Left Abducens Nerve Palsy Research Articles

Sphenoclival fibrous dysplasia with cyst formation causing abducens nerve palsy in an older patient: illustrative case.

Fibrous dysplasia (FD) is typically asymptomatic but is known to occasionally cause neurological symptoms such as visual disturbances. However, FD-associated abducens nerve palsy is extremely rare. A 76-year-old woman had a left abducens nerve palsy due to mass effect on the abducens nerve at Dorello's canal caused by FD and its associated cyst, which was resolved by resection of the cyst through an endoscopic transnasal route. Although FD is generally an asymptomatic lesion occurring in younger patients, it can occasionally cause abducens nerve palsy even in older patients. In particular, cystic degeneration within FD tends to trigger a mass effect and thus can require surgical decompression. https://thejns.org/doi/10.3171/CASE24424.

Cranial neuropathy following coronavirus disease 2019 vaccination in kidney transplant recipients.

Understanding of adverse reactions to coronavirus disease 2019 vaccines remains limited. Case 1: A 52-year-old woman, post-kidney transplantation, experienced sudden vision loss in her left eye after receiving a second dose of coronavirus disease 2019 vaccine. She was diagnosed with ischemic optic neuropathy.Case 2: A 53-year-old woman, post-kidney transplantation, presented with worsening diplopia and left eye pain following the second dose of coronavirus disease 2019 vaccine. She was diagnosed with left abducens nerve palsy. Vigilance is essential for recognizing the potential for delayed cranial neuropathy in immunocompromised individuals after coronavirus disease 2019 vaccination.

Diplopia as the presenting symptom of a post‐transplant lymphoproliferative disorder in a bilateral inferior lim transplanted young adult

Aims/Purpose: To present a unique case of post‐transplant lymphoproliferative disorder (PTLD) after bilateral inferior limb transplantation, which is still controversial due to the absence of precedents in the literature and the unclear risk–benefit ratio.Methods: Case report.Results: A 22‐year‐old male presented to the emergency room complaining of double vision. He had a history of bilateral inferior limb amputation 8 years ago and received a bilateral lower limb transplantation 2 years later. He had normal graft function and was receiving cytomegalovirus prophylaxis. During neurological examinations left abducens nerve (CN VI) palsy was noted. Other cranial nerves affected produced facial hypoesthesia (CN V) and mild paresis (CN VII), hearing loss and tinnitus in left side (CN VIII) and soft palate asymmetry (CN X). He was otherwise neurologically intact, alert and orientated. MRI revealed a lesion located at the facial colliculus at the floor of 4th ventricle. In the context of immunosuppression, the differential diagnosis included abscess versus PTLD. Biopsy of the lesion was performed. Morphologically atypical lymphocytic cells suggestive of B lymphoma were identified. Monoclonal rearrangement for heavy chain Igen and immunoglobulins were identified with molecular biology techniques. The histology features were consistent with a diagnosis of CNS B lineage primary lymphoma. Two days later, bilateral supracondylar amputation was performed. Following the explant, immunosuppression was withdrawn. His underlying condition was finally diagnosed as PTLD, for which he started a chemotherapeutic regimen with radical intention (methotrexate and cytosine arabinoside). The patient recovered fully and up to date, 10 years later, is doing well.Conclusions: Due to its diverse aetiologias, a proper workup must be done of the patients presenting with diplopia to the emergency room. PLTD must be a condition to always be considered in patients with history of solid organ transplant; since the early diagnosis is crucial for a successful management of this often‐fatal condition.

10249-PEDT-8 CURRENT STATUS AND ISSUES OF PEDIATRIC DIFFUSE INTRINSIC PONTINE GLIOMA (DIPG)

