Sudden Onset Of Diplopia Research Articles

Abducens palsy with facial nerve palsy: A rare clinical presentation

Abstract Multiple cranial nerve palsies are commonly due to brainstem lesions and the presence of abducens nerve palsy with peripheral facial nerve palsy is very rare and the exact etiologies are not defined. Very few reports of pontine infarct are associated with the condition. We report a young male presented with sudden onset diplopia with left-side abduction deficit and inadequate eyelid closure of the left eye. Upon evaluation, it was found to have high blood pressure recordings and imaging revealed the presence of an ipsilateral pontine infarct. The patient was managed with antihypertensives and antiplatelet agents and symptomatic resolution was found after 2 months of follow-up. This report describes a very rare association of abducens and facial nerve palsy in pontine infarct and to our best knowledge only a few reports are available on the same.

An unusual cause of diplopia and blurred vision in a patient with renal insufficiency.

We report the interesting case of a 75-year-old patient who presented with sudden onset of diplopia and a blurred vision of the right eye. Recurrent headaches in the temporal region were reported as further symptoms. Clinical examination revealed abduction restriction on the right side and bilateral vascular congestion in the anterior segment of the eye, and marked chemosis. As relevant pre-existing conditions, the patient had severe hypertension, long-standing nicotine abuse (>50py), 3-vessel coronary artery disease, myocardial infarction 33 years ago, and cardiac bypass surgery five years ago. Furthermore, the patient suffered from chronic renal failure stage G5D, according to Kidney Disease Outcomes Quality Initiative (K/DOQI), with the need for dialysis. For this reason, a polytetrafluoroethylene loop shunt was created on the right cubital region five years ago, which underwent thrombectomies one year ago and three months ago. During the last revision, the shunt was extended to the internal jugular vein to improve patency, which was not reported by the patient with a language barrier, and no documentation from the external hospital was available.

Demographic and clinical characteristics of age-related distance esotropia

Age-related distance esotropia (ARDE), is an acquired, small, comitant esodeviation that is greater at distance than at near. It occurs in older adult patients without a history of neurological event or prior strabismus. It has been observed more frequently in White adults than in other racial groups. The purpose of this study was to assess the demographic and clinical characteristics of patients with ARDE presenting at a tertiary neuro-ophthalmology clinic. In this retrospective study, ICD-9/10 (ICD-9 378.85 and ICD-10 H51.8) codes were used to identify all patients with ARDE from 2005 to 2020 seen in a single tertiary neuro-ophthalmology clinic. ARDE was defined as esotropia greater at distance than near with associated clinical signs of adnexal tissue laxity. Patients with history or findings compatible with other etiologies of strabismus, such as thyroid eye disease, neuromuscular disorders, sensory deviations, sudden onset of diplopia, and high myopia, as well as those with prior strabismus surgery, were excluded. A total of 89 patients (59 females [66%]) met inclusion criteria. Mean patient age was 76.6 years. All patients were White except for a single patient of African descent. Mean follow-up time was 25.2 months. Mean esodeviation at distance on presentation was 6.6Δ. Of the 87 patients electing nonsurgical treatment, 80 achieved remission of diplopia symptoms with prism therapy alone. Of the 89 patients, 59 had no neuroimaging. ARDE in our neuro-ophthalmology clinic population was diagnosed almost exclusively in older White adults. Prism therapy was effective for a majority of our patients.

E015 A case of ocular myasthenia gravis seen by rheumatology as suspected mononeuritis multiplex in an adult male shortly after Pfizer-BioNTech SARS-CoV-2 vaccination

