Cranial Neuralgias.

OBJECTIVE: The cranial neuralgias are relatively rare, but recognizing these syndromes and distinguishing among them is critical to reducing unnecessary pain and disability for affected patients. Despite their distinctive features, cranial neuralgias may go undiagnosed or misdiagnosed for several years. A notable proportion of cranial neuralgia presentations are due to secondary causes and require targeted treatment. The purpose of this article is to review the diagnosis and management of cranial neuralgias encountered in clinical practice. LATEST DEVELOPMENTS: In 2020, the International Classification of Orofacial Pain was released for the first time. Modeled after the International Classification of Headache Disorders, it includes updated terminology for cranial neuralgias. The underlying pathophysiology of the cranial neuralgias is currently believed to be rooted in both peripheral and central nociceptive systems. In addition, a growing number of familial cases are being identified. Recent therapeutic advancements include a better understanding of how to utilize older therapies and procedures more effectively as well as the development of newer approaches. ESSENTIAL POINTS: Cranial neuralgia syndromes are rare but important to recognize due to their debilitating nature and greater likelihood of having potentially treatable underlying causes. While management options have remained somewhat limited, scientific inquiry is continually advancing the understanding of these syndromes and how best to address them.

Cranial nerve involvement, visual complications and headache syndromes in Lyme disease.

PURPOSE OF REVIEW: To provide a summary of the visual manifestations and cranial neuropathies seen in Lyme disease. RECENT FINDINGS: Lyme facial palsy remains the most common manifestation of Lyme neuroborreliosis. Recent investigations show likely evidence of vagal involvement in Lyme disease. SUMMARY: The literature on Lyme neuroborreliosis continues to evolve. Lyme disease can affect nearly any cranial nerve in addition to causing various headache syndromes. The most common manifestation is Lyme disease facial palsy, occurring in up to 5-10% of patients with documented Lyme disease. Headache syndromes are common in the context of facial palsy but can occur in isolation, and more specific headache syndromes including trigeminal and geniculate neuralgias can occur rarely. Signs and symptoms indicative of vestibulocochlear nerve involvement are relatively common, although it could be that these represent other vestibular involvement rather than a specific cranial neuropathy. Optic neuritis is a controversial entity within Lyme disease and is likely overdiagnosed, but convincing cases do exist. Physicians who see any cranial neuropathy, including optic neuritis, in an endemic area can consider Lyme disease as a possible cause.

Retropharyngeal abscess due to Fusobacterium necrophorum complicated by progressive internal carotid artery stenosis and multiple cranial nerve palsies.

BACKGROUND: A case of retropharyngeal abscess complicated by both artery and nerve injury has rarely been reported. METHODS: A 36-year-old woman suddenly presented with right eye visual loss, dilated pupil, reduced direct light reflex, ptosis and ocular motility disorder on the side of inflammation progression, and was diagnosed with retropharyngeal abscess due to Fusobacterium necrophorum. The patient was treated only with antibiotics and, no further surgery was necessary but tracheotomy. Four months later, MRA showed right ICA occlusion and left ICA stenosis. MRI revealed continuous spread of inflammation due to the abscess from the retropharyngeal to the intracranial space. RESULTS: These severe complications would be attributed to an endothelial damage to the arterial wall and an ischemic neuropathy caused by inflammation and thrombogenesis due to Fusobacterium necrophorum. CONCLUSIONS: This case should provide a better understanding of the mechanism of vascular and cranial nerve injury due to retropharyngeal infections, and highlights the need for early antibiotic therapy and repeated vascular evaluation.

