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1.
Semin Cell Dev Biol ; 156: 210-218, 2024 03 15.
Article in English | MEDLINE | ID: mdl-37507330

ABSTRACT

The vagus nerve vitally connects the brain and body to coordinate digestive, cardiorespiratory, and immune functions. Its efferent neurons, which project their axons from the brainstem to the viscera, are thought to comprise "functional units" - neuron populations dedicated to the control of specific vagal reflexes or organ functions. Previous research indicates that these functional units differ from one another anatomically, neurochemically, and physiologically but have yet to define their identity in an experimentally tractable way. However, recent work with genetic technology and single-cell genomics suggests that genetically distinct subtypes of neurons may be the functional units of the efferent vagus. Here we review how these approaches are revealing the organizational principles of the efferent vagus in unprecedented detail.


Subject(s)
Neurons, Efferent , Vagus Nerve , Vagus Nerve/metabolism , Neurons/physiology
2.
Brain ; 147(4): 1399-1411, 2024 Apr 04.
Article in English | MEDLINE | ID: mdl-37972275

ABSTRACT

The most frequent neurodegenerative proteinopathies include diseases with deposition of misfolded tau or α-synuclein in the brain. Pathological protein aggregates in the PNS are well-recognized in α-synucleinopathies and have recently attracted attention as a diagnostic biomarker. However, there is a paucity of observations in tauopathies. To characterize the involvement of the PNS in tauopathies, we investigated tau pathology in cranial and spinal nerves (PNS-tau) in 54 tauopathy cases [progressive supranuclear palsy (PSP), n = 15; Alzheimer's disease (AD), n = 18; chronic traumatic encephalopathy (CTE), n = 5; and corticobasal degeneration (CBD), n = 6; Pick's disease, n = 9; limbic-predominant neuronal inclusion body 4-repeat tauopathy (LNT), n = 1] using immunohistochemistry, Gallyas silver staining, biochemistry, and seeding assays. Most PSP cases revealed phosphorylated and 4-repeat tau immunoreactive tau deposits in the PNS as follows: (number of tau-positive cases/available cases) cranial nerves III: 7/8 (88%); IX/X: 10/11 (91%); and XII: 6/6 (100%); anterior spinal roots: 10/10 (100%). The tau-positive inclusions in PSP often showed structures with fibrillary (neurofibrillary tangle-like) morphology in the axon that were also recognized with Gallyas silver staining. CBD cases rarely showed fine granular non-argyrophilic tau deposits. In contrast, tau pathology in the PNS was not evident in AD, CTE and Pick's disease cases. The single LNT case also showed tau pathology in the PNS. In PSP, the severity of PNS-tau involvement correlated with that of the corresponding nuclei, although, occasionally, p-tau deposits were present in the cranial nerves but not in the related brainstem nuclei. Not surprisingly, most of the PSP cases presented with eye movement disorder and bulbar symptoms, and some cases also showed lower-motor neuron signs. Using tau biosensor cells, for the first time we demonstrated seeding capacity of tau in the PNS. In conclusion, prominent PNS-tau distinguishes PSP from other tauopathies. The morphological differences of PNS-tau between PSP and CBD suggest that the tau pathology in PNS could reflect that in the central nervous system. The high frequency and early presence of tau lesions in PSP suggest that PNS-tau may have clinical and biomarker relevance.


Subject(s)
Alzheimer Disease , Pick Disease of the Brain , Supranuclear Palsy, Progressive , Tauopathies , Humans , Supranuclear Palsy, Progressive/pathology , tau Proteins/metabolism , Pick Disease of the Brain/pathology , Alzheimer Disease/pathology , Tauopathies/pathology , Spinal Nerves , Biomarkers
3.
Genet Med ; : 101216, 2024 Jul 17.
Article in English | MEDLINE | ID: mdl-39033378

