8th Cranial Nerve Research Articles

Early Childhood Onset Vanishing White Matter Disease with Multiple Cranial Nerve Enhancement: A New Consensus Criteria?

Vanishing White Matter Disease (VWM), also known as Childhood Ataxia with Central Nervous System Hypomyelination (CACH), although a rare neurological condition, is a prevalent hereditary leukoencephalopathy with a characteristic phenotype of gradual neurologic deterioration with ataxia being a prominent feature. It is characterized by a wide range of onset, from antenatal and infantile periods to early childhood and later adulthood periods. The early childhood form is considered the prevailing form, characterized by a preceding phase of normal development until the second or 3rd year of life, followed by progressive neurologic deterioration, accentuated by certain stressors, such as infections or minor trauma. Diagnosis is achieved based on the clinical pretext combined with distinctive MRI brain features, confirmed by DNA analysis detection of elf2B mutation. Our case is a healthy 18-month-old male who after a preceding upper respiratory tract infection, developed an ataxic gait, encephalopathy, and recurrent generalized seizures. Physical assessment revealed signs of generalized spasticity and hyperreflexia. Neuroimaging revealed diffuse symmetric supratentorial and infratentorial diffuse white matter hypomyelination. Interestingly, there was notable enhancement of the 3rd and 5th cranial nerves. Differential diagnosis included acute demyelinating leukoencephalopathies; and neuromatobolic disorders with acute presentations (including leukodystrophies, hypomyelinating disorders, and mitochondrial encephalopathies). In the early course of his management, he received immunomodulatory therapy in the form of pulse steroids, intravenous immunoglobulins, and oral tapering course of steroids. He had limited response to these interventions. Whole exome sequencing yielded a homozygous mutation in EIF2B3, confirming the diagnosis of VWMD/CACH. The presence of enhancing cranial neuropathies represent an atypical phenotype reported in other case reports and should alert the physician to avoid unnecessary intervention with immunosuppressive therapies.

Basaloid squamous cell carcinoma clinically and radiologically masquerading as a head and neck paraganglioma: a case report and review of the literature

BackgroundThis paper reports the first case of basaloid squamous cell carcinoma clinically and radiologically masquerading as a head and neck paraganglioma.Case presentationA 66-year-old Sinhalese male with unilateral hearing impairment and 7th–12th (excluding 11th) cranial nerve palsies was diagnosed radiologically with a head and neck paraganglioma by magnetic resonance imaging of the brain, which revealed a hypointense and hyperintense punctate mass centered at the jugular fossa with intracranial extension. The ascending pharyngeal artery, recognized as the major feeder, was embolized by percutaneous embolization following digital subtraction angiography. Gross total resection of the tumor was followed by an uneventful postoperative recovery. Combined immunohistochemistry and histopathological morphology revealed a basaloid squamous cell carcinoma, following which the patient completed radiotherapy and is at 3-month follow-up currently.ConclusionThis case report discusses the diagnostic pitfalls and management challenges of this rare entity on the basis of prior evidence, as well as a literature review and clinical and surgical analysis.

P026 Neurofascin antibodies complicating a case of severe systemic lupus erythematosus

