A Case of Isolated Unilateral Glossopharyngeal Nerve Palsy.

Isolated palsy of the glossopharyngeal nerve is rare. We report the case of an elderly patient with unilateral right glossopharyngeal nerve palsy secondary to extra cranial ischemia. On examination there was no other deficit other than an absent right gag reflex. She was diagnosed clinically with ischemic stroke of the ninth nerve, and her daily dose of aspirin was increased from 81 mg to 325 mg. Magnetic resonance imaging of the brain showed a normal brainstem and cerebellum with patent intracranial circulation. Total resolution of the paralysis was seen 2 months later. The possible mechanisms suspected were diabetic or hypertensive stenosis of the vasa nervorum or compression of the ninth nerve by an internal carotid artery dissection or aneurysm. This article discusses the various etiologies and mechanisms of this rare condition. It is unique because of the nerve's location and relationship to other structures.

Bilateral taste disorders in patients with Ramsay Hunt syndrome and Bell palsy.

OBJECTIVE: Ramsay Hunt syndrome (RHS) and Bell palsy (BP) are typically known as facial nerve motor syndromes and are primarily unilateral. The aim of this study was to challenge this assertion, because both conditions are also known to be associated with viruses that typically affect several nerves. METHODS: Ten participants with RHS, 12 with BP, all clinically unilateral, and 12 healthy controls were prospectively enrolled. Electrogustometric thresholds were measured bilaterally in the areas of the chorda tympani, the glossopharyngeal, and the major petrosal nerve. Also bilaterally, the taste function was tested using chemogustometry with different tastant concentrations. Again bilaterally, the morphology of the mucosa and the vessels of the anterior fungiform papillae were examined by contact endoscopy. Statistically, RHS and BP participants were compared with the healthy controls, and the paretic sides of RHS and BP were compared pairwise with their mobile sides. RESULTS: Electrogustometrically, perception was reduced bilaterally in RHS (10-19dB, p < 0.001) and BP (3-5dB, p = 0.011-0.030) in all 3 innervation areas. Chemogustometrically, it was also reduced bilaterally in RHS (20-70%) and BP (8-50%). Papillary atrophies were increased 100% in RHS (p = 0.001) and BP (p < 0.001). They were more increased on the paretic side in RHS (30%, p = 0.078) and BP (83%, p < 0.001). INTERPRETATION: In these 2 clinically unilateral conditions, the gustatory perception and morphology are bilaterally affected, more in RHS and more on the paretic side. BP, known as an isolated motor condition, appears to be a cranial polyneuritis. A bilateral examination and therapeutic gustatory monitoring might follow these observations in evidence-based practice. Ann Neurol 2018;83:807-815.

Proton Pump Inhibitor Ameliorates Taste Disturbance among Patients with Laryngopharyngeal Reflux: A Randomized Controlled Study.

Patients with laryngopharyngeal reflux (LPR) were reported to suffer from hypogeusia that affects quality of life. Proton pump inhibitor (PPI) is a useful drug in the treatment of LPR, but its effect on hypogeusia is not known. We therefore assessed the effects of PPI or a histamine H2 receptor antagonist (H2 blocker) on hypogeusia among patients with LPR. Both PPI and H2 blocker could inhibit acid reflux. LPR was diagnosed with reflux finding score and reflux symptom index. The visual analogue scale (VAS) of taste disturbance symptoms and the gustatory tests were assessed before and 8 weeks after treatment with esomeprazole, a PPI (20 patients, aged 50.0 ± 1.7 years) or famotidine, a H2 blocker (20 patients, aged 47.1 ± 1.8 years). There were no significant differences in VAS scores and recognition thresholds for four basic tastes between the two groups before treatment. Only PPI therapy significantly decreased the VAS scores, suggesting the improvement of taste perception. Moreover, PPI therapy significantly decreased recognition thresholds for bitter taste in the anterior tongue (chorda tympani nerve area) and the thresholds in the posterior tongue (glossopharyngeal nerve area) for salty, sour, and bitter tastes. By contrast, H2-blocker therapy caused no significant changes of thresholds in the anterior tongue, but improved the threshold only for bitter in the posterior tongue, the value of which was however significantly higher than that in PPI group. In conclusion, PPI could ameliorate hypogeusia by improving bitter, salty, and sour tastes among patients with LPR.

