
Vol 58 (6) ◽  
pp. E1212-E1212 ◽  
Author(s):  
Mahmoud H. Kamel ◽  
Nassir H. Mansour ◽  
Chris Mascott ◽  
Kristian Aquilina ◽  
Steven Young

Abstract OBJECTIVE: The rostral ventrolateral medulla is thought to serve as a final common pathway for the integration of central cardiovascular information and to be important for the mediation of central pressor responses. An association between essential hypertension and neurovascular compression of the rostral ventrolateral medulla has been reported. This may be mediated by an increase in sympathetic tone. CLINICAL PRESENTATION: Schwannomas arising from the lower cranial nerves (Cranial Nerves IX-XI) are rare, constituting only 3% of all intracranial schwannomas unassociated with neurofibromatosis. The majority of these tumors present as jugular foramen lesions and, less commonly, they occur along the extracranial course of these nerves. An intracisternal location is extremely rare. Fewer than 15 cases of pathologically proven intracisternal vagal schwannomas in the absence of neurofibromatosis have been reported. INTERVENTION: We report a case of vagal schwannoma in the cerebellomedullary cistern causing distortion of the vagal root entry zone and presenting with refractory neurogenic hypertension. Total microsurgical excision of this tumor, arising from one of the rootlets of the vagus nerve, was achieved. Immediately postoperatively, blood pressure decreased markedly, and despite our effort to maintain the blood pressure with fluids, the patient developed a cerebral infarction in the watershed zone. CONCLUSION: We discuss the proposed mechanism of hypertension, and the perioperative management, stressing blood pressure control. A review of the literature regarding vagal schwannomas is also presented. To the best of our knowledge, this is the first case report of a cerebellomedullary cistern vagal schwannoma presenting with neurogenic hypertension.


1991 ◽  
Vol 75 (3) ◽  
pp. 388-392 ◽  
Author(s):  
Shinji Nagahiro ◽  
Akira Takada ◽  
Yasuhiko Matsukado ◽  
Yukitaka Ushio

✓ To determine the causative factors of unsuccessful microvascular decompression for hemifacial spasm, the follow-up results in 53 patients were assessed retrospectively. The mean follow-up period was 36 months. There were 32 patients who had compression of the seventh cranial nerve ventrocaudally by an anterior inferior cerebellar artery (AICA) or a posterior inferior cerebellar artery. Of these 32 patients, 30 (94%) had excellent postoperative results. Of 14 patients with more severe compression by the vertebral artery, nine (64%) had excellent results, three (21%) had good results, and two (14%) had poor results; in this group, three patients with excellent results experienced transient spasm recurrence. There were seven patients in whom the meatal branch of the AICA coursed between the seventh and eighth cranial nerves and compressed the dorsal aspect of the seventh nerve; this was usually associated with another artery compressing the ventral aspect of the nerve (“sandwich-type” compression). Of these seven patients, five (71%) had poor results including operative failure in one and recurrence of spasm in four. The authors conclude that the clinical outcome was closely related to the patterns of vascular compression.


Neurosurgery ◽  
1989 ◽  
Vol 25 (4) ◽  
pp. 630-632 ◽  
Author(s):  
Bernardo Fraioli ◽  
Vincenzo Esposito ◽  
Luigi Ferrante ◽  
Lanfranco Trubiani ◽  
Pierpaolo Lunardi

Abstract Three patients with excruciating glossopharyngeal neuralgia underwent microsurgical operations in the posterior cranial fossa. In each patient, neurovascular compression was found involving the posteroinferior cerebellar artery and involving the 9th and 10th cranial nerves. In two of the patients, the compression was caused by arachnoiditis and in the other by an arterial loop. In each patient, neuralgia was successfully eliminated by microvascular decompression and by section of the upper rootlets of the vagus nerve. In one patient, partial section of the 9th cranial nerve was also performed. Because of the frequent involvement of the vagus nerve in the pathogenesis of this condition, open surgery should be preferable to percutaneous thermorhizotomy, which is unable to act selectively on the 10th cranial nerve.


2021 ◽  
Vol 12 ◽  
pp. 570
Author(s):  
Tejas Arvind Sardar ◽  
Viren S. Vasudeva ◽  
M. Neil Woodall

Background: Glossopharyngeal neuralgia is a rare neurovascular compression syndrome that can lead to paroxysmal craniofacial pain and sometimes cardiovascular symptoms.[1,2] The characteristic pathology involves a vessel (commonly a branch/loop of PICA) compressing the nerve at the root entry/exit zone at the brainstem.[1] Microvascular decompression is a commonly used treatment approach for patients that have failed conservative measures.[2] Case Description: A 72-year-old male presented to the ED following four episodes of syncope. The patient had a multi-year history of right-sided burning/stabbing pain involving the submandibular area and posterior throat. His syncope was related to symptomatic bradycardia that would occur during episodes of pain. His pain was exacerbated by speaking and swallowing and could be triggered by placing his finger in the right external auditory meatus. Interestingly, this maneuver would also trigger his bradycardia. The patient had failed previous pharmacotherapy, and a pacemaker had been placed to protect him from periods of hypotension. MRI/MRA of the brain and cervical spine were unremarkable. Due to his profoundly symptomatic status, the patient was offered a right retrosigmoid craniotomy for microvascular decompression of the right glossopharyngeal nerve. The patient had complete resolution of his pain and bradycardia immediately post-operatively. He was discharged on the second postoperative day and his pacemaker was ultimately removed. The patient continues to be pain free and off medication. Conclusion: Here we present a video case report of microvascular decompression with favorable outcome for an interesting presentation of glossopharyngeal neuralgia. The patient gave informed consent for surgery and video recording.


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