ABSTRACT

PURPOSE: To identify genetic etiologies and genotype/phenotype associations for unsolved ocular congenital cranial dysinnervation disorders (oCCDDs). METHODS: We coupled phenotyping with exome or genome sequencing of 467 probands (550 affected and 1108 total individuals) with genetically unsolved oCCDDs, integrating analyses of pedigrees, human and animal model phenotypes, and de novo variants to identify rare candidate single nucleotide variants, insertion/deletions, and structural variants disrupting protein-coding regions. Prioritized variants were classified for pathogenicity and evaluated for genotype/phenotype correlations. RESULTS: Analyses elucidated phenotypic subgroups, identified pathogenic/likely pathogenic variant(s) in 43/467 probands (9.2%), and prioritized variants of uncertain significance in 70/467 additional probands (15.0%). These included known and novel variants in established oCCDD genes, genes associated with syndromes that sometimes include oCCDDs (e.g., MYH10, KIF21B, TGFBR2, TUBB6), genes that fit the syndromic component of the phenotype but had no prior oCCDD association (e.g., CDK13, TGFB2), genes with no reported association with oCCDDs or the syndromic phenotypes (e.g., TUBA4A, KIF5C, CTNNA1, KLB, FGF21), and genes associated with oCCDD phenocopies that had resulted in misdiagnoses. CONCLUSION: This study suggests that unsolved oCCDDs are clinically and genetically heterogeneous disorders often overlapping other Mendelian conditions and nominates many candidates for future replication and functional studies.

4.
Ophthalmology ; 131(3): 288-301, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37832727

ABSTRACT

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.


Subject(s)
Alzheimer Disease , Cranial Nerve Diseases , Humans , Child , Cohort Studies , Retrospective Studies , Risk Factors , Alzheimer Disease/diagnosis
5.
Ophthalmology ; 131(1): 78-86, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37634758

ABSTRACT

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.


Subject(s)
COVID-19 , Cataract , Cranial Nerve Diseases , Glaucoma , Optic Neuritis , Humans , Female , Middle Aged , Male , COVID-19/epidemiology , COVID-19/prevention & control , COVID-19 Vaccines , Diplopia/diagnosis , Diplopia/epidemiology , Pandemics/prevention & control , Retrospective Studies , Optic Neuritis/diagnosis , Optic Neuritis/epidemiology , Paralysis , COVID-19 Testing
6.
J Vasc Surg ; 2024 Aug 12.
Article in English | MEDLINE | ID: mdl-39142450

ABSTRACT

OBJECTIVE: The aim of this prospective monocentric cohort study was to analyze the risk of otolaryngologist-assessed cranial nerve injuries (CNIs) following carotid endarterectomy (CEA) in our academic center during a 15-year period, and to identify possible risk factors for CNI development. METHODS: From January 2007 to December 2022, 3749 consecutive CEAs were performed and their data prospectively recorded in a dedicated database. CNIs were assessed and defined according to a standardized protocol. Instrumental ear, nose, and throat (ENT) evaluations were conducted within 30 days before intervention and before discharge. Preoperative neurological assessments were carried out in all patients with symptomatic carotid stenosis, whereas postoperative neurological evaluations were performed in all patients. Patients with newly onset CNIs underwent follow-up assessments at 30 days and, if necessary, at 6, 12, and 24 months. Perioperative results, including mortality, major central neurological events, and postoperative CNIs, were analyzed. Regression or persistence of lesions during follow-up visits was assessed, and multivariate analysis (binary logistic regression) was conducted to evaluate clinical, anatomical, and surgical technique factors influencing the occurrence of CNIs. RESULTS: CEAs were performed more frequently in male patients (2453 interventions; 65.5%) than in females (1296 interventions; 34.5%). The interventions were performed in asymptomatic patients in 3078 cases (82%). In 66 cases, the interventions followed a previous ipsilateral CEA. At preoperative ENT evaluation, no cases of ipsilateral pre-existent CNI were recorded. The 30-day stroke and death rate was 1%. In 113 patients (3%), a postoperative neck bleeding requiring surgical revision and drainage was noted. Pre-discharge ENT evaluations identified 259 motor CNIs, accounting for 6.9% of the entire study group. Eighteen patients had lesions in more than one cranial nerve. ENT and neurological evaluations at 30 days showed the complete resolution of 161 lesions, whereas in 98 cases (2.6%), the CNI persisted. At 1 year, the rate of persistent CNI was 0.4% (10 patients), whereas at 2 years, it was 0.25% (6 cases), in all but one asymptomatic. At multivariate analysis, urgent intervention in unstable patients, secondary intervention, a clamping time >40 minutes, a hematoma requiring revision, and a postoperative stroke were independent predictors of CNIs. CONCLUSIONS: Data from this prospective monocentric cohort study showed that the occurrence of CNI following CEA was low, even when an independent multi-specialist evaluation was performed. The percentage of persistent lesions at 2 years was negligible and, in most cases, asymptomatic.

