Eculizumab in myasthenia gravis: A review.

Eculizumab, a monoclonal antibody against complement C5, is a novel therapy to treat refractory myasthenia gravis (MG). The present review was undertaken to study the role of eculizumab in MG. This includes the drug's mechanism, pharmacokinetics, clinical trial findings, tolerability, side effects, safety, dosage, administration, and cost. An English-language search for relevant items was undertaken using Embase and PubMed from 1946 to present. Clinical trial registries/databases and websites were also searched for relevant data. Keywords were eculizumab and MG. The present review found 103 articles after initial screening. Current data support eculizumab as an effective, safe, and tolerable drug in cases of refractory MG. However, its cost can prevent it from being widely accessible to a majority of the general population.

Mixed Aphasia Caused by Bilateral Cerebellar Infarcts: a Case Report.

Although neuroanatomical and physiological understanding of the cerebellum has evolved over recent decades and continues to develop, there is much that remains to be expounded upon, especially with regard to nonmotor roles. Neurocognitive and language processing is one area where involvement of the cerebellum is no longer in question, but the extent and mechanism of this relationship have yet to be defined. For example, which of the cerebellar hemispheres is involved continues to be debated. We present a case wherein a thrombus in the basilar artery led to bihemispheric cerebellar strokes with profound mixed effects on the patient's language and cognition. To the authors' knowledge, this is the first reported case of bilateral cerebellar strokes resulting in a mixed aphasia reported in scientific literature. This demonstrates the importance of continued research into a model for cerebellar function and the clinical impact of lesions to various cerebellar regions.

Fourth Nerve Palsy Due to Neurocysticercosis.

Neurocysticercosis is the most common disease of the nervous system caused by a parasite. Ophthalmological manifestations of cysticercosis typically are due to direct intraorbital or intraocular involvement, parenchymal brainstem involvement, or secondary to hydrocephalus or increased intracranial pressure. We describe a patient with a unilateral fourth nerve palsy, a rare presentation of neurocyticercosis.

Reversible Vision Loss Due to Transependymal Oedema of the Optic Apparatus Secondary to Ventriculoperitoneal Shunt Malfunction.

A 29-year-old male with a history of pilocytic astrocytoma status post-multiple ventriculoperitoneal shunt (VPS) revisions presented with vision loss, ocular motor deficits, and headaches. His shunt was revised but he experienced persistent vision loss despite resolution of his headaches and diplopia. Magnetic resonance imaging (MRI) of the head revealed hydrocephalus and T2 hyperintensity consistent with transependymal oedema surrounding the midbrain, pons, and optic apparatus. His shunt was again revised, but was complicated by an infection that required removal. He underwent an endoscopic third ventriculostomy and an external ventricular device was temporarily placed. Repeat MRI revealed marked improvement of the transependymal oedema and hydrocephalus. His vision returned to baseline and remained stable at 6-months follow-up.

Postoperative Horner Syndrome After Lung Transplantation.

BACKGROUND: Horner syndrome arises from a disruption along the oculosympathetic efferent chain and can be caused by a variety of pathological and iatrogenic etiologies. We present 3 cases of postoperative Horner syndrome after bilateral lung transplantation. METHODS: The electronic health records of 3 patients with iatrogenic Horner syndrome after lung transplantation were examined, including notes from each patient's medical history, operative and postoperative records, and ophthalmology consultation results. A literature review was performed. RESULTS: All 3 of our patients displayed anisocoria and ptosis, symptoms consistent with Horner syndrome, and the patients from Cases 1 and 2 showed reversal of anisocoria after an application of topical apraclonidine. CONCLUSIONS: Ophthalmologists should be aware of the risk of Horner syndrome after lung transplantation.

Atypical presentations of intracranial dysgerminoma mimicking central nervous system inflammatory or demyelinating disease.

OBJECTIVES: The aim of this study is to report a case series of atypical presentations of intracranial dysgerminoma in which the diagnosis was delayed due to clinical and radiographic findings initially suggestive of CNS inflammatory or demyelinating diseases, such as MS. METHODS: This study is a case series detailing the history, clinical presentations, radiographic and laboratory results, and management of three patients with biopsy-proven intracranial dysgerminoma. RESULTS: All three patients demonstrated hyperintense lesions on MRI that were more suggestive of demyelinating or inflammatory diseases, including lesions involving the midbrain and corpus callosum. All three patients were serum positive for oligoclonal bands and negative for both AFP and beta-hCG (these two markers are commonly seen in dysgerminoma cases). One case involved a steroid-responsive tumor whereas the other two cases either did not respond to steroids or steroids were withheld due to uncertainty of etiology. Following biopsy, all three results were consistent with dysgerminoma. CONCLUSION: Clinicians should be aware that dysgerminoma may mimic the clinical and radiographic presentations of demyelinating diseases such as MS. These lesions can cause acute visual loss or diplopia, have MRI and CSF findings that might mimic MS, and have been shown to respond to steroids. Atypical clinical (e.g., headache, dorsal midbrain syndrome, bilateral optic neuropathy) or atypical radiographic features (e.g., mass effect, hydrocephalus) should prompt consideration for repeat imaging and possible biopsy even if serum or CSF tumor markers (beta-hCG and AFP) are negative for dysgerminoma.

