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In amyotrophic lateral sclerosis (ALS), prognosis is usually based on death or tracheostomy as outcomes. Nevertheless, the early course is known to be a reliable indicator of the whole course of the disease.1 The identification of outcomes in early stages would determine better planning of interventions and more appropriate stratification of newly diagnosed patients for randomised clinical trials (RCTs). The time of disease spread is probably linked to the rate of progression and the intensity of neuro-axonal degeneration,2 and it has already been related to survival in ALS.3 The aim of the present study was to evaluate if time to generalisation (TTG) may predict survival in ALS. All patients with a diagnosis of possible, probable or definite ALS, according to El Escorial criteria,4 and an onset of the disease between January 2004 and December 2007, referred to the Centre of Motor Neuron Disease, University of Bari, were enrolled in the current study. The study was terminated in April 2012. Death or tracheostomy was used as outcome measure to study the 4-year and 5-year mortality. Considered clinical variables were: (1) age at symptom onset (AAO); (2) site of symptom onset (bulbar region or spinal region); (3) time of diagnosis; (4) onset-diagnosis interval (ODI), defined as the time from the onset of the first symptom to diagnosis of ALS; (5) score of the revised ALS Functional Rating Scale (ALSFRS-r) at baseline;5 (6) TTG, considered as the time of spreading of the clinical signs from spinal (cervical, thoracic, or lumbosacral regions), or bulbar localisation or both. To detect generalisation, all the lower and upper motor neuron signs listed in the El Escorial criteria for the four body regions were investigated;4 TTG was censored at the time of the last follow-up visit; (7) overall survival (OS), defined as …

Objective The frontal assessment battery (FAB) is a quick and reliable method of screening to evaluate frontal lobe dysfunction in amyotrophic lateral sclerosis (ALS). However, previous studies were generally conducted on small samples representing different stages of disease and severity. We assessed the diagnostic accuracy of the FAB in detecting executive functions and its association with demographic and clinical features in ALS without dementia. Design Retrospective observational study. Setting A multidisciplinary tertiary centre for motor neuron disease. Participants We enrolled 95 consecutive patients with ALS diagnosed with El Escorial criteria in the period between January 2006 and December 2010. Main outcome measures We screened the patients with ALS using the FAB. An Executive Index (EI) was also calculated by averaging the Z scores of analytic executive tests evaluating information-processing speed (Symbol Digit Modalities Test—Oral version), selective attention (Stroop test) and semantic memory (Verbal Fluency Test). Results The FAB detected executive dysfunction in 13.7% of the patients with ALS. Moreover, using the EI standardised cut-off, 37.9% of the patients with ALS showed executive dysfunction. The receiver-operating characteristic curve showed that the optimal cut-off for the FAB in the whole sample was 16, with a sensitivity of 0.889 (95% CIs 0.545 to 1.000), a specificity of 0.593 (95% CI 0.450 to 0.907) and a moderate overall discriminatory power of 0.809. Different levels of respiratory function, duration of disease and depressive symptoms did not affect the FAB validity. Conclusions In patients with ALS without dementia, a high prevalence of executive dysfunction was present. The FAB showed good validity as a screening instrument to detect executive dysfunction in these patients and may be used when a complete neuropsychological assessment is not possible.