Abstract Background/Aims Myasthenia gravis (MG) is an antibody-mediated autoimmune disease targeting proteins at the postsynaptic membrane of the neuromuscular junction. MG is thought to occur in genetically susceptible individuals following an environmental trigger. SARS-CoV-2 infection has been associated with new-onset autoimmune disease, new-onset MG, and exacerbations of pre-existing MG, with molecular mimicry between SARS-CoV-2 epitopes and autoantigen-induced autoreactivity thought to be part of the underlying mechanism. We report a case of new-onset ocular MG following first dose Pfizer-BioNTech BNT162b2 SARS-COV2 vaccination which was referred to rheumatology as suspected mononeuritis multiplex. Methods A 53-year-old man of East Asian ethnicity presented to the emergency department (ED) with sudden onset diplopia and left lateral gaze restriction 7 days after receiving his first dose of the Pfizer-BioNTech BNT162b2 SARS-COV2 vaccination. He had longstanding myopia and dry eyes but no other medical history, no regular medications or significant family history. He was a current smoker, with a 50-pack year history. He did not drink alcohol or use any recreational drugs. He was found to have an isolated left VI cranial nerve (CN) palsy with an otherwise normal ocular and physical examination. Blood tests were unremarkable apart from raised cholesterol, and he was discharged with a suspected self-limiting microvascular CN lesion. Three weeks later he presented to ED with worsening diplopia, increasingly restricted eye movements, headache, nausea, vomiting and blurred vision. Ophthalmology assessment noted new right sided CN III and VI palsy, persistent left CN VI palsy, and vertical diplopia in all fields of gaze. Neurological and physical examination were normal. Bloods including an autoimmune screen were unremarkable. SARS-CoV-2 Spike antibodies were positive consistent with SARS-CoV-2 vaccination but not infection. Intracranial and thoracic imaging were unremarkable. He was referred to and seen by both rheumatology and neurology as a case of suspected mononeuritis multiplex. Results A diagnosis of ocular MG was confirmed with positive serum acetylcholine receptor antibodies, and he was started on prednisolone, and pyridostigmine to good effect. Daily forced vital capacity (FVC) showed no respiratory muscle involvement, and nerve conduction studies and electromyography were normal, excluding secondary generalisation. Conclusion A review of the literature found 14 reported cases of new-onset MG all within 4 weeks following SARS-CoV-2 vaccine. Whilst these cases provide interesting insights into the pathogenesis of autoimmune conditions such as MG, they are not epidemiological studies to inform vaccine safety. Ultimately, current evidence suggests that the risks of SARS-COV-2 infection outweigh the risk of vaccine-related adverse events, therefore we suggest clinicians should be aware of potential new-onset autoimmune conditions, but support the safety of SARS-COV2 vaccination. Further, research into possible immunological mechanisms behind this phenomenon, including identifying potential epitopes inducing molecular mimicry, could help establish the likelihood of a causative link. Disclosure A. Storrie: None. R.M. Hum: None. J. Lilleker: None. P. Ho: None.

Eight-and-a-half syndrome as the first presentation of multiple sclerosis in an Asian male: a case report

BackgroundMultiple sclerosis is a diffuse chronic demyelinating disease of the central nervous system. It is relatively uncommon in the Asian population and even more so in males. Despite the usual involvement of the brainstem, eight-and-a-half syndrome remains a rare first presentation in multiple sclerosis. Only a few cases have been reported previously, but none involving the Asian population. Eight-and-a-half syndrome, a neuro-ophthalmological condition, is characterized by one-and-a-half syndrome with ipsilateral lower facial nerve palsy, which localizes lesions to the pontine tegmentum. This case report demonstrates the first case of eight-and-a-half syndrome as the first presentation of multiple sclerosis in an Asian male.Case presentationA healthy 23-year-old Asian man presented with sudden onset of diplopia followed by left-sided facial asymmetry for 3 days. Assessment of extraocular movement revealed left conjugate horizontal gaze palsy. On right gaze, there was limited left eye adduction and horizontal nystagmus of the right eye. These findings were consistent with a left-sided one-and-a-half syndrome. Prism cover test revealed left esotropia of 30 prism diopters. Cranial nerve examination showed left lower motor neuron facial nerve palsy, while other neurological examination was normal. Magnetic resonance imaging brain showed multifocal T2 fluid attenuated inversion recovery hyperintense lesions, involving bilateral periventricular, juxtacortical, and infratentorial regions. A focal gadolinium contrast-enhanced lesion with open ring sign on T1 sequence was seen at the left frontal juxtacortical region. Multiple sclerosis was diagnosed on the basis of the clinical and radiological evidence, which fulfilled the 2017 McDonald criteria. Positive oligoclonal bands in cerebrospinal fluid analysis further confirmed our diagnosis. He had a complete resolution of symptoms 1 month after a course of pulsed corticosteroid therapy, and was subsequently placed on maintenance therapy with interferon beta-1a.ConclusionThis case illustrates eight-and-a-half syndrome as the first presentation of a diffuse central nervous system pathology. A wide range of differential diagnoses needs to be considered in such a presentation as based on the patient’s demographics and risk factors.