ANCA-associated vasculitis presenting with isolated neurological manifestations in a patient with cocaine abuse: a case report and literature review.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare conditions predominantly affecting small vessels of skin, musculoskeletal, pulmonary, renal, and rarely central and peripheral nervous systems. Isolated neurological manifestations of AAV are uncommon and challenging to diagnose. Cocaine has been reported as a potential trigger for the development of AAV. There are only a few case reports of isolated neurological involvement in cocaine-induced AAV with poorly characterized histopathological features. We present a unique case of AAV with isolated neurological manifestations presenting with multiple cranial neuropathies, leptomeningeal enhancement on imaging and histopathologic evidence of small-vessel vasculitis in the leptomeninges and brain and extensive dural fibrosis in a patient with cocaine abuse. The patient's progressive neurological deficits were controlled after starting immunosuppression with rituximab and prednisone. We also reviewed the literature to provide the diagnostic overview of AAV and evaluate intervention options. To our knowledge, this is the first case of AAV with isolated neurological manifestations and histopathologic evidence of small-vessel vasculitis in a patient with cocaine abuse. Patients with multiple cranial neuropathies and meningeal involvement should be screened for AAV, especially if they have a history of cocaine abuse.

Intraoperative hybrid technique for excision of temporal bone paraganglioma: A case report.

BACKGROUND: Temporal bone paragangliomas are vascularized neoplasms. Although preoperative angioembolization serves as a valuable approach to reduce intraoperative blood loss, it comes with an elevated risk of cranial neuropathies, offers no assurance of complete hemostasis, and precludes real-time adjustments during surgery. METHODS: A 74-year-old patient presented with recurrent episodes of ear bleeding. On examination, a vascular lesion obstructed her external auditory canal. It had the clinical and radiological characteristics of a paraganglioma. Angiography revealed that it had three feeding vessels. RESULTS: The patient was successfully scheduled for hybrid, intraoperative angiography and temporary balloon occlusion of the feeding vessels supplying the lesion instead of preoperative angioembolization. CONCLUSIONS: Utilizing hybrid intraoperative angiography with temporary balloon occlusion during the surgical removal of temporal bone paragangliomas represents an innovative technique that reduces the risk of permanent cranial neuropathies while providing the capacity for real-time adjustments and improved hemostasis.

Comparison of Perioperative Safety of Carotid Artery Stenting and Endarterectomy in the Treatment of Carotid Artery Stenosis: A Meta-Analysis of Randomized Controlled Trials.

BACKGROUND: Current management guidelines for the treatment of carotid stenosis are controversial. We performed this meta-analysis to evaluate the perioperative safety of carotid artery stenting (CAS) and endarterectomy. METHODS: We systematically searched EMBASE, PubMed, Web of Science, and the Cochrane Library from inception to November 10, 2022, for randomized controlled trials that compared CAS with carotid endarterectomy (CEA) among patients with carotid stenosis. The analyzed outcomes mainly included stroke, death, myocardial infarction (MI), cranial nerve palsy, the cumulative incidence of mortality, stroke, or MI and the cumulative incidence of death or stroke in the perioperative periods. The risk ratio (RR) and 95% confidence interval (95% CI) were calculated and pooled. Subgroup analyses were based on whether patients were symptomatic or asymptomatic. We assessed the certainty of evidence using the Grading of Recommendations Assessment, Development and Evaluation framework. RESULTS: Seventeen randomized controlled trials with 12,277 participants (6514 and 5763 in the CAS and CEA groups, respectively) were included. Pooled analysis demonstrated that compared with CEA, CAS was associated with decreased risks of perioperative MI (RR = 0.47, 95% CI = 0.29∼0.77) and perioperative cranial nerve palsy (RR = 0.02, 95% CI = 0.01∼0.06) but higher risks of perioperative stroke (RR = 1.48, 95% CI = 1.18∼1.87) and cumulative incidence of death or stroke (RR = 1.52, 95% CI = 1.20∼1.93). CONCLUSIONS: The perioperative safety was equivalent between CAS and CEA. However, CEA may be preferred when considering both procedural safety and long-term efficacy in preventing recurrent stroke.

Optic Neuritis and Cranial Neuropathies Diagnosis Rates before Coronavirus Disease 2019, in the Initial Pandemic Phase, and Post-Vaccine Introduction.