7.
J Neurooncol ; 170(1): 119-128, 2024 Oct.
Article in English | MEDLINE | ID: mdl-39088156

ABSTRACT

PURPOSE: Cranial Nerve Neuropathies (CNNs) often accompany Cavernous Sinus Meningioma (CSM), for which Stereotactic Radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSR) are established treatments. This study assesses CNNs recovery in CSM patients treated with LINAC, offering insight into treatment effectiveness. METHODS: This study was conducted on 128 patients with CSM treated with LINAC-based SRS/FSR between 2005 and 2020 at a single institution. 46 patients presented with CNNs. The study analyzed patients' demographics, clinical parameters, SRS/FSR treatment characteristics, post-treatment CNNs recovery duration, status, and radiological control on their last follow-up. RESULTS: The median follow-up duration was 53.4 months. Patients were treated with SRS (n = 25) or FSR (n = 21). The mean pretreatment tumor volume was 9.5 cc decreasing to a mean end-of-follow-up tumor volume was 5.1 cc. Radiological tumor control was achieved in all cases. CNN recovery was observed in 80.4% of patients, with specific nerve recoveries documented as follows: extra-ocular nerves (43.2%), trigeminal nerve (32.4%), and optic nerve (10.8%). A higher CNNs recovery rate was associated with a smaller pre-treatment tumor volume (p < 0.001), and the median time-to-improvement was 3.7 months. Patients with tumor volumes exceeding 6.8 cc and those treated with FSR exhibited prolonged time-to-improvement (P < 0.03 and P < 0.04 respectively). CONCLUSIONS: This study suggests that SRS/FSR for CSM provides good and sustainable CNNs recovery outcomes with excellent long-term radiological control. A higher CNNs recovery rate was associated with a smaller pre-treatment tumor volume. while shorter time-to-improvement was identified in patients treated with SRS compared to FSR, particularly in those with small pre-treatment tumor volume.


Subject(s)
Cavernous Sinus , Cranial Nerve Diseases , Meningeal Neoplasms , Meningioma , Radiosurgery , Humans , Radiosurgery/adverse effects , Radiosurgery/methods , Meningioma/surgery , Meningioma/radiotherapy , Meningioma/pathology , Male , Female , Middle Aged , Cavernous Sinus/pathology , Cavernous Sinus/surgery , Meningeal Neoplasms/surgery , Meningeal Neoplasms/radiotherapy , Meningeal Neoplasms/pathology , Aged , Cranial Nerve Diseases/etiology , Adult , Follow-Up Studies , Retrospective Studies , Recovery of Function , Aged, 80 and over , Treatment Outcome
8.
Eur J Neurol ; 31(10): e16398, 2024 Oct.
Article in English | MEDLINE | ID: mdl-39030970

ABSTRACT

BACKGROUND AND PURPOSE: Cervical artery dissection (CAD) represents a leading cause of unilateral lower cranial nerve IX-XII palsy, known as Collet-Sicard syndrome (CSS). High-resolution magnetic resonance imaging (HR-MRI) is widely used in the evaluation of patients with CAD, providing information regarding vessel wall abnormalities and intraluminal thrombus. METHODS: We present a patient with palsy of multiple lower cranial nerves in the context of CSS, attributed to unilateral spontaneous internal carotid artery dissection. RESULTS: We describe a 68-year-old man with unremarkable previous history, who presented with subacute, gradually worsening dysphagia and hoarse voice. Clinical examination revealed right-sided palsy of cranial nerves IX-XII. Three-dimensional fat-saturated black-blood T1-weighted high-resolution vessel wall imaging disclosed spontaneous dissection with intramural hematoma along the distal right internal carotid artery. Neck MRI showed inward displacement of right aryepiglottic fold, right pyriform sinus dilatation, and right true vocal cord in middle position, indicative of right vagus nerve palsy, atrophy of right trapezius and sternocleidomastoid muscles, due to right spinal accessory nerve palsy, and unilateral tongue atrophy with fatty infiltration, characteristic for right hypoglossal nerve palsy. CONCLUSIONS: This case highlights the utility of high-resolution vessel wall imaging and especially fat-saturated T1-weighted black-blood SPACE (sampling perfection with application-optimized contrast using different flip-angle evolutions) sequences in the accurate diagnosis of CAD, revealing the characteristic mural hematoma and intimal flap. HR-MRI is also valuable in the recognition of indirect signs of lower cranial nerve compression.