Percutaneous transorbital embolization of a carotid cavernous fistula.

This case report highlights utilization of image-guided, percutaneous transorbital direct cavernous sinus puncture to embolize an anteriorly draining carotid cavernous fistula (CCF) when conventional transarterial and transvenous approaches were not feasible. An 86-year-old man with a known posterior draining CCF developed acute unilateral proptosis, pain, and vision loss ("red-eyed shunt"). Cerebral angiogram revealed the dural CCF to be draining anteriorly into partially thrombosed ophthalmic veins. After failed transarterial and transvenous attempts, a percutaneous transorbital approach was used to successfully embolize the fistula using the Onyx Liquid Embolic System according to the visual needle path generated by the Seimens Syngo iGuide. To our knowledge, this is the first reported case of percutaneous transorbital direct embolization of a CCF utilizing the Seimens Syngo iGuide.

Petroclival Meningioma Presenting With an Ipsilateral Sixth Nerve Palsy and a Contralateral Homonymous Quadrantanopia: A Unique and Topographically Localizing Syndrome.

An 84-year-old woman reported onset of headaches, diplopia, and blurred vision. On examination, she was found to have a left sixth nerve palsy and an incongruous right homonymous hemianopia. Brain MRI demonstrated a left petroclival meningioma, causing this unusual combination of clinical findings. The patient was treated with radiation therapy and has remained stable over 4 years of follow-up.

Not the usual suspect.

An 82-year-old woman presented with right-sided proptosis, chemosis, and a supraorbital bruit. A dural carotid-cavernous fistula was suspected, but catheter angiography revealed an intraorbital inferior ophthalmic vein arteriovenous fistula supplied by the right ophthalmic artery and infraorbital branch of the internal maxillary artery. A primary orbital arteriovenous fistula can mimic the clinical and radiographic features of the more common carotid-cavernous fistula.

Neuro-Ophthalmic Sarcoidosis.

Background: In the absence of confirmatory biopsy, the criteria for diagnosis of neuro-ophthalmic sarcoidosis are not well established. Diagnostic criteria for both intraocular sarcoidosis and neurosarcoidosis have been proposed, but the diagnosis of neuro-ophthalmic sarcoidosis remains challenging. It is our intention to augment what is currently known about the diagnosis of neuro-ophthalmic sarcoidosis by providing a series of biopsy-proven cases that contribute to the continued development of diagnostic criteria for this enigmatic condition. Methods: Case series of four Caucasian women with biopsy-proven neuro-ophthalmic sarcoidosis. Results: The first patient was initially diagnosed with traumatic optic neuropathy following a fall. Years later, the presence of pathologic submandibular lymphadenopathy was identified and biopsied, revealing non-caseating granulomas. The second and third cases involved sarcoidosis of the extraocular muscles without clear or common systemic features of sarcoidosis. In the fourth and final case, the patient presented with a Horner syndrome attributed to sarcoid infiltration of the ipsilateral sympathetic chain. Bronchoscopy with biopsy showed non-caseating granulomas consistent with sarcoidosis. Conclusions: We describe four cases of neuro-ophthalmic sarcoidosis and propose possible neuro-orbital and neuro-ophthalmic criteria both with and without diagnostic biopsy.

Third Nerve Palsy as the Presenting Sign of Metastatic Endometrial Carcinoma with Lymphovascular Space Invasion.

We present a case of endometrial carcinoma (EC) presenting with a third cranial nerve palsy due to metastasis to the cavernous sinus. Although the tumour was only stage 1B, histopathologic features including higher grade and lymphovascular space invasion (LVSI) increase the risk of recurrent and metastatic disease. To our knowledge, this is the first case of EC presenting as a third cranial nerve palsy in the English-language ophthalmic literature. Clinicians should be aware that the risk of metastatic EC including neuro-ophthalmic presentations depends upon not only stage and grade but also the presence of unique histopathological findings such as LVSI.