Refractory glossopharyngeal neuralgia successfully treated with onabotulinumtoxinA: A case report.

BACKGROUND: Glossopharyngeal neuralgia is a rare but severe and disabling pain condition often caused by vascular compression of the glossopharyngeal nerve. Treatment is similar to that of trigeminal neuralgia, but some patients may be refractory to both medical and surgical approaches. Here we present a case of refractory glossopharyngeal neuralgia that responded well to onabotulinumtoxinA (BTX-A). CASE: We report a case of a 65-year-old man with well-controlled human immunodeficiency virus disease with glossopharyngeal neuralgia symptoms since 2015. He had partial response to medications but was limited by side-effects. He underwent microvascular decompression twice with initial relief both times, but experienced recurrence of attacks 1-3 years after each surgery. He was treated with BTX-A using the chronic migraine PREEMPT protocol (i.e., 31-39 injection sites in head and neck muscles), which led to significant relief of his glossopharyngeal neuralgia pain. CONCLUSIONS: This is the first case to our knowledge of glossopharyngeal neuralgia treated with BTX-A. BTX-A can be an effective treatment for glossopharyngeal neuralgia, even when injections are not administered directly over the sensory distribution of the glossopharyngeal nerve.

Intravenous Fosphenytoin Therapy as an Acute Rescue Treatment for Glossopharyngeal Neuralgia Crisis in Patients Awaiting Neurosurgical Procedures: A Case Series.

INTRODUCTION: Few treatments exist for acute attacks of glossopharyngeal neuralgia (GPN). We investigated the efficacy of intravenous fosphenytoin therapy (IFT) during GPN crisis. CASE PRESENTATION: We evaluated records of 4 consecutive patients with GPN awaiting microvascular decompression (MVD) who received IFT (total, 750 mg). Pain severity was evaluated using a Numerical Rating Scale (NRS). The score was 10 (maximum pain) before treatment. Case 1 (a 52-year-old woman, left GPN): for 12 hours after IFT, pain was eliminated (NRS 0/10); however, severe pain recurred 2 days later. She received MVD 9 days after IFT. Case 2 (a 72-year-old woman, right GPN): pain score reduced to 0/10 immediately after IFT and remained so for 2 days. Severe pain recurred, and she underwent MVD 4 days after IFT. Case 3 (a 69-year-old woman, right GPN): pain was reduced (NRS, 5/10) immediately after IFT and nearly eliminated (1/10) 1 hour later. After 6 hours, severe pain recurred; she received a second IFT 3 days later, and pain score dropped to 1/10. She was pain-free for 24 hours but intermediate pain recurred in 2 days. Microvascular decompression was performed 9 days after the second IFT. Case 4 (a 32-year-old woman, right GPN): Pain score reduced to 0/10 immediately after IFT and remained so for 4 days. She underwent MVD 4 days after IFT. No evidence of recurrence was found throughout the 24-, 22-, 20-, and 5-month follow-ups. CONCLUSIONS: These results provide new insights into the innovative therapeutic option of intravenous fosphenytoin and contribute to advancements in treating acute GPN crisis.

Stridor Due to Cranial Nerve X Palsy Progressing to Polyneuropathy in a Teenager With COVID-19.

The neurologic manifestations of coronavirus disease 2019 (COVID-19) are wide-ranging, including various cranial neuropathies, beyond anosmia and dysgeusia, the exact neuropathological mechanism of which are yet unknown. Acute cranial nerve (CN) X neuritis with vocal cord paralysis has not been reported in COVID-19 and is a rare presentation of neuropathy in general. A girl aged 14 years was admitted with stridor. She was diagnosed with symptomatic COVID-19 8 days before. By presentation, fever had resolved, but she had developed stridor; sore throat with dysphagia; chest, shoulder, and back pain; and generalized weakness. Neurologic examination and laryngoscopy were consistent with isolated left CN X palsy. Steroids were started, but neurologic disease progressed with subjective pain, right lower face numbness, and eye fatigability. Respiratory distress increased, and she was intubated for airway protection. MRI revealed abnormal enhancement of CNs III, V, XII, and X. Cerebrospinal fluid studies were normal. Nasopharyngeal severe acute respiratory syndrome coronavirus 2 polymerase chain reaction test result was positive. She was treated with intravenous immunoglobulin, a total of 2 g/kg, and steroids were continued. She made a full neurologic recovery and was discharged after 9 days of hospitalization. This is a case of a teenager who presented with an acute, life-threatening CN X palsy and development of a progressive polyneuropathy in the setting of COVID-19. Although there was concern for Guillain-Barre syndrome, a definitive diagnosis could not be made, and the unusual features of this case, including presentation with stridor and predominate CN involvement seem to indicate a separate symptomatic COVID-19-associated polyneuritis.

Glossopharyngeal Neuralgia: A Multidimensional Challenge.

Glossopharyngeal neuralgia is an underreported condition characterized by discomfort elicited through involvement of the ninthcranial nerve. Triggering phenomena and associated vagal nerve involvement creates the potential for an unexpected clinical presentation. In this case report, we present a 60-year-old male who described a shock-like pain throughout his neck and jaw. The patient initially responded to carbamazepine but the clinical course was complicated by cardiac pauses with syncope requiring pacemaker implantation. Failure of pharmacologic treatment led to surgical intervention.