Vernet syndrome resulting from varicella zoster virus infection-a very rare clinical presentation of a common viral infection.

Vernet syndrome is a unilateral palsy of glossopharyngeal, vagus, and accessory nerves. Varicella zoster virus (VZV) infection has rarely been described as a possible cause. A 76-year-old man presented with 1-week-long symptoms of dysphonia, dysphagia, and weakness of the right shoulder elevation, accompanied by a mild right temporal parietal headache with radiation to the ipsilateral ear. Physical examination showed signs compatible with a right XI, X, and XI cranial nerves involvement and also several vesicular lesions in the right ear's concha. He had a personal history of poliomyelitis and chickenpox. Laringoscopy demonstrated right vocal cord palsy. Brain MRI showed thickening and enhancement of right lower cranial nerves and an enhancing nodular lesion in the ipsilateral jugular foramen, in T1 weighted images with gadolinium. Cerebrospinal fluid (CSF) analysis disclosed a mild lymphocytic pleocytosis and absence of VZV-DNA by PCR analysis. Serum VZV IgM and IgG antibodies were positive. The patient had a noticeable clinical improvement after initiation of acyclovir and prednisolone therapy. The presentation of a VZV infection with isolated IX, X, and XI cranial nerves palsy is extremely rare. In our case, the diagnosis of Vernet syndrome as a result of VZV infection was made essentially from clinical findings and supported by analytical and imaging data.

Fetal alcohol exposure reduces responsiveness of taste nerves and trigeminal chemosensory neurons to ethanol and its flavor components.

Fetal alcohol exposure (FAE) leads to increased intake of ethanol in adolescent rats and humans. We asked whether these behavioral changes may be mediated in part by changes in responsiveness of the peripheral taste and oral trigeminal systems. We exposed the experimental rats to ethanol in utero by administering ethanol to dams through a liquid diet; we exposed the control rats to an isocaloric and isonutritive liquid diet. To assess taste responsiveness, we recorded responses of the chorda tympani (CT) and glossopharyngeal (GL) nerves to lingual stimulation with ethanol, quinine, sucrose, and NaCl. To assess trigeminal responsiveness, we measured changes in calcium levels of isolated trigeminal ganglion (TG) neurons during stimulation with ethanol, capsaicin, mustard oil, and KCl. Compared with adolescent control rats, the adolescent experimental rats exhibited diminished CT nerve responses to ethanol, quinine, and sucrose and GL nerve responses to quinine and sucrose. The reductions in taste responsiveness persisted into adulthood for quinine but not for any of the other stimuli. Adolescent experimental rats also exhibited reduced TG neuron responses to ethanol, capsaicin, and mustard oil. The lack of change in responsiveness of the taste nerves to NaCl and the TG neurons to KCl indicates that FAE altered only a subset of the response pathways within each chemosensory system. We propose that FAE reprograms development of the peripheral taste and trigeminal systems in ways that reduce their responsiveness to ethanol and surrogates for its pleasant (i.e., sweet) and unpleasant (i.e., bitterness, oral burning) flavor attributes.NEW & NOTEWORTHY Pregnant mothers are advised to avoid alcohol. This is because even small amounts of alcohol can alter fetal brain development and increase the risk of adolescent alcohol abuse. We asked how fetal alcohol exposure (FAE) produces the latter effect in adolescent rats by measuring responsiveness of taste nerves and trigeminal chemosensory neurons. We found that FAE substantially reduced taste and trigeminal responsiveness to ethanol and its flavor components.

Glossopharyngeal insufflation and breath-hold diving: the more, the worse?

