Cranial nerve palsies and intracranial aneurysms: A narrative review of patterns and outcomes.

Background: Cranial nerve palsy (CNP) in patients with intracranial aneurysms (IAs) can impose significant burdens on a patient's quality of life. The literature has a paucity of reviews addressing patterns of overall reported cranial nerve (CN) involvement and outcomes in patients with IA. Methods: The literature systematically reviewed CNP at presentation in the setting of IA using PubMed, Web-of-Science, and Scopus according to the PRISMA guidelines. Results: Fifty-two studies reported a total of 513 patients with IA and 630 CNPs observed at presentation: oculomotor (58.25%), abducent (15.87%), optic (12.06%), trochlear (8.7%), and trigeminal (1.9%). Most common aneurysms are located in a posterior communicating artery (46%) and cavernous internal carotid artery (29.2%). Trends of CNP based on the rupture status of IAs showed that 80% were associated with unruptured IAs and 20% with ruptured IAs. Post-treatment of IA, 55% of patients had complete resolution of CNP, with most (89%; n = 134) resolving within the first 6 months. Stratified by CNP type: Complete resolution rate is 100% in CN VII-IX, 60% in CN VI, 59% in CN IV, 54% in CN III, 45% in CN V, and 43% in CN II. Conclusion: In patients with cranial nerve palsies attributed to IAs, the location and rupture status of the aneurysm could determine the type and severity of the nerve palsy. Most patients experienced favorable outcomes in terms of their resolution and long-term function of the CNP after treatment of the IA.

Neuro-Ophthalmic Manifestations of Carotid Cavernous Fistulas: A Systematic Review and Meta-Analysis.

Carotid-cavernous fistulas (CCFs) are pathologic, arteriovenous communications between the carotid artery and cavernous sinus. They cause various complex neuro-ophthalmic symptoms by shunting the flow of arterial blood into the venous system. In this study, a systematic review is conducted on the neuro-ophthalmic presentations associated with CCFs. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 guidelines were followed during the systematic review. We searched PubMed, Scopus, and Web of Science from inception to December 31, 2023. Articles written in English on patients with confirmed CCFs reporting clinical features, diagnostic modalities, treatment approaches, and outcomes were included. Abstracted data included demography, clinical presentations, venous flow dynamics, trauma history, investigative methodology, approaches to treatment, and outcomes. Overall, 33 studies with a total number of 403 patients were included. The mean age at presentation was 42.99 years for patients with direct CCFs and 55.88 years for those with indirect CCFs. Preponderance was observed in male patients with direct CCFs, constituting 51.56%, while females predominated in those with indirect CCFs, at 56.44%. The clinical symptoms in all patients with CCFs were proptosis in 58 cases (14.39%), conjunctival congestion in 29 patients (7.20%), diplopia in nine patients (2.23%), vision blurring in four patients (0.99%), eyelid swelling in five patients (1.24%), pain in the eye in three patients (0.74%), and an upper lid mass in one patient (0.25%). Endovascular treatments, including coil and Onyx embolization, have been effective in relieving clinical symptoms and arresting the progression of these symptoms. In conclusion, the common clinical features in CCFs usually underline proptosis, congestion, and diplopia, necessitating a comprehensive neuro-ophthalmological review. Prompt identification of the symptoms of blurred vision is crucial to avoid permanent damage. Lid swelling, ocular pain, and an upper lid mass are less common but equally essential presentations for comprehensive evaluation. The recognition of these variable presentations is essential not only for timely intervention but also for the improvement in patient outcomes, thus emphasizing the role of clinician awareness in managing CCF cases.

A Rare Case of Adult-Onset Bilateral Nonspecific Orbital Inflammation (NSOI) With Concomitant Unilateral Third Cranial Nerve Palsy.