PURPOSE: To compare rates of diagnosis of neuro-ophthalmic conditions across the Coronavirus Disease 2019 (COVID-19) pandemic with pre-pandemic levels. DESIGN: Multicenter, retrospective, observational study. PARTICIPANTS: Patients seen for eye care between March 11, 2019, and December 31, 2021. METHODS: A multicenter electronic health record database, Sight Outcomes Research Collaborative (SOURCE), was queried for new diagnoses of neuro-ophthalmic conditions (cranial nerve [CN] III, IV, VI, and VII palsy; diplopia; and optic neuritis) and new diagnoses of other ophthalmic conditions from January 1, 2016, to December 31, 2021. Data were divided into 3 periods (pre-COVID, pre-COVID vaccine, and after introduction of COVID vaccine), with a 3-year look-back period. Logistic regressions were used to compare diagnosis rates across periods. Two-sample z-test was used to compare the log odds ratio (OR) of the diagnosis in each period with emergent ocular conditions: retinal detachment (RD) and acute angle-closure glaucoma (AACG). MAIN OUTCOME MEASURES: Diagnosis rate of neuro-ophthalmic conditions in each study period. RESULTS: A total of 323 261 unique patients (median age 59 years [interquartile range, 43-70], 58% female, 68% White) across 5 academic centers were included, with 180 009 patients seen in the pre-COVID period, 149 835 patients seen in the pre-COVID vaccine period, and 164 778 patients seen in the COVID vaccine period. Diagnosis rates of CN VII palsy, diplopia, glaucoma, and cataract decreased from the pre-COVID period to the pre-vaccine period. However, the optic neuritis diagnoses increased, in contrast to a decrease in RD diagnoses (P = 0.021). By comparing the diagnosis rates before and after widespread vaccination, all eye conditions evaluated were diagnosed at higher rates in the COVID vaccination period compared with pre-COVID and pre-vaccine periods. The log OR of neuro-ophthalmic diagnosis rates across every period comparison were largely similar to emergency conditions (RD and AACG, P > 0.05). However, the log OR of cataract and glaucoma diagnoses were different to RD or AACG (P < 0.05) in each period comparison. CONCLUSIONS: Neuro-ophthalmic diagnoses had a similar reduction in diagnosis rates as emergent eye conditions in the first part of the pandemic, except optic neuritis. After widespread COVID-19 vaccination, all ophthalmic diagnosis rates increased compared with pre-pandemic rates, and the increase in neuro-ophthalmic diagnosis rates did not exceed the increase in RD and AACG diagnosis rates. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Spinal procedures, pneumocephalus, and cranial nerve palsies: A review of the literature.

Purpose: Minimally invasive and surgical spine procedures are commonplace with various risks and complications. Cranial nerve palsies, however, are infrequently encountered, particularly after procedures such as lumbar punctures, epidural anesthesia, or intrathecal injections, and are understandably worrisome for clinicians and patients as they may be interpreted as secondary to a sinister etiology. However, a less commonly considered source is a pneumocephalus which may, in rare cases, abut cranial nerves and cause a palsy as a benign and often self-resolving complication. Here, we present the case of a patient who underwent an intrathecal methotrexate infusion for newly diagnosed non-Hodgkin's T-cell lymphoma and subsequently developed an abducens nerve palsy due to pneumocephalus. We highlight the utility of various imaging modalities, treatment options, and review current literature on spinal procedures resulting in cranial nerve palsies attributable to pneumocephalus presenting as malignant etiologies.

Early-Onset Ocular Motor Cranial Neuropathy Is a Strong Predictor of Dementia: A Nationwide, Population-Based Cohort Study.

