Case Report: Recurrent Transient Monocular Vision Loss Secondary to Protein C Deficiency.

SIGNIFICANCE: Protein C deficiency is a thrombophilic condition that increases the risk of venous and arterial thrombi, the latter of which can cause transient monocular vision loss. In cases of recurrent transient monocular vision loss, in which the typical stroke workup has been unrevealing, investigation for hypercoagulable states is warranted. PURPOSE: This study reports a case of transient monocular vision loss secondary to protein C deficiency in a patient with no known personal or family history of venous thromboembolism and highlights the eye care provider's role in helping with diagnosis of this condition. CASE REPORT: A 59-year-old woman presented with recurrent transient monocular vision loss of the right eye. Her history was remarkable for suffering an ischemic stroke with hemorrhagic conversion shortly after experiencing episodes of transient monocular vision loss. These episodes initially waned but recurred 3 months later. Extensive workup at the time of recurrence of her visual symptoms was unrevealing. Given the timing of her visual symptoms and history of stroke, her presentation was suggestive of transient ischemic attacks. Her previous extensive workup and chronicity of symptoms did not necessitate emergent evaluation. However, additional workup for hypercoagulable conditions was initiated. The testing revealed protein C deficiency, which prompted initiation of oral anticoagulants for stroke prophylaxis. CONCLUSIONS: Transient monocular vision loss is a symptom commonly encountered by eye care providers, which necessitates emergent evaluation to reduce stroke risk if the symptom appears vascular in origin. Testing for hypercoagulable conditions is indicated in patients demonstrating recurrent transient monocular vision loss, even if there is no known personal or family history of venous thromboembolism. Eye care providers need to be aware of this association between hypercoagulable conditions and transient vision loss to aid in prompt diagnosis and treatment with the goal of preventing stroke and permanent vision loss.

Topical Review: An Update of Diagnostic and Management Algorithms for Acquired Blepharoptosis.

SIGNIFICANCE: Acquired ptosis is a condition of the upper eyelid that has negative cosmetic and functional effects but is likely underdiagnosed and undertreated. Given the evolving understanding of the condition and expanding therapeutic options, this review reappraised published evidence and clinical experience regarding diagnosis and treatment of acquired ptosis.The authors met over two structured virtual working sessions to review current evidence and develop timely recommendations for acquired ptosis identification, differential diagnosis, characterization, and treatment selection. Diagnostic algorithms, plus management and referral guidelines, are presented. Eyelid evaluation and, when needed, ptosis diagnostic workup are essential in the comprehensive eye examination. Acquired ptosis can be efficiently identified via patient questionnaire, history, and photograph review combined with assessment of eyelid position and symmetry using established methods. When ptosis is present, it is essential to evaluate onset, symptoms, pupil diameter, and extraocular muscle function to identify or rule out serious underlying conditions. If signs of serious underlying etiology are present, immediate referral/follow-up testing is required. After ruling out serious underlying causes, masquerade conditions, and pseudoptosis, pharmacologic or surgical treatment should be selected based on the clinical evidence. Effectively managing acquired ptosis requires practice-wide commitment to thorough eyelid evaluation, accurate diagnosis, and adoption of new treatment modalities. Aided by evolving pharmacologic therapeutic options, shifting from a "detect and refer" to a "diagnose and manage" approach can support identification and treatment of more patients with acquired ptosis, particularly mild-to-moderate cases.

Case Report: Bilateral Cranial Nerve VI Palsy in Miller Fisher Syndrome.