Subject(s)
Magnetic Resonance Imaging , Humans , Male , Aged , Carotid Artery, Internal, Dissection/diagnostic imaging , Carotid Artery, Internal, Dissection/complications , Cranial Nerve Diseases/etiology , Cranial Nerve Diseases/diagnostic imaging
9.
Neuroradiology ; 66(6): 955-961, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38407581

ABSTRACT

PURPOSE: Cranial nerve involvement (CNI) influences the treatment strategies and prognosis of head and neck tumors. However, its incidence in skull base chordomas and chondrosarcomas remains to be investigated. This study evaluated the imaging features of chordoma and chondrosarcoma, with a focus on the differences in CNI. METHODS: Forty-two patients (26 and 16 patients with chordomas and chondrosarcomas, respectively) treated at our institution between January 2007 and January 2023 were included in this retrospective study. Imaging features, such as the maximum diameter, tumor location (midline or off-midline), calcification, signal intensity on T2-weighted image, mean apparent diffusion coefficient (ADC) values, contrast enhancement, and CNI, were evaluated and compared using Fisher's exact test or the Mann-Whitney U-test. The odds ratio (OR) was calculated to evaluate the association between the histological type and imaging features. RESULTS: The incidence of CNI in chondrosarcomas was significantly higher than that in chordomas (63% vs. 8%, P < 0.001). An off-midline location was more common in chondrosarcomas than in chordomas (86% vs. 13%; P < 0.001). The mean ADC values of chondrosarcomas were significantly higher than those of chordomas (P < 0.001). Significant associations were identified between chondrosarcomas and CNI (OR = 20.00; P < 0.001), location (OR = 53.70; P < 0.001), and mean ADC values (OR = 1.01; P = 0.002). CONCLUSION: The incidence of CNI and off-midline location in chondrosarcomas was significantly higher than that in chordomas. CNI, tumor location, and the mean ADC can help distinguish between these entities.


Subject(s)
Chondrosarcoma , Chordoma , Skull Base Neoplasms , Humans , Female , Male , Retrospective Studies , Middle Aged , Chordoma/diagnostic imaging , Chordoma/pathology , Adult , Chondrosarcoma/diagnostic imaging , Chondrosarcoma/pathology , Aged , Skull Base Neoplasms/diagnostic imaging , Contrast Media , Adolescent , Magnetic Resonance Imaging/methods
10.
Am J Emerg Med ; 83: 47-53, 2024 Sep.
Article in English | MEDLINE | ID: mdl-38959601

ABSTRACT

INTRODUCTION: Cavernous sinus thrombosis (CST) is a serious condition that carries with it a high rate of morbidity and mortality. OBJECTIVE: This review highlights the pearls and pitfalls of CST, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. DISCUSSION: CST is a potentially deadly thrombophlebitic disease involving the cavernous sinuses. The most common underlying etiology is sinusitis or other facial infection several days prior to development of CST, though other causes include maxillofacial trauma or surgery, thrombophilia, dehydration, or medications. Staphylococcus aureus, streptococcal species, oral anaerobic species, and gram-negative bacilli are the most frequent bacterial etiologies. The most prevalent presenting signs and symptoms are fever, headache, and ocular manifestations (chemosis, periorbital edema, ptosis, ophthalmoplegia, vision changes). Cranial nerve (CN) VI is the most commonly affected CN, resulting in lateral rectus palsy. Other CNs that may be affected include III, IV, and V. The disease may also affect the pulmonary and central nervous systems. Laboratory testing typically reveals elevated inflammatory markers, and blood cultures are positive in up to 70% of cases. Computed tomography of the head and orbits with intravenous contrast delayed phase imaging is recommended in the ED setting, though magnetic resonance venography demonstrates the highest sensitivity. Management includes resuscitation, antibiotics, and anticoagulation with specialist consultation. CONCLUSION: An understanding of CST can assist emergency clinicians in diagnosing and managing this potentially deadly disease.


