Editorial Sue Buckley

The first paper, entitled Françoise, a fifteen year follow-up, from Jean Rondal and his colleagues M. Elbouz, M. Ylieff, and L. Docquier, at the University of Liège in Belgium presents an individual case study that describes in detail the changes in language and memory function of Françoise, a 47 year old lady with Down syndrome. Françoise will already be well known to many in this field as she developed exceptionally good spoken language abilities in her earlier years and Rondal published a detailed study of her language, memory and cognitive skills in 1995.[1] In 2000, the day centre that Françoise attended became concerned about changes in her behaviour and requested further investigation. This case study is of particular interest because Françoise had exceptionally good language abilities for a person with Down syndrome. It is therefore possible to identify subtle changes in her abilities which would not be evident in adults with more typical language abilities. The assessments revealed that in 2002, Françoise had relatively preserved articulatory skills though more dysfluencies and slower speech rate than previously. She showed considerable word finding difficulties and a significant deterioration in syntactic comprehension abilities. Her MLU had decreased but she still had basically preserved morphosyntax in her spontaneous speech, maintaining correct French word order and correctly marking gender, number, case, tense or aspect. Her short-term memory span for digits and words had decreased, and her sentence span had decreased very significantly. Rondal and colleagues review in detail the findings with regard to loss of language and memory skills in Alzheimer patients in the typical population and identify that Françoise’s profile is similar – relatively spared syntactic production skills but greater difficulty with semantic comprehension of sentences. The authors suggest that the difficulty in sentence comprehension tasks may be linked to impairment of central executive function rather than verbal short-term memory per se. They also provide a useful review of the studies of ageing and cognitive change in individuals with Down syndrome, identifying well preserved skills in the majority. They discuss possible explanations of the profile of language deterioration seen in both Françoise and typical adults with probable Alzheimer disease and suggest that differential effects on implicit or explicit memory systems or differential effects on a modular language system could be explanations at this point in our knowledge of the processes involved. This paper is a unique and valuable addition to the literature on dementia in Down syndrome.