Abstract that predominantly occurs in childhood, with a median survival of less than one year. Herein we present a recent case of DIPG and discuss the current status and future challenges. The patient was a 7-year-old boy who complained of tinnitus, headaches, and unstable gait. The MRI showed diffuse enlargement of the pons, and the contrast-enhanced T1-weighted image exhibited an irregular ring-like enhancement. Neurologically, he presented with left abducens nerve palsy, horizontal gaze nystagmus, significant left ataxia, and truncal ataxia. As the lesion extended to the left cerebellar peduncle, a stereotactic biopsy was performed. However, postoperative bleeding occurred, resulting in transient consciousness disturbance, left facial palsy, speech disturbance, and dysphagia, necessitating enteral nutrition management. The pathological diagnosis was high-grade glioma, IDH wild type, H3K27M mutation (-), TERTp wild type, BRAF V600E mutation (-), BRAF fusion (-), and MGMT unmethylated. After surgery, the patient received radiation therapy (54Gy/30fr), and his symptoms improved, allowing him to resume oral intake. Post-irradiation, the lesion shrank, and he was discharged home without assistance. However, six months later, his symptoms worsened, and he started taking temozolomide orally, but the effect was limited. Nine months later, he became unable to take oral medication, and a central venous port was placed. We coordinated with home care physicians to provide intravenous and palliative treatments, but respiratory and consciousness disturbances gradually worsened, and he died 10 months after the initial diagnosis. The median survival of DIPG patients treated at our hospital is currently 10.5 months, and this case followed a typical course. The development of novel treatments is desired, but due to the rarity of the disease, conducting comparative trials is challenging. Hence, registry data forming a control group are necessary. Additionally, transitioning to home-based palliative care and sharing information on supportive therapies are also critical tasks.

Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuropathy after Ad26.COV2.S COVID-19 Vaccination (P13-8.006)

<h3>Objective:</h3> NA <h3>Background:</h3> Acute onset demyelinating neuropathy has been reported after coronavirus disease 2019 (COVID-19) vaccination. Only a limited number of cases continued progression and became chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), mostly after ChAdOx1 nCoV-19 vaccination. Here, we report the first case of acute-onset CIDP after Ad26.COV2.S (Johnson &amp; Johnson) vaccination. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 67-year-old man presented with one-week history of progressive left facial droop, unsteady gait, slurred speech, double vision, and patchy numbness; he reported Ad26.COV2.S vaccination three weeks before presentation. On initial examination, he had left facial palsy, partial left abducens nerve palsy, generalized areflexia, severe lower extremities ataxia, and diminished vibratory sensation in toes. Cerebrospinal fluid analysis showed albuminocytologic dissociation. He received intravenous immunoglobulin (IVIG) for five days for presumptive diagnosis of Guillain-Barré syndrome (GBS). Electrodiagnostic studies demonstrated impersistent F-waves, decreased recruitment in lower extremities, and bilaterally absent blink reflexes. He developed right facial weakness on day three and asymmetric appendicular muscle weakness on day five of treatment. Seven weeks after symptom onset, his lower extremities weakness worsened, and received a second cycle of IVIG. The lower extremity weakness did not improve but remained stable until seventeen weeks after symptom onset when he returned with progressive upper extremities weakness and dyspnea. Electrodiagnostic studies demonstrated demyelinating features consistent with CIDP. Neuromuscular ultrasound demonstrated multifocal median nerve enlargements at non-entrapment sites. At this point, given the clinical course, electrodiagnostic and ultrasonographical findings, CIDP diagnosis was established. <h3>Conclusions:</h3> This patient’s acute-onset CIDP and other reported GBS cases after adenoviral vector COVID-19 vaccinations shared similar and atypical presenting symptoms, which were not seen in GBS after mRNA COVID-19 vaccinations. While a causal relationship cannot be established to his COVID-19 vaccination, this case adds to the evolving literature of COVID-19 vaccination-associated neurological events. <b>Disclosure:</b> Dr. Bendi has nothing to disclose. Dr. Chen has nothing to disclose. Dr. Danielson has nothing to disclose.

Neurofibroma of the internal carotid artery cavernous sympathetic plexus: illustrative case.

Intracranial carotid sympathetic plexus (CSP) nerve sheath tumors have rarely been reported in the literature. This study describes the first reported case of a CSP neurofibroma and the first case of a CSP nerve sheath tumor treated via an endoscopic endonasal approach followed by adjuvant radiosurgery. A 53-year-old man presented with 3 days of headaches and diplopia and was found to have a complete left abducens nerve palsy. Computed tomography (CT) revealed a smoothly dilated left carotid canal, CT angiography revealed a superiorly displaced left internal carotid artery (ICA), and magnetic resonance imaging revealed a T2-hyperintense and avidly enhancing lesion in the left cavernous sinus encasing the ICA. The patient underwent subtotal resection via an endoscopic transsphenoidal transcavernous approach followed by Gamma Knife radiosurgery. Nerve sheath tumors arising from the CSP are extremely rare but need to be considered when assessing unusual cavernous sinus lesions. The clinical presentation is dependent on the anatomical location of the tumor and its relationship to the ICA. The optimal treatment paradigm is unknown.

