West Nile Virus: A Neglected Cause of Bell's Palsy?

The West Nile virus (WNV) is the leading cause of mosquito-borne disease in the United States. Bell's palsy (BP) is a clinical syndrome associated with viral infections, but an association with West Nile virus (WNV) is not well-described, with only two cases reported in the literature. We present a case of a 68-year-old woman presenting with fevers and encephalopathy. Cerebrospinal fluid was positive for WNV. Following improvement, she developed facial weakness and was diagnosed with BP secondary to the WNV infection. Identifying BP associated with WNV infection may have significant clinical implications, but further studies are needed to fully characterize a causative relationship.

Cranial Neuropathy As Initial Manifestation of Primary Sjögren's Syndrome: A Case Series With Literature Review.

The clinical spectrum of primary Sjögren's syndrome (PSS) extends beyond its classical manifestations. This work explores an unusual aspect of PSS, namely the initial presentation of cranial neuropathy. The study was conducted over a period of 22 months, from January 2022 to October 2023. Of 58 PSS patients, only five (four women and one man) had cranial neuropathy as their initial manifestation. Only one patient had sixth cranial nerve involvement, three had acute optic neuritis (second cranial nerve), and three had fifth cranial nerve involvement. The diagnosis of PSS was retained according to the 2016 ACR-EULAR criteria. All patients received symptomatic and immunosuppressive treatments. The course was favorable for all patients. The purpose of this case series is to show that cranial neuropathy can be the initial manifestation of PSS, which should be systematically investigated after the elimination of the most common etiologies of cranial neuropathy, particularly in the elderly.

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.

Imaging spectrum and complications of otogenic infections: insights into delayed diagnosis.

Skull base osteomyelitis (SBO) is a late manifestation of complicated otogenic infections that presents a diagnostic challenge. Delayed or missed diagnoses lead to high morbidity and mortality and can be attributed to non-specific symptoms, subtle early radiologic findings, radiologic mimicry of nasopharyngeal carcinoma (NPC), and under-recognition from clinician and radiologists. This pictorial review aims to emphasize on early imaging recognition and distinction between SBO and NPC.

Electromyography findings in radiation-induced unilateral tongue immobility.

BACKGROUND: We used electromyography to characterize hypoglossal nerve function among radiation-treated head and neck cancer survivors with later onset unilateral tongue immobility. METHODS: Patients with unilateral tongue immobility without evidence of recurrent cancer were seen at a tertiary academic institution between February and September 2021. All patients were at least 2 years post-treatment with radiation therapy for head and neck squamous cell carcinoma. Participants were under annual surveillance and displayed no evidence of operative injury to the hypoglossal nerve. RESULTS: The median symptom-free interval for the 10 patients included in this study was 13.2 years (range 2-25 years). Myokymia alone was present in 3 of 10 patients, fibrillation potentials alone were present in 3 of 10 patients, and 1 subject displayed both fibrillation and myokymia. Three out of 10 patients had normal hypoglossal nerve function. DISCUSSION: These findings highlight how disparate mechanisms may underlie similar clinical presentations of radiation-induced neuromuscular dysfunction.

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.

Tongue electrical impedance myography correlates with functional, neurophysiologic, and clinical outcome measures in long-term oropharyngeal cancer survivors with and without hypoglossal neuropathy: An exploratory study.

BACKGROUND: This pilot study analyzed correlations between tongue electrical impedance myography (EIM), standard tongue electromyography (EMG), and tongue functional measures in N = 4 long-term oropharyngeal cancer (OPC) survivors. METHODS: Patients were screened for a supportive care trial (NCT04151082). Hypoglossal nerve function was evaluated with genioglossus needle EMG, functional measures with the Iowa oral performance instrument (IOPI), and multi-frequency tissue composition with tongue EIM. RESULTS: Tongue EIM conductivity was higher for patients with EMG-confirmed cranial nerve XII neuropathy than those without (p = 0.005) and in patients with mild versus normal EMG reinnervation ratings (16 kHz EIM: p = 0.051). Tongue EIM correlated with IOPI strength measurements (e.g., anterior maximum isometric lingual strength: r2 = 0.62, p = 0.020). CONCLUSIONS: Tongue EIM measures related to tongue strength and the presence of XII neuropathy. Noninvasive tongue EIM may be a convenient adjunctive biomarker to assess tongue health in OPC survivors.

Polyneuritis cranialis as a unique initial presentation of juvenile systemic lupus erythematosus: Case report.

