Optic atrophy secondary to minocycline-induced idiopathic intracranial hypertension.

An early adolescent female presented with blurry vision, ocular 'fullness', pulsatile tinnitus and gait difficulty due to poor vision. She was found to have florid grade V papilloedema, 2 months after the use of minocycline for the treatment of confluent and reticulated papillomatosis for 2 months. MRI of the brain without contrast showed fullness of the optic nerve heads concerning for increased intracranial pressure, which was confirmed on lumbar puncture with an opening pressure greater than 55 cm H2O. She was initially started on acetazolamide, but due to high opening pressure and severity of visual loss, a lumboperitoneal shunt was placed in 3 days. This was complicated by a shunt tubal migration 4 months later, leading to worsening vision of 20/400 in both eyes for which she underwent shunt revision. By the time she presented to the neuro-ophthalmology clinic, she was legally blind with her exam consistent with bilateral optic atrophy.

Genomic sequencing of a pregnancy associated symptomatic meningioma of the diaphragma sellae: a case report.

Pregnancy-associated meningiomas have unique considerations and features regarding their pathophysiology, location, genetic profile, and neurosurgical management. These tumours have been reported to undergo rapid growth during gestation and regression post-partum, implicating a role for female sex hormones in tumour physiology. In addition, these tumours occur at a higher incidence in the skull base compared to sporadic meningiomas in the general population, often impinging neurovascular structures and requiring emergent resection. While the genomics of sporadic meningiomas have been described, there are no reports characterizing the genetic features of those associated with pregnancy. Here we describe a patient diagnosed with a diphragma sellae meningioma early in the third trimester after presenting with rapidly deteriorating vision. At 32 weeks gestation the baby was delivered by caesarean section and the tumour subsequently removed. Genomic profiling of the tumour sample revealed variants of unknown significant (VUS) in six genes, none of which were in canonical meningioma drivers. We describe our surgical approach and discuss the relevant pathology and genomics, as well as medical and surgical management considerations of meningiomas in pregnancy.

Not All Cases of Visual Snows are Benign: Mimics of Visual Snow Syndrome.

Visual snow syndrome (VSS) is a clinical disorder characterized by pan-field visual disturbance. It is a diagnosis of exclusion since its pathophysiology remains unknown. Excluding other mimics is of great significance since some serious pathologies can have secondary visual snow (VS) as an initial presentation. Delayed or incorrect diagnosis of these VSS mimics may lead to permanent vision loss or even death. The purpose of this review is to help physicians distinguish VSS mimics promptly to avoid bad outcomes. The authors performed a PubMed literature search of articles, case reports, and reviews describing VS symptoms in patients with underlying diseases other than VSS. The red flags of secondary VS symptoms were highlighted, such as new-onset or intermittent VS, unilateral or quadrant VS, and accompanied ocular or neurological deficits. There are four main categories of VSS mimics, ie, including neurological disorders, ocular pathologies, drug-related VS, and other systemic diseases. The physicians could largely exclude most etiologies based on history taking, ophthalmologic and neurologic examinations, and neuroimaging. Further research in VS should carefully define and unify the inclusion and exclusion criteria of this disorder and investigate these secondary VS conditions and their pathogenesis.

A tearfully painful darkness.

A 70-year-old woman presented with new onset of left eye and facial pain. Ophthalmic and neurological examinations, magnetic resonance imaging brain, erythrocyte sedimentation rate, and C-reactive protein were unrevealing. A few days later, she developed vision loss in her left eye. Examination revealed decreased visual acuity with a relative afferent pupillary defect in the left eye and a diffuse mild swelling of the left optic nerve head. Repeat magnetic resonance imaging showed T2 hyperintensity and enhancement of the intraorbital optic nerve and surrounding tissues with no other intracranial abnormalities. Serum studies showed elevated myelin oligodendrocyte glycoprotein IgG titer. She was treated with IV methylprednisolone 1000 mg daily for 3 days and was discharged on prolonged prednisone taper with return of vision to baseline.

Reversal of Iris Heterochromia in Adult-Onset Acquired Horner Syndrome.

ABSTRACT: Iris heterochromia is typically seen in association with congenital Horner syndrome. A man in his 40s with congenital iris heterochromia, blue in the right and brown in the left, presented with left-sided Horner syndrome. This was associated with recent change in color of his brown left iris to blue similar to the right iris. This case demonstrates a unique case of adult-onset Horner syndrome with reversal of iris heterochromia.