CNSC-03. NEURONAL INTERMEDIATE FILAMENT (NIF) ANTIBODY POSITIVE PARANEOPLASTIC SYNDROME IN A PATIENT WITH METASTATIC NEUROENDOCRINE PANCREATIC CARCINOMA AND TREATMENT WITH IPILIMUMAB/NIVOLUMAB

Abstract Immune checkpoint inhibitors, such as ipilimumab (CTL-4) and nivolumab (PD-1) have shown effectiveness in many types of neoplasia. This combination has been used as salvage immunotherapy for high grade metastatic pancreatic carcinoma. Nevertheless, severe neurologic immune-mediated adverse effects can result from the use of this treatment. We present a case of a 64-year-old woman with metastatic neuroendocrine pancreatic carcinoma with abrupt left facial and oculomotor cranial nerve palsy as well spastic tetraplegia after one dose of ipilimumab/nivolumab. She had a history of sudden onset diplopia, fever and ground-level fall a few hours after her initial dose of immunotherapy. Initial laboratory assessment showed pronounced lymphocyte predominant leukocytosis and mild chronic hyponatremia. In the following hours, she developed right mydriasis, right sided fast phase nystagmus and right central facial paralysis along with weakness and spasticity in four extremities. Neuroimaging of brain, orbits and CTL spine showed enhancement of intra-orbital and intracranial optic nerves as well chiasm, linear enhancement from C4-C7 and cauda equina, as well as abnormal signal along thoracic spinal cord and conus medullaris. Routine EEG demonstrated non-etiology specific encephalopathy. Lumbar puncture had normal opening pressure, mild pleocytosis, hyper-proteinorrhachia and normal glucose. Viral panel and cytology in the CSF were negative, and myeline basic protein was elevated. She was treated with high dose steroids which improved overall strength and CN palsies. Twenty days after initial presentation, she suddenly developed hypotension, tachypnea, and altered cognition. The patient and family opted for comfort measures only. She passed away that same day. Late report of CSF antibody panels showed positive cell-based assay for heavy and light chain NIF with a titer of 1:512. This case emphasizes the importance of recognition of anti-body mediated paraneoplastic syndromes in patients treated with immunotherapy, their appropriate treatment and prognosis.

Pontine hemorrhage presenting as Foville’s syndrome

Foville’s syndrome, also known as inferior medial pontine syndrome is one of the rare brainstem stroke syndromes with only few cases reported worldwide occurring due to involvement of the infero-medial aspect of pons. Condition is characterised by various cluster of neurological features as a result of defect in multiple vital areas like cortico spinal tract, medial lemniscus, middle cerebral peduncle, facial nerve and abducens nerve involvement. We reported one such rare case of a patient with no known systemic co-morbidity, who presented with sudden onset diplopia, lagophthalmos and contralateral weakness of limbs. On evaluation with computed tomography imaging, hemorrhage at the level of inferior pons was found. Patient subsequently treated and commenced on physiotherapy for rehabilitation.