Abstract Background/Aims Juvenile-onset systemic lupus erythematosus (jSLE) is an autoimmune disease that results in increased disease activity and damage compared to adult-onset SLE. There is increased medication burden, severe organ manifestations and higher incidence of renal, cardiovascular and neuropsychiatric (NP) involvement. Anti-nervous system (NS) antibodies are seen across immune mediated diseases including SLE. Myelin oligodendrocyte glycoprotein (MOG) and neurofascin (NF) 186 antibodies are particularly associated with higher frequencies of NP-SLE. These patients can present with the distinct clinical phenotype associated with that antibody (i.e., demyelination with MOG, neuropathy with NF186). Methods We present an SLE patient with progressive neurological features and anti-NF antibodies who responds well to ocrelizumab after modest response to initial immunotherapy. Results An 18 year old female has active jSLE including malar rash, alopecia, arthralgia, aphthous ulcers, headaches. She presents with lower limb and ascending abdominal paraesthesia and left 6th cranial nerve palsy alongside a recent respiratory tract infection. ANA 1:5120 homogenous pattern, high dsDNA, low C3 and C4, thrombocytopenia and antibodies to Sm, Ro, RNP, anti-Rib-P and strongly positive lupus anticoagulant antibodies and low titre anti-cardiolipin (aCL) IgG and IgM. Lumbar puncture is unremarkable. Nerve conduction studies (NCS) demonstrate progressive predominant motor inflammatory neuropathy without demyelination. CT, MRI brain and MRI spine are normal. She is treated as atypical Guillain-Barre Syndrome (GBS)/ Miller-Fischer with 5 days IV immunoglobulin 2g/kg and also for active SLE with 3 days 500mg IV methylprednisolone. She is intubated following worsening dysphagia, dysarthria, muscle weakness and reduced consciousness. Plasma exchange and pulsed cyclophosphamide are subsequently given. Eye and facial movements worsen with NCS showing worsening patchy axonal sensorimotor polyneuropathy. Serial MRI brain is suggestive of posterior reversible encephalopathy syndrome (PRES). Autonomic instability is seen with labile blood pressure and tachycardia. Given the unusual presentation, anti-NF antibodies are tested and identified at 1:3200 positive, NF155 and 186, both IgG1 and IgG2. Given previous adverse reaction to rituximab, she received ocrelizumab which lead to a marked improvement in her sensorimotor symptoms and functionality. C3 and C4 levels show considerable improvement which is sustained and dsDNA levels normalise. B cell depletion therapy was chosen following a small case series of patients with neurofascin antibodies who developed severe autoimmune neuropathies. Half of these patients responded well to rituximab. One proposed mechanism being the targeting of immature CD20+ B cells inhibits plasmablast generation, which may be responsible for neurofascin antibody production early on in the disease. Conclusion Anti-NS antibodies are seen in SLE patients and their presence can suggest increased frequency of NP-SLE suggesting a potential future role as a biomarker. One should consider anti-NF antibody testing in patients with a GBS-like illness with progressive neuropathy, respiratory involvement and autonomic dysfunction despite standard immunotherapy. Disclosure P. Yee: None. M. Mouyis: None.

Progressive Bilateral Ptosis in Adduction Deficit: True Internuclear Ophthalmoplegia (INO) or Pseudo-INO?

Abstract
 Introduction : Bilateral INO is a rare disease caused by a lesion in the medial longitudinal fasciculus (MLF) in the pons, resulting in adduction deficit. Progression to ptosis is rare and may confuse with pseudo-INO as in ocular myasthenia gravis (OMG). This case aims to report progressive ptosis in bilateral INO that mimics pseudo-INO.
 Case Illustration : A 36-year-old woman complained sudden binocular diplopia 4 days before admission. Bilateral adduction deficits (-3 and -2 of right and left eye, respectively) with nystagmus were seen. There was no ptosis. The patient was diagnosed with bilateral INO. One month later, the patient returned with bilateral ptosis. The marginal reflex distance 1 (MRD1) were 0 on both eyes. Post fatigue and ice pack test showed 2mm difference of MRD1. The single fiber electromyography (EMG) result came out negative with jitter <10%. Brain MRI revealed lesions in the posterior pons and periaqueductal on the level of midbrain.
 Discussion : The MLF controls 6th and contralateral 3rd cranial nerve nucleus for horizontal gaze. Ptosis in INO can occurred due to central caudal subnucleus involvement in midbrain that innervates levator palpebra. The result of fatigue and ice pack test may be misleading, as those are not pathognomonic for OMG. The single-fiber EMG are valuable modalities to exclude pseudo-INO in this case.
 Conclusion : Progressive bilateral ptosis may occur in bilateral INO. Fatigue and ice pack test may give false- positive results leading to confusion with pseudo-INO, rather than true INO. Supporting examinations are needed to confirm the diagnosis.