Subject(s)
Cavernous Sinus Thrombosis , Humans , Cavernous Sinus Thrombosis/diagnosis , Cavernous Sinus Thrombosis/therapy , Cavernous Sinus Thrombosis/etiology , Emergency Service, Hospital , Tomography, X-Ray Computed , Prevalence , Anticoagulants/therapeutic use , Anti-Bacterial Agents/therapeutic use
11.
Neurosurg Rev ; 47(1): 236, 2024 May 28.
Article in English | MEDLINE | ID: mdl-38802695

ABSTRACT

Pituitary apoplexy is a rare and potentially life-threatening clinical syndrome. Patients may present with severeneuro-ophthalmologic or endocrine symptoms. Current evidence is unclear whether conservative or surgicalmanagement leads to the best neuroendocrine outcomes. This study aimed to compare neuroendocrine outcomesbetween surgical and conservative treatments in a single center. Cases of patients with pituitary apoplexy whoreceived transsphenoidal surgery or conservative management in Songklanagarind Hospital between January 1,2005 and December 31, 2022 were retrospectively reviewed. A propensity score matching method was used toadjust bias from treatment selection (surgery or conservative treatment). Differences in visual field, visual acuity,cranial nerve, and endocrine outcomes between the surgical and conservative treatment groups were analyzedusing logistic regression analysis. This study included 127 patients, with 98 and 29 patients in the surgical and theconservative treatment group, respectively. The optimal matching method was used for propensity score matching.Compared to the conservative group, the surgically treated patients had a significantly higher rate of visual fieldrecovery (odds ratio (OR): 12.89, P = 0.007). However, there were no statistical differences in the recovery rate ofpreoperative visual acuity, cranial nerve, and endocrine deficits between the groups. Transsphenoidal surgery wasassociated with a higher rate of visual field recovery when compared to the conservative treatment for pituitaryapoplexy patients. Careful selection of appropriate treatment based on the patient's presentation andneuroendocrine status will result in the best outcomes while avoiding unnecessary surgical intervention.


Subject(s)
Conservative Treatment , Pituitary Apoplexy , Propensity Score , Humans , Male , Female , Middle Aged , Pituitary Apoplexy/surgery , Pituitary Apoplexy/therapy , Conservative Treatment/methods , Aged , Adult , Retrospective Studies , Treatment Outcome , Neurosurgical Procedures/methods , Visual Acuity/physiology , Pituitary Neoplasms/surgery , Recovery of Function
12.
Neurosurg Rev ; 47(1): 358, 2024 Jul 26.
Article in English | MEDLINE | ID: mdl-39060848

ABSTRACT

OBJECTIVES: This study aimed to investigate the association between intracerebral aneurysms and cranial nerve (CN) palsies, focusing on nerves other than the oculomotor nerve. It sought to determine the prevalence, risk factors, and clinical outcomes of these nerve palsies and compare the effectiveness of microsurgical clipping versus endovascular coiling in restoring nerve function. METHODOLOGY: Following PRISMA guidelines, a comprehensive literature search was conducted using databases like PubMed, Scopus, and Google Scholar, covering studies from 1975 to April 2024. The inclusion criteria targeted patients with non-oculomotor nerve palsies diagnosed with cerebral aneurysms. Studies published before 1975 and non-English studies were excluded. Data extraction included study design, patient characteristics, and intervention outcomes. The Joanna Briggs Institute and Newcastle-Ottawa scales were used to assess study quality. Data were synthesized narratively and statistically analysed using SPSS v27. RESULTS: The analysis included 47 patients (53.2% female, mean age 44.8 years). The internal carotid artery (ICA) was the most common aneurysm site (44.7%), and the abducent nerve (CN VI) was most frequently affected. Ruptured aneurysms had better recovery outcomes (88.9%) than unruptured ones (66.7%). Hypertension was present in 9.2%. Unilateral aneurysms were seen in 80.9%, with 76.6% having a single nerve palsy. Non-ruptured aneurysms accounted for 58.1%, and ruptured for 41.9% of associated cranial nerve palsies. Treatment included microsurgical approaches (42.6%), endovascular approaches (34%), combined approaches (6.4%), and conservative management (17%). Recovery of the palsy was observed in 75.6%, with endovascular procedures showing higher recovery (93.3%) compared to conservative treatment (28.6%). CONCLUSION: Intracerebral aneurysms are significantly associated with non-oculomotor CN palsies. Endovascular procedures yield higher recovery rates than conservative management, particularly in ruptured aneurysms. Timely and appropriate treatment is crucial for improving nerve function recovery in these patients.