A Perforating Artery Compressing the Nerve Rootlet and Causing Glossopharyngeal Neuralgia.

BACKGROUND: A surgical procedure for glossopharyngeal neuralgia (GPN) was selected from microvascular decompression, glossopharyngeal and upper vagal rhizotomy, or a combination of these procedures based on the presence of arteries compressing the glossopharyngeal and vagal rootlets. The offending artery is usually a main trunk or branch of the cerebellar arteries. A perforating artery is a known but uncommon variation of the offending artery that causes GPN. The appropriate procedure for such cases is unknown. OBJECTIVE: To analyze the clinical significance of the perforating artery in GPN, we describe 2 patients with a perforating artery compressing the rootlet, and its mobilization relieved neuralgia. We examined the validity of decompressing a perforating artery as an alternative to rhizotomy in such cases. METHODS: We independently reviewed 12 GPN patients treated with microvascular decompression. The patients' pain severity, medication doses, preoperative imaging studies, intraoperative findings, and outcomes were examined. RESULTS: Eleven patients had neurovascular compression of the glossopharyngeal nerve. In 2 of the patients, a perforating artery compressed the rootlet, thereby generating an indentation and creating a discoloration of the rootlet. Mobilizing the perforating artery with no additional rhizotomy provided complete pain relief with no significant complications and allowed the discontinuation of medications. CONCLUSION: Even a small perforating artery can cause GPN when it compresses the rootlet. In such cases, mobilization of the perforating artery with no additional rhizotomy is an effective surgical option.

[Pediatric reversible acute velar palsy: A case report]. / Paralysie vélaire aiguë réversible de l'enfant : à propos d'un cas.

INTRODUCTION: Approximately 40 cases of acute idiopathic velopharyngeal reversible paralysis in the pediatric population have been reported in the literature. OBSERVATION: We present the case of a 12-year-old boy who had consulted in pediatric emergency departments for symptomatology including rhinolalia, nasal regurgitation, and deviation of the labial commissure. Paraclinical explorations helped diagnose rhombencephalitis with enterovirus. The introduction of oral corticosteroids was followed by rapid clinical improvement in 3 days. Monitoring 1 month later showed complete regression of symptoms. DISCUSSION: Similar cases in the literature describe the occurrence of nasal regurgitation and rhinolalia, sometimes associated with other cranial nerve impairment. The pathogenesis is rarely highlighted and the imaging results are always normal. Ad integrum recovery with or without corticosteroids is the rule. In light of this literature review, it is possible to conclude that the occurrence of such a suggestive clinical picture should limit the often costly and unnecessary additional tests.

Peripheral glycerol injection: an alternative treatment of children with glossopharyngeal neuralgia.

Glossopharyngeal neuralgia is much less common in children and more difficult to relief its pain symptoms than the adults. We report an experience with peripheral glycerol injection for the control of pain in eight sick children with glossopharyngeal neuralgia. At the latest follow-up, 5 cases had a complete pain-free result after the treatment. There were two sick children who were recurred within three months, of which one child was respond to additional injections. It is concluded that the peripheral glycerol injection is safe and effective in the control of pain symptom among the children with glossopharyngeal neuralgia.

Idiopathic cranial polyneuropathy with unilateral IX and X and contralateral XI nerve palsy in a 4-year-old boy.

A 4-year-old boy presented with a sudden onset of nasal escape of fluids, nasal speech, and difficulty placing his left arm through a sleeve. Neurologic examination indicated a unilateral cranial IX and X and contralateral XI nerve palsy that was considered idiopathic. Palsy of cranial nerves IX, X, and XI is rare in childhood, and few reports have described this condition. Our patient received prednisolone for 1 week and demonstrated complete recovery within several weeks. We suggest that aggressive therapy is unnecessary for patients with idiopathic cranial polyneuropathy. The pathogenesis of this condition may involve an immunologic mechanism.

Antiepileptic drugs for the treatment of neuropathic pain: A systematic review

Many therapies have been proposed for the management of neuropathic pain, and they include the use of different antiepileptic drugs. However, the lack of high quality studies indicates that results on the different neuropathic disorders under study do not recommend a particular drug treatment. This study makes a systematic review of the published literature on the use of several antiepileptic drugs to treat neuropathic pain, and has the objective of considering both its clinical characteristics and pharmacological use, which will depend on their level of scientific evidence and will follow the principles of evidence-based dentistry. The articles were stratified according to their scientific evidence using the SORT criteria (Strength of Recommendation Taxonomy), and it included those articles that only have level 1 or 2. Randomized clinical trials were stratified according to their level of quality using the JADAD scale, an instrument described by Jadad et al. (7). to assess the quality of clinical trials, while studies with a level below 3 were discarded. Recently, type A or B recommendations are given in favor or against the use of antiepileptic drugs to treat neuropathic pain on the basis of their scientific quality (AU)

Complex pattern of convulsive syncope in glossopharyngeal neuralgia: video/EEG report and short review.