OBJECTIVE: The glossopharyngeal insufflation maneuver (lung packing) is largely performed by competitive breath-hold divers to improve their performance, despite observational evidence of fainting and loss of consciousness in the first seconds of apnea. METHODS: We describe here the time course of hemodynamic changes, induced by breath-holding with and without lung packing, in 2 world-class apnea competitors. RESULTS: When compared with apnea performed after a deep breath (100% vital capacity), lung packing leads to a decrease in cardiac output, blood pressure, and cerebral blood flow during the first seconds after the beginning of apnea. The major hemodynamic disorders were observed in diver 1, who exhibited the greater increase in pulmonary volume after lung packing (+22% for diver 1 vs +10% for diver 2). After the initial drop in both cardiac output and blood pressure, the time course of hemodynamic alterations became quite similar between the two apneas. CONCLUSIONS: Some recommendations, such as limiting the number of maneuvers and performing lung packing in the supine position, should be expressed to avoid injuries secondary to the use of glossopharyngeal insufflation.

Electrogustometry thresholds, tongue tip vascularization, and density and morphology of the fungiform papillae in diabetes.

OBJECTIVES: The aim of this study was to investigate, in parallel, changes in electrogustometric (EGM) thresholds, the morphology and density of the fungiform papillae (fPap), and the shape and density of the vessels at the tip of the human tongue in patients with diabetes mellitus (DM). METHODOLOGY: In 36 patients (19 females, 17 males; 12 subjects with type 1 DM and 24 subjects with type 2 DM), we recorded bilateral EGM-thresholds at the areas innervated by the chorda tympani, the glossopharyngeal nerves, and the greater petrosal nerves. We examined the morphology and density of the fPap and blood vessel density and morphology at the tip of the tongue with contact endoscopy (CE). A group of 36 healthy, age-matched, non-smoking individuals served as controls. RESULTS: The fPap density measured by CE was significantly (p < 0.05) reduced in DM compared to control groups. EGM-thresholds were significantly higher in the DM group than in the control group (p < 0.05). Gender did not have a significant impact on CE and EGM findings within the DM group. Body mass index did not significantly affect EGM-thresholds or the morphology and vascularization of fPap. CONCLUSION: These results suggested that DM significantly reduced gustatory function, based on EGM, and impaired the gustatory anatomical structures, based on CE. Both EGM and CE may be useful in clinical settings to monitor taste disorders in patients with DM.

Glossopharyngeal neuralgia.

In this review, the clinical characteristics, differentiating features from other forms of neuralgia, etiology and treatment options of glossopharyngeal neuralgia will be discussed.

A case of leprosy with multiple cranial neuropathy mimicking Melkerson Rosenthal syndrome.

Involvement of cranial nerves is not uncommon in leprosy with trigeminal and facial nerves being commonly affected. Other cranial nerves can also be involved especially in longstanding cases of leprosy towards the lepromatous pole. Herein, we report a case of leprosy with multiple cranial neuropathy mimicking Melkerson Rosenthal syndrome.

Neuralgia of the glossopharyngeal and vagal nerves: long-term outcome following surgical treatment and literature review.

This study describes our experience in the surgical treatment of neuralgia of the glossopharyngeal and vagal nerves. Over the last 19 years, 21 patients underwent surgery. Their case notes were reviewed to obtain demographic information, clinical presentation, surgical findings and early results. All patients were then contacted by telephone for long-term results and complications. Independent analysis of results was carried out by a Neurology team. Ten patients had microvascular decompression (MVD). Four patients had MVD and nerve section. In the remaining seven patients, the glossopharyngeal and first two rootlets of the vagal nerve were sectioned. Nineteen (90%) of 21 patients experienced complete relief of pain immediately after surgery. The remaining patients reported an improvement in their symptoms. There were no mortalities. Four patients experienced short-term complications, which resolved. Two patients were left with a persistent hoarse voice. At follow-up (mean duration of 4 years), there was no recurrence in symptoms. In our experience, surgery is safe and effective for the treatment of vago-glossopharyngeal neuralgia.

Ameliorating Effect of Microvascular Decompression on Patients with Coexistence of Hemifacial Spasm and Glossopharyngeal Neuralgia: A Retrospective Study.