453 Changed Innervation Upon Loss of Mandibular Division of Trigeminal Nerve to Muscles of Mastication

INTRODUCTION: The gastrulation brain homeobox (Gbx) genes are essential for patterning and maintenance of neurons along the anteroposterior axis of the developing neural tube. Knockout (ko) of Gbx2 results in neonatal lethality associated with neurological and other defects. To understand pathologies, ko studies are not realistic as gene loss usually results in death before birth. Gbx2 neo/neo mutant mice express 6-10% of the wildtype Gbx2 expression levels. They have milder malformations than ko mice but lack the cerebellar vermis and mandibular division of the trigeminal nerve (3rd division of 5th cranial nerve = V3), among other defects. These Gbx2 neo/neo mutant mice die perinatally as they are unable to suckle due to lack of motor innervation via V3 to the muscles of mastication. Muscle cells develop largely without interaction with their motor nerve. However, the final differentiation of myocytes requires interactions with nerve fibers. Unexpectedly, the muscles of mastication appear normal in Gbx2 neo/neo mutant mice, despite the lack of V3. METHODS: We performed microdissections of neonate wildtype and Gbx2neo/neo mutant mice. Additionally, we embedded mutant and wildtype mouse embryos in paraffin, serial sectioned them (7 μm), stained the sections with Azan staining, and analyzed specimen microscopically. RESULTS: Current analysis of the data to identify where nerve fibers in the muscles of mastication originate is in process. We favor the origin of these fibers from the facial nerve (7th cranial nerve), which has several overlapping territories with the trigeminal nerve. CONCLUSIONS: Muscles require innervation for their final differentiation steps. The loss of a nerve can result in the invasion of another nerve into the territory of the lost one, which rescues muscle differentiation but not muscle function. IACUC Ziermann Med-20-02, Funding NSF #2000005 to Ziermann.

Contralateral Nasofrontal Trephination: A Novel Corridor for a "Dual Port" Approach to the Petrous Apex.

Expanded endonasal approaches (EEAs) have proven safe and effective in treating select petrous apex (PA) pathologies. Angled endoscopes and instruments have expanded indications for such approaches; however, the complex neurovascular anatomy surrounding the petrous region remains a significant challenge. This study evaluates the feasibility, anatomic aspects, and limitations of a contralateral nasofrontal trephination (CNT) route as a complementary corridor improving access to the PA. Expanded endonasal and CNT approaches to the PA were carried out bilaterally in 15 cadaveric heads with endovascular latex injections. The distance to the PA, angle between instruments through the 2 approach portals, and surgical freedom were measured and compared. Three-dimensional DICOM-based modeling and visualization indicate that the CNT route reduces the distance to the target located within the contralateral PA by an average of 3.33 cm (19%) and affords a significant increase in the angle between instruments (15.60°; 54%). Furthermore, the vertical vector of approach is improved by 28.97° yielding a caudal reach advantage of 2 cm. The area of surgical freedom afforded by 3 different approaches (endonasal, endonasal with an endoscope in CNT portal, and endonasal with an instrument in CNT portal) was compared at 4 points: the dural exit point of the 6th cranial nerve, jugular foramen, foramen lacerum, and petroclival fissure. The mean area of surgical freedom provided by both approaches incorporating the CNT corridor was superior to EEA alone at each of the surgical targets ( P = <.001). The addition of a CNT portal provides an additional avenue to expand on the classical EEA to the PA. This study provides insight into the anatomic nuances and potential clinical benefits of a dual-port approach to the PA.