SIGNIFICANCE: Miller Fisher syndrome, a variant of Guillain-Barré syndrome, is a condition characterized by ophthalmoplegia, ataxia, and areflexia. Diplopia, particularly secondary to a bilateral abduction deficit, is the most common presenting symptom. The telltale neurologic symptoms associated with this condition can easily be overlooked by eye care providers, delaying timely diagnosis and treatment. PURPOSE: This study aimed to report a case of diplopia secondary to an uncommon condition (Miller Fisher syndrome) and to highlight the eye care provider's role in helping with diagnosis and management of this condition. CASE REPORT: A 31-year-old woman presented to the emergency eye care service because of a 2-day history of sudden-onset diplopia, for which no cause was found 1 day prior at a local hospital emergency department. She also reported weakness in her legs, difficulty walking, balance problems, and reduced sensation of her left hand for the past 2 days. Clinical testing revealed bilateral abduction deficits, ataxia, and areflexia, the combination of which suggested Miller Fisher syndrome. Because of the acute onset and progressive severity of her neurologic symptoms, she was referred to a different hospital emergency department for confirmatory diagnosis and treatment of Miller Fisher syndrome. CONCLUSIONS: Diplopia is a symptom commonly encountered by eye care providers, regardless of their mode of practice. Although there are many potential etiologies of diplopia, performing a comprehensive eye examination combined with a neurologic evaluation can potentially pinpoint the specific cause. Miller Fisher syndrome is one such condition in which the diagnostic triad can be uncovered with in-office ocular motility testing and neurologic examination. Eye care providers need to be aware of the clinical features of Miller Fisher syndrome to aid in prompt diagnosis and treatment for patients with this acute condition.

Case Report: Transient Dorsal Midbrain Syndrome as the Initial Presentation of Multiple Sclerosis.

SIGNIFICANCE: Prompt neuroimaging is important to identify multiple sclerosis lesions in the appropriate clinical setting. However, despite a normal brain MRI finding, a diagnosis of multiple sclerosis still must be considered in cases of dorsal midbrain syndrome, even if it is transient. PURPOSE: The purpose of this case report is to present a patient with a transient presentation of dorsal midbrain syndrome, resolving within 1 week of initial symptoms, which was ultimately attributed to multiple sclerosis in the setting of a normal enhanced brain MRI study. CASE REPORT: A 33-year-old man with new-onset visual complaints was found to have upgaze paresis, eyelid retraction, and pupillary light-near dissociation suggestive of dorsal midbrain syndrome. Within days, enhanced brain MRI was completed and showed a normal finding, and the clinical features of dorsal midbrain syndrome had resolved. Subsequent spine imaging and lumbar puncture lead to an ultimate diagnosis of multiple sclerosis. CONCLUSIONS: There have been a few reported cases of dorsal midbrain syndrome as the presenting feature of multiple sclerosis. This case is unique because it reports a transient presentation of dorsal midbrain syndrome, documented to have resolved only days after initial presentation, which was ultimately attributed to multiple sclerosis.

Progressive visual and hearing loss secondary to neurosyphilis.

PURPOSE: There is a rising incidence of syphilis in economically advanced countries. Early diagnosis can spare the patient devastating vision and hearing loss, as well as other significant morbidity. Ocular presentations of neurosyphilis are varied and numerous, requiring syphilis to be considered in many clinical situations, even if a negative medical history is reported. Features of Argyll Robertson pupils, however, are virtually pathognomonic of syphilis; therefore, careful pupil testing can be the key to making a correct and timely diagnosis. CASE REPORT: A 51-year-old woman with a history of unexplained hearing loss presented for a third opinion for progressive vision loss OU, which was previously labeled as functional loss. A review of records indicated significant worsening of visual acuity and visual fields over 6 months, with minimal remaining visual function. She exhibited features of optic neuropathy as well as 2 mm miotic pupils, with evident light-near dissociation bilaterally. Evaluation showed a reactive fluorescent treponemal antibody absorption and rapid plasma reagin (1:8 titer) and positive cerebral spinal fluid venereal disease research laboratory test. A diagnosis of neurosyphilis was made, and a 2-week course of intravenous penicillin treatment was completed. Despite treatment, her vision decreased to no light perception OU owing to the advanced course of the disease at the time of treatment. CONCLUSIONS: Neurosyphilis can occur at any stage of infection and needs to be considered in the differential diagnosis for many visual and ocular problems, especially in the setting of other systemic signs and symptoms. In this case, the combination of light-near dissociation and optic neuropathy with hearing loss was instrumental in dismissing the past diagnosis of functional vision loss and in pursuing the diagnosis of neurosyphilis. A timely diagnosis of neurosyphilis needs to be made to prevent devastating vision loss as seen in this case.

Anterior temporal chordoid meningioma causing compressive optic neuropathy.

