Our objective was to measure direct (hospital and NHS) and indirect (patient/caregiver) costs of following up in-home compliance to non-invasive ventilation via wireless modem. We constructed a prospective controlled trial of 40 consecutive ALS home-ventilated patients, randomly assigned according to their residence area to G1 (nearby hospital, office-based follow-up) and G2 (outside hospital area, telemetry device-based follow-up). Total NHS direct cost encompassed costs related to outpatients' visits (office and emergency room) and hospitalizations. Hospital direct costs included transportation to/from hospital, office visit per hour cost and equipment maintenance. Non-medical costs considered days of wages lost due to absenteeism. G1 included 20 patients aged 60 ± 10 years and G2 included 19 patients aged 62 ± 13 years. Results showed that no differences were found regarding clinical/demographic characteristics at admission. NHS costs showed a 55% reduction in average total costs with a statistically significant decrease of 81% in annual costs per patient in G2. Hospital costs were found to be significantly higher in G2 with regard to total costs (64% average increase) but not annual costs (7%). No statistical difference was found with regard to expenses from absenteeism. In conclusion, at the cost of an initial financial constraint to the hospital per year (non-significant), telemonitoring is cost-effective, representing major cost savings to the NHS in the order of 700 euros/patient/year.

Our objective was to investigate functional outcome in primary lateral sclerosis (PLS). We followed a group of 24 patients with PLS. Clinical (ALSFRS), respiratory, and neurophysiological (electromyography and transcranial magnetic stimulation) evaluations were performed at entry and regularly over the follow-up period. The time taken for a greater than 10% decrease in ALSFRS compared to the first assessment was defined as the dichotomous outcome; prognostic factors were evaluated using the Cox proportional hazard model. Results demonstrated that the median age at symptom onset was 54 years (range 28-75 years). In 46% symptoms began in the lower limbs, in 21% in the upper limbs and in 33% in bulbar muscles. The median symptom duration at first visit was 3.1 years (range 0.9-11.7 years). At last follow-up the median disease duration was 9.9 years (range 4.2-17.6 years). The median follow-up time was 4.6 years (range 2.1-11 years). We excluded from final analysis three patients with positive family history. Older age at onset (p = 0.019) was related to more rapid functional impairment; gender, forced vital capacity, region of onset and neurophysiological changes did not predict outcome. In conclusion, age is the most critical prognostic factor for functional outcome in PLS.

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