A case of cerebellopontine angle meningioma presenting as neurovascular compression syndrome of the 8th cranial nerve

A 54-year-old female patient presented with paroxysmal tinnitus and vertigo for 2 years, which have repeatedly occurred while rotating her neck, and lasted about 10 seconds. An anticonvulsant medication was prescribed with a diagnosis of microvascular compression syndrome on the 8th cranial nerve and audiovestibular evaluation and magnetic resonance imaging (MRI) scan were appointed a week later. In her next visit, she reported the disappearance of paroxysmal audiovestibular symptoms after medication. In the left ear, canal paresis and abnormal auditory brainstem response were observed. In MRI, a large meningioma in the cerebellopontine angle in the vicinity of the internal auditory canal orifice was detected, that was surgically resected by a neurosurgeon. After surgical removal of the tumor, she reported continuous dizziness due to vestibular nerve injury, but the paroxysmal attack of tinnitus and vertigo disappeared without anticonvulsant medication. This case suggests that an imaging study is mandatory when diagnosing microvascular compression syndrome on the 8th cranial nerve.

A case of vertebrobasilar dolichoectasia manifesting as sudden sensorineural hearing loss with vertigo

Vertebrobasilar dolichoectasia (VBD) is a rare vascular condition characterized by the elongation, widening, and tortuosity of the vertebrobasilar artery. It can lead to various symptoms due to compression of cranial nerves and brainstem structures. We report a rare case of VBD presenting as sudden sensorineural hearing loss (SSNHL) with vertigo and spontaneous downbeat nystagmus in a 65-year-old woman with a history of hypertension. Magnetic resonance imaging revealed a tortuous and dilated right vertebrobasilar artery compressing the brainstem and left 8th cranial nerve root entry zone, without signs of acute cerebellar stroke. The compression of the anterior inferior cerebellar artery and cervicomedullary junction was also noted, suggesting a vascular cause for her symptoms. Following high-dose steroid treatment, the patient showed significant hearing improvement. This case underscores the importance of considering VBD in patients with unexplained SSNHL and vertigo, highlighting the role of detailed vascular imaging in the diagnosis and management of such cases.

A Case Study Photobiomodulation As The Solution For Bell's Palsy

Often affecting one side, Bell's palsy, also known as "acute facial palsy of unknown cause," is a common cranial neuropathy that results in abrupt, complete paralysis or face muscle paresis. Over the period of 48 hours, the condition may get worse. It is brought on by damage or inflammation to the 7th cranial nerve, the face nerve, or any of its branches along its course, primarily in the bony canal. This results in dysfunction of the facial nerve. It is equally prevalent in both sexes and has no age restriction, however as people age, so does the incidence. A increased risk is associated with diabetes, hypertension, obesity, pregnancy, and upper respiratory tract infections. It is mainly believed to be idiopathic and is diagnosed by ruling out other potential causes. Bell's palsy sufferers and their family may experience severe consequences, including psychological and physical problems. Therefore, a timely cause determination and early diagnosis are essential to efficient treatment. However, the exact etiology of Bell's palsy remains unknown, which affects the course of treatment. To adopt a customized therapy strategy, however, a comprehensive examination and a comprehensive history are necessary to determine the likely cause and risk factors. Most patients heal on their own in less than three weeks, even without treatment. Still, there's always a chance of lingering paresis after therapy or recovery.

Facial Palsy in Post COVID-19 Invasive Fungal Sinusitis.

This study aimed to study the various presentations of post COVID-19 invasive fungal sinusitis with 7th cranial nerve palsy and to correlate topodiagnostic tests and radiological findings to identify the site of lesion. A retrospective study was conducted at a tertiary care hospital where 11 patients with post COVID-19 invasive fungal sinusitis with facial palsy presented to our institute. Detailed history, clinical examination, radiological imaging were done for all patients. A total of 11 patients with post COVID-19 invasive fungal sinusitis with facial palsy were included in this study. The mean age of the patients was 44.8, with a male to female ratio of 4.5:1. Diabetes Mellitus was a major factor contributing to immunosuppression in our study with 9 patients who were known cases of diabetes mellitus and 2 newly diagnosed cases. On MRI, 10 patients had involvement of the Pterygopalatine fossa and 4 patients had involvement of the petrous apex. Post COVID-19 invasive fungal sinusitis can present with facial palsy, and diabetes mellitus is a major contributing factor to immunosuppression in these patients. The involvement of the petrous apex is a possible site of involvement, and MRI can aid in localizing the site of the lesion. Further studies are required to identify the exact mechanism of facial nerve palsy in invasive fungal sinusitis.