PURPOSE: To assess the risk of dementia in individuals with newly diagnosed ocular motor cranial neuropathy (OMCN). DESIGN: A nationwide, population-based cohort study using authenticated data from the Korean National Health Insurance Service (KNHIS). PARTICIPANTS: This study included 60 781 patients with OMCN who received a diagnosis between 2010 and 2017 and were followed up through 2018, with an average follow-up of 3.37 ± 2.21 years with a 1-year lag. After excluding patients with disease related to oculomotor dysfunction preceding the OMCN diagnosis, a total of 52 076 patients with OMCN were established. Of these, 23 642 patients who had participated in the National Health Screening Program (NHSP) within 2 years before the OMCN diagnosis were included. After applying the exclusion criteria, the final cohort comprised 19 243 patients and 96 215 age and sex-matched control participants without OMCN. METHODS: We identified patients with newly diagnosed OMCN in the KNHIS database and collected participant characteristics from the health checkup records of the NHSP. The study end point was determined by the first claim with a dementia diagnostic code and antidementia medications. The association of OMCN with dementia risk was examined using Cox proportional hazards regression analysis, adjusting for potential confounding factors. MAIN OUTCOME MEASURES: The main outcome measures were hazard ratios (HRs) and 95% confidence intervals (CIs) for all-cause dementia (ACD), Alzheimer's disease (AD), and vascular dementia (VaD) development in patients with OMCN relative to those without OMCN. RESULTS: Patients with newly diagnosed OMCN demonstrated higher metabolic comorbidities than those without OMCN. New OMCN was associated with an elevated risk of ACD (HR, 1.203; 95% CI, 1.113-1.300), AD (HR, 1.137; 95% CI, 1.041-1.243), and VaD (HR, 1.583; 95% CI, 1.286-1.948), independent of potential confounding factors. The younger age groups exhibited a stronger association between OMCN and ACD (HR, 8.690 [< 50 years] vs. 1.192 [≥ 50 years]; P = 0.0004; HR, 2.517 [< 65 years] vs. 1.099 [≥ 65 years]; P < 0.0001). CONCLUSIONS: This nationwide population-based study assessed the association between OMCN and dementia risk. Our results demonstrated a robust relationship between OMCN and the risk of dementia, particularly in the younger population. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Traumatic cavernous sinus syndrome - A peculiar presentation of multiple cranial nerve neuropathies following a minor head injury: Case report and literature review.

BACKGROUND: In patients with traumatic head injuries, the percentage of cranial nerve injuries (CNI) range from4.3 to 17.6% in which majority are isolated CNI[1-5].In present literature, moderate to severe types of head injuries are often studied which may result in a lack of representation and description of CNI associated with minor head injuries (MHI). Alongside this peculiar case of a traumatic cavernous sinus syndrome (CSS) that is non-thrombotic and non-fistulous in nature, this paper aims to analyse traumatic CNI in non-severe head injuries and the surrounding literature. CASE REPORT: A 65-year-old man who had sustained a minor head injury was found to have CNI of III, IV and VI.Brain imaging showed scattered traumatic subarachnoid haemorrhage and a non-displaced right zygomatic arch fracture. Despite the short course of high dose dexamethasone, he showed only partial recovery of his CNI after one year. CONCLUSION: We present a case of traumatic CSS likely secondary to tractional injury from a MHI. Injury to the extraocular nerves wasfound to be one of the more commonly observed combination of CNI from the literature review conducted. In patients with MHI, multiple CNI is less common. Hence, consideration should be given to work upfor secondary causes such as tumours. There is presently no known clear identifiable pattern of CNI associated with MHI. CT brain findings of skull base fractures and early onset of cranial nerve palsies are generally associated with worse outcomes. More remains to be studied about tractional CNI in non-severe head injuries.

Effective cochlear implantation for idiopathic hypertrophic pachymeningitis with bilateral profound hearing loss: A case report.

