Magnetic Oculomotor Prosthetics for Acquired Nystagmus.

PURPOSE: Acquired nystagmus, a highly symptomatic consequence of damage to the substrates of oculomotor control, often is resistant to pharmacotherapy. Although heterogeneous in its neural cause, its expression is unified at the effector-the eye muscles themselves-where physical damping of the oscillation offers an alternative approach. Because direct surgical fixation would immobilize the globe, action at a distance is required to damp the oscillation at the point of fixation, allowing unhindered gaze shifts at other times. Implementing this idea magnetically, herein we describe the successful implantation of a novel magnetic oculomotor prosthesis in a patient. DESIGN: Case report of a pilot, experimental intervention. PARTICIPANT: A 49-year-old man with longstanding, medication-resistant, upbeat nystagmus resulting from a paraneoplastic syndrome caused by stage 2A, grade I, nodular sclerosing Hodgkin's lymphoma. METHODS: We designed a 2-part, titanium-encased, rare-earth magnet oculomotor prosthesis, powered to damp nystagmus without interfering with the larger forces involved in saccades. Its damping effects were confirmed when applied externally. We proceeded to implant the device in the patient, comparing visual functions and high-resolution oculography before and after implantation and monitoring the patient for more than 4 years after surgery. MAIN OUTCOME MEASURES: We recorded Snellen visual acuity before and after intervention, as well as the amplitude, drift velocity, frequency, and intensity of the nystagmus in each eye. RESULTS: The patient reported a clinically significant improvement of 1 line of Snellen acuity (from 6/9 bilaterally to 6/6 on the left and 6/5-2 on the right), reflecting an objectively measured reduction in the amplitude, drift velocity, frequency, and intensity of the nystagmus. These improvements were maintained throughout a follow-up of 4 years and enabled him to return to paid employment. CONCLUSIONS: This work opens a new field of implantable therapeutic devices-oculomotor prosthetics-designed to modify eye movements dynamically by physical means in cases where a purely neural approach is ineffective. Applied to acquired nystagmus refractory to all other interventions, it is shown successfully to damp pathologic eye oscillations while allowing normal saccadic shifts of gaze.

Description and evaluation of the first national patient and public involvement day for thyroid eye disease in the United Kingdom.

BACKGROUND: To ensure appropriate patient-focused outcomes, the National Institute for Health Research (NIHR) in the United Kingdom has made consultation with patients, caregivers, and the public a prerequisite to providing research funding. One method of encouraging engagement with research is through patient and public involvement (PPI) events. We describe the planning and implementation of a PPI day for thyroid eye disease (TED) and evaluate our own event using feedback from delegates. METHODS: The Moorfields NIHR Biomedical Research Centre, in partnership with TED charities, arranged the first national PPI day for TED in the United Kingdom. The event included didactic lectures; pre-event and postevent questionnaires; an exhibition with stalls, posters, and an interactive voting wall to determine research priorities; focus group sessions to explore patient experiences and perceptions of research; and one-on-one interviews recording individual patient stories. RESULTS: Of 100 attendees, 70 completed questionnaires. When asked whether the day had provided what they wanted, 48 of 52 (92%) said yes; 3 of 52 (6%) said no. Overall 6 of 52 (12%) rated the event as good; 28 of 52 (54%), very good; and 18 of 52 (34%), excellent. Thirty-six patients registered to participate in further research, identifying "finding the cause for TED," "improving psychological support," and "achieving a better cosmetic outcome" as key priorities. A poor understanding of TED among medical professionals was a common complaint. CONCLUSIONS: The event received positive feedback and achieved its key objective of engaging patients, researchers, and clinicians in a two-way discussion about research priorities and improved study design. An invaluable insight was gained into patients' needs for a better quality of life, and we have demonstrated that important data can be captured from such events, providing an evidential basis consistent with the NIHR principles of patient-centered research.

Protocol for the combined immunosuppression & radiotherapy in thyroid eye disease (CIRTED) trial: a multi-centre, double-masked, factorial randomised controlled trial.

BACKGROUND: Medical management of thyroid eye disease remains controversial due to a paucity of high quality evidence on long-term treatment outcomes. Glucocorticoids are known to be effective initially but have significant side-effects with long-term use and recrudescence can occur on cessation. Current evidence is conflicting on the efficacy of radiotherapy and non-steroid systemic immunosuppression, and the majority of previous studies have been retrospective, uncontrolled, small or poorly designed.The Combined Immunosuppression and Radiotherapy in Thyroid Eye Disease (CIRTED) trial was designed to investigate the efficacy of radiotherapy and azathioprine in combination with a standard course of oral prednisolone in patients with active thyroid eye disease. METHODS/DESIGN: Patients with active thyroid eye disease will be randomised to receive (i) azathioprine or oral placebo and (ii) radiotherapy or sham-radiotherapy in this multi-centre, factorial randomised control trial. The primary outcome is improvement in disease severity (assessed using a composite binary measure) at 12 months and secondary end-points include quality of life scores and health economic measures. DISCUSSION: The CIRTED trial is the first study to evaluate the role of radiotherapy and azathioprine as part of a long-term, combination immunosuppressive treatment regime for Thyroid Eye Disease. It will provide evidence for the role of radiotherapy and prolonged immunosuppression in the management of this condition, as well as pilot data on their use in combination. We have paid particular attention in the trial design to establishing (a) robust placebo controls and masking protocols which are effective and safe for both radiotherapy and the systemic administration of an antiproliferative drug; (b) constructing effective inclusion and exclusion criteria to select for active disease; and (c) selecting pragmatic outcome measures. TRIAL REGISTRATION: Current controlled trials ISRCTN22471573.

Pleomorphic lipoma of the orbit: a case series and review of literature.

OBJECTIVE: Pleomorphic lipomas are rare benign tumors that can resemble a variety of malignant soft tissue tumors on histologic examination. Six cases of patients with orbital pleomorphic lipoma, one of which was proven to be bilateral, are presented. DESIGN: Retrospective, noncomparative, interventional case series with clinicopathologic correlation. METHODS: Clinical and histologic review of 6 patients with pleomorphic lipomas of the orbit and histologic review of fat from 22 exenteration specimens and 20 other orbital procedures. MAIN OUTCOME MEASURES: Evidence of histologic abnormalities in histologic specimens. RESULTS: Pleomorphic spindle cells and multinucleated cells with nuclei arranged in a floret-like pattern were present in 7 specimens from 6 patients presenting with a clinical diagnosis of orbital fat prolapse, but there were no similar cell types present in the adipose tissue of 22 exenteration or 20 other orbital specimens. CONCLUSION: Pleomorphic lipoma may arise in the orbit, presenting as what was hitherto considered to be age-related epibulbar prolapse of orbital fat.