Isolated Intracranial Hypertension as a Presentation of Pediatric Lyme Borreliosis: A Case Report and Literature Review

BackgroundIt is extremely rare for Lyme borreliosis to present solely with features of increased intracranial pressure. The treatment of pediatric Lyme neuroborreliosis with oral versus intravenous antibiotics remains controversial. MethodsCase report and literature review. ResultsA 13-year-old male presented with five days of binocular diplopia, several weeks of headache, and a history of multiple tick bites six weeks prior. His examination showed a left eye abduction deficit and bilateral optic disc edema. Magnetic resonance imaging (MRI) of the brain with contrast showed tortuosity of the optic nerves, prominence of the optic nerve sheaths, and enhancement of the left fifth and bilateral sixth cranial nerves. Lumbar puncture showed an elevated opening pressure and a lymphocytic pleocytosis. Lyme IgM and IgG antibodies were positive in the serum and cerebrospinal fluid. The patient was treated with intravenous ceftriaxone for two days empirically followed by doxycycline by mouth for 19 days. Symptoms began improving after 48 hours. The strabismus resolved after two weeks, and the papilledema improved slowly with complete resolution at six months. ConclusionsLyme neuroborreliosis can present as isolated intracranial hypertension in the pediatric population; it can be differentiated from idiopathic intracranial hypertension on MRI, and lumbar puncture and can be confirmed with serum antibody testing. Oral doxycycline can be considered for Lyme neuroborreliosis in children.

Isolated 12th Cranial Nerve Palsy Due to Skull Base Synovial Cyst.

Isolated 12th Cranial Nerve Palsy Due to Skull Base Synovial Cyst.

Study of etiological profile of patients with paralytic strabismus in a tertiary care hospital in India: a cross-sectional study

Abstract Introduction: Paralytic strabismus, a type of strabismus caused by paralysis of one or more extraocular muscles, can lead to a variety of ocular and psychosocial sequelae. Etiology varies in different types of paralytic strabismus. Numerous studies have shown that ocular motor cranial nerve palsies have a variety of causes including vascular disease, head trauma, intracranial tumour or aneurysm and inflammatory disorders.Materials and Methods: A cross-sectional study was conducted at a tertiary eye hospital to evaluate Paralytic squint by Neuroimaging in patients presenting to the Squint department of Sarojini Devi Eye Hospital Hyderabad from June 2021 to May 2022. Paralytic strabismus cases with limitation of ocular motility were enrolled and a detailed examination was performed to know the etiology. Results: 190 patients (1.35%) were diagnosed with paralytic strabismus. Ischemia constituted to be the major cause of paralytic strabismus i.e. (42.1%) followed by trauma (20%). The 3rd cranial nerve was the most commonly involved (34.73%), followed by the 6th cranial nerve (32.63%). Multiple cranial nerves were involved in 20% of cases. The 3rd CN was involved, primarily due to ischemia in both males and females while the 6th CN was affected with trauma, neoplasm, and ischemia being prominent causes. Conclusion: The study showed that ischemia, trauma and idiopathy are some of the major etiologies of paralytic strabismus with male predominance. The cranial nerve involvement also varied with the etiology of paralytic strabismus.

