Reading and Writing: An Interdisciplinary Journal 15: 589–612, 2002.
© 2002 Kluwer Academic Publishers. Printed in the Netherlands.
A linguistic dissociation in Williams syndrome: Good at gender
agreement but poor at lexical retrieval
SANDRINE MONNERY, ALIX SEIGNEURIC, DANIEL ZAGAR &
Université de Bourgogne, LEAD-CNRS, Dijon, France
Abstract. Williams syndrome (WS) is a rare neuro-developmental disorder characterised
by spared language in the face of serious deﬁcits in nonlinguistic cognitive abilities. We
conducted a study on ten WS individuals in which gender agreement and gender assignment
were assessed by means of two tasks: gender categorisation and gender concord. Subjects
performed gender categorisation to real nouns whose gender was regular or exceptional given
their ending, and to invented nouns which were composed of nonword or word stems and real
word-endings. The same material was used in the gender concord task in which subjects had
to match the items with the appropriate form of the article and the adjective carrying gender
agreement. In the gender categorisation of real words, WS were lower than controls but both
groups demonstrated a similar sensitivity to gender-ending regularities. In the gender categor-
isation of nonwords, the results showed a clear dissociation. The WS subjects produced more
ending-consistent responses than the controls. Contrary to the controls, WS relied heavily on
the gender clue provided by the ending even when the gender of the word evoked by the
stem of the invented word was opposed to that evoked by the ending. Participants with WS
were not inﬂuenced by the speciﬁc word evoked by the stem of the invented word. In the
second task (concord task), the WS subjects performed well although lower than the controls.
We concluded that the WS people were not impaired in gender agreement which relies on
syntactic rules and/or on the extraction of regularities but experienced difﬁculties in retrieving
Key words: Gender-ending regularities, Grammatical agreement, Grammatical gender,
Morphosyntax, Williams syndrome
Williams syndrome (WS) is a relatively rare genetic disorder (Mari, Amatti,
Mingarelli, Gianotti, Sebastio, Colloridi et al. 1995; Morris, Thomas &
Greenberg 1993). It has been ﬁrst described as infantile hypercalcemia
(Beuren, Apitz & Harmjanz 1962; Williams, Barratt-Boyces & Lowe 1961).
Since the works of Morris and colleagues, it has been widely recognized as
a microdeletional genetic disorder with, in particular, the lack of Elastin and
LIM-kinase 1 genes (Morris, Loker, Ensing & Stock 1993; Morris, Thomas &
Greenberg 1993; Nickerson, Greenberg, Keating, McCaskill & Shaffer 1995).
The WS patients display three symptomatic elements: (1) a heart defect,