Spontaneous complete resolution of a giant cavernous carotid aneurysm in the setting of ipsilateral ICA occlusion

Background. Cavernous carotid aneurysms (CCA) are rare aneurysms with a relatively benign natural history. The association between CCA aneurysm and ipsilateral Internal carotid artery (ICA) thrombosis or occlusion has not been described previously. The management of patients with these dual lesions is a challenging problem. Case description. In this report, we describe an 18-year-old man who presented with left abducent nerve palsy of 3 weeks duration, and imaging revealed left CCA with left ICA occlusion. The patient was managed conservatively with clinical and imaging follow-up. The patient recovered well with complete resolution of clinical symptoms and disappearance of left CCA. Conclusions. The association of giant CCA and ICA occlusion on the same side is a rare phenomenon with no current consensus on the appropriate follow-up and management strategy. In this report, we described the first case of spontaneous complete disappearance of a giant CCA in the setting of ipsilateral ICA occlusion with complete resolution of symptoms at nine months of follow-up.

Clival Chondroma with Brainstem Invasion Findings in MRI: A Case Report

Chordoma is a rare neoplasm derived from remnants of the fetal notochord; the most commun locations are the lumbosacral spineand the clivus. Clival chordomas represent 35 to 40% of cases and are very difficult to treat because of the localization and high recurrence rate. We present the case of 60 years old male patient with no previous history consulting for diplopia and headach that worsened in the last week before the diagnosis, with a walking ability disorder. Neurological examination found a raised intracranial pressure syndrome with static cerebellar syndrome. However, he presented left hemifacial numbness due to left fifth cranial nerve palsy and diplopia at left gaze due to left sixth cranial nerve palsy. The diagnosis was made by MRI using the different sequences especially T1 SE, T1 GE, T2 FLAIR, DWI and spectroscopy.

A RARE CASE OF CONCOMITANT ABDUCENS AND CONTRALATERAL LOWER MOTOR NEURON FACIAL NERVE PALSIES FOLLOWING BLUNT HEAD TRAUMA ASSOCIATED WITH TEMPORAL BONE FRACTURE

This case report describes a case of concomitant abducens and contralateral lower motor neuron facial nerve palsies following blunt head trauma associated with left temporal bone fracture. A 11year old boy was brought to the emergency department with alleged history of fall from swing set. Detailed ocular examination and assessment of function of all the twelve cranial nerves of the patient revealed left oculomotor nerve paresis, left abducens nerve palsy and right sided lower motor neuron (LMN) facial nerve palsy. The patient was run through various neuroimaging investigations which showed longitudinal fracture of the left temporal bone. The patient was treated aggressively with systemic and oral steroids. On discharge, the left oculomotor nerve paresis had healed completely with no improvement in the other two affected cranial nerves. During one month follow up, the abducens and facial nerve palsies still persisted.

Gummatous neurosyphilis in an elderly patient in the Australian outback: a case report

IntroductionNeurosyphilis is an infection caused by the spirochete Treponema pallidum, which causes infiltration and thickening of brain meninges. Despite being an Old World disease, the rates of infection continue to rise. This clinical challenge involves early and accurate diagnosis, as neurosyphilis masquerades with various clinical symptoms and is often missed during initial presentation to the hospital. A comprehensive history and clinical examination are essential to detect suspicious cases early for further cerebrospinal fluid examination and neuroimaging. Patients treated with benzylpenicillin for a specific duration often show promising clinical and cognitive improvement, thus emphasizing the need for constant vigilance in our day-to-day practice.Case presentationA 77-year-old Caucasian gentleman presented to our hospital repeatedly with multiple episodes of presyncope and cognitive impairment. He also demonstrated bilateral deafness, tabes dorsalis, and left sixth cranial nerve palsy. His cerebrospinal fluid examination showed a nonreactive venereal disease research laboratory test, and magnetic resonance imaging of the brain revealed a gumma.ConclusionThe diagnosis of neurosyphilis in the elderly requires a combination of clinical vigilance and a high index of suspicion, along with multimodal investigations, including cerebrospinal fluid examination and brain imaging.

Extracranial arteriovenous malformation and subsequent contralateral cavernous sinus dural arteriovenous fistula showing abducens nerve palsy: illustrative case.

