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Childhood onset motor neuron diseases or neuronopathies are a clinically heterogeneous group of disorders. A particularly severe subgroup first described in 1894, and subsequently called Brown-Vialetto-Van Laere syndrome, is characterized by progressive pontobulbar palsy, sensorineural hearing loss and respiratory insufficiency. There has been no treatment(More)
Adults who give proxy consent for medical treatment for adolescents must decide how much weight to give to adolescents' own preferences. There is evidence that some adolescents choose treatments different from what adults see as most reasonable. It is argued that adolescents choose according to age-specific values, i.e. values they hold, as adolescents, and(More)
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