OBJECTIVE: Microvascular decompression (MVD) has been widely accepted for treating hemifacial spasm (HFS) and glossopharyngeal neuralgia (GN); an effective surgical treatment of coexistent HFS and GN still remains to be determined, however. In this paper we discuss the operative strategy of MVD for patients with coexistent HFS and GN. METHODS: This was a retrospective study. All cases of HFS with or without GN at China-Japan Friendship Hospital from January 2014 to June 2016 have been included. All patients underwent MVD and have been followed up for an average of 1.5 years. RESULTS: A total of 5375 cases of HFS were included, wherein 8 cases coexist with GN. Eight patients had same offending vessel(s) compressing the root entry zone of glossopharyngeal nerve and facial nerve. Posterior inferior cerebellar artery was identified as at least 1 of the offending arteries in all 8 patients. After MVD, spasm ceased in all 8 cases, with 7 cases ceasing immediately and 1 within 2 months. Pain disappeared also in all cases, with 7 cases immediately and 1 case after 4 days. No recurrence or complication was observed during the follow-ups. CONCLUSIONS: HFS combined with ipsilateral GN was rare. MVD could be performed to effectively relieve nerve root compression and associated symptoms for coexistent HFS and GN. Sufficient exposure of root entry zones of both nerves and fully decompression of offending blood vessels and exploratory sequences of different nerve roots are critical points for improving operative effect and reducing complications.

Glial vascular endothelial growth factor overexpression in rat brainstem under tolerable hypoxia: evidence for a central chemosensitivity.

The ventilatory response to hypoxia is mediated by peripheral inputs arising from the arterial chemoreceptors. In their absence, hypoxic adaptation can be achieved, possibly as a result of central cellular reorganization. To study this reorganization, we used chemodenervated rats to investigate the expression and localization of vascular endothelial growth factor (VEGF) in the brainstem. VEGF is a target gene of hypoxia-inducible factor (HIF) that is responsible for the morphofunctional remodeling induced by hypoxia. Intact and chemodenervated rats were subjected to normoxia or hypoxia for 6 hr (10% O(2) in N(2)). VEGF protein was quantified in micropunches of brainstem tissue. Only chemodenervated animals showed an increased VEGF expression in response to hypoxia, whereas, in normoxia, VEGF expression was not modified by chemodenervation. The same hypoxic condition was repeated for 8 days before immunocytochemical staining with anti-VEGF; antiglial fibrillary acidic protein (GFAP), a marker of astrocytes; and anti-rat endothelial cell antigen-1 (anti-RECA-1) that recognizes endothelial cells. Confocal analysis showed a cellular colocalization of GFAP and VEGF, indicating that VEGF was overexpressed predominantly in astrocytes. Increased RECA-1 immunolabeling indicated an enhanced angiogenesis in chemodenervated rats subjected to hypoxia. These results indicate that glial cells and the vascular network contribute to the brainstem remodeling. The peripheral chemodenervation reveals a central O(2) chemosensitivity involving a cascade of gene expression triggered by hypoxia, which in intact animals may act synergically with peripheral chemosensory inputs.

The physiology and pathophysiology of human breath-hold diving.

This is a brief overview of physiological reactions, limitations, and pathophysiological mechanisms associated with human breath-hold diving. Breath-hold duration and ability to withstand compression at depth are the two main challenges that have been overcome to an amazing degree as evidenced by the current world records in breath-hold duration at 10:12 min and depth of 214 m. The quest for even further performance enhancements continues among competitive breath-hold divers, even if absolute physiological limits are being approached as indicated by findings of pulmonary edema and alveolar hemorrhage postdive. However, a remarkable, and so far poorly understood, variation in individual disposition for such problems exists. Mortality connected with breath-hold diving is primarily concentrated to less well-trained recreational divers and competitive spearfishermen who fall victim to hypoxia. Particularly vulnerable are probably also individuals with preexisting cardiac problems and possibly, essentially healthy divers who may have suffered severe alternobaric vertigo as a complication to inadequate pressure equilibration of the middle ears. The specific topics discussed include the diving response and its expression by the cardiovascular system, which exhibits hypertension, bradycardia, oxygen conservation, arrhythmias, and contraction of the spleen. The respiratory system is challenged by compression of the lungs with barotrauma of descent, intrapulmonary hemorrhage, edema, and the effects of glossopharyngeal insufflation and exsufflation. Various mechanisms associated with hypoxia and loss of consciousness are discussed, including hyperventilation, ascent blackout, fasting, and excessive postexercise O(2) consumption. The potential for high nitrogen pressure in the lungs to cause decompression sickness and N(2) narcosis is also illuminated.