BACKGROUNDExtracranial arteriovenous malformations (AVMs) are rare clinical entities and on rare occasions cause neurological symptoms. The authors report a case of an extracranial pterygoid AVM and a subsequent contralateral cavernous sinus dural arteriovenous fistula (dAVF) presenting with abducens nerve palsy.OBSERVATIONSAn 80-year-old woman was referred to the authors’ hospital with left abducens nerve palsy followed by right ophthalmalgia. Magnetic resonance imaging (MRI) showed abnormal vessel staining in the left pterygoid and the right inferior petrosal sinus (IPS). Cerebral angiography revealed a left pterygoid AVM draining into the right IPS via the cavernous sinus (CS). A dAVF in the right CS was also revealed. The right ophthalmalgia disappeared spontaneously, and, 4 months later, the left abducens nerve palsy also disappeared after conservative management. Follow-up MRI showed spontaneous regression of the AVM and dAVF. The disappearance of the dAVF was considered to be due to spontaneous regression of the left pterygoid AVM and the consequent decrease in venous pressure of the CS, and the symptoms eventually disappeared.LESSONSThe authors treated an extremely rare case of extracranial AVM with dramatic changes in vascular structure and symptoms. Understanding of the pathophysiology between symptoms and dynamic changes in the vascular structure is essential for providing the appropriate treatment.

Atypical Presentation of Frontal Lobe Tumor (Glioblastoma) in an Adolescent: A Case Report

<strong>Introduction:</strong> Malignant brain tumors in childhood occur less frequently than nonmalignant ones and glioblastoma multiforme has been identified as the most common and aggressive [1]. These tumors usually present with typical neurologic symptoms including signs of raised intracranial pressure [2]. The case study describes an atypical presentation of glioblastoma in a previously healthy adolescent male with several distractors that could have led to a delay in diagnosis. This highlights the need to have a high index of suspicion for serious underlying illness when managing pediatric patients presenting with apparently trivial symptoms. <strong>Case Presentation:</strong> A 15-year-old previously healthy adolescent male presented with a 2-week history of non-projectile, non-bilious, non-bloody vomiting, usually post-prandial, about 4–5 episodes per day. He had associated periumbilical pain, burning sensation during voiding, chills, poor feeding, weakness, and weight loss. Physical examination was significant for dry mouth and generalized abdominal tenderness. He was initially managed with famotidine and polyethylene glycol in the pediatrician’s clinic prior to presentation in the ED for persistent symptoms. Results of investigations, including complete blood count, complete metabolic panel, urine analysis, culture, and toxicology were unremarkable. He tested positive for Influenza A and Respiratory Syncytial Virus. Abdominal x-ray was suggestive of constipation. On admission, he received intravenous fluids, pantoprazole, famotidine, and oseltamivir with some improvement in symptoms. On day 2, he complained of throat pain and intermittent low intensity bitemporal headache rated 5/10 which subsided with acetaminophen. On day 3, urinary and fecal incontinence were noted. Additional history of recent travel to Ecuador and prolonged contact with a family member with gastrointestinal tuberculosis was obtained so Quantiferon Gold was sent. Psychiatry was consulted because of his flat affect and poor communication and assessment of adjustment disorder from medical illness was made. On day 4 of admission, neurologic exam revealed significant neck stiffness and suspicion of left abducens nerve palsy. Urgent brain computed tomography scan without contrast showed a large slightly hyperdense mass centered within the left frontal lobe that appeared to cross the anterior corpus callosum and measured roughly 7.6 × 5.8 × 4.3 cm (<em><strong>Figure 1</strong></em>). The patient was transferred to the Pediatric Intensive Care Unit where he received a dose of intravenous Dexamethasone and then transferred to a tertiary hospital with a Pediatric Neurosurgery service. The patient had persistent raised intracranial pressure with waxing and waning neurologic findings. He underwent tumor resection on day 6 of admission. Frozen section was suggestive of glioblastoma multiforme and pathology showed grade IV glioblastoma. Surgery was followed by radiation and chemotherapy with temozolomide [3]. <strong>Conclusion:</strong> Vomiting, typically early morning projectile vomiting, is a classic presenting symptom of increased intracranial pressure. However, this patient had several red herrings on presentation and history that could explain his symptoms. Premature closure and anchoring can lead to missing serious diagnoses presenting as a seemingly routine pediatric illness.

Teaching NeuroImage: Abducens Nerve Palsy With Ipsilateral Excessive Eye Tearing Attributed to an Internal Carotid Artery Sympathetic Plexus Schwannoma.

