The Declarative/Procedural Model of Pinker, Ullman and colleagues claims that the basal ganglia are part of a fronto-striatal procedural memory system which applies grammatical rules to combine morphemes (the smallest...The Declarative/Procedural Model of Pinker, Ullman and colleagues claims that the basal ganglia are part of a fronto-striatal procedural memory system which applies grammatical rules to combine morphemes (the smallest meaningful units in language) into complex words (e.g. talk-ed, talk-ing). We tested this claim b y investigating whether striatal damage or loss of its dopaminergic innervation is reliably associated with selective regular past tense deficits in patients wi th subcortical cerebrovascular damage, Parkinson’s disease or Huntington’s dis ease.We focused on past tense morphology since this allows us to contrast the re gular past tense (jump-jumped), which is rulebased,with the irregular past tens e (sleep-slept), which is not We used elicitation and priming tasks to test pat ients’ability to comprehend and produce inflected forms. We found no evidence o f a consistent association between striatal dysfunction and selective impairment of regular past tensemorphology, suggesting that the basal ganglia are not esse ntial for processing the regular past tense as a sequence of morphemes, either i n comprehension or production, in contrast to the claims of the Declarative/Proc edural Model. All patient groups showed normal activation of semantic and morpho logical representations in comprehension, despite difficulties suppressing seman tically appropriate alternatives when trying to inflect novel verbs. This is con sistent with previous reports that striatal dysfunction spares automatic activat ion of linguistic information, but disrupts later language processes that requir e inhibition of competing alternatives.展开更多
【目的】探讨8个家族性特发性基底节钙化（FIBGC）家系的临床特点及遗传规律。【方法】收集8个FIBGC家系，根据临床表现分为运动受损组和精神症状组，绘制家系图，分析先证者及家系其他患者的发病年龄、临床表现、基底节区钙化的体积，...【目的】探讨8个家族性特发性基底节钙化（FIBGC）家系的临床特点及遗传规律。【方法】收集8个FIBGC家系，根据临床表现分为运动受损组和精神症状组，绘制家系图，分析先证者及家系其他患者的发病年龄、临床表现、基底节区钙化的体积，总结遗传规律。【结果】8个家系均呈常染色体显性遗传，先证者的性别比：男／女-4／4；患者的性别比：男／女-18／17，两组先证者人数构成比：运动受损／精神症状-4／4，运动受损组与精神症状组性别比（男／女）无显著性差异[（3／1）vs（1／3），P〉0．05]。两组先证者的发病年龄[（43．00±3．16）岁vs（29．50±6．95）岁]和基底节区钙化的体积[（1.526±0．679）cm3 vs （0．233±0．114）cm3]比较差异具有统计学意义（P〈0．05）。临床特点：患者均表现为一个系统损害的症状，运动系统受损或者精神症状。以运动受损的4个家系其他成员发病的症状也以运动受损为特点，精神症状为主的4个家系仅5人有精神症状，其他成员均没有临床症状。【结论]FIBGC临床表现具有明显的异质性，以运动受损的患者其病情严重程度与基底节区钙化病灶的大小相关，且发病年龄较晚，家系成员临床症状具有遗传性；以精神症状为主的患者其基底节区钙化病灶小，发病年龄早，家系成员临床症状遗传性不明显。展开更多
文摘The Declarative/Procedural Model of Pinker, Ullman and colleagues claims that the basal ganglia are part of a fronto-striatal procedural memory system which applies grammatical rules to combine morphemes (the smallest meaningful units in language) into complex words (e.g. talk-ed, talk-ing). We tested this claim b y investigating whether striatal damage or loss of its dopaminergic innervