Anti-Ma2 Antibody-Mediated Paraneoplastic Cerebellar Degeneration and Myeloneuropathy Secondary to Lymphoma.

ABSTRACT: A 61-year-old woman with a history of untreated low-grade B-cell lymphoma presented with blurry vision, unsteadiness, and worsening pain on touching skin of the upper trunk was enrolled. Blurry vision was attributed to oscillopsia from downbeat nystagmus, which later evolved into macrosaccadic oscillations. MRI brain and spine showed mild, longitudinally extensive T2 hyperintensity in the central gray matter of the spinal cord extending from the medulla to T11 level. Serum paraneoplastic panel was negative; however, she had very high titers of anti-Ma2 antibodies in cerebrospinal fluid. The diagnosis of paraneoplastic neurological syndrome was made. Empiric treatment with high dose of intravenous steroids followed by intravenous immunoglobulin infusions did not improve her symptoms. An extensive search for an underlying tumor commenced and was initially unrevealing. However, two-month follow-up positron emission tomography scan showed increased uptake in a right pulmonary nodule, which when biopsied confirmed diagnosis of extranodal marginal zone lymphoma. The final diagnosis was anti-Ma2 antibody-mediated paraneoplastic cerebellar degeneration and myeloneuropathy secondary to lymphoma.

Poor Visual Outcome After First Attack in a Cohort of Patients With Myelin Oligodendrocyte Glycoprotein-Related Optic Neuritis.

BACKGROUND: Myelin oligodendrocyte glycoprotein-associated optic neuritis (MOG-ON) is typically a highly treatable condition that responds quickly to treatment with high doses of corticosteroids. We reviewed the cohort of patients with MOG-ON to identify patients who had poor visual outcome after the first attack despite rapid initiation of treatment. METHODS: Records of all patients diagnosed with MOG-ON seen in a tertiary neuro-ophthalmology practice were reviewed to identify and describe those with poor visual recovery (final visual acuity of 20/200 or worse in at least one eye) after the first attack despite initiation of treatment within 1 week of symptoms onset. RESULTS: Two patients of 36 fulfilled inclusion criteria: both had bilateral severe optic neuritis at presentation, and both were seen within 7 days of symptoms onset and treated immediately with pulse doses of intravenous corticosteroids followed by very-slow oral taper. Plasma exchange (PLEX) was performed 2 weeks after symptoms onset in both patients because of poor response to steroids, followed by monthly intravenous immunoglobulin infusions. Despite the use of all available treatment modalities, final visual outcome was poor in both patients. In both patients, there was enhancement of intracanalicular portion of optic nerve in the worse-seeing eye. CONCLUSIONS: In this cohort of patients with MOG-ON, 2 (5.6%) had very poor visual outcome after the first attack despite immediate initiation of treatment with pulse doses of corticosteroids and subsequent treatment with PLEX. These cases highlight that despite immediate initiation and escalation of treatment, some patients with MOG-ON can have very poor visual outcomes after the initial attack that may be because of the involvement of the intracanalicular portion of the optic nerves.

Treatment of Cancer-Associated Retinopathy: A Systematic Literature Review.

TOPIC: Systematic literature review of treatment efficacy of previously used protocols in treating patients with proven cancer-associated retinopathy (CAR). CLINICAL RELEVANCE: There is no universally accepted treatment algorithm for CAR and visual prognosis is very poor. We describe a patient with CAR with dramatic improvement in vision after treatment with high doses of corticosteroids followed by plasma exchange (PLEX) and present results of a systematic literature review of treatment efficacy of previously used protocols in treating patients with proven CAR. METHODS: We describe a 70-year-old man with CAR who demonstrated dramatic improvement in vision after treatment with high doses of systemic corticosteroids followed by 7 sessions of PLEX. We then report the results of a systematic review of all previously published English literature discussing visual outcomes of various treatment regimens used for patients with antibody-proven CAR. RESULTS: The index patient is a rare case of CAR with sustained significant improvement in vision after treatment with high doses of corticosteroids followed by PLEX. The systematic review identified 28 antibody-proven cases of CAR, 27 of which were treated with steroids, which resulted in varying degrees of improvement in visual acuity in 59% (16 of 27). The time from symptom onset to initiation of treatment and the dose of steroids did not influence the visual outcome. Three patients were also treated with PLEX in addition to steroids, and 2 of 3 patients demonstrated improvement in vision; however, there was no difference in visual outcome in patients treated with steroids only versus those treated with steroids + PLEX. CONCLUSION: Treatment with steroids or steroids + PLEX resulted in some improvements in visual acuity in 59% of patients. Removal of antirecoverin antibodies with PLEX can arrest the immune attack on the photoreceptors and potentially improve visual function; thus, it should be considered in addition to steroids. Further studies with larger cohorts are needed to establish a treatment protocol and further determine the effectiveness of the different approaches. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Characteristics of 110 Patients With Functional Visual Loss.

