Survival of Patients with Amyotrophic Lateral Sclerosis in a Population-Based Registry

Abstract

Objective: To evaluate the survival of patients with amyotrophic lateral sclerosis (ALS) in an Italian population and to assess the effects of selected prognostic indicators on survival. Background: Median survival of ALS patients has been reported to range between 12 and 23 months from diagnosis and between 23 and 36 months from onset of symptoms. Although several negative prognostic factors have been identified, the overall picture still needs clarification. Methods: We included patients enrolled in an Italian ALS Regional Register (population 4,529,003) during the calendar year 1998. The diagnosis was confirmed by an ad hoc committee using the original El Escorial criteria. Each case was regularly followed up until death or December 31, 2002, whichever came first. Survival was assessed with the Kaplan-Meier method in the whole sample, by level of diagnostic certainty, and by selected prognostic indicators (age, sex, bulbar or spinal onset, and disease duration). Multivariate analysis was done with the Cox proportional hazard function. Results: The sample comprised 79 patients (33 female; 46 male) aged 28–85 years (mean age 64.4 years). Onset of symptoms was bulbar in 30% of cases. Mean symptom duration at diagnosis was 13.3 months. ALS was definite in 43%, probable in 29%, possible in 6%, and suspected in 22%. By December 31, 2002, 56 cases (71%) had died. The cumulative probability of surviving after diagnosis was 78% at 12 months, 56% at 24 months, and 32% at 48 months. Median survival from onset was 39.2 months and from diagnosis 30.6 months. Multivariate analysis confirmed definite ALS at diagnosis and older age as adverse prognostic factors. Conclusions: Survival of ALS patients in the present sample was slightly longer than previously reported. Better palliative care and supportive treatment may explain the difference. Older age and the presence of definite ALS at diagnosis are poor prognostic predictors.

Blood Lead, Bone Turnover, and Survival in Amyotrophic Lateral Sclerosis

“…Older age at diagnosis, bulbar onset, lower forced vital capacity, and shorter diagnostic delay (i.e., time interval between first symptom onset and ALS diagnosis) are all consistently related to a worse prognosis (1)(2)(3)(4). Female sex, higher diagnostic certainty, lower score on the ALS Functional Rating Scale, and greater rate of disease progression after diagnosis may be associated with a worse prognosis, but the associations are less consistent (5)(6)(7)(8). More recently, rate of decline of ALS functional rating score before diagnosis and executive dysfunction have also been discussed as predictors of poor prognosis for ALS (9).…”

Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS

“…ALS typically causes death within 3-5 y of diagnosis; there is no cure and treatment options are very limited (3)(4)(5). Ten percent of ALS cases are familial (fALS), with 20% of fALS being linked to mutations in the homodimeric protein Cu/Zn superoxide dismutase 1 (SOD1) (6)(7)(8).…”

The Present and the Future of Neuroimaging in Amyotrophic Lateral Sclerosis

“…4,5 In ALS, the absence of a disease marker for UMN and LMN involvement has 2 main negative consequences. First, the delay from onset of the disease to diagnosis of ALS can vary between 13 and 18 months 6,7 …”

“…4,5 In ALS, the absence of a disease marker for UMN and LMN involvement has 2 main negative consequences. First, the delay from onset of the disease to diagnosis of ALS can vary between 13 and 18 months 6,7 ; and the diagnostic delay may even be greater in patients who present with isolated LMN signs. 8 Such a delay precludes early initiation of neuroprotective treatments.…”