PURPOSE: Optic neuropathy associated with proptosis and ocular motility restrictions is suggestive of a compressive etiology. This raises concern for a mass lesion involving the orbit or orbital apex. Meningiomas, which account for 4% of all intraorbital tumors and 20 to 30% of all intracranial tumors, are capable of compressing critical anatomic structures within the orbit or orbital apex, and thereby impairing both afferent and efferent visual function. The purpose of this case report is to present a case of chordoid meningioma associated with compressive optic neuropathy. METHODS: A 51-year-old woman presented with clinicopathological features pathognomonic of compressive optic neuropathy and orbital apex syndrome. Radiologic and neuropathologic evaluation revealed a large right anterior temporal atypical meningioma with chordoid features associated with frontal intraparenchymal edema. The therapeutic intervention consisted of orbitozygomatic craniotomy and radiotherapy. RESULTS: Postoperatively, the patient progressed very well with considerable improvement of her visual and oculomotor function. The MIB-1 labeling index of 5 to 7% calls for close observation because of an increased probability of recurrence. CONCLUSIONS: This case highlights the importance of prompt diagnosis and treatment, because chordoid meningiomas exhibit a more aggressive biological behavior, with the potential to cause significant morbidity because of mass effect and higher risk of recurrence. To our knowledge, this is the first report of a rare case of a chordoid meningioma in the anterior temporal lobe that presented as a compressive optic neuropathy. The details of this case are presented with a review of relevant literature.

Progressive sixth nerve palsy secondary to intracavernous arachnoid cyst and complicated by contralateral optic nerve sheath meningioma.

INTRODUCTION: Optic nerve sheath meningiomas and intracavernous arachnoid cysts are both fairly rare conditions, and to the best of our knowledge, have not been previously reported to co-occur in the same patient. Both can cause diplopia, but only ONSMs have been documented to demonstrate progressive worsening of ocular motility. CASE REPORT: A 67-year-old woman with blur and diplopia demonstrated a right optic neuropathy and limited ductions bilaterally. Neuroimaging revealed a right optic nerve sheath meningioma and left intracavernous arachnoid cyst. She was conservatively managed with neurosurgical surveillance for 1.5 years, until her diplopia worsened. Ocular motility re-evaluation demonstrated a worsening left abduction deficit, suggesting interval change of the intracavernous cyst, rather than the meningioma. CONCLUSION: There are only a few reported cases of cranial nerve VI palsy secondary to a cavernous sinus arachnoid cyst. However, this is the first reported case in a patient with a concurrent optic nerve sheath meningioma, and the first case demonstrating progressive worsening of a sixth cranial nerve palsy from an intracavernous arachnoid cyst. Determining which comorbidity caused worsening of symptoms played a critical role in the management of this patient.

Pupil-sparing third cranial nerve palsy with aberrant regeneration secondary to cavernous sinus arachnoid cyst.

A 66 year-old woman presented with a pupil-sparing partial third cranial nerve palsy with aberrant regeneration. Imaging revealed a cystic lesion in the right cavernous sinus, demonstrating signal characteristics consistent with arachnoid cyst. Oculomotor synkinesis and a pupil-sparing third cranial nerve palsy from an arachnoid cyst of the cavernous sinus have not been previously reported. Intracavernous arachnoid cysts are in the differential diagnosis of cranial nerve III palsies, with and without synkinesis or pupil involvement.

The influence of non-modifiable and modifiable factors on cardiac biomarkers after marathon running.

BACKGROUND: This study investigated the influence of modifiable (training) and non-modifiable factors (age and gender) on cardiac troponin I (cTnI) and B-type natriuretic peptide (BNP) levels post-marathon. METHODS: Thirteen female and nine male recreational runners participated in the 2015 Hartford Marathon. A venous blood draw was taken from each subject at 24 hours pre-race, immediately post-race and 24 hours post-race. RESULTS: Weekly mileage and weekly long runs were recorded for a 12-week period prior to the marathon. No association was found between age and BNP (P=0.11, P=0.50) or cTnI (P=0.69, P=0.28) for either post-race time points. No association was found between gender and cTnI for either post-race time points (P=0.09, P=0.57). However, BNP elevation, at 24 hours post-race was more pronounced in females than males (P=0.047). For cTnI levels immediately post-race, a negative association was found for average weekly mileage (P=0.006), while a positive association was found for the number of long runs exceeding 20 miles (P=0.05). No association between training and BNP were found. CONCLUSIONS: These results suggest that female runners may experience greater cardiac stress than males. In addition, runners with greater weekly training mileage experienced less cardiac stress post-race, while runners who ran too many 20+ mile long runs, experienced more cardiac stress post-marathon.