SUN-273 A Rare Case of IgG4-Related Hypophysitis

Introduction:Hypophysitis is an acute or chronic inflammation of the pituitary gland and is an important diagnostic consideration in a patient with a sellar lesion. The annual incidence of hypophysitis is estimated to be 1 in 7–9 million and it accounts for approximately 0.4% of pituitary surgery cases. The following highlights a rare case of isolated IgG4-related hypophysitisClinical Case:A 63-year-old Caucasian female presented with sudden onset of diplopia and decreased visual acuity. This was associated with a 3-month history of headaches and 5-lbs weight loss. Past medical history was significant for hypertension and a 1.5cm sellar/suprasellar mass incidentally discovered during the work-up for persistent headaches 1-month prior. Initial anterior pituitary hormone evaluation was normal and the patient was scheduled for endoscopic endonasal resection of a presumed non-functioning pituitary adenoma. Family history was negative for pituitary tumors or hyperparathyroidism. Physical examination was notable for medial deviation of her left eye but neurologic examination was otherwise normal. Laboratory studies were notable for a normal TSH [1.769 uIu/ml (normal: 0.3–5.0)] and low free T4 [0.44 ng/ml (normal: 0.89–1.78)] consistent with central hypothyroidism; an inappropriately normal FSH for a postmenopausal woman [5.6 mIu/ml (normal: 0.3–10.5), and a normal prolactin level [16 ng/ml (0.6–20)]. An 8am cortisol was low at 2mcg/dL (5–21) with an ACTH level of 10 pg/mL (9–46). IGF-1 was normal at 89 ng/mL (41–279). Repeat pituitary MRI imaging demonstrated a homogenously enhancing sellar/suprasellar mass measuring 3.8 cm with displacement of the optic chiasm. Serum IgG4 levels were normal. The patient was started on 50mg IV hydrocortisone every 8 hours for central adrenal insufficiency and levothyroxine 88 mcg daily for central hypothyroidism and underwent an endoscopic endonasal biopsy of the lesion. Surgical pathology was notable for plasma cell-rich lymphohistiocytic hypophysitis and IgG4 plasma cells constituted >40% of the total plasma cell population. The patient subsequently received 1g of rituximab and repeat imaging one week later showed marked improvement in the size and extent of the lesion. The patient was discharged on prednisone and levothyroxine and received a second dose of rituximab at follow-up. The patient reports a decrease in the frequency of her headaches but continues to endorse diplopia.Conclusion:IgG4-related hypophysitis typically presents as part of a multifocal systemic process. This case highlights a rare entity of IgG4-related hypophysitis without other features of systemic disease and with normal serum levels of IgG4. Although glucocorticoids are universally regarded as the first line of therapy, an immunosuppressive agent or B-cell depletion therapy such as Rituximab may improve remission and decrease the risk of relapse.

WEBINO syndrome (wall-eyed bilateral internuclear ophthalmoplegia) secondary to ischemic stroke, about a case

WEBINO (wall-eyed bilateral internuclear ophthalmoplegia) syndrome is characterized by bilateral adduction impairment, nystagmus of the abducting eye, and primary gaze exotropia. We present the case of a 68 year-old man who was initially attended in emergency department with sudden onset diplopia. Neurological exploration revealed WEBINO and gait ataxia. Relevant medical history included liver transplantation and subsequent tacrolimus prescription. Complementary exams revealed ischemic lesion in mesencephalic tegmentum, involving medial longitudinal fasciculus and pretectal area. WEBINO syndrome is unfrequent. Among its etiologies, ischemic and demyelinating are the most frequent. In our case, iatrogenic etiology was also considered. Clinical recognition of this syndrome is required to perform adequate exams in order to reach diagnosis.

Síndrome de WEBINO (wall-eyed bilateral internuclear ophthalmoplegia) secundario a ictus isquémico, a propósito de un caso

WEBINO (wall-eyed bilateral internuclear ophthalmoplegia) syndrome is characterized by bilateral adduction impairment, nystagmus of the abducting eye, and primary gaze exotropia. We present the case of a 68 year-old man who was initially attended in emergency department with sudden onset diplopia. Neurological exploration revealed WEBINO and gait ataxia. Relevant medical history included liver transplantation and subsequent tacrolimus prescription. Complementary exams revealed ischemic lesion in mesencephalic tegmentum, involving medial longitudinal fasciculus and pretectal area. WEBINO syndrome is unfrequent. Among its etiologies, ischemic and demyelinating are the most frequent. In our case, iatrogenic etiology was also considered. Clinical recognition of this syndrome is required to perform adequate exams in order to reach diagnosis.

Nothnagel Syndrome: A rare case of Ischemic Brain Stem Syndrome

‘Nothnagel Syndrome' is a brainstem syndrome which is often underdiagnosed by the physicians due to rarity of the disease and complex clinical presentation. We present a case of brainstem midbrain syndrome caused by ischemic stroke. The male hypertensive diabetic patient presented with sudden onset of diplopia, ptosis and gait imbalance. Right-sidedgaze palsy with nystagmus and ataxic gait were the clinical findings. Brain imaging (MRI and DWI) showed high signal intensity at upper midbrain. The patient was finally diagnosed as ischemic stroke in the brainstem called Nothnagel syndrome. Up to now, there was no case reported as Nothnagel Syndrome due to ischemic stroke.
 J MEDICINE JAN 2020; 21 (1) : 62-64