Cranial neuropathy is a rare presentation in juvenile (j) SLE and being multiple is even rarer. We describe here an adolescent girl presenting with polyneuritis cranialis (PNC) as an initial presentation of SLE which had not been reported before in literature. She presented with symptoms suggestive of bilateral abducent and hypoglossal neuropathy with nerve conduction studies showing partial axonal neuropathy of left facial and accessory nerves, 6 weeks after common cold. The condition was not associated with any other neurological or systemic manifestations nor features of Sjogren's syndrome. Her condition responded well to pulsed methylprednisolone therapy and plasma exchange. After exclusion of the common causes and owing to the initial positive ANA results and mild proteinuria, renal biopsy was taken and revealed histopathological features of class III lupus nephritis for which mycophenolate mofetil was started at 1200 mg per m2. Our case highlights the importance of considering collagen disorders including SLE in the differential diagnosis of children presenting with PNC in order to allow adequate management and proper follow-up.

Clinico-Radiological Evaluation for Longitudinal Assessment in Central Skull Base Osteomyelitis: Proposal of Novel Scoring System.

This study aims to evaluate clinical, radiological and laboratory parameters for longitudinal assessment and prognostication in central skull base osteomyelitis (CSBO). Novel radiological score and cranial nerve assessment score (CNAS) have been proposed and analysed along with pain score (VAS), ESR, CRP, WBC count, and HbA1c for utility in disease-monitoring and predicting outcome in CSBO. CSBO cases managed in a tertiary care centre from January 2018 to November 2020, with a minimum follow-up of 6 months were included. The parameters were recorded at presentation, 3-month, 6-month postoperative follow-up, and at completion of therapy, for statistical analysis. Significant positive correlation was found amongst pain score, CNAS, radiological score, ESR, and CRP at different timelines. On longitudinal assessment, there was a statistically significant reduction in above-mentioned parameters, in the cases who recovered. Those with initial radiological score < 30, pain score ≤ 7, and CNAS < 10 showed early clinical improvement, required shorter duration of antimicrobial therapy, and exhibited higher probability of becoming disease-free at an earlier time, compared to those presenting with higher scores. We propose the use of pain score, a novel cranial nerve assessment score, and a novel radiological score for longitudinal assessment in CSBO. The trend in these parameters along with ESR and CRP are useful to monitor the disease process. The initial assessment scores can predict duration of antimicrobial therapy and probability of early recovery. WBC count and HbA1c were neither useful for disease-monitoring nor predicting outcome.

Isolated Lymphomatoid Granulomatosis of the Central Nervous System Mimicking Trigeminal Neuropathy, Bell's Palsy, and Glioblastoma in an Epstein-Barr-Negative Immunocompetent Host: A Case Report.

Lymphomatoid granulomatosis is an Epstein-Barr virus-associated lymphoproliferative B-cell neoplasm that typically involves multiple organ systems. This disease is exceedingly rare when confined to the central nervous system (CNS), usually presenting as a mass lesion or diffuse disease, with no existing standard of care. We present the case of a 67-year-old patient who had a unique and insidious course of isolated CNS lymphomatoid granulomatosis. The disease first presented with cranial neuropathies involving the trigeminal and facial nerves that were responsive to steroids both clinically and radiographically. Two years later, the disease manifested as a parietal mass mimicking high-grade glioma that caused homonymous hemianopsia. The patient underwent craniotomy for resection and was treated with rituximab after surgery. The patient has achieved progression-free survival more than three years after the surgery. Surgical debulking and post-procedural rituximab resulted in favorable survival in a case of isolated CNS lymphomatoid granulomatosis. An intracranial mass preceded by steroid-responsive cranial neuropathies should raise suspicion for lymphoproliferative disorder.

MRI Features of Multiple Cranial Neuropathies in Guillain-Barré Syndrome Occurring after COVID-19 Vaccination: A Case Report.

Guillain-Barré syndrome (GBS) is an immune-mediated demyelinating polyneuropathy characterized by progressive, ascending, and symmetrical paralysis. It is known to be triggered by an antecedent infection or vaccination. Recently, GBS development following coronavirus disease 2019 (COVID-19) vaccination has been reported. Cranial neuropathies in typical GBS patients usually involve the facial and the lower cranial nerves (from IX to XII). We report a rare case of multiple cranial neuropathies involving trigeminal, abducens, and facial nerves in a patient who developed GBS following COVID-19 vaccination on the basis of obvious MRI features.

Jugular Foramen Syndrome Caused by Varicella Zoster Virus Infection.

Jugular foramen syndrome (JFS) is a lower cranial neuropathy syndrome characterized by dysphonia and dysphagia. The syndrome is caused by dysfunction of the glossopharyngeal, vagus, and spinal accessory nerves at the level of the pars nervosa and pars vascularis within the jugular foramen. There are numerous etiologies for JFS, including malignancy, trauma, vascular, and infection. Here, we present the case of a healthy adult man who developed JFS secondary to an atypical presentation of Varicella Zoster meningitis, and was promptly diagnosed and treated with rapid symptom resolution. We diagnosed the patient using specialized skull-based imaging which detailed the jugular foramen, as well as CSF analysis. This case highlights the clinical value of detailed structural evaluation, consideration for infection in the absence of systemic symptoms, and favorable outcomes following early identification and treatment.

