Periocular and Orbital Amyloidosis. Clinical Characteristics, Management, and Outcome

Objective: To present the clinical features and management outcome in a large series of patients with periocular and orbital amyloidosis. Design: Retrospective, noncomparative, interventional case series. Patients: All patients diagnosed with periocular and orbital amyloidosis in 6 oculoplastic and orbital units. Methods: Clinical records of all patients were reviewed. Main Outcome Measures: Clinical presentation, radiological and histological findings, treatment modalities, and outcome. Results. The study included 24 patients (15 female, 9 male) with a mean age of 57 17 years. Nineteen cases were unilateral, and 5 were bilateral. Clinical signs and symptoms included a visible or palpable periocular mass or tissue infiltration (95.8%), ptosis (54.2%), periocular discomfort or pain (25%), proptosis or globe displacement (21%), limitations in ocular motility (16.7%), recurrent periocular subcutaneous hemorrhages (12.5%), and diplopia (8.3%). Seven cases had orbital involvement, and 17 were periocular. Immunohistochemistry in 7 patients showed B cells or plasma cells producing monoclonal immunoglobulin chains that were deposited as amyloid light chains. Only 1 patient was diagnosed with systemic amyloid light chain amyloidosis. Treatment modalities were mainly observation and surgical debulking. During a mean follow-up period of 39 months, 21% showed significant progression after treatment, whereas 79% were stable or showed no recurrence after treatment. Conclusion: Periocular and orbital amyloidosis may present with a wide spectrum of clinical findings and result in significant ocular morbidity. Complete surgical excision is not feasible in many cases, and the goal of treatment is to preserve function and to prevent sight-threatening complications.

Development of a multi-tiered speech annotation system for accented English

This paper discusses methodological issues in the development of a multitiered, phonetic annotation system, intended to capture pronunciation variation in the speech of second language learners and to serve in construction of a data base for training ASR models to recognize major pronunciation variants in the assessment of accented English.

High-level and phonological awareness abilities of children following management for supratentorial tumour: Part II

Background. Limited information is available regarding the impact of childhood tumour on the cerebral hemispheres and supratentorial cranial fossa. However, a recent study found that children managed for a tumour located in this region may demonstrate reduced general language abilities. However, the indirect or direct impact of a tumour in this region on higher-level language abilities in childhood is at present largely unknown. Materials and methods. The present study examined the higher language and phonological awareness abilities of five children treated for supratentorial tumour ranging in age from seven to fourteen years in age. Assessments included measures of receptive and expressive semantic abilities, inferencing, figurative language, and problem solving, as well as a comprehensive pre-literacy test. Results. As a group, reductions were evident in problem solving, and in the ability to receive and decode content of high-level language when compared to a group of age- and gender-matched peers. At an individual level, only two of five children managed for supratentorial tumour demonstrated language deficits. These two cases were noted to be the same children previously identified as also having general language deficits. More widespread findings were noted in phonological awareness, with four of the five children previously managed for supratentorial tumour demonstrating weaknesses in one or more areas. Conclusions. Findings demonstrated that weaknesses in general language ability in children managed for supratentorial tumour may indicate higher-level language difficulties. Language abilities beyond general measures of language should be monitored, as well as long-term consideration of phonological awareness abilities in this population.