Subject(s)
Cranial Nerve Diseases , Intracranial Aneurysm , Humans , Intracranial Aneurysm/surgery , Intracranial Aneurysm/complications , Cranial Nerve Diseases/epidemiology , Female , Endovascular Procedures/methods , Male , Adult , Middle Aged , Aneurysm, Ruptured/surgery , Aneurysm, Ruptured/complications
13.
Eur Spine J ; 33(7): 2878-2885, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38376559

ABSTRACT

BACKGROUND: Neurosarcoidosis is rare, and among its manifestations, nerve root involvement has been reported in only a few cases. Therefore, magnetic resonance imaging (MRI) findings of neurosarcoidosis, particularly those involving nerve roots, are scarce in the literature. METHODS: We presented the case of neurosarcoidosis involving cervical nerve roots and cranial nerves, alongside a systematic literature review. RESULTS: A 28-year-old female suddenly developed right facial numbness as well as left upper extremity and left hand pain. Initial brain and spine MRI showed a bulging mass of T2 iso-to-high signal intensity in the left Meckel's cave/trigeminal nerve, as well as diffuse enlargement of the right C6 and C7 nerve roots. Follow-up MRI at 2 months revealed a reduction in the size of the initial lesion and the appearance of new similar lesions on the contralateral side (right Meckel's cave, left C3-C8 nerve roots). In particular, the lesions involving the nerve roots demonstrated central enlargement along the nerve roots, without involvement of the adjacent spinal cord. All these lesions exhibited enhancement, leading to the differentiation between sarcoidosis and lymphoma. Sarcoidosis was subsequently confirmed through biopsy of a hilar lymph node. CONCLUSIONS: This report presents a distinctive MRI feature of neurosarcoidosis involving spinal nerve roots, representing the first of its kind, and describes the evolution of MRI findings throughout the clinical course.


Subject(s)
Central Nervous System Diseases , Magnetic Resonance Imaging , Sarcoidosis , Spinal Nerve Roots , Humans , Sarcoidosis/diagnostic imaging , Female , Adult , Central Nervous System Diseases/diagnostic imaging , Spinal Nerve Roots/diagnostic imaging , Spinal Nerve Roots/pathology , Cervical Vertebrae/diagnostic imaging
14.
World J Surg Oncol ; 22(1): 112, 2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38664798

ABSTRACT

BACKGROUND: Carotid body paraganglioma (CBP) is a rare, highly vascularized, and slow-growing neuroendocrine tumor. Surgical resection is the definitive treatment for CBP, however, it remains challenging due to the tumor's proximity to critical blood vessels and cervical cranial nerves. This study aimed to document the characteristics of CBP and examine the clinical outcomes of patients following surgical extirpation of CBP. METHODS: This is a single-center retrospective review analyzed patients who underwent CBP extirpation. We examined the patient demographics, preoperative clinical features, tumor characteristics, levels of catecholamines and their metabolites in the serum and urine. Surgeries were performed by one vascular surgeon with follow-ups at 1,3,6 months and yearly thereafter. Logistic regression analysis was conducted to identify risk factors associated with the occurrence of either permanent or temporary cervival cranial nerve palsy (CNP). RESULTS: From September 2020 to February 2023, this study examined 21 cases of CBP removal surgeries that were carried out in 19 patients. The mean age of the patients was 38.9 ± 10.9 years and the percentage of males was 57.1% (n = 12). The most common preoperative clinical feature was painless neck mass (n = 12; 57.1%). Complete resection was achieved in 20 cases; excluding one case with pathologically proven sclerosing paraganglioma. Vascular procedures were performed in four cases (ECA resection, n = 2; primary repair of ICA tear without carotid shunting, n = 1; and ICA patch angioplasty with carotid shunting, n = 1). Temporary cranial neurologic complications, specifically aspiration and hoarseness occurred in four (19.0%), and three (14.3%) cases, respectively. Hoarseness associated with permanent CNP persisted for more than 6 months in two cases (9.5%). No recurrence or mortality was observed during the follow-up period. CONCLUSIONS: Surgical resection is the primay treatment approach for CBP; however, it poses risks of vascular or cervical CNP. The intraoperative estimated blood loss was the only identified risk factor for CNP.