A 65-year-old man developed subacute horizontal diplopia due to left abducens nerve (AN) palsy and excessive left eye tearing. Brain MRI revealed a hyperintense T2 lesion with an elongated course within the left carotid canal, presenting homogenous contrast enhancement (figure 1). The imaging findings were characteristic for an internal carotid artery sympathetic plexus (ICSP) schwannoma compressing the left AN. Subsequent irritation of the deep petrosal nerve originating directly from ICSP and continuing as the Vidian nerve may have led to the lacrimal gland edema and excessive left eye tearing (figure 2). Thorough case presentation and a figure demonstrating the relevant anatomy are available from Dryad at [doi.org/10.5061/dryad.pzgmsbck6][1]. [1]: https://doi.org/10.5061/dryad.pzgmsbck6

Abducens Nerve Palsy as the First Manifestation of Type Two Diabetes Mellitus

Abstract Introduction: Diabetes mellitus has varied presentations at different times from onset. One of the uncommon presentations is cranial nerve palsy secondary to microvascular ischemia. Approximately 20% of isolated abducens nerve palsies are secondary to vascular microangiopathy. Clinical Case: A 53-year-old female with no significant past medical history presented to the emergency department with concerns of double vision. The patient first noticed her symptoms five days prior to presentation. The patient endorsed blurry vision and stated that she has double vision in certain gazes that resolves with shutting either eye. Furthermore, she has also developed headaches during that period, described as a pressure-like sensation in her forehead and behind her eyes. She otherwise denied any tearing, redness, or pain. On review of systems, she denied numbness, tingling, changes in hearing, changes in speech, or extremity weakness. Due to the persistence of symptoms the patient presented to the emergency department. On exam, the patient’s vitals were normal. Pupils were equal and briskly reactive to light with no relative afferent pupillary defect. External examination was unremarkable without scalp tenderness, proptosis, or ptosis. Color vision was intact. Ocular motility testing revealed limited abduction of the left eye causing double vision on lateral gaze. Confrontation visual fields were full in each eye. Bilateral lower extremity exam revealed decreased sensation in the sole of the foot. The rest of the physical exam was unremarkable. Laboratory work revealed blood glucose level of 305 mg/dl (Reference range 70–99 mg/dl). HBA1C was 12.3% (Reference range 3.8–5.6%). CT head and CTA of the neck was performed and were unremarkable. Given the normal imaging and findings on examinations, her ocular motor findings were attributed to diabetes mellitus. The patient was started on insulin, aspirin, and received diabetic education for lifestyle modification, and was scheduled for outpatient follow up. The patient’s acute isolated left sixth cranial nerve palsy was most likely owing to microvascular ischemia from previously undiagnosed diabetes mellitus. A study of 59 patients with an isolated sixth cranial nerve palsy showed a 6-fold increase in the odds of having diabetes compared with controls.(1) Conclusion: Given the infrequent presentation of Diabetes with Abducens nerve palsy, diagnosis is usually delayed with the expense of ordering costly investigations that put a financial and psychological burden on patients. Thus, we urge clinicians’ awareness when encountering cases of isolated cranial nerve palsies.

Hypopituitarism as an Initial Presentation of Silent Squamous Cell Carcinoma of Lung