Management of Ardita through Nasya with special reference to Bell's Palsy : A Single Case Study

In modern era, prevalence of neurological disorders are rising due to fast-paced and stressful lifestyle. Ayurveda categorizes neurological disorder as Vata Vyadhi. Of the 80 Nanatmaj Vatavyadhi, Ardita is the one. Ardita is the disease characterized by Mukhardha Vakrata &amp; results in Facial asymmetry &amp; dysfunction. Ardita can be correlated to Bell's Palsy. Bell's Palsy is a neurological condition that involves the 7th cranial nerve &amp; results in facial drooping and weakness. As per Acharya Charaka, Nasya is the specific treatment for Ardita. Nasya is one of the Panchakarma procedure in which the drug (medicated oil / ghee / decoction / powder, etc.) is administered through Nasa, essentially to eliminate the vitiated Dosha present in Shira. The present case deals with the 50 years old man diagnosed with Ardita and treated with Ayurvedic Shaman Aushadh along with Nasya. Patient got complete relief in all symptoms with appreciable changes. Criteria of assessment was based on House-Brackmann facial paralysis scale. To explore and elaborate the role of Nasya in Ardita with special reference to Bell's Palsy, various Samhitas along with contemporary books, research articles available on Nasya and Ardita are studied. Panchakarma procedure like Nasya proves to be the promising Ayurvedic management in Ardita.

Abstract #1608724: 12th Cranial Nerve Palsy: A Very Rare Presentation of Pituitary Macroadenoma

Abstract #1608724: 12th Cranial Nerve Palsy: A Very Rare Presentation of Pituitary Macroadenoma

The sound and musical preferences of tympanic chord

The tympanic chord is a part of the 7th cranial nerve (facial nerve), from which it branches off, runs through the middle ear and then joins the lingual nerve, a part of the 5th cranial nerve (mandibular nerve/trigeminal nerve). Despite its course through the ear, the tympanic chord does not carry auditory fibers, but rather sensory and parasympathetic fibers to the front part of the tongue and to the salivary glands.The cranial nerves V and VII have a common phylogenetic origin, establishing a close relationship between auditory and gustative sense.In German language "Geschmack" means both gustative sense and the preference for different ways of thinking or styles in music, art or fashion. "Musikgeschmack" is the preference for special music styles or composers. "Musikgeschmack" therefore forms an interface between the anatomy of the tympanic chord and music preferences.The effect of parallel processing of music and taste is used, for example, in music therapy or as part of marketing strategies. But it could also be used to treat Covid-19 patients who suffer from a loss of sense of smell and taste. However, there are still no studies on this specific question.