Subject(s)
Carotid Body Tumor , Humans , Male , Female , Carotid Body Tumor/surgery , Carotid Body Tumor/pathology , Retrospective Studies , Adult , Middle Aged , Republic of Korea/epidemiology , Follow-Up Studies , Prognosis , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Treatment Outcome
15.
Acta Neurochir (Wien) ; 166(1): 198, 2024 Apr 29.
Article in English | MEDLINE | ID: mdl-38684564

ABSTRACT

BACKGROUND: Trigeminal schwannomas (TSs) are intracranial tumors that can cause significant brainstem compression. TS resection can be challenging because of the risk of new neurologic and cranial nerve deficits, especially with large (≥ 3 cm) or giant (≥ 4 cm) TSs. As prior surgical series include TSs of all sizes, we herein present our clinical experience treating large and giant TSs via microsurgical resection. METHODS: This was a retrospective, single-surgeon case series of adult patients with large or giant TSs treated with microsurgery in 2012-2023. RESULTS: Seven patients underwent microsurgical resection for TSs (1 large, 6 giant; 4 males; mean age 39 ± 14 years). Tumors were classified as type M (middle fossa in the interdural space; 1 case, 14%), type ME (middle fossa with extracranial extension; 3 cases, 43%), type MP (middle and posterior fossae; 2 cases, 29%), or type MPE (middle/posterior fossae and extracranial space; 1 case, 14%). Six patients were treated with a frontotemporal approach (combined with transmastoid craniotomy in the same sitting in one patient and a delayed transmaxillary approach in another), and one patient was treated using an orbitofrontotemporal approach. Gross total resection was achieved in 5 cases (2 near-total resections). Five patients had preoperative facial numbness, and 6 had immediate postoperative facial numbness, including two with worsened or new symptoms. Two patients (28%) demonstrated new non-trigeminal cranial nerve deficits over mean follow-up of 22 months. Overall, 80% of patients with preoperative facial numbness and 83% with facial numbness at any point experienced improvement or resolution during their postoperative course. All patients with preoperative or new postoperative non-trigeminal tumor-related cranial nerve deficits (4/4) experienced improvement or resolution on follow-up. One patient experienced tumor recurrence that has been managed conservatively. CONCLUSIONS: Microsurgical resection of large or giant TSs can be performed with low morbidity and excellent long-term cranial nerve function.


Subject(s)
Cranial Nerve Neoplasms , Microsurgery , Neurilemmoma , Trigeminal Nerve Diseases , Humans , Male , Female , Neurilemmoma/surgery , Adult , Middle Aged , Cranial Nerve Neoplasms/surgery , Cranial Nerve Neoplasms/pathology , Retrospective Studies , Microsurgery/methods , Trigeminal Nerve Diseases/surgery , Trigeminal Nerve Diseases/pathology , Neurosurgical Procedures/methods , Cranial Nerves/surgery , Cranial Nerves/pathology , Treatment Outcome , Young Adult
16.
Acta Neurochir (Wien) ; 166(1): 16, 2024 Jan 16.
Article in English | MEDLINE | ID: mdl-38227056

ABSTRACT

BACKGROUND: Cavernous malformations (CMs) are clusters of thin-walled sinusoidal vessels without well-defined walls. Though they can occur anywhere in the neuroaxis, cranial nerve (CN) CMs are rare. METHOD: We report a 47-year-old male with gradual CN III palsy. Initial imaging showed no significant findings, but a follow-up MRI revealed a growing lesion along CN III. Intraoperative findings confirmed a CN III CM. Diagnosing and treating CN III CM are complex. Radiological findings lack specificity, requiring consideration of various diagnoses for patients with isolated CN III palsy and abnormal radiological findings. CONCLUSION: Surgery is the gold standard, aiming for complete lesion removal while minimizing neurological complications.


Subject(s)
Oculomotor Nerve Diseases , Oculomotor Nerve , Humans , Male , Middle Aged , Cranial Nerves , Oculomotor Nerve Diseases/etiology , Oculomotor Nerve Diseases/surgery , Paralysis
17.
Article in English | MEDLINE | ID: mdl-39127799