Multiple cranial neuropathy due to varicella zoster virus reactivation without vesicular rash: a challenging diagnosis.

Ramsay Hunt syndrome is due to reactivation of varicella zoster virus (VZV) dormant in the geniculate ganglion of the facial nerve. The diagnosis is typically based on clinical triad of ipsilateral facial paralysis, otalgia, and vesicles in the auditory canal or the auricle. However, Ramsay Hunt syndrome may occur without skin eruption in up to one third of patients. Moreover, the involvement of other cranial nerves in addition to the facial nerve has been also reported. Herein, we reported a case report of a man who developed a multiple cranial neuropathy caused by VZV reactivation without skin vesicular eruption. The present case underlines a possible diagnostic challenge that clinicians may hit when facing a common disorder such as peripheral facial palsy. Indeed, clinicians must be aware that Ramsay Hunt syndrome may develop without skin vesicular eruption as well it may be complicated by multiple cranial nerve involvement. Antiviral therapy is effective in VZV reactivation for recovery of nerve function.

Hereditary gelsolin amyloidosis: a rare cause of cranial, peripheral and autonomic neuropathies linked to D187N and Y447H substitutions.

INTRODUCTION: Hereditary gelsolin (AGel) amyloidosis is a systemic disease that is characterised by neurologic, ophthalmologic, dermatologic, and other organ involvements. We describe the clinical features with a focus on neurological manifestations in a cohort of patients with AGel amyloidosis referred to the Amyloidosis Centre in the United States. METHODS: Fifteen patients with AGel amyloidosis were included in the study between 2005 and 2022 with the permission of the Institutional Review Board. Data were collected from the prospectively maintained clinical database, electronic medical records and telephone interviews. RESULTS: Neurologic manifestations were featured in 15 patients: cranial neuropathy in 93%, peripheral and autonomic neuropathy in 57% and bilateral carpal tunnel syndrome in 73% of cases. A novel p.Y474H gelsolin variant featured a unique clinical phenotype that differed from the one associated with the most common variant of AGel amyloidosis. DISCUSSION: We report high rates of cranial and peripheral neuropathy, carpal tunnel syndrome and autonomic dysfunction in patients with systemic AGel amyloidosis. The awareness of these features will enable earlier diagnosis and timely screening for end-organ dysfunction. The characterisation of pathophysiology will assist the development of therapeutic options in AGel amyloidosis.

A Unique Case of Unilateral Oculomotor Nerve Palsy Secondary to Dengue Fever.

A wide range of ocular complications may arise from the mosquito-borne illness, dengue fever. We report a case of isolated unilateral oculomotor nerve palsy due to complications of dengue fever. A 50-year-old male with serologically confirmed dengue fever presented with a sudden onset of double vision with left eyelid drooping and left eye outward deviation on his day 8 of illness. Ocular examination revealed binocular diplopia with complete left eye ptosis and restriction of all left eye movements except for abduction. His left eye pupil was 8 mm dilated with a negative relative afferent pupillary defect (RAPD). A clinical diagnosis of left eye oculomotor nerve palsy with pupil involvement was established. Urgent contrasted brain imaging tests were performed and revealed to be normal. He was managed conservatively and had complete resolution of symptoms with good vision recovery within 3.5 months. Cranial mononeuropathy may be one of the various complications following dengue fever, as demonstrated in this case report. As it is an uncommon presentation, there is a need to exclude other acute causes of cranial nerve palsy. Visual prognosis is still favorable with judicious monitoring and without any treatment of steroids or immunoglobulin.

ANCA-associated refractory vasculitis with multiple systemic involvement: A rare case report.

We report the case of a 65 year old female patient, presenting with a combination of bilateral hearing loss, otalgia, and hyperacusis. Pure tone audiometry revealed mixed bilateral hearing loss. Conventional cranial imaging tests failed to show a significant brain pathology, but fat-suppressed T1-weighted gadolinium-enhanced magnetic resonance imaging scan displayed a diffuse infiltrative skull base process, extending from the nasopharynx to the jugular fossa, and encasing the internal carotid artery. The latter findings, besides elevated inflammatory markers and a positive perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) led to the diagnosis of ANCA-associated vasculitis. Additional disease manifestations sequentially appeared, including a right peripheral nerve palsy, aortitis, hepatitis, peripheral neuropathy, and uveitis. Therapy with corticosteroids, azathioprine, and then cyclophosphamide brought no evident benefit, but rituximab led to impressive clinical and radiologic improvement.

Neuro-Leptospirosis: Experience from a tertiary center of North India.