A 65-year-old woman presented with three "convulsive" events that were preceded by stabbing pain extending from the left submandible zone to the neck and ipsilateral ear. Video-electroencephalography captured a typical attack, where electrocardiography showed bradycardia for 17 seconds and asystole for at least 9 seconds. The patient lost consciousness while her head/gaze turned right. She then manifested tonic extension of her left limbs followed by adduction of her left limb and flexion of her right upper limb. Her gaze deviated upward and her left upper limb manifested swimming-like automatisms. The full episode lasted about 70 seconds, and the EEG showed progressive diffuse high-amplitude slowing. A diagnosis of convulsive syncope resulting from classic glossopharyngeal neuralgia was made. Carbamazepine led to steady remission. Glossopharyngeal neuralgia is a rare condition (incidence of 0.7/100.000/year), whereas the occurrence of syncope is about 20%, and that of convulsive syncope is about 5%.

[Glossopharyngeal neuralgia after resection of a glossopharyngeal schwannoma]. / Glossopharyngeusneuralgie nach Resektion eines Glossopharyngeusschwannoms.

Glossopharyngeal neuralgia is a rare condition and the origin is mostly idiopathic. Causes of symptomatic glossopharyngeal neuralgia can be tumors, infarction or trauma. We report the case of a 28-year-old patient who developed glossopharyngeal neuralgia after resection of a glossopharyngeal schwannoma, which is an extremely rare tumor. Treatment consisted of orally administered pregabalin and a series of injections of buprenorphine in the superior cervical ganglion (ganglionic local opioid application/analgesia, GLOA) which led to a substantial decrease in the frequency of pain attacks. This improvement was maintained at 1-year follow-up. This is the first report of development of glossopharyngeal neuralgia after resection of a glossopharyngeal schwannoma.

Medical management of trigeminal neuropathic pains.

IMPORTANCE OF THE FIELD: Although trigeminal neuralgia has traditionally been considered the prime neuralgic condition in the face region, other forms of neuropathic pain are now being more frequently recognized and require recognition and a different management approach. AREAS COVERED IN THIS REVIEW: This review principally covers medical management of trigeminal neuralgia; but also included is glossopharyngeal neuralgia, trigeminal neuropathic pain (atypical odontalgia) and burning mouth syndrome. Systematic reviews and guidelines will be discussed. WHAT THE READER WILL GAIN: An update will be provided of drug therapy for these relatively rare facial pains. TAKE HOME MESSAGE: Trigeminal neuralgia continues to be best managed using anticonvulsant drugs, the primary ones being carbamazepine and oxcarbazepine; baclofen may be helpful and, of the newly emerging drugs, pregabalin has potential. Glossopharyngeal neuralgia remains managed in the same way as trigeminal neuralgia. Trigeminal neuropathic pain is probably best managed according to guidelines used for the management of neuropathic pain, which include the use of tricyclic antidepressants, gabapentin, pregabalin, duloxetine, venalafaxine and topical lidocaine. Burning mouth syndrome is a neuropathic pain managed initially with topical clonazepam and then with other neuropathic drugs. Patients need to be involved in their management.

Pharmacological treatment of neuropathic facial pain in the dutch general population.

UNLABELLED: Few drugs are registered for treatment of neuropathic facial pain (NFP), and not much is known about treatment choices for NFP in daily practice. Patients with NFP were identified in the IPCI-database with longitudinal electronic general practitioner (GP) records. We described prescription patterns of pain medication following first symptoms. Off-label, off-guideline use, failure and reasons for failure were assessed. Failure was defined as treatment switch, exacerbation, adverse event, or invasive treatment for NFP. Of 203 NFP cases, 160 (79%) received pharmacological pain treatment. Most patients (90%) were initially treated by a GP with anti-epileptic drugs (55%) or NSAIDs (16%) as monotherapy. The median treatment delay was 0 days (range 0 to 2,478 days). Adverse events were experienced by 16 (10%) of patients. Sixty-two percent of first prescriptions were in adherence to guidelines and 59% were considered on-label while 34% of prescriptions were both off-label and off-guideline. Of the first therapy, 38% failed within 3 months. The median duration until failure was 251 days. General practitioners usually are the first to treat NFP. They usually prescribe drugs licensed for NFP and according to guidelines, but the extent of off-label use is substantial. Initial treatment often failed within a short period after starting therapy. PERSPECTIVE: This drug-utilization study describes the pharmacological treatment of different forms of neuropathic facial pain in daily practice. Although treatment is mostly initiated rapidly by general practitioners in a correct way, it often contains off-label or off-guideline medication. Failure of the initial treatment is common and occurs rapidly as well.

Glossopharyngeal neuralgia as onset of multiple sclerosis.

OBJECTIVES AND METHODS: Glossopharyngeal neuralgia is a painful condition, affecting the ninth cranial nerve, rarely described in the course of multiple sclerosis. Here we describe a case of multiple sclerosis presenting with glossopharyngeal neuralgia. RESULTS AND DISCUSSION: We suggest the presence of demyelinating areas at the nerve root entry zone as principal trigger mechanism.