Ocular myasthenia gravis in a person with tetraplegia presenting challenges in diagnosis and management

We report the first case of ocular myasthenia gravis (OMG) in a patient with complete tetraplegia, highlighting diagnostic and management challenges. Spinal multidisciplinary rural clinic and specialised inpatient Spinal Cord Injury Unit, NSW, Australia. A 61-year-old man with established C5 AIS A tetraplegia, presented with sudden onset of diplopia and bilateral ptosis, later diagnosed as OMG, in context of other complex co-morbidities, including a cervical cord syrinx, obstructive sleep apnoea and labile blood pressure. Clinical findings were consistent with fluctuating bilateral partial third and sixth nerve palsies. Acetylcholine receptor antibodies were negative, but electromyography demonstrated muscle fatigue. The ocular signs responded well to pyridostigmine. Medications taken before diagnosis, including solifenacin for neurogenic bladder overactivity, were ceased to avoid attenuating the anti-cholinesterase effect. However, the unopposed anti-cholinesterase activity led to frequent and painful abdominal spasms, associated with uncontrolled detrusor hyperreflexia and worsening autonomic dysreflexia (AD). A trans-vesical phenol block to treat this provided only short-lasting benefit. Pyridostigmine was ceased to avoid provoking his abdominal spasms and his regular medications were recommenced. It was decided that the most appropriate treatment for his distressing diplopia was an eye patch. After discharge home, he continued to experience problems with recurrent urinary tract infections, abdominal spasms, episodic postural hypotension and AD. After 5 months, the patient died from an acute myocardial infarction. This case report contributes new knowledge about the rare presentation of OMG in a person with chronic tetraplegia.

Idiopathic Orbital Myositis in a 9-Year-Old Girl: A Case Report.

Idiopathic Orbital Myositis in a 9-Year-Old Girl: A Case Report.

Alternating IV Nerve Palsy and Ptosis as a First Sign of Childhood Ocular Myasthenia Gravis

A previously healthy 5-year-old girl was referred because of sudden-onset diplopia. Her examination demonstrated moderate right ptosis, left inferior oblique overaction and superior oblique underaction, and a positive Bielschowsky head tilt test to the left. This suggested a left fourth nerve palsy, but the right ptosis remained undefined (Figure). Ophthalmic and neurological examination and cranial magnetic resonance imaging were otherwise normal. Ten days later, a right fourth nerve palsy with a right ptosis and left adduction deficit was evident.

Sudden onset diplopia in an elderly male patient : answer

Brain diffusion-weighted imaging revealed a small hyperintense lesion involving the median dorsal midbrain (Fig. 1). Aspirin and clopidogrel were prescribed. He was discharged 8 days after onset, at which point his diplopia was slightly improved. Binocular double vision suggests the dysfunction of extraocular muscles, the neuromuscular junction, cranial nerves, cranial nerve nuclei, or internuclear and supranuclear connections [1]. Accurate localization of the underlying lesion is the first step towards the correct diagnosis. Each extraocular muscle is innervated by the corresponding cranial nerve, which originates from the individual cranial nerve nucleus. In complete oculomotor nerve palsy, there is ptosis due to the dysfunction of the levator palpebrae superioris muscle. The eye is deviated down and out, with impaired elevation, depression, and adduction. Pupil involvement usually indicates a compressive lesion, while sparing of the pupil is more likely due to ischemia [1]. The oculomotor nucleus is a paired structure that is located in the midbrain, adjacent to the medial longitudinal fasciculus (MLF) and reticular formation, ventral to the aqueduct of Sylvius and the fourth ventricle. The oculomotor nucleus lesion typically causes ipsilateral ophthalmoparesis and pupil dilatation with bilateral impairment of ocular elevation and bilateral ptosis. Isolated weakness of a single muscle is possible with an oculomotor nucleus lesion and isolated bilateral oculomotor nuclear palsy is extremely rare [2]. Classic signs of internuclear ophthalmoplegia (INO) are the impairment of adduction of the ipsilateral eye and the preservation of abduction of the contralateral eye when the patient tries to gaze in the contralateral direction [3]. It is induced by a lesion involving the MLF in the brainstem. One-and-a-half syndrome consists of lateral gaze palsy in one direction and INO in the other direction with a contralateral gaze. It occurswith lesions in the dorsal pontine tegmentum that impair both the ipsilateral paramedian pontine reticular formation or the abducens nucleus and the ipsilateralMLF.