RATIONALE: Hypertrophic pachymeningitis (HP) is a local or diffuse fibrous thickness of the dura mater of the brain or spinal cord, caused by infection or connective tissue disease. Headache is the most common clinical symptom, followed by various cranial nerve disorders such as visual impairment, diplopia, and hearing loss. HP can be classified into secondary and idiopathic. Here, we report a case of bilateral progressive profound sensorineural hearing loss diagnosed in a patient with idiopathic HP, where a cochlear implant was effectively used. PATIENT CONCERNS: The patient was a 77-year-old woman. Hearing loss gradually progressed bilaterally, and magnetic resonance imaging showed a space-occupying lesion with a continuous contrast enhancement in the bilateral internal auditory canals, and diffused dural thickening from the middle to the posterior cranial fossa. DIAGNOSES: A trans-labyrinthine biopsy was conducted, and a definite diagnosis of idiopathic HP was made. Thickening of the dura mater in the bilateral internal auditory canals was thought to cause profound hearing loss. INTERVENTIONS AND OUTCOMES: A cochlear implant was implemented 4 months after biopsy, and a favorable hearing response was obtained postoperatively. LESSONS: This is the first report of a cochlear implant in a patient with idiopathic HP. Cochlear implantation was considered a good treatment for profound hearing loss due to idiopathic HP, which provides a reference for patients to receive timely and correct treatment.

Cranial nerve Palsy following COVID-19 Vaccination Responsive to Plasma Exchange.

BACKGROUND: Globally, mass COVID-19 vaccine administration has revealed various adverse effects of the vaccine, such as various neurological symptoms, which are currently identified as a result of an excessive immune response. CASE REPORT: A 70-year-old woman presented with progressive unilateral oculomotor nerve palsy and decreased visual acuity 12 days after receiving the Moderna COVID-19 vaccine. In adults, such palsy is typically caused by microvascular disease (ischemia) or compressive tumors. Given the temporal relationship between vaccination and symptoms and the exclusion of other possible causative factors, the patient's oculomotor nerve palsy and optic nerve involvement was considered to be related to the vaccination. Cranial nerve palsy following COVID-19 vaccination was diagnosed, and after pulse steroid and plasma exchange, the patient showed steady recovery. CONCLUSION: Our patient with cranial nerve palsy following COVID-19 vaccination responded well to plasma exchange after limited improved toward steroid. This case highlights the importance of early identification and treatment of the immunological effects of COVID-19 vaccines on cranial nerves.

Lateral Transorbital Endoscope-Assisted Approach to the Cavernous Sinus.

BACKGROUND AND OBJECTIVES: Surgical access to the cavernous sinus (CS) poses a unique challenge to the neurosurgeon given the concentration of delicate structures in the confines of a very small anatomic space. The lateral transorbital approach (LTOA) is a minimally invasive, keyhole approach that can provide direct access to the lateral CS. METHODS: A retrospective review of CS lesions treated by a LTOA at a single institution was performed between 2020 and 2023. Patient indications, surgical outcomes, and complications are described. RESULTS: Six patients underwent a LTOA for a variety of pathologies including a dermoid cyst, schwannoma, prolactinoma, craniopharyngioma, and solitary fibrous tumor. The goals of surgery (ie, drainage of cyst, debulking, and pathological diagnosis) were achieved in all cases. The mean extent of resection was 64.6% (±34%). Half of the patients with preoperative cranial neuropathies (n = 4) improved postoperatively. There were no new permanent cranial neuropathies. One patient had a vascular injury repaired endovascularly with no neurological deficits. CONCLUSION: The LTOA provides a minimal access corridor to the lateral CS. Careful case selection and reasonable goals of surgery are critical to successful outcome.

Bilateral vocal cord palsy as complication of CNS tuberculosis.