Background: Symptomatic metastasis to the pituitary gland is a rare, life-threatening condition. A better understanding of its clinical presentation could lead to early diagnosis and also improve quality of life. In this unusual case, we present a patient with hypopituitarism as the first manifestation of undiagnosed squamous cell carcinoma of the lung. Clinical Case: A 56-year-old Caucasian female with past medical history of pre-diabetes, hypertension, hyperlipidemia, and a chronic smoker for 40 years presented with recent onset of headache for two weeks and diplopia for three days. Initial CT head was negative but MRI of brain pituitary scan revealed a large heterogeneously enhancing sellar/suprasellar mass (1.5 x 2.8 x 2.4 cm) extending into the prepontine cistern with probable bilateral cavernous sinus invasion and contacting the optic chiasm without significant compression. Physical examination showed left abducens nerve (CN VI) palsy. Lab results showed elevated prolactin at 67.4 ng/ml (normal postmenopausal: 1.8 – 20.3 ng/ml), normal IGF-1 at 136 ng/ml (normal 48- 235 ng/ml), low TSH at 0.129 µIU/ml (normal 0.3 – 4.5 µIU/ml), low normal Free T4 at 0.50 ng/dl (normal 0.5 – 1.26 ng/dl), low ACTH at 5.1 pg/ml (normal 7.2 – 63.3 pg/ml), low am cortisol at 2.6 mcg/dl (normal 8.7 – 22.4 mcg/dl), low FSH at 2.6 mIU/ml (normal postmenopausal 23.0-116.3 mIU/ml) and low LH at 0.2 mIU/ml (normal postmenopausal 15.9 - 54.0 mIU/ml). She subsequently began treatment for hypopituitarism. Given her smoking history and unusual presentation of cranial nerve palsy, a chest x-ray was ordered and revealed a left upper lobe mass. CT chest confirmed the left upper lobe mass (4.0 x 3.3 x 2.9 cm), and biopsy reported poorly differentiated squamous cell carcinoma. Decompression of the pituitary macroadenoma was performed for symptomatic improvement but was complicated with the development of central diabetes insipidus. Pathology report disclosed metastatic pituitary carcinoma, compatible with primary lung carcinoma. Following surgical resection and radiation therapy to the pituitary gland, the patient remains stable and is currently tolerating all treatment. Conclusion: In a patient with occult malignancy, pituitary metastasis is an exceedingly rare and challenging diagnosis that carries a poor prognosis. The purpose of this abstract is to raise clinical suspicion for sellar metastasis in a patient presenting with hypopituitarism and cranial nerve palsy.

Ventrally exophytic pontine mass in a patient with dysarthria, dysphagia and diplopia – Question

A 44-year-old man with no significant past medical history presented with a one-month long history of dysarthria, dysphagia and diplopia of left lateral gaze. Examination showed a left abducens nerve palsy and hemifacial numbness. He was hyper-reflexic in the upper and lower limbs on the right side, with bilateral ankle clonus and upgoing plantar reflexes. Routine blood tests showed no abnormalities. Magnetic resonance imaging (MRI) of the brain demonstrated a large ventral pontine mass extending to the left cerebellopontine angle (Fig. 1A). The lesion demonstrated moderately high signal on FLAIR sequences (Fig. 1B) and intermediate to low signal on T1 weighted images. Internal cystic components were seen, and the mass demonstrated patchy contrast enhancement (Fig. 1C). There was no associated diffusion restriction. Encasement of the basilar artery was noted without loss of the flow void. The most likely diagnosis is:A)Left Abducens Nerve SchwannomaB)High Grade GliomaC)SarcoidosisD)Serpentine Fusiform Basilar Artery AneurysmE)Epidermoid CystF)Exophytic Pilocytic Astrocytoma Answer on page: 310. Both C.O and T.O conceptualised the paper. C.O prepared the initial draft. This draft was revised based on input from T.O and S.S.

Unraveling a cephalalgic quagmire from a cavern to a cave.

Headache in women in their late forties can be primary or secondary. We report a 48-year-old female with chronic slowly progressive left temporal headache for 1 year. She also had ipsilateral eye pain and facial numbness for 1 month, with restricted abduction in the left eye and diplopia. On neurological examination, she had isolated left abducent nerve palsy, with loss of corneal and conjunctival reflexes, localizing the pathology to the cavernous sinus or its adjacent structures. Anatomically, cranial nerves V and VI are in close proximity to each other in the region of Meckel's cave. In view of her age, insidious onset, progressive symptoms and clinical findings, the provisional diagnosis in this patient was a Meckel's cave tumor. Magnetic resonance (MR) imaging revealed a 2 cm × 2 cm × 1.7 cm enhancing dumb-bell-shaped mass lesion with mild restricted diffusion in the Meckel's cave projecting into cavernous sinus with alanine, myoinositol and glutamine peaks on MR spectroscopy. Intradural debulking was done; lesion was confirmed by histopathology and patient was cured of her symptoms. An algorithm for diagnosing this entity at the bedside is presented.

Oculopalatal tremor and hypertrophic olivary degeneration

Background: Oculopalatal tremor is characterized by synchronous occurrence of palatal tremor and pendular nystagmus. Oculopalatal tremor (OPT) follows a destructive lesion in the Guillain- Mollaret triangle (GMT) and is associated with hypertrophic inferior olive degeneration (HOD). Case presentation: We report a case of 45 yearsold patient with palatal tremor and pendular nystagmus without ear click. He has had a brainstem stroke 11 months back. He had right side pyramidal drift, left side lower motor neuron type facial weakness and left abducens nerve palsy and mild gait ataxia in addition to oculopalatal tremor. MRI showed hypertrophic inferior olive degeneration. Conclusion: Ear click is an infrequent finding in symptomatic palatal tremor. Additional clinical features may be present in OPT depending on topography of initial structural lesion. There is a wide variety of time intervals between the occurrence of anatomical lesions and recognition of palatal tremor.