Sudden Sensorineural Hearing Loss from a Jugular Bulb Diverticulum

A 19-year-old woman presented with an 11-month history of sudden-onset left sided hearing loss accompanied by vertigo and headache. Audiometric testing revealed profound left- sided hearing loss. A contrast-enhanced MRI of the internal auditory canal performed 5 months after symptom onset was interpreted as showing a vascular loop, probably the anterior inferior cerebellar artery, abutting and indenting on the left vestibulocochlear nerve; and a prominent and high-riding left jugular bulb. In this study, the internal auditory canals were assessed to be of normal width, with walls that were smooth and sharply defined. A cerebral CT angiogram subsequently performed did not show any abnormal findings related to the previously identified vascular loop. On the basis of these radiologic findings, the patient was advised surgery by physicians at a tertiary- care institution, presumably to address the identified vascular loop. A second opinion was sought by the patient.&#x0D; Review of the MRI initially focused on the axial high-resolution T2-weighted sequence (T2-DRIVE), as the fast spin-echo T2-weighted sequence has been recommended as a reliable and cost-effective MR screening protocol for the detection of masses in the IAC.1 In contrast to the official radiology report, stenosis of the left internal auditory canal by a protrusion (Figure 1, white asterisk) originating from the posteromedial wall of the internal auditory canal was noted. This protrusion, which had no MR signal intensity, appeared to abut and compress the cranial nerves within the IAC. Reconstruction of the images in non-orthogonal planes aligned with the orientation and direction of the left 8th cranial nerve showed the protrusion causing upward compression on and distortion of the nerve. (Figure 2, white arrows)&#x0D; Attention was directed to the axial high-resolution T1-weighted sequence (T1W-3D FFE), which revealed that the protrusion contained an isointense soft tissue structure (Figure 3, white asterisk) located within the petrous bone medial to the posterior semicircular canal. This structure appeared to be an upward extension of the jugular bulb.&#x0D; The axial high-resolution contrast-enhanced T1-weighted sequence (T1W-3D TFE Gd) showed smooth, vivid enhancement of the identified structure (Figure 4, black asterisk), which connected with the sigmoid sinus in lower cuts. This confirmed the presence of a high-riding jugular bulb that encroached on the internal auditory canal.&#x0D; Any doubt as to its true nature was dispelled by a review of the temporal bone structures on high-resolution CT which was fortunately available in the cerebral CT angiogram. This revealed a protrusion of the high-riding jugular bulb with a waist-like margin (Figure 5A and B, black arrows), allowing further characterization of the lesion as a jugular bulb diverticulum.2&#x0D; A high-riding jugular bulb that projects into the middle ear is not an uncommon anatomic variation. On the other hand, a jugular bulb diverticulum, which is an outpouching of the jugular bulb that can extend superiorly, medially, and posteriorly in the petrous bone, is a true venous anomaly that has been described rarely in the medical literature.3,4 When symptomatic, patients with this anomaly can present with sensorineural hearing loss, tinnitus, vertigo and auricular pain.3-5 Proper identification of a jugular bulb diverticulum in the evaluation of a patient with neurotologic symptoms is necessary to avoid inappropriate medical and surgical intervention.&#x0D; As demonstrated in this patient, a jugular bulb diverticulum may not be identified by a screening MRI that utilizes only a T2-weighted sequence. T1-weighted MRI sequences with and without contrast are necessary to demonstrate its soft tissue imaging characteristics. Although not the initial imaging study of choice for sudden sensorineural hearing loss, high-resolution bone-window CT may be necessary to delineate the bony anatomy of the jugular foramen and confirm the presence of this anomaly.

Cardiovascular surgery perspective on carotid body tumors: Retrospective study

Objectives: In this study, we aimed to share the results of the patients we operated on due to carotid body tumors and our preoperative, perioperative, and postoperative perspective. Patients and Methods: The medical records of patients who underwent surgery due to carotid body tumor between January 2004 and December 2020 were retrospectively reviewed. Seventeen patients (2 male, 15 female; mean age 52.4±15.4 years; range 25 to 75 years) who underwent surgery were evaluated based on age, sex, complaints, Shamblin classification, surgical procedures, and complications. Results: The smallest tumor was 15x15x11 mm, and the largest tumor was 75x55x45 mm. There were five Shamblin I, nine Shamblin II, and three Shamblin III patients. External carotid artery ligation and tumor excision were performed in one patient, while en blok tumor excision was performed in other patients without the need for vascular repair. No complications occurred, except for the ninth and 12th cranial nerve injuries that developed in one patient. Conclusion: Although follow-up and radiotherapy are recommended for high surgical risk, elderly, and comorbid patients, we believe that a careful and rigorous surgery by experienced surgeons, in accordance with the necessary multidisciplinary approach and thorough preoperative assessment, could be safely and effectively performed with low mortality and morbidity for carotid body tumors.