Nonspecific orbital inflammation (NSOI), also known as orbital pseudotumor, is a condition characterized by inflammation in the tissues around the eye socket (orbit) without a clearly identifiable cause. This inflammatory disorder can affect various structures within the orbit, including muscles, fat, and connective tissues, leading to symptoms such as pain, swelling, and changes in vision. A 74-year-old man with a history of previous orbital trauma presented with acute-onset head and orbital pain, followed by restricted left eye movements in all directions, left ptosis, and a dilated left pupil. Orbital imaging revealed bilateral inflammation of the lateral rectus muscles and orbital fat, suggestive of bilateral NSOI, while brain and laboratory studies ruled out other differential diagnoses. The presence of left ptosis, a dilated pupil, and limited upward, downward, and inward movements in the left eye suggested intraorbital involvement of both the superior and inferior divisions of the left third nerve. The complete resolution of orbital symptoms and third nerve function after systemic corticosteroid therapy supported the inflammatory nature of the nerve involvement in this case. The case is notable in terms of bilateral involvement in adult-onset NSOI, the possible role of previous orbital trauma in the development of the disease, and the inflammatory involvement of third nerve divisions following the extension of inflammation into the orbital tissues. NSOI can mimic other, more serious conditions, making accurate diagnosis crucial for effective management and treatment. Understanding its presentation, potential causes, and appropriate diagnostic approaches is essential in providing optimal care for patients affected by this complex inflammatory condition.

A Case of Neurosyphilis Presenting with Isolated Abducens Nerve Palsy.

We herein report a case of neurosyphilis that presented with isolated bilateral abducens nerve palsy. A 39-year-old man was referred to our department with diplopia. He had a history of homosexual relationships and showed only bilateral abducens nerve palsy upon a neurological examination. Positive syphilis tests in the serum and cerebrospinal fluid and a contrasting effect on the abducens nerve on magnetic resonance imaging (MRI) confirmed the diagnosis of active neurosyphilis. When a patient manifests isolated abducens nerve palsy, neurosyphilis can be a differential diagnosis, although rare, and contrast-enhanced MRI may help diagnose the disease.

Skull Base Osteomyelitis: A 5-Year Review and Prognostic Outcome in a Single Tertiary Institution.

Objective: The primary objective of this study is to review the clinical parameters associated with skull base osteomyelitis (SBO), with a secondary aim of studying their association with patient outcomes 1 and 6 months after treatment initiation. Study Design: This is a single-center restrospective observational study. Setting: The study was conducted from January 2018 to December 2022 at the University Malaya Medical Center in Kuala Lumpur. Methods: Patients aged over 15 years with a diagnosis of SBO were included in the study. Clinical parameters, investigations, and follow-up records were recorded. The disease outcomes were analyzed at 1 and 6 months after treatment initiation using multivariable analyses. Results: The study identified 31 patients with SBO, the majority of whom were elderly males with comorbidities such as diabetes and hypertension. Otalgia and otorrhea were the most common symptoms, and computed tomography scans were used for diagnosis. Pseudomonas aeruginosa was the most commonly identified pathogen, and intravenous broad-spectrum antimicrobials were used to treat all patients. Surgical intervention was required for 25% of patients, and underlying ischemic heart disease, anemia, and single nerve palsy were significantly associated with an unfavorable prognosis. Patients with higher body mass index and elevated C-reactive protein showed poorer outcomes after 1 and 6 months of treatment, respectively. Conclusion: Early recognition, prompt treatment, better control of comorbidities, nutrition, and monitoring can improve SBO outcomes and reduce complications. Therefore, as the prevalence of SBO increases, diagnostic criteria or management guidelines should be established to guide the best clinical practice.

Utility of Sodium Fluorescein During Peripheral Nerve Tumor Surgery: A Scoping Review of Indications, Techniques, and Outcomes.

OBJECTIVE: The primary treatment for peripheral nerve tumors involves maximal surgical resection while preserving nerve function. Sodium fluorescein shows potential for enhancing the safety and efficacy of nerve tumor surgery. This review evaluates the advantages and limitations of sodium fluorescein in this context. METHODS: PubMed, EMBASE, Web-of-Science, and Scopus were searched following the PRISMA-ScR guidelines to include studies reporting the use of sodium fluorescein in peripheral nerve tumors surgery. Intervention-related outcomes (i.e., extent of resection, clinical outcomes, complication rates, recurrence rates, and duration of surgery) were evaluated and summarized. RESULTS: A total of 4 studies encompassing 166 patients with 168 tumors were included. Patients were mostly female (98; 53.6%), 101 (69.2%) had sporadic (non-syndromic) tumors, and at histopathology, 114 (67.9%) tumors were WHO grade-1 schwannomas. Gross total resection was achieved in 146 (86.9%) tumors. Postoperative complications were reported in 16 cases (10.2%%), none related to side effects of the fluorescent dye. High tumor fluorescence was reported in 150 (94.3%) tumors, while absent and low parent nerve fluorescence was reported in 121 (79.6%) and 27 (17.8%), respectively. The median duration of surgery was 51.5 (range: 24-92) minutes. CONCLUSION: Sodium fluorescein shows promise as assisting tool in nerve tumor surgery by facilitating differentiation between the tumor, parent nerve, and surrounding soft tissue. However, multi-center randomized controlled trials are necessary to determine its effect on extent of resection rates, clinical outcomes, postoperative complication rates, and surgical duration in comparison to current standard of care.