MRI findings as markers of idiopathic intracranial hypertension.

PURPOSE OF REVIEW: Negative findings on neuroimaging are part of the diagnostic criteria for idiopathic intracranial hypertension (IIH), a syndrome characterized by increased intracranial pressure (ICP). Some positive neuroimaging findings are associated with increased ICP, but their role in diagnosis of IIH has not been established. We provide an overview of these findings and their relevance for diagnosis of raised intracranial pressure. RECENT FINDINGS: MRI acquisition techniques have significantly improved in the last few decades leading to better characterization of the intracranial changes associated with IIH, including empty sella turcica, optic nerve tortuosity, distension of the optic nerve sheath, posterior globe flattening, slit-like ventricles, and venous sinus stenosis. These may be MRI biomarkers of increased ICP. Prevalence difference between people with and without increased ICP, and reversibility of these MRI findings following treatment of increased ICP inform evaluation of their diagnostic potential. SUMMARY: MRI and magnetic resonance venography findings are important tools in the diagnosis of IIH. Empty sella turcica, optic nerve protrusion, distension of the optic nerve sheath, optic nerve tortuosity, posterior globe flattening, and transverse sinus stenosis have been found to be the most promising diagnostic markers for IIH, although absence of these findings does not rule out the diagnosis.

Dural venous sinus stenting for idiopathic intracranial hypertension: An updated review.

BACKGROUND: Dural venous sinus stenting (DVSS) is an accepted treatment option in selected patients with medically refractory idiopathic intracranial hypertension and obstructive venous outflow physiology prior to cerebrospinal flow diversion (CSFD) surgery. There are no randomized controlled studies focusing on outcomes and complication rates for dural venous sinus stenting. PURPOSE: We present the largest comprehensive meta-analysis on DVSS for idiopathic intracranial hypertension (IIH) focusing on success rates, complications, and re-stenting rates to date. We also present a simplified approach to direct retrograde internal jugular vein (IJ) access for DVSS that allows for expedited procedures. MATERIALS AND METHODS: We performed a retrospective electronic PubMed query of all peer-reviewed articles in the last 15 years between 2003 to 2018. We included all patients who underwent dural venous sinus stenting for a medically refractive IIH and excluded articles without sufficient data on outcomes, complication rates and re-stenting rates. We also evaluated and compared outcomes in patients undergoing direct retrograde IJ access DVSS to traditional transfemoral vein access. RESULTS: A total of 29 papers and 410 patients who underwent DVSS met criteria for inclusion. DVSS was associated with high technical success [99.5%], low rates of repeated procedure [10%], and low major complication rates [1.5%]. CONCLUSION: Our retrospective comprehensive review of DVSS for medically refractory IIH suggests that stenting in appropriately chosen patients is associated with low complication rates, high technical success, and low repeat procedure rates.

Opinion and Special Articles: Amateur fundus photography with various new devices: Our experience as neurology residents.

Times are changing in the way we secure and share patient fundus photographs to enhance our diagnostic skills in neurology. At the recent American Academy of Neurology meeting, the use of a fundus camera and smartphones to secure good-quality fundus photographs of patients presenting with headache to the emergency department (ED) was presented. We were enthusiastic to replicate the success of the Fundus Photography vs Ophthalmoscopy Trial Outcomes in the Emergency Department (FOTO-ED) study in our neurology department, but encountered problems in terms of cost, setup, feasibility, and portability of the device. As neurology residents, we came up with 3 easier options. We present these 3 options as our personal experience, and hope to reignite enthusiasm among neurology trainees to find their own means of performing ophthalmoscopy routinely in the hospital, as it appears that the Internet market is now thriving with many other devices to make this examination easier and more rewarding. Of the options explored above, the Handheld Fundus Camera was a clear favorite among the residents, and we have placed one in our call room for routine use. It travels to the clinic, floor, intensive care unit, and ED when needed. It has enhanced the way we approach the fundus examination and been a fun skill to acquire. We look forward to further advances that will make it possible to carry such a device in a physician's pocket.

Indirect carotid cavernous fistula mimicking ocular myasthenia.