Sudden onset diplopia in an elderly male patient question

Sudden onset diplopia in an elderly male patient question

Akute Diplopie

Sudden onset diplopia may occur secondary to something as simple as uncorrected refractive error or as complicated as brainstem disorders in conjunction with other symptoms. Therefore, all complaints of diplopia must be a cause for concern. Ophthalmologists have to determine if diplopia is the first sign of a systemic or neurological disorder, which needs to be referred to a specialist or can be managed by the practitioner. In this paper the importance of the case history, primary diagnostic options, the indications for supplementary testing with computed tomography (CT) or magnetic resonance imaging (MRI) as well as treatment options when a patient complains of sudden onset diplopia are discussed.

An Adult Case of Fisher Syndrome Subsequent to Mycoplasma pneumoniae Infection

Reported herein is an adult case of Fisher syndrome (FS) that occurred as a complication during the course of community-acquired pneumonia caused by Mycoplasma pneumoniae. A 38-yr-old man who had been treated with antibiotics for serologically proven M. pneumoniae pneumonia presented with a sudden onset of diplopia, ataxic gait, and areflexia. A thorough evaluation including brain imaging, cerebrospinal fluid examination, a nerve conduction study, and detection of serum anti-ganglioside GQ1b antibody titers led to the diagnosis of FS. Antibiotic treatment of the underlying M. pneumoniae pneumonia was maintained without additional immunomodulatory agents. A complete and spontaneous resolution of neurologic abnormalities was observed within 1 month, accompanied by resolution of lung lesions.

ABDUCENS NERVE PALSY AND THROMBOSIS OF THE CEREBRAL VEINS AND SINUSES - A DIAGNOSTIC PITFALL

Thrombosis of the cerebral veins and sinuses is an infrequent cerebrovascular disorder. Because the highly variable symptoms, recent neuroimaging plays a key role in the diagnosis. Abducens nerve palsy as a focal neurological deficit is a rare clinical manifestation in these patients. We present two cases with sudden onset of diplopia and headache. Case 1: A 3-year old girl with B cell lymphoblastic leukemia developed bilateral abducens deficit and bilateral optic disc edema after treatment including L-asparaginase. Thrombosis of the right jugular vein, sagittal and right sigmoid sinuses was visualized on magnetic resonance imaging (MRI) and magnetic resonance venography (MRV). Symptoms gradually resolved after treatment with enoxiparine and MRV demonstrated recanalization. Case 2: A 75-year old female with medical history of arterial hypertension presented with headache and sudden left abduction deficit. Computerized tomography (CT) scan was normal. MRI and MRV revealed aging brain and disruption of venous flow at the left internal jugular vein, suspecting thrombosis. Extracranial colour duplex sonography and CT angiography proved haemodinamic equivalent of left internal jugular vein thrombosis due to sclerotic pathology of aortic arch. Our first case illustrates the role of improved neuroimaging techniques as the best method for diagnosis of cerebral veins and sinuses thrombosis, presenting with abducens nerve palsy. With second case the potential neuroimaging pitfalls concerning the accurate diagnosis of these cerebrovascular disorders with neuro-ophthalmologic manifestation are discussed.

Cerebral venous sinus thrombosis presenting with diplopia in pregnancy: a case report

IntroductionCerebral venous sinus thrombosis is a rare condition. The most frequent symptoms and signs are headache, focal seizures with or without secondary generalization, unilateral or bilateral paresis and papilledema. We report a case of transverse sinus and superior sagittal sinus thrombosis that presented with diplopia in a pregnant woman.Case presentationA 34-year-old Malay woman, gravida 3 para 2 at 8 weeks of pregnancy, was admitted for hyperemesis gravidarum, presented with sudden onset of diplopia, blurring of vision and headache. A magnetic resonance scan of her brain showed the presence of cerebral edema with no space occupying lesion, but magnetic resonance venography ultimately revealed right transverse sinus and superior sagittal sinus thrombosis. The patient was treated with anticoagulation for 1 year, after which the patient recovered fully.ConclusionDue to its diverse and varied neurological presentation, cerebral venous sinus thrombosis should be considered in almost any brain syndrome.