ABSTRACT

INTRODUCTION: Facial nerve palsies may develop during the postoperative period of microsurgical removal of vestibular schwannomas (VSs), even after normal facial function for days or weeks after surgery. The aim of this study was to identify the pathomechanism and predictive factors of delayed palsy. MATERIAL AND METHOD: The clinical data of 193 patients who underwent vestibular schwannoma surgery between 2012 and 2021 were retrospectively analyzed. A total of 134 patients were included. The patients showed intact facial nerve function up to 24 h after surgery. All patients (n = 20) with palsy from postoperative day 4 were included and collectively referred to as delayed facial nerve palsy (DFNP). Various factors were checked using a binomial regression analysis. RESULTS: The mean age of patients with DFNP was 57.8 years (55% female, 45% male). 70% had VS with KOOS ≥ 3, and 60% underwent surgery via a translabyrinthine approach Among the 16 patients with DFNP-related neurotropic pathogens, 25% were seropositive for herpes simplex virus. Most patients (n = 9/20) experienced onset of palsy between postoperative days 6 and 10. Of the four variables included in the significance test, three were significant: KOOS ≥ 3 (p < .04), ipsilateral vestibular organ failure (p < .05), and age group (p < .03). After therapy, 100% of patients recovered almost complete facial nerve function. The parameters mentioned above (KOOS classification and ipsilateral vestibular dysfunction) could be proven risk factors for the occurrence of DFNP.

18.
J Emerg Med ; 66(3): e338-e340, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38413284

ABSTRACT

BACKGROUND: This case report describes a 34-year-old woman who developed diplopia and strabismus 2 weeks after a vaginal delivery and epidural anesthesia. CASE REPORT: A 34-year-old women presented to the emergency department (ED) with continued headache and new-onset diplopia after having undergone epidural anesthesia for a vaginal delivery 2 weeks prior. During that time, she underwent two blood patches, rested supine, drank additional fluids, and consumed caffeinated products for her spinal headache. When she developed double vision from a cranial nerve VI palsy, she returned to the ED. At that time, she had a third blood patch performed, and she was evaluated by a neurologist. The medical team felt the cranial nerve VI palsy was due to the downward pull of the brain and stretching of the nerve. Magnetic resonance imaging and neurosurgical closure of the dura were considered as the next steps in treatment; however, they were not performed after being declined by the patient. All symptoms were resolved over the next 3 weeks. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case illustrates the uncommon complication of a cranial nerve VI palsy from a persistent cerebrospinal fluid leak after a dural puncture. Emergency physicians must be aware that diplopia can be a rare presenting symptom after patients undergo a lumbar puncture. Furthermore, emergency physicians should be aware of the multiple treatment options available. Knowledge of the timeline of resolution of the diplopia is necessary to make shared decisions with our patients about escalating care.


Subject(s)
Abducens Nerve Diseases , Anesthesia, Epidural , Humans , Female , Adult , Diplopia/etiology , Diplopia/therapy , Blood Patch, Epidural/adverse effects , Blood Patch, Epidural/methods , Anesthesia, Epidural/adverse effects , Abducens Nerve Diseases/etiology , Headache/etiology , Paralysis , Cranial Nerves
19.
Radiol Med ; 129(1): 118-132, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37882918

ABSTRACT

Cranial nerve enhancement is a common and challenging MRI finding that requires a meticulous and systematic evaluation to identify the correct diagnosis. Literature mainly describes the various pathologies with the associated clinic-radiological characteristics, while the radiologist often needs a reverse approach that starts from the radiological findings to reach the diagnosis. Therefore, our aim is to provide a new and practical pattern-based approach to cranial nerve enhancement, which starts from the radiological findings and follows pattern-driven pipelines to navigate through multiple differential diagnoses, guiding the radiologist to reach the proper diagnosis. Firstly, we reviewed the literature and identified four patterns to categorize the main pathologies presenting with cranial nerve enhancement: unilateral linear pattern, bilateral linear pattern, unilateral thickened pattern, and bilateral thickened pattern. For each pattern, we describe the underlying pathogenic origin, and the main radiological features are displayed through high-quality MRI images and illustrative panels. A suggested MRI protocol for studying cranial nerve enhancement is also provided. In conclusion, our approach for cranial nerve enhancement aims to be an easy tool immediately applicable to clinical practice for converting challenging findings into specific pathological patterns.


Subject(s)
Cranial Nerves , Magnetic Resonance Imaging , Humans , Magnetic Resonance Imaging/methods , Radiography
20.
Wilderness Environ Med ; : 10806032241272116, 2024 Sep 24.
Article in English | MEDLINE | ID: mdl-39314184

ABSTRACT

This case report describes the presentation and management of a patient with an isolated right sixth nerve palsy while trekking in Nepal. Consideration is made of the anatomy of the sixth nerve and the differential diagnoses afforded to this isolated sign, including high altitude cerebral edema. The case stresses the need to exclude life-threatening pathologies for any symptoms associated with altitude and includes decision-making processes on whether to monitor the patient in the field or evacuate them to a definitive care facility.

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