71-year-old woman with progressive left-sided, monocular diplopia and ptosis. Her symptoms mimicked ocular myasthenia, but she had an indirect carotid cavernous fistula (CCF). She was diagnosed with monocular myasthenia gravis (negative acetylcholinesterase antibody) after a positive ice test and started on Mestinon and underwent a thymectomy complicated by a brachial plexus injury. Months later, she developed left-sided proptosis and ocular bruit. She was urgently referred to neuro-interventional surgery and was diagnosed with an indirect high-flow left CCF, which was treated with Onyx liquid and platinum coil embolisation. Mestinon was discontinued. Her ophthalmic symptoms resolved. However, she was left with a residual left arm and hand hemiparesis and dysmetria secondary to a brachial plexus injury. Indirect CCF usually can present with subtle and progressive symptoms leading to delayed diagnosis or misdiagnosis. It is important for ophthalmologists to consider this differential in a patient with progressive ocular symptoms.

Dural venous sinus stenting for medically and surgically refractory idiopathic intracranial hypertension.

Background Idiopathic intracranial hypertension (IIH) is a syndrome defined by elevated intracranial hypertension without radiographic evidence of a mass lesion in the brain. Dural venous sinus stenosis has been increasingly recognized as a treatable cause, and dural venous sinus stenting (DVSS) is increasingly performed. Methods A 5 year single-center retrospective analysis of consecutive patients undergoing DVSS for medically refractory IIH. Results There were 43 patients with a mean imaging follow-up of 6.5 months and a mean clinical follow-up period of 13.5 months. DVSS was performed as the first procedure for medically refractory IIH in 81.4% of patients, whereas 18.6% of patients included had previously had a surgical procedure (ventriculoperitoneal (VP) shunt or optic nerve sheath fenestration (ONSF)). Headache was present in all patients and after DVSS improved or remained stable in 69.2% and 30.8%, respectively. Visual acuity changes and visual field changes were present in 88.4% and 37.2% of patients, respectively. Visual field improved or remained unchanged in 92%, but worsened in 8% after stenting. There was a stent patency rate of 81.8%, with an 18.2% re-stenosis rate. Of the 43 procedures performed, there was a 100% technical success rate with zero major or minor complications. Conclusion Based on this single-center retrospective analysis, DVSS can be performed with high technical success and low complication rates. A majority of patients presented primarily with headache, and these patients had excellent symptom relief with DVSS alone. Patients presenting with visual symptoms had lower success rates, and this population, if stented, should be carefully followed for progression of symptoms.

Vessel perforation during withdrawal of Trevo ProVue stent retriever during mechanical thrombectomy for acute ischemic stroke.

The authors report a case of an intracranial extravasation during the withdrawal of a Trevo ProVue stent retriever device in a patient being treated for acute ischemic stroke. An 82-year-old woman developed sudden left hemiparesis and aphasia during an urgent cardiac catheterization procedure for a non-ST elevation myocardial infarction. She had a baseline National Institutes of Health Stroke Scale (NIHSS) score of at least 10 and no improvement with intravenous administration of tissue plasminogen activator (tPA). Cerebral angiography was performed with conscious sedation, confirming an occlusion of the superior division of the right middle cerebral artery (MCA). Using standard technique, a Trevo thrombectomy device was deployed across the clot. Post-thrombectomy control angiography demonstrated complete revascularization of the right MCA. The device was then gently withdrawn without difficulty. Immediately afterward the patient's blood pressure showed a sudden and significant elevation. Immediate posttreatment angiography demonstrated active extravasation from the posterior wall of the communicating segment of the right internal carotid artery. Rapid cessation of bleeding was achieved with intravenous administration of protamine and induced hypotension. Immediate neurological assessment was performed, which showed motor improvement. An immediate postintervention CT scan confirmed a moderate-sized subarachnoid hemorrhage and contrast in the prepontine cistern. The patient was discharged home on postoperative Day 3 with an NIHSS score of zero. At 6-month follow-up in the neurointerventional clinic, her NIHSS and modified Rankin Scale scores were both zero. Endovascular stent retriever devices are increasingly being used as first-line thrombectomy devices in acute embolic strokes. A unique and previously undescribed complication is vessel perforation during withdrawal of a stent retriever. Conservative treatment options and reversal of anticoagulation should be the first line of treatment for such complications. In the authors' case, performing the procedure without anesthesia was helpful in assessing the patient's neurological status.