SUN-923 Combination Immune Checkpoint Inhibitor Therapy for ACTH-Secreting Pituitary Carcinoma

IntroductionPituitary carcinoma is a rare yet serious entity with poor prognosis despite multimodal therapies. Cerebrospinal and/or systemic metastases are present by definition, making adjuvant systemic therapy necessary. Novel treatments are urgently needed for refractory cases. Immunotherapy with immune checkpoint inhibitors (ICI) targeting cytotoxic T-lymphocyte antigen-4 (CTLA-4), programmed cell death 1 (PD-1) or its ligand (PD-L1) has been a revolution in multiple malignancies. The expression of CTLA-4 and PD-L1 has been elucidated in pituitary adenomas and could be implicated in pituitary carcinomas as well. Hypophysitis is also a frequent endocrine immune-related adverse event, especially during CTLA-4 blockade (with ipilimumab) or combination ICI. However, the efficacy of ICI in the treatment of refractory pituitary tumors has yet to be established. In 2018, Lin et al. successfully treated a first case of a hypermutated aggressive ACTH-secreting pituitary carcinoma with ipilimumab (anti-CTLA-4) and nivolumab (anti-PD-1) combination immunotherapy.Clinical CaseWe report a 40-year old male, diagnosed with an invasive ACTH-secreting pituitary macroadenoma in 2012, initially treated by transsphenoidal and transcranial surgery, followed by adjuvant stereotactic radiotherapy and several courses of ketoconazole. In 2017, he presented to our clinic for a recurrent Cushing’s phenotype despite maximal dosing of ketoconazole. Therapy both with pasireotide and cabergoline was unable to normalize cortisol levels and a bilateral (subtotal) adrenalectomy was performed. In June 2018, he presented to our emergency department with acute diplopia due to a left abducens nerve palsy. Imaging revealed recurrent invasion of the tumor into the sella turcica and cavernous sinus, together with cerebellar and drop metastases at the cervical spine. Temozolomide (TMZ) was initiated for a total of 9 cycles. Progressive disease was observed with development of new onset right oculomotor nerve palsy after the last TMZ cycle, and persistence of elevated serum ACTH-cortisol and urinary cortisol levels, despite the absence of radiological progression. Therefore, he was started in a compassionate use setting with a combination ICI therapy with ipilimumab 3 mg/kg and nivolumab 1 mg/kg (for 4 cycles), followed by maintenance nivolumab therapy (240 mg) every two weeks. He has stable disease (both radiographically and hormonally) five months after the initiation of the immunotherapy.Clinical Lesson(s) or Conclusion(s)We report the second case of ACTH-secreting pituitary carcinoma treated with combination ICI therapy. The disease status of the patient is stable up until now, suggesting at least disease control by the immunotherapy. Checkpoint blockade inhibitors are a promising novel treatment modality for refractory pituitary tumors and should be further studied.

Bilateral Giant Opthalmic Carotid Artery Aneurysms Presenting as Unilateral Diplopia: A Case Report

Introduction: The etiology of giant aneurysms is multifactorial. It becomes symptomatic in 9/100.000, most frequently in the fifth and sixth decades of the life. Intracavernous carotid artery aneurysms (ICAAs) represent less than 1% of intracranial aneurysms and show a slight female predominance. Case-Report: We report an interesting case of bilateral ICAA in 42-year-old female presented as acute diplopia and emphasize the need of a thorough systemic evaluation in young patients with diplopia and treated with endovascular coil embolization. A 42-year-old woman was admitted to the neurology department suffering from the unilateral diplopia. Neurological examination showed the left sixth cranial nerve palsy and had no further general motor or sensory symptoms. Digital Subtraction Angiogram (DSA) showed bilateral ophthalmic artery aneurysm. The patient was treated with endovascular loose packing coil embolization and Derivo 3.5x30mm flow diverter stent. Conclusion: It should be kept in mind that intracavernous carotid artery aneurysms may be bilateral in patients with unilateral cranial nerve symptoms.