BACKGROUND: Tuberculous meningitis, a rare but severe form of extrapulmonary tuberculosis, frequently affects cranial nerves. While nerves III, VI and VII are commonly involved, involvement of caudal cranial nerves is rarely described. Here, we report a rare case of bilateral vocal cord palsy secondary to caudal cranial nerve involvement in tuberculous meningoencephalitis, that occurred in Germany, a country with low tuberculosis incidence. CASE PRESENTATION: A 71-year-old woman was transferred for further treatment of hydrocephalus as a complication of presumed bacterial meningitis with unknown pathogen at that time. Because of decreased consciousness, intubation was performed and an empiric antibiotic therapy with ampicillin, ceftriaxone and acyclovir was initiated. Upon admission to our hospital, an external ventricular drainage was placed. Cerebrospinal fluid analysis revealed Mycobacterium tuberculosis as the causative pathogen, and antitubercular treatment was initiated. Extubation was possible one week after admission. Eleven days later, the patient developed inspiratory stridor that worsened within a few hours. Flexible endoscopic evaluation of swallowing (FEES) revealed new-onset bilateral vocal cord palsy as the cause of respiratory distress, which required re-intubation and tracheostomy. The bilateral vocal cord palsy persisted despite continued antitubercular therapy on the follow-up examination. CONCLUSION: Considering the aetiology of infectious meningitis, cranial nerve palsies may be suggestive for tuberculous meningitis as underlying disease given their rarity in other bacterial forms of meningitis. Nevertheless, intracranial involvement of inferior cranial nerves is rare even in this specific entity, as only extracranial lesions of inferior cranial nerves have been reported in tuberculosis. With this report of a rare case of bilateral vocal cord palsy due to intracranial involvement of the vagal nerves, we emphasize the importance of timely initiation of treatment for tuberculous meningitis. This may help to prevent serious complications and associated poor outcome since the response to anti-tuberculosis therapy may be limited.

Prevalence of and risk factors for cranial neuropathy in diabetic and non-diabetic patients.

OBJECTIVE: Diabetes mellitus (DM) is a major global health concern and is associated with high morbidity and mortality as well as poor quality of life. This health burden is mostly due to complications associated with DM. Cranial nerve neuropathy is not a well-studied complication of DM. In this study, we aimed to study the prevalence and risk factors for the development of cranial neuropathy in diabetic patients. PATIENTS AND METHODS: This is a cross-sectional study among diabetics who are attending Almanhal Primary Healthcare Center, Abha, Aseer Province, Saudi Arabia. A total of 714 subjects were included, 238 of them were in the study group and 476 were controls chosen randomly from the same community. SPSS program was used to calculate demographic, clinical, and biochemical parameters as well as to measure the statistically significant differences. Analysis was conducted using the SPSS statistical package and p-value lower than or equal to 0.05 was considered statistically significant. RESULTS: The diabetic patients were significantly older than the control group; the mean standard deviation (SD) age was 59.78 (8.26), and 34.04 (9.45) for both study and control groups, respectively. The prevalence of cranial neuropathy was higher in diabetic patients. Among diabetic patients, hyperlipidemia, gestational DM, compliance with DM treatment, and the presence of microvascular complications of DM are significant risk factors for the development of cranial neuropathy. CONCLUSIONS: Our results indicate that the prevalence of cranial neuropathy is higher in the diabetic population than in the non-diabetic population. The oculomotor and trigeminal nerves were the most commonly affected nerves in diabetic patients compared to the abducent and facial nerves in non-diabetic patients.

Third cranial nerve palsy due to COVID-19 infection.

We report a case of a previously healthy man in his 40s who presented with mild SARS-CoV-2 infection (COVID-19) concomitant with acute onset of left third cranial nerve palsy with restricted supraduction, adduction and infraduction. Our patient did not present any history of hypertension, hyperlipidaemia, diabetes mellitus or smoking. The patient recovered spontaneously without any antiviral treatment. To our knowledge, this is the second report of third cranial nerve palsy spontaneously resolved without any risk factors of vascular disease, specific image findings, nor any possible causes other than COVID-19. In addition, we reviewed 10 other cases of third cranial nerve palsy associated with COVID-19, which suggested that the aetiology varies greatly. As a clinician, it is important to recognise COVID-19 as a differential diagnosis for third cranial nerve palsy. Finally, we aimed to encapsulate the aetiologies and the prognosis of the third cranial nerve palsy associated with COVID-19.