Leptospirosis is a common zoonotic disease, especially in agricultural countries. Neurological manifestations of leptospirosis (neuroleptospirosis) have been reported in a study with a small number of patients. Here we report seven consecutive patients with neuroleptospirosis admitted to a neurology ward. All seven patients had a meningoencephalitis-like presentation. Leptospirosis was confirmed by polymerase chain reaction. None of the patients had systemic involvement. All patients responded significantly to intravenous ceftriaxone and oral doxycycline, recovering completely. Diagnosis of neuroleptospirosis should always be considered in patients with acute meningoencephalitis along with bacterial and viral encephalitis. Prognosis is good with early diagnosis and appropriate treatment.

ACR Appropriateness Criteria® Cranial Neuropathy: 2022 Update.

Cranial neuropathy can result from pathology affecting the nerve fibers at any point and requires imaging of the entire course of the nerve from its nucleus to the end organ in order to identify a cause. MRI with and without intravenous contrast is often the modality of choice with CT playing a complementary role. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer-reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances in which peer-reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

Isolated abducens nerve palsy: Comparison of microvascular and other causes.

OBJECTIVE: Abducens nerve paralysis is the most common ocular motor neuropathy. In this article, we aimed to compare the causes of isolated abducens nerve palsy in terms of demographic, clinical features, and prognosis. METHODS: Thirty-six isolated abducens nerve palsy patients were prospectively enrolled in the study. The demographic, clinical features, and prognosis compared in two etiological groups as microvascular and other causes. RESULTS: The most common etiology was microvascular, which was seen in 16/36 (44.4%) patients. Mean clinical recovery time was 2.5±1.3 months (range, 10 days-6 months). When etiological groups were compared as microvascular and other causes, the mean age of the microvascular group was significantly higher (62.8±13.3 vs. 44.5±16.4, p=0.001). Diabetes mellitus was seen significantly higher in the microvascular group than other causes group (p=0.001), but no significant difference was observed in terms of other atherosclerotic risk factors (p>0.05). The fasting blood glucose and hemoglobin A1c value were significantly higher in the microvascular group (p=0.02 and p=0.02, respectively). There was no significant difference in terms of clinical improvement and clinical recovery times between groups (p>0.05). CONCLUSION: There is no difference between microvascular group and other causes in terms of clinical outcome, while the mean age and presence of diabetes were higher in the microvascular group. The presence of diabetes should be questioned in cases with isolated abducens nerve palsy.

Surgical and Functional Outcome after Resection of 64 Petroclival Meningiomas.

Objective: The management of petroclival meningiomas (PCMs) remains notoriously difficult due to their close association with neurovascular structures and their complex anatomy, hence the surgical paradigm change from radical to functional resection in the past. With this study, we aimed to analyze surgical and functional outcomes of a modern consecutive series of patients with PCMs. Methods: We reviewed patient charts and imaging data of 64 consecutive patients from 2006 to 2018 with a PCM resected at our institution and compared surgical and functional outcomes between subgroups stratified by surgical approach. Results: Females comprised 67.2% of patients (n = 43), with a mean age of 55 years (median 56; range 21-84). Follow-up data were available for 68.8% and reached a mean of 42.3 months (range 1-129) with a median of 28.5 months. The mean tumor diameter was 37.3 mm (standard deviation (SD) 15.4; median 37.0). Infiltration of the cavernous sinus was observed in 34 cases (53.1%), and the lesions affected the brain stem in 28 cases (43.8%). Preoperative cranial nerve palsy was observed in 73.4% of cases; trigeminal neuropathy (42.2%), hearing loss (32.8%), and impairment of vision (18.8%) were the most common. A retrosigmoid approach was employed in 47 cases (78.1%), pterional in 10 (15.6%), combined petrosal in 2 (3.1%), and transnasal and subtemporal in 1 (1.6%). Fifteen cases (23.4%) were resected in a two-staged fashion. Gross total resection (GTR) was attempted in 30 (46.9%) cases without cavernous sinus infiltration and was achieved in 21 (70.0%) of these cases. Surgical complications occurred in 13 cases (20.3%), most commonly meningitis (n = 4; 6.3%). Postoperatively, 56 patients (87.5%) developed new cranial nerve palsy, of which 36 (63.6%) had improved or resolved on last follow up. Achieving GTR was not significantly associated with higher rates of surgical complications (chi-square; p = 0.288) or postoperative cranial nerve palsy (chi-square; p = 0.842). Of all cases, 20 (31.3%) underwent postoperative radiation. Tumor progression was observed in 10 patients (15.9%) after a mean 102 months (median 124). Conclusions: Surgical resection remains the mainstay of treatment for PCMs, with perioperative cranial neuropathies exhibiting favorable recovery rates. Most essentially, the preselection of patients with hallmarks of brain stem affection and cavernous sinus infiltration should dictate whether to strive for a functionally oriented strategy in favor of radical resection.