Cranial nerve injuries post carotid endarterectomy: a 15-year prospective study with routine otolaryngologist and neurological evaluation.

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.

Delayed facial nerve palsy after vestibular schwannoma resection: risk factors, extent and prognosis.

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.

Recovery of cranial nerve neuropathies after LINAC-based stereotactic radiosurgery for benign cavernous sinus meningioma.

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.

Surgical results, technical notes and complications of jugular foramen lesions via retroauricular infratemporal fossa approach.

OBJECTIVE: The objective of this study was to evaluate the clinical effect and safety of the postauricular infratemporal fossa approach (ITFA) in resecting jugular foramen lesions. METHODS: All 25 patients undergoing microsurgery via postauricular ITFA from March 2015 to May 2023 in the Department of Neurosurgery, Tangdu Hospital, Air Force Military Medical University were included. The clinical and radiological data were retrospectively analyzed. Regular follow-up was carried out. RESULTS: The mean age of all patients was 50.5±8.9 years, and 14 of them were female and 11 were male. Among the cases, lower cranial nerve schwannoma accounted for 60 % (15/25) of all tumors, jugular foramen paraganglioma accounted for 20 % (5/25), and the remaining 20 % included meningioma, chondrosarcoma, plasmacytoma, and salivary gland tumors. Total tumor resection was performed in 18 cases, subtotal tumor resection in 7 cases and partial resection in 1 case. Seven patients underwent gamma knife radiotherapy after surgery. Transient lower cranial nerve dysfunction occurred in 8 patients, and permanent lower cranial nerve dysfunction occurred in 2 patients after surgery. One patient developed facial paralysis, and one patient presented hearing loss. CONCLUSIONS: The postauricular ITFA achieved a relatively high total tumor resection rate and a lower incidence of neurological functional disorders. It is an alternative and suitable surgical approach for resecting jugular foramen lesions. Maximizing the preservation of neurological function is preferred, especially when radical resection cannot be achieved. Stereotactic radiotherapy could be used for residual tumors.

Clinical and Genomic Profile of Primary Cranial Neurolymphomatosis.

Primary cranial neurolymphomatosis (PCNL) is a rare subtype of primary CNS lymphoma (PCNSL) in which infiltrative lymphomatous involvement is confined to cranial nerves. Here, we report a case of PCNL with successful genomic profiling. A 57-year-old male had a lengthy prediagnostic phase spanning approximately 30 months, characterized by multiple episodes of cranial neuropathies managed by steroids. At the time of diagnosis, the patient had right-sided cranial neuropathies involving cranial nerves (CN) V, VI, and VII. Pathological findings of the right cavernous lesion biopsy were consistent with large B-cell lymphoma-infiltrating nerve fibers. The clinical course was aggressive and refractory, characterized by relentless progression with the development of cervical spinal neurolymphomatosis, cerebrospinal fluid involvement, and ependymal and intraparenchymal cerebral involvement, despite multiple lines of therapy, including chemoimmunotherapy, Bruton's tyrosine kinase inhibitor, radiation, autologous stem cell transplant, chimeric antigen receptor T-cell therapy (CAR-T), and whole-brain radiation. The patient survived for 22 months from the time of the initial diagnosis and 52 months after the first episode of cranial neuropathy. Next-generation sequencing identified mutations (MYD88, CD79b, and PIM1) that are frequently observed in PCNSL. The unusual findings included a total of 22 mutations involving PIM1, indicating a highly active aberrant somatic hypermutation and two missense CXCR4 mutations. CXCR4 mutations have never been described in PCNSL and may have implications for disease biology and therapeutic interventions. We provide a literature review to further elucidate PCNL.