Microvascular decompression for glossopharyngeal neuralgia: a long-term retrospectic review of the Milan-Bologna experience in 31 consecutive cases.

OBJECTIVE: To examine surgical findings and results of microvascular decompression (MVD) for glossopharyngeal neuralgia (GN). METHODS: Between 1990 and 2007, 31 consecutive patients affected by drug-resistant GN underwent MVD through a retromastoid keyhole in the supine position with the head rotated to the opposite side. A retrospective analysis was performed that paid particular attention to the relationship among surgical technique, pain control and side effects. RESULTS: A vascular compression of the glossopharyngeal nerve was found in all cases. Twenty-eight out of 31 patients (90.3%) were found to be pain free without medication at long-term follow-up (1-17 years, mean 7.5 years). Three patients (9.7%) were found to require medication to control pain paroxysms that were less frequent and less severe than those observed preoperatively. Two patients required repeated surgery for a drug-resistant recurrence of pain for a total of 33 MVDs. We observed no mortality and did not find any long-term surgical morbidity. Cranial nerve impairment, when observed, always resolved in the following months. CONCLUSIONS: MVD is a safe and effective treatment for GN in patients of all ages.

IVIG- responsive multiple cranial neuropathy: a pharyngo-facial variant of Guillain-Barré syndrome.

We report a case with symptoms of facial swelling, bilateral facial paralysis, dysphagia and aspiration. On electrophysiological studies, the right facial nerve was not excitable. The left facial nerve compound muscle action potential (CMAP) amplitude was severely dispersed and latency was mildly prolonged, consistent with demyelination. Cerebrospinal fluid examinations were normal. Anti-ganglioside antibodies and tumor markers were negative. Bickerstaff brainstem encephalitis, stroke, diabetes mellitus, vasculitis, sarcoidosis, Sjögren's syndrome, Melkersson-Rosenthal Syndrome, trauma, infectious diseases, toxicity, neoplasm, facial onset sensory and motor neuronopathy (FOSMN) and other degenerative diseases were excluded. Intravenous immunoglobulin therapy resolved symptoms of lower cranial nerve dysfunction. Clinically incomplete improvement of bilateral facial paralysis was observed. We conclude that IVIg therapy may improve the symptoms of multiple cranial nerve palsies due to pharyngo-facial variant of Guillain-Barré syndrome.

[Electrophysiological monitoring of cranial motor nerves (V, VII, IX, X, XI, XII)]. / Evaluation électrophysiologique des nerfs crâniens moteurs (V, VII, IX, X, XI, XII).

In various neurosurgical operations, there is a risk of cranial nerve lesion that can be avoided or minimized with intraoperative electrophysiological monitoring. Regarding motor function of the cranial nerves, stimulodetection techniques are used, including electrical stimulation of nerve trunks and electromyographic recording of evoked motor responses. These techniques can be used for monitoring the trigeminal nerve (Vth cranial nerve), facial nerve (VIIth), glossopharyngeal nerve (IXth), pneumogastric nerve (Xth), spinal accessory nerve (XIth), and hypoglossal nerve (XIIth), in particular during surgical removal of tumors of the cerebellopontine angle or skull base. When beginning an operation, electrical stimulation is only used to identify the nerve structures. As removal of the tumor progresses, the goal is to verify that a surgical injury to the nerve is avoided by looking for the absence of any change regarding amplitude, morphology, and latency of motor responses. Intraoperative electromyographic monitoring can also be applied during the surgical treatment of primary hemifacial spasm by microvascular decompression. An effective decompression is usually associated with the disappearance of "lateral spread" motor responses to facial nerve branch stimulation. Therefore, the intraoperative disappearance of the lateral spread responses can be considered a predictive factor of good postoperative clinical outcome, even if this assertion remains a matter of debate.

Laryngeal manifestations of cranial nerve IX/X compression at the brainstem.