Aggressive Osteoblastoma of Temporal Bone Causing Facial Palsy in a 9-year-old Child: A Case Report Based on 2020 WHO Classification of Bone Tumors

AbstractAggressive osteoblastoma (AO) is an uncommon bone tumor that represents a borderline lesion between osteoblastoma and osteosarcoma. The vertebral column, the sacrum, the pelvis, and jaw/craniofacial bones are primarily affected. Aggressive osteoblastoma does not metastasize and is treated by surgical resection. The authors report a case of AO in a 9-year-old female patient presenting with 5th and 7th cranial nerve palsy. Prior pathological history included resection of an expansile nodule in the left temporal bone. Conventional radiological examination and computed tomography (CT) of the skull revealed an osteoblastic lesion arising in the petrous portion of the left temporal bone, measuring 5.2 cm in the largest dimension. The patient was subjected to partial surgical resection of the process. Microscopy revealed a primary neoplastic bone composed of numerous epithelioid round osteoblasts disposed in solid sheets and with mild atypia, large eosinophilic cytoplasm, and an eccentric, ovoid nucleus. The process exhibited loose stroma, low mitotic index, osteoid formation, and a few osteoclast-like multinucleated giant cells. The diagnosis of AO was thus established. After 5 months of clinical follow-up, the patient is asymptomatic, without evidence of tumoral growth on CT scans.

THU375 A Case Of Right Eye VI Nerve Palsy In A Patient With Uncontrolled Type 2 Diabetes Mellitus

Abstract Disclosure: C. Musurakis: None. A. Havrylyan: None. J.L. Gilden: None. Background: Uncontrolled type 2 diabetes is one of the most common etiologies of acquired VI nerve palsy. Clinical Case: A 40-year-old Mexican female with uncontrolled type 2 diabetes mellitus (DM), diabetic retinopathy and hypertension was admitted to the hospital due to the new onset of diplopia, headache, and right eye pain. Her symptoms started 2 weeks before seeking medical care and had worsened when she looked towards the right. Duration of DM was more than 10 years, with long-standing history of diet and medication non-compliance. She had never taken the prescribed metformin and insulin, nor did she monitor her blood glucose levels at home. Four years previously, she had received laser therapy to both eyes for diabetic retinopathy. Although she denied numbness or tingling of her extremities, she had a left 4th toe amputation in the past, due to infection, which was complicated by chronic osteomyelitis. There was no prior history of cerebrovascular or cardiac disease. Family history was positive for type 2 DM in both parents. Upon hospital admission, HbA1c was 13.2% (n&amp;lt;5.7). BMI 23.7. Vitals: BP 184/104, HR 110. Labs: blood glucose was 439 mg/dL (n=70-99), CO2 23 mEq/L (n=21-31), AG 12 mmol/L (n=4-11), Beta-Hydroxybutyrate/Acetate 2.0 mmol/L (n=0.1-0.3), venous pH 7.36 (n=7.35-7.45). Physical exam: no conjunctival redness, discharge or swelling around the eye, but inability to abduct the right eye laterally. CT head wo contrast: significant atherosclerotic calcifications within the intracranial segment of right vertebral artery and no acute intracranial abnormality. CTA brain w/wo contrast: no obvious mass effect on 6th cranial nerve territories. MRI of brain and orbit w/wo contrast showed no macroscopic anatomic abnormality or impingement on right abducens nerve but showed minimal white matter changes on the centrum bilaterally. She was diagnosed with an isolated VI facial nerve palsy in the setting of longstanding uncontrolled diabetes mellitus and hypertension. She was started with basal-bolus insulin and antihypertensive therapies and recommended to continue this medication regimen after discharge. She was also advised that gradual recovery of vision may be achieved with glycemic control of DM and hypertension. Conclusion: Although the etiology of acquired VI nerve palsy may be idiopathic, or result from hypertension, trauma, multiple sclerosis, neoplasm, CVA, postsurgical complication, or aneurysm, it is also caused by uncontrolled diabetes mellitus combined with hypertension. It is to be noted that when neoplasm or aneurysm is the cause, the history and examination often reveals the presence of other neurologic symptoms or signs. Reference: Patel SV, Holmes JM, Hodge DO, Burke JP. Diabetes and hypertension in isolated sixth nerve palsy: a population-based study. Ophthalmology. 2005 May;112(5):760-3. doi: 10.1016/j.ophtha.2004.11.057. PMID: 15878054. Presentation: Thursday, June 15, 2023