Outcomes and Factors Associated with Successful Strabismus Surgery for Abducens Nerve Palsies: A Retrospective Study and Literature Review.

Purpose: Several studies reported surgical outcomes for abducens nerve palsy, but information on factors that affect treatment success remains lacking. These factors are crucial for developing a treatment plan and providing disease counseling. This study aimed to investigate the outcomes of strabismus surgery for abducens nerve palsy and determine the factors that influence its success, including a review of relevant literature. Methods: This retrospective analysis included abducens nerve palsy cases, focusing on surgical interventions and relevant patient data, at the outpatient clinics of Phramongkutklao Hospital from April 1, 2012, to April 30, 2022. A relevant literature review included the surgical success rate and factors that influence surgical outcomes. Results: This study enrolled 32 patients, including 19 with partial and 13 with complete abducens nerve palsy. The overall success rate of strabismus surgery was 78.1%. Trauma was the leading cause of abducens nerve palsy in this population (28.13%). Fisher's exact and Mann-Whitney U-tests revealed that shorter abducens nerve palsy onset and smaller preoperative angle were significantly associated with successful surgical outcomes of strabismus surgery among the groups. In contrast, subgroup analysis revealed that only preoperative smaller angles were significantly associated with good surgical outcomes in horizontal strabismus surgery. However, the vertical rectus muscle transposition group demonstrated no significant factors. The literature review revealed that the success rate of surgery in abducens nerve palsy was 25%-82.6% for horizontal rectus muscle surgery and 46.2%-91% for rectus muscle transposition. Conclusion: The surgical success rate for abducens nerve palsy reached 78.1%, including 78.95% for partial and 76.92% for complete abducens nerve palsy. Notably, a shorter onset preceding surgery and a smaller preoperative angle significantly correlated with successful surgical outcomes one year postoperatively.

Numerous studies have been conducted to determine the effectiveness of eye muscle surgery for abducens nerve palsy. However, there is still a lack of information on the various factors that can influence the success rate of the treatment. Understanding these factors is crucial for developing appropriate treatment plans and guidance for individuals with this condition. Our study aimed to examine the effectiveness of strabismus surgery for abducens nerve palsy and identify the factors that affect its success. This was achieved by reviewing relevant literature and analyzing cases from Phramongkutklao Hospital between April 1, 2012, and April 30, 2022. We included 32 patients with partial or complete abducens nerve palsy, with trauma being the leading cause of the condition in our group. The results showed that the surgery was successful in 78.1% of cases. Our analysis revealed that getting surgery sooner after the palsy started and having a smaller misalignment angle before surgery were associated with better outcomes. However, these factors only mattered for horizontal eye muscle surgery, not vertical muscle transposition. Previous studies have reported success rates for this condition ranging from 25% to 91%. Our study concludes that earlier surgery and certain preoperative factors can improve outcomes for individuals with abducens nerve palsy after eye muscle surgery.

Presumed Fourth Nerve Palsy in a Healthy and Asymptomatic Child with COVID-19 Infection.

COVID-19 can cause a wide range of ocular manifestations. The most common ocular manifestation is conjunctivitis. Neuro-ophthalmic presentations of COVID-19 are rare. Case reports suggest that COVID-19 infection can cause cranial nerve palsy, including nerves that regulate ocular movements. The present studypresented a case of fourth nerve palsy in a healthy and asymptomatic COVID-19-infected child. A healthy 10-year-old boy was referred to our eye clinic with a complaint of recent abnormal head posture and squint. His past medical history was unremarkable, and he had not received any medication or vaccinations within the last few weeks. No history of ocular or head trauma was observed. The patient was afebrile and had no respiratory symptoms. A comprehensive ocular examination was performed. All examinations, including slit-lamp, pupils, eyelids, and optic nerve heads, were normal. In ocular motor evaluations, left eye hyperdeviation was observed. Because of the history of COVID-19 in the mother of the child, he was referred to an infectious disease specialist and was tested for SARS-COV-2 with a nasopharyngeal swab specimen. The test was positive and SARS-COV-2 was detected. In addition, the patient was referred to a pediatric neurology department. Brain and orbital MRI was performed, and it was unremarkable. The post-viral fourth nerve palsy is uncommon, and post-COVID-19 has not been reported before. Clinicians should consider this infection in any recent strabismus in pediatrics. The children rarely complain of diplopia, and a recent abnormal head posture may be a sign of acquired strabismus.

