The cognitive and behavioural profile of amyotrophic lateral sclerosis: application of the consensus criteria.

OBJECTIVE: The study aims to assess the spectrum of cognitive and behavioural disorders in patients affected by Amyotrophic Lateral Sclerosis (ALS) according to the recent consensus criteria [9]. The study also intends to assess the impact of physical disability on cognitive and behavioural abnormalities. METHODS: Detailed neurological, neuropsychological and neurobehavioral evaluations were administered to 23 ALS patients, 11 Lower Motor Neuron Disease (LMND) patients and 39 healthy controls. Strong et al.'s criteria [9] were applied to diagnose the presence of cognitive/behavioural impairment. Clinical and neuropsychological scores were used for group comparisons and correlation analyses. RESULTS: In comparison with LMND and controls, a subgroup of ALS patients (∼30%) manifested executive dysfunction, which was severe enough to classify them as cognitively impaired. Action naming difficulties and short-term memory deficits were also observed. Aspontaneity, disorganization and mental rigidity reached clinical relevance in 20% of ALS patients. A small percentage of ALS patients (13%) also had comorbid dementia. The cognitive or behavioural status was not related to the clinical features of ALS. CONCLUSION: The use of consensus criteria for cognitive and behavioural impairment and the comparison with the LMND group proved useful in defining the spectrum of non-motor manifestations of ALS.

Experience of donation and quality of life in living kidney and liver donors.

Italian guidelines on living donation demand that we ascertain the donor's free and informed consent. Assessments to do so have to be conducted by an independent 'third party' who has nothing to do with the medical team treating the recipient. From February 2002 to December 2006, the Veneto Regional Authority's Third Party Commission evaluated 201 living liver and kidney donors. A sample of these were contacted after their surgery to assess their living donation experience and quality of life (QoL); 81 were eligible for the assessment and 69 (85.2%) responded. All donors involved in the study completed an anonymous document that included the SF-36 and a questionnaire on their donation experience. The majority (96%) of the sample expressed a positive global opinion of the experience. We concluded that the donation had positive effects on their QoL and that family support had a fundamental influence on their general well-being, and their psychic balance in particular. Some crucial issues emerged, however, i.e. 11% of donors judged the information received before the operation inadequate, 17% reported a subjective perception of bodily changes after the operation and 14% were concerned about their current health: these findings emphasize the importance of informing potential donors thoroughly before they submit to surgery.