OBJECTIVE: We report an association between lower cranial nerve (CN IX/X) vascular compression at the brainstem with laryngeal symptoms utilizing a stepwise algorithm that systematically evaluates and eliminates all other common etiologies. Our experiences with retromastoid craniectomy with lower cranial nerve (LCN) decompression versus non-neurosurgical treatments are detailed. STUDY DESIGN: Retrospective chart review at a tertiary care academic medical center with follow-up telephone survey. METHODS: Baseline demographics, clinical characteristics, quality-of-life surveys, and treatment outcomes were recorded for patients with laryngeal symptoms associated with LCN compression at the brainstem. RESULTS: Forty-nine patients demonstrated LCN compression at the brainstem on imaging and presented with chief complaints of dysphonia (25 of 49, 51%), chronic cough (19 of 49, 39%), dysphoric breathing (3 of 49, 6%), and dysphagia (2 of 49, 4%). Poor initial scores were noted for Voice-Related Quality of Life (V-RQOL), Reflux Symptom Index, and Glottal Closure Index. Twenty-four patients underwent LCN decompression, of which 21 of 24 (88%) reported partial, near-complete, or complete improvement. Major perioperative complications occurred in four of 24 patients (17%). Patients who had undergone decompression were more likely to obtain complete/near-complete symptom resolution (10 of 24 patients, 42%) compared to those undergoing conservative treatments (2 of 25 patients, 8%) (P = 0.02). V-RQOL scores improved more in surgical patients [mean change score, 33.0 (standard deviation [SD], 31.2) than nonsurgical patients (mean change score 9.6, SD 20.9) (P = 0.03) (mean follow-up 3.0 years, SD 2.0). CONCLUSION: Lower cranial nerve compression at the brainstem should be considered when all other etiologies are excluded. Retromastoid craniectomy with LCN decompression demonstrates an acceptable safety profile. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:2105-2111, 2019.

Electrophysiology of Cranial Nerve Testing: Cranial Nerves IX and X.

The cranial nerves IX and X emerge from medulla oblongata and have motor, sensory, and parasympathetic functions. Some of these are amenable to neurophysiological assessment. It is often hard to separate the individual contribution of each nerve; in fact, some of the techniques are indeed a composite functional measure of both nerves. The main methods are the evaluation of the swallowing function (combined IX and X), laryngeal electromyogram (predominant motor vagal function), and heart rate variability (predominant parasympathetic vagal function). This review describes, therefore, the techniques that best evaluate the major symptoms presented in IX and X cranial nerve disturbance: dysphagia, dysphonia, and autonomic parasympathetic dysfunction.

Acute effects of glossopharyngeal insufflation in people with cervical spinal cord injury.

OBJECTIVES: To evaluate acute effects of glossopharyngeal insufflation (GI) on lung function, airway pressure (Paw), blood pressure and heart rate (HR) in people with cervical spinal cord injury (CSCI). DESIGN: Case-control design. SETTING: Karolinska Institutet, Stockholm, Sweden. PARTICIPANTS: Ten participants with CSCI suffering from lesions between C4 and C8, and ASIA classification of A or B were recruited. Ten healthy particpants familiar with GI were recruited as a reference group. OUTCOME MEASURES: Spirometry, mean arterial blood pressure (MAP), Paw, and HR were measured in a sitting and a supine position before, during, and after GI. RESULTS: GI in the study group in a sitting position increased total lung capacity (TLC) by 712 ml: P < 0.001, vital capacity (VC) by 587 ml: P < 0.0001, Paw by 13 cm H2O: P < 0.01, and HR by 10 beats/min: P < 0.001. MAP decreased by 25 mmHg, P < 0.0001. Significant differences were observed between groups comparing baseline with GI. The reference group had a higher increase in; TLC (P < 0.01), VC (P < 0.001), Paw (P < 0.001) and HR (P < 0.05) and a higher decrease in MAP (P < 0.001). With GI in a sitting compared to a supine position, TLC, MAP, HR, Paw remained unchanged in the study group, while residual volume decreased in the supine position (P < 0.01). CONCLUSION: There was a difference between the groups in the increase in TLC; VC; Paw, HR and in the decrease in MAP with GI, however MAP, HR and Paw responded in similar way in both groups in a sitting as well as a supine position. If performed correctly, the risks of GI resulting in clinically significant hemodynamic changes is low, although syncope may still occur.