Intracranial aneurysms and abducent nerve palsy.

Background: Cranial nerve (CN) palsy may manifest as an initial presentation of intracranial aneurysms or due to the treatment. The literature reveals a paucity of studies addressing the involvement of the 6th CN in the presentation of cerebral aneurysms. Methods: Clinical patient data, aneurysmal characteristics, and CN 6th palsy outcome were retrospectively reviewed and analyzed. Results: Out of 1311 cases analyzed, a total of 12 cases were identified as having CN 6th palsy at the presentation. Eight out of the 12 were found in the unruptured aneurysm in the cavernous segment of the internal carotid artery (ICA). The other four cases of CN 6th palsy were found in association with ruptured aneurysms located exclusively at the posterior inferior cerebellar artery (PICA). For the full functional recovery of the CN 6th palsy, there was 50% documented full recovery in the eight cases of the unruptured cavernous ICA aneurysm. On the other hand, all four patients with ruptured PICA aneurysms have a full recovery of CN 6th palsy. The duration for recovery for CN palsy ranges from 1 to 5 months. Conclusion: The association between intracranial aneurysms and CN 6th palsy at presentation may suggest distinct patterns related to aneurysmal location and size. The abducent nerve palsy can be linked to unruptured cavernous ICA and ruptured PICA aneurysms. The recovery of CN 6th palsy may be influenced by aneurysm size, rupture status, location, and treatment modality.

Perineural Invasion of Cranial Nerves in Cutaneous Squamous Cell Carcinoma: Unraveling Its Complexities, Diagnostic Challenges, and Multifaceted Treatment Approaches.

Cutaneous squamous cell carcinoma is the second most common neoplasm among non-melanoma skin cancers. When associated with perineural invasion of the cranial nerves, with clinical features often observed in trigeminal and facial nerves due to their cutaneous extension, it may lead to a worse prognosis. This paper introduces a rare case of an 81-year-old male, with a history of a moderately differentiated invasive carcinoma of the left frontal region with perineural invasion on the left trigeminal cranial nerve. The case underscores the aggressive nature of the intraneural infiltration by squamous cell carcinoma and the challenges in managing such advanced malignancies.

Analyzing the relationship between cerebral aneurysms and Non-oculomotor cranial nerve palsies: a systematic review.

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.

Soft Tissue Dissection in the Transcondylar Approach: A Modified Layered Technique to Maximize Lateral Access.

Objective While the transcondylar approach is technically challenging, it provides generous ventral and caudal exposure to the craniovertebral junction. This approach requires navigation around multiple eloquent neurovascular structures including the lower cranial nerves, vertebral artery and its branches, and the brainstem. Superficial exposure, including incision location and muscle dissection, can dramatically affect the surgical angle and maneuverability at depth. Methods We demonstrate the transcondylar approach in a step-by-step fashion in a formalin-embalmed, latex-injected cadaver head. Dissection within each layer of the suboccipital muscles was performed. A small cohort with an illustrative case is also included herein. Results The sternocleidomastoid (SCM) muscle was retracted anteriorly; the splenium capitis, semispinalis capitis, and longissimus capitis muscles were disconnected from the superior nuchal line and reflected inferomedially. The suboccipital muscle group was fully exposed. The superior and inferior oblique muscles were disconnected from the transverse process of C1. The superior oblique and the rectus capitis posterior major muscles were then dissected off the inferior nuchal line, and the suboccipital muscle group was retracted inferomedially en bloc . The greater auricular nerve was retracted laterally with the SCM, and the greater occipital nerve was retracted inferomedially with the suboccipital muscle group. Conclusion This technique avoids the obstructive muscle bulk that results from a myocutaneous approach while maximizing deep exposure. Understanding the detailed muscular anatomical relationship with the insertion location and suboccipital nerves is key to complete and safe extracranial dissection. Diligent dissection helps minimize postoperative pain and muscle spasm while optimizing the closure technique.

Collet-Sicard syndrome due to cervical artery dissection disclosed by high-resolution magnetic resonance imaging.

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.

Expanding the genetics and phenotypes of ocular congenital cranial dysinnervation disorders.

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.