Stiff person syndrome with Anti-GAD65 antibodies within the national veterans affairs health administration.

INTRODUCTION: Stiff person syndrome (SPS) is a neurological disorder characterized by muscle rigidity primarily in the truncal muscles, commonly associated with autoantibodies to the glutamic acid-decarboxylase 65 kD receptor (GAD65). There is limited epidemiological information on patients with SPS. METHODS: We performed a retrospective case review using the National United States Veterans Affairs Health Administration electronic medical record system. We analyzed prevalence, demographics, disease characteristics, and treatment outcomes in SPS patients who were anti-GAD65 antibody positive. RESULTS: Fifteen patients met our criteria. Point prevalence was 2.06 per million, and period prevalence was 2.71 per million. Men to women ratio was 14:1. All patients benefitted from treatment with symptomatic antispasmodic agents. Ten of 15 patients received intravenous immunoglobulin, with a majority demonstrating stable or improved modified Rankin scores. DISCUSSION: This investigation was a large North American epidemiological study of SPS with predominantly male patients. Symptomatic therapy was beneficial for most patients, with less clear sustained benefit of immunotherapy. Muscle Nerve 58:801-804, 2018.

A Community Needs Assessment of Urban Utah American Indians and Alaska Natives.

The needs of Urban American Indian/Alaska Native (AI/AN) populations are often not well understood. In order to inform programs and services, the Urban Indian Center of Salt Lake (UIC-SL), in cooperation with researchers from the University of Utah, undertook a community needs assessment of AI/AN living along Utah's Wasatch Front. A 60-item questionnaire was developed to capture information about health status, services used, unmet needs, and common sources of information about AI/AN community events and activities. Study participants (n = 336) were a convenience sample. Descriptive statistics, including mean, standard deviation, percentage, and 95% confidence intervals, were calculated. The most common health provider diagnosed medical conditions included hypertension, obesity, and diabetes. The prevalence of violence, substance abuse, and mental health conditions were also high among study participants and their families. Compared to the general Utah population, study participants experienced disparities relative to diabetes prevalence, cognitive and visual disabilities, and access to health care. In addition to health care services, respondents indicated a need for cultural, social, and educational programs. They also reported high levels of technology use, suggesting a possible avenue for communicating with this population. This community-based participatory research project provided rich information about the unmet needs of this urban AI/AN population. The results will be used to guide UIC-SL strategic planning, and a database created for this project will be available for future data collection, allowing for comparison of results between sites and over time.

Lambert-Eaton myasthenic syndrome: Epidemiology and therapeutic response in the national veterans affairs population.

INTRODUCTION: One nationwide study (The Netherlands) of Lambert-Eaton myasthenic syndrome (LEMS) has been published. We report LEMS epidemiology and its therapeutic response in the United States Veterans Affairs (VA) population. METHODS: Medical records for all active patients (12.5 million) in the VA health system were queried for relevant ICD-9 codes for the period October 1, 1999 to September 30, 2013. Clinical, electrophysiologic, and serologic features were evaluated to confirm diagnosis; epidemiologic and treatment data were collected. RESULTS: Point prevalence was estimated at 2.6 per 1,000,000 (confirmed cases) and 3.3 per 1,000,000 (combined confirmed and probable cases). Crude prevalence was similarly estimated at 9.2 and 10.9 per 1,000,000 respectively. A total of 18 of 48 (38%) patients received 3,4-diaminopyridine (3,4-DAP); 14 of 18 (78%) improved. CONCLUSIONS: This investigation was a large North American epidemiologic study of LEMS. LEMS prevalence in the national VA population was found to be similar to previously published rates in other large international populations. Most patients experienced improvement with therapy, including a majority with 3,4-DAP. Muscle Nerve 56: 421-426, 2017.