PURPOSE: Functional visual loss (FVL) is characterized by complaints of visual impairment without evidence of an organic cause. Physicians are often reluctant to diagnose FVL; thus, little is known about health care utilization in patients with FVL. DESIGN: Retrospective case series. METHODS: A total of 110 patients seen at 2 university-affiliated neuro-ophthalmology practices who were diagnosed with FVL were included. Medical records were evaluated, and data were collected on demographics, clinical presentation, ophthalmologic examination, neuroimaging, ancillary tests, and other health care provider visits and treatments. RESULTS: More than 70% of patients with FVL were women with a mean age of 37 ± 15 years. The presenting complaint in 71.8% (79/110) of participants was decreased vision, which was bilateral in >50% of cases. Close to half (53/110) endorsed at least 1 coexisting psychiatric or neurologic diagnosis. The mean number of different medical specialists seen before neuro-ophthalmic consultation was 3.7 ± 2.6, and the average number of health care visits was 4.6 ± 4.4. Each patient had 2.2 ± 1.8 neuroimaging studies performed. Fifteen percent of patients underwent unnecessary treatments, including receiving steroids, visual therapy, and prisms. CONCLUSIONS: Patients with FVL typically see at least 3 different health care providers across 4 different visits and undergo at least 2 neuroimaging studies before having neuro-ophthalmic consultation. To avoid this undue burden on patients and the health care system, clinicians should refer patients with suspected FVL to a neuro-ophthalmologist to confirm the diagnosis of FVL and appropriately counsel the patient.

Asymptomatic Fellow Eye Involvement in Nonarteritic Anterior Ischemic Optic Neuropathy.

BACKGROUND: Sequential nonarteritic anterior ischemic optic neuropathy (NAION) has been reported to occur in approximately 15% of patients within 5 years of the first episode. However, the incidence of presumed previous asymptomatic episode of NAION in fellow eye of patients presenting with acute NAION has not been previously reported. We reviewed charts of patients with acute NAION seen over a 5-year period to determine the frequency of visual field (VF) defect in the fellow eye secondary to presumed previous asymptomatic episode of NAION. METHODS: Retrospective chart review of all patients presenting to single, tertiary university-affiliated neuro-ophthalmology practice from January 2016 to September 2021 with diagnosis of acute NAION. Patients were determined to have had a presumed previous episode of asymptomatic NAION in the fellow eye if VF defect and corresponding optic nerve head pallor as well as thinning on peripapillary ocular coherence tomography (OCT) and ganglion cell analysis of macular complex were present and alternate causes of VF were excluded. RESULTS: One hundred ninety-two patients with the diagnosis of acute NAION were identified. One hundred thirty-nine had reliable VFs and were included in this study. VF defects in the fellow eye were present in 63 patients (45.4%). Of these, 54 (39%) were determined to represent previous NAION. In 14 of 139 patients (10%), a presumed episode of previous NAION in the fellow eye was asymptomatic. The most prevalent defect in asymptomatic eye was inferior altitudinal defect sparing fixation (7 of 14, 50%). The presence of obstructive sleep apnea, hypertension, diabetes, age, or sex was not predictive of previous episode of asymptomatic NAION. CONCLUSION: Unrecognized presumed previous episode of NAION occurred in a significant proportion of patients with acute NAION (14 of 139, 10.1%). In 100% of cases, the VF defect in the asymptomatic fellow eye was in a hemifield where there was a new loss in the symptomatic eye.

Severe Orbital Congestion After a Brow Lift Due to an Osteodural Arteriovenous Fistula.

The authors describe a 73-year-old woman who developed worsening proptosis, binocular diplopia, and periorbital pain shortly after undergoing an open subperiosteal brow lift procedure. She was found to have a pre-existing osteodural fistula (ODF) within left frontal bone, which was draining through supraorbital vein (SOV) in cavernous sinus. When SOV was inadvertently severed during a brow lift, blood from the fistula was now redirected in the orbit. This is the first report of a decompensated ODF after a brow lift procedure. It underscores the complexity of orbital venous drainage and the importance in preserving the supraorbital and supratrochlear veins during brow lift.

Intracranial rheumatoid nodule causing recurrent transient neurological deficits.

A 67-year-old man with rheumatoid arthritis developed recurrent acute onset of stereotyped focal neurological abnormalities. Cerebral imaging showed a mass lesion in the left parieto-occipital lobe. Imaging did not show the time evolution expected in stroke and so he underwent an extensive workup, which was inconclusive. Brain biopsy identified a rheumatoid nodule causing an extensive inflammatory reaction that mimicked a mass. Following treatment with intravenous corticosteroids and rituximab infusions, his clinical condition improved. While rheumatoid meningitis is well recognised, a rheumatoid nodule in the brain rarely presents as a mass lesion. Nevertheless, it is important to consider rheumatoid nodule in the differential diagnosis of a cerebral mass lesion in patients with rheumatoid arthritis.

Update on Micropulse Transscleral Cyclophotocoagulation.

Micropulse transscleral cyclophotocoagulation is a promising and relatively new technique that uses repetitive micropulses of diode laser energy, delivered with the Cyclo G6 Glaucoma Laser System (Iridex Corporation), in an off-and-on cyclical manner. This review article provides an update on the latest data available for this technique, including a discussion of the aspects in which there is still limited data, such as the precise mechanism of action, the ideal laser parameters based on total energy levels, as well as an overview of other potentially relevant variables that may be playing an important role in outcomes.