OBJECTIVE: Dizziness is a common reason patients present to doctors, but effective diagnostic tests and treatments for dizziness are underused. The internet is a way to disseminate medical information and is emerging as an intervention platform. The objective of this study was to describe internet searches for dizziness terms to assess the possible consumer demand for internet-based dizziness diagnostic and treatment tools. STUDY DESIGN/METHODS: Google AdWords and Google Insights for Search were used for keyword search data on the following generic terms: vertigo, dizzy, dizziness, lightheaded, and lightheadedness. Data collected included keyword ideas (i.e., additional keywords identified by Google as being related search terms), global and US only monthly search frequencies, as well as trends in top searches related to dizziness terms from 2004 to 2012. Keywords suggestive of benign paroxysmal positional vertigo (BPPV) or BPPV processes were identified. RESULTS: Of the five generic dizziness terms, vertigo had the most global searches per month (1.83 million) and lightheadedness had the least (90,500). Four BPPV-specific terms had more than 100,000 global searches per month. Three BPPV terms (positional vertigo, benign vertigo, and benign positional vertigo) have been in the list of top searches related to vertigo every quarter since 2004. CONCLUSION: Substantial demand exists for dizziness information via the internet. Future studies should seek to better characterize the population seeking this information. The magnitude of this potential demand suggests that validated and tested diagnostic and treatment tools could contribute to healthcare efficiencies and patient outcomes.

Importance

Understanding the natural history of familial amyotrophic lateral sclerosis (ALS) caused by SOD1 mutations (ALS^SOD1) will provide key information for optimising clinical trials in this patient population.

Objective

To establish an updated natural history of ALS^SOD1.

Design, setting and participants

Retrospective cohort study from 15 medical centres in North America evaluated records from 175 patients with ALS with genetically confirmed SOD1 mutations, cared for after the year 2000.

Main outcomes and measures

Age of onset, survival, ALS Functional Rating Scale (ALS-FRS) scores and respiratory function were analysed. Patients with the A4V (Ala-Val) SOD1 mutation (SOD1^A4V), the largest mutation population in North America with an aggressive disease progression, were distinguished from other SOD1 mutation patients (SOD1^non-A4V) for analysis.

Results

Mean age of disease onset was 49.7±12.3 years (mean±SD) for all SOD1 patients, with no statistical significance between SOD1^A4V and SOD1^non-A4V (p=0.72, Kruskal-Wallis). Total SOD1 patient median survival was 2.7 years. Mean disease duration for all SOD1 was 4.6±6.0 and 1.4±0.7 years for SOD1^A4V. SOD1^A4V survival probability (median survival 1.2 years) was significantly decreased compared with SOD1^non-A4V (median survival 6.8 years; p<0.0001, log-rank). A statistically significant increase in ALS-FRS decline in SOD1^A4V compared with SOD1^non-A4V participants (p=0.02) was observed, as well as a statistically significant increase in ALS-forced vital capacity decline in SOD1^A4V compared with SOD1^non-A4V (p=0.02).

Conclusions and relevance

SOD1^A4V is an aggressive, but relatively homogeneous form of ALS. These SOD1-specific ALS natural history data will be important for the design and implementation of clinical trials in the ALS^SOD1 patient population.