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Few studies have examined the use of a short cognitive screening of ALS patients in order to establish which patients should undergo a more comprehensive neuropsychological assessment. We tested 20 patients with early ALS with four cognitive screening instruments and subsequently with an extensive neuropsychological assessment. Sixty percent of patients showed a deficit in at least two scores of tests administered, while 40% had three abnormal tests. Dysexecutive syndrome was the most common neuropsychological impairment. The Frontal Assessment Battery (FAB) proved a useful indicator of the presence of cognitive dysfunction to complete neuropsychological evaluation. In conclusion, the FAB can be considered a sensitive cognitive screening tool in these patients. These data will be verified on a larger sample of patients. [ABSTRACT FROM AUTHOR]

Effective treatments for amyotrophic lateral sclerosis (ALS) have remained elusive. Only riluzole, a drug thought to affect glutamate metabolism, improves survival albeit to modest extent. Explanations for the negative results of therapeutic trials include a likely heterogeneity, both in disease susceptibility and pathogenic mechanisms, and faulty methodology of clinical trials. Further understanding of these factors will lead to improvements in patient stratification, and in the design of future clinical trials. [ABSTRACT FROM AUTHOR]

We have performed a systematic review to summarize current knowledge concerning factors related to survival in ALS and to evaluate the implications of these data for clinical trials design. The median survival time from onset to death ranges from 20 to 48 ;months, but 10–20% of ALS patients have a survival longer than 10 ;years. Older age and bulbar onset are consistently reported to have a worse outcome. There are conflicting data on gender, diagnostic delay and El Escorial criteria. The rate of symptom progression was revealed to be an independent prognostic factor. Psychosocial factors, FTD, nutritional status, and respiratory function are also related to ALS outcome. The effect of enteral nutrition on survival is still unclear, while NIPPV has been found to improve survival. There are no well established biological markers of progression, although some are likely to emerge in the near future. These findings have relevant implications for the design of future trials. Randomization, besides the type of onset, should take into account age, respiratory status at entry, and a measure of disease progression pre-entry. Alternative trial designs can include the use of natural history controls, the so-called minimization method for treatment allocation, and the futility approach. [ABSTRACT FROM AUTHOR]

Monomelic amyotrophy (MA) is a variant of motor neuron disease (MND), characterized by muscle weakness and atrophy restricted to one limb. We describe the case of a 56-year-old Italian patient who developed a segmental muscular atrophy limited to the lower left limb. After 11 years of clinical stability he developed progressive spread of the disease to all limbs and to bulbar and respiratory muscles. The patient died from respiratory failure 15 years after disease onset. This case demonstrates that monomelic amyotrophy may rarely evolve to a diffuse fatal MND, even after more than a decade of clinical stability. Our findings support the idea that MA is part of the clinical continuum of MND. [ABSTRACT FROM AUTHOR]