CHAPTER ONE INTRODUCTION
1.1 Motivation
Inspired by the conversation between a Prader-Willi Syndrome (PWS) patient and her elder sister, I decided to embark upon an odyssey of exploring the language deficits of the patient. What follows is an excerpt of their conversation.
(The patient was named AH; and A was her older sister)
A: 你吃什麼?
ni chi shemo you eat what
„What are you eating?‟
AH: 吃放(飯?)啊!
chi fang (fan?) a
eat put (rice?) exclamation mark (EM)
„I am eating dinner!‟ [fan(rice) was the intended word, but it was imprecisely articulated due to the anomaly of her articulatory structure.
fan(rice)=> fang(put): hypernasality].
A: 吃飯喔!?配菜喔!? 1
chi fan o pei cai o eat rice EM match vegetable EM
„Eating your dinner with some dishes (vegetables)!?‟
AH: 對呀!
dui a yes EM
„Yes!‟
1 In Taiwanese/Chinese culture, “吃飯 (chi fan)” has a broad sense that is similar to “having a meal”
in English. However, in this conversation, the idea of “吃飯 (chi fan)” was more restricted to “having boiled rice only”; this interpretation was triggered mainly owing to the influence of the contextual phrase “配菜 (pei cai)”. “配什麼菜 (pei shemo cai)” means “what kinds of dishes (e.g. vegetables, meat, or fish) that someone has “to go with boiled rice, which is usually unpalatable if eaten alone. As to a Chinese/Taiwanese, the concept of “吃飯 (chi fan)” is usually composed of “ having plain boiled rice”, “eating accompanying dishes”, and sometimes “having soup”.
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A: 配什麼菜?
pei shemo cai
match what vegetable
„What vegetables are you eating to go with the rice?‟
AH: 什麼?
shemo what
„What?‟
A: 配什麼菜?
pei shemo cai match what vegetable
„What vegetables are you eating to go with the rice?‟
AH: 什麼(青浦?)菜?
shemo (qingpu) cai what (qingpu?) vegetable
„What vegetable?‟
A: 我說你配什麼菜?
wo shuo ni pei shemo cai I say you match what vegetable
„I said, “What vegetables are you eating to go with the rice?”‟ [The older sister sounded impatient after asking the same question three times.]
AH: 配菜喔?
pei cai o match vegetable EM
„What vegetables am I eating?‟ [AH confirmed the question that she had heard.]
A: 嘿呀!
hei a yep
„Yep!‟ [The implicature that “Thank God! You finally understood my question.”
could be inferred from her tone.]
AH: 配那個菜呀!
pei nage cai a match that vegetable EM
„I am eating “THAT” vegetable!‟
A: 什麼菜呀?
Shemo cai a What vegetable EM
„What vegetable?‟
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AH: 那個那個，那個阿公種的菜呀!
nage nage nage agong zhong de cai a that that that grandpa grow de vegetable EM
„That, that, that vegetable that Grandpa grows!‟
A: 什麼菜呀?菜那麼多種，高麗菜，筍子也是菜呀!
Shemo cai a cai namo duo zhong what vegetable EM vegetable that many kind gaolicai sunzi ye shi cai a (white) cabbage bamboo shoot also is vegetable EM
„What vegetable? There are “SO” many kinds; (white) cabbage, or bamboo shoot is a vegetable, too.‟
AH: 高麗菜啊!
gaolicai a (white) cabbage EM
„(White) cabbage!‟
A: 是哦?!
shi o yes/really EM
„Really?!‟
Based on the conversation above, it was noted that AH had some difficulties with general language abilities, including hypernasality, excessive fillers “that, that, and that… (那個那個，那個…)”, repetitiveness in speech, and some pragmatic cue insensitiveness. And word-retrieving difficulties could be obviously observed in this conversation, too. AH tended to use adjectives or relative clauses to describe the specific object(s) that she could not name/label. Her disabilities of specifying the basic level exemplars (i.e. she seemed only able to give the superordinate category
“VEGETABLE” as a response instead of specifying what vegetables she ate) under a superordinate category (VEGETABLE) should be analyzed and investigated along three dimensions: whether she had no stored knowledge for “types” of vegetables at all (i.e. conceptual representation of the exemplars under “VEGETABLE”), or she could conceptualize vegetables/vegetable kinds but did not have a label for them (i.e.
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linguistic representation). Alternatively, she might have both the conceptual and nominal knowledge, but just could not retrieve it on the spot (i.e. access problem), which led to her naming difficulties.
In terms of Prader-Willi syndrome (hereafter PWS), it is a neuro-developmental or multisystem genetic disorder (cf. Prader, Labhart & Willi, 1956). Major
characteristics associated with PWS include mental retardation, infant hypotonia, hypogonadism, short stature and typical face traits (small forehead, almond-shaped eyes, small mouth, and thin upper lip). Since PWS was recognized as a distinct genetic syndrome, many studies (Butler, Bittel, Kibiryeva, Talebizadeh & Thompson, 2004; Cassidy & Driscoll, 2009; Conners, Rosenquist, Atwell & Klinger, 2000) have been conducted, which focus particularly on medical, genetic and behavioral
(cognitive/intellectual/psychological) aspects of the syndrome. A dimension that has received comparatively less attention is language functioning. Even though retarded developments and delayed abilities in language and speech have been reported (Å kefeldt, Å kefeldt & Gillberg, 1997; Branson, 1981; Defloor, Van Borsel & Curfs, 2000; Downey & Knutson, 1995; Edmonston, 1982; Hall & Smith, 1972; Kleppe, Katayama, Shipley & Foushee, 1990; Lewis, 2006; Lewis, Freebairn, Heeger &
Cassidy, 2002; Lewis, Freebairn, Sieg & Cassidy, 2000), the number of papers on language functions in PWS is still a drop in the bucket. Among the available papers on language, most of the investigators focused on the presence of articulation problems in PWS individuals, sometimes extending their discussion to phonetic or phonological problems (Å kefeldt, Å kefeldt & Gillberg, 1997; Kleppe, Katayama, Shipley & Foushee, 1990; Lewis, 2006). Some papers also reported morphological or morphosyntactic disorders (Kleppe, Katayama, Shipley & Foushee, 1990; Weiss, Lillywhite & Gordon, 1980), but virtually there have been no studies investigating the conceptual system of PWS individuals. Therefore, the goal of this thesis is to explore
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the vertical (superordinate vs. basic level categories) and horizontal
(prototypical/central vs. peripheral items; semantic related vs. semantic unrelated items) aspects of the conceptual system in a PWS patient, which includes how well, compared with a group of normal controls, is the patient‟s taxonomical hierarchical structure (semantic structure) preserved, how prototypicality and semantic relatedness affect her categorization and recognition respectively.