Prostate cancer metastasis to clivus causing cranial nerve VI palsy.

BACKGROUND: An abduction deficit can have many potential etiologies. Clinical testing can help distinguish a neurogenic from a restrictive process. For any patient with a current or past history of cancer, even in the setting of vasculopathic risk factors, a further workup is necessary to rule out a metastatic process. CASE REPORT: A 66-year-old man reported sudden blurry vision but did not describe a definite diplopia. Clinical evaluation found left cranial nerve (CN) VI palsy. Although he did have vasculopathic risk factors, neuroimaging found prostate cancer metastasis to the mid to left clivus, extending to the left cavernous sinus region as well as a smaller metastasis to the left temporal lobe. The patient underwent radiation treatment with improvement in his clinical presentation and symptoms. His prostate cancer was subsequently treated more aggressively, and 2 years later, despite spinal metastases, he was doing relatively well. CONCLUSION: Prostate cancer commonly metastasizes, with a high propensity to invade bone. CN VI runs along the midline-structured bony clivus, between the pons and the cavernous sinuses. Therefore, a metastatic lesion to the clivus can be responsible for unilateral or bilateral CN VI palsy. In men, a common primary site of cancer metastasis to the clivus is the prostate. Eye doctors must look closely for evidence of even subtle abduction deficits in all patients with a history of prostate cancer. Early detection can lead to improved medical treatment and extended life expectancy.

Fronto-ethmoid sinus mucocele: a case report.

BACKGROUND: Mucoceles are epithelium-lined cavities in the paranasal sinuses filled with mucus. They develop because of scarring and obstruction of the sinus ostium, whether from chronic sinusitis, trauma, or surgery. They commonly erode the bony sinus wall and can have serious complications of brain and orbital invasion, with potential for abscess and rupture. CASE REPORT: A 39-year-old woman had diplopia in left gaze and a hard nodule above the right eye for 1 year. She recently noted a bulging right eye, nasal congestion, and occasional headaches. She was 9 years post-sinus surgery. All aspects of the afferent optic nerve function were intact. Right-sided proptosis was evident as was a right adduction and supraduction deficit, prompting immediate imaging, both with a computed tomography scan and magnetic resonance imaging of the orbits. These results showed a large right ethmoid sinus mucocele, with bony erosion and orbital invasion, prompting an immediate surgical referral. CONCLUSION: Mucoceles may arise from any of the paranasal sinuses and, because of the close proximity of these spaces to the orbit, may initially manifest with visual and ocular signs and symptoms. Therefore, eye care providers need to be aware of this entity and the need for immediate referral or workup if a mucocele is suspected.

Giant cell arteritis.

Giant cell arteritis has been considered an enigmatic disease. It is characterised by chronic granulomatous inflammation of the walls of large and medium-sized arteries. The process has a predilection for the extradural cranial arteries, which include the ophthalmic and the posterior ciliary arteries. It is a multi-symptom disease of older individuals and patients often present with challenging issues and diagnostic dilemmas. We review the literature and latest protocols for the diagnosis and management of giant cell arteritis.

Ocular manifestations uncover chronic subdural hematoma secondary to abuse.

BACKGROUND: An abduction deficit or cranial nerve VI palsy can have many potential etiologies. However, a cranial nerve VI palsy in the setting of disc edema suggests increased intracranial pressure, requiring emergent work-up. CASE REPORT: An 18-year-old woman presented with significant headaches and horizontal diplopia, which she claimed began after giving birth 3 months prior. Her examination found a right cranial nerve VI palsy and mild disc edema of the left eye. This combination of findings was suggestive of increased intracranial pressure. Emergent workup uncovered a large chronic subdural hematoma requiring immediate neurosurgical intervention. She ultimately admitted to being abused by her partner. CONCLUSION: Diplopia and disc edema in a young, nonoverweight patient with an altered mental state is very concerning. Meningitis and complications of spinal anesthesia, although rare, need to be considered in patients who have recently given birth. However, other causes of increased intracranial pressure, including subdural hemorrhage secondary to trauma, also have to be considered, even if the patient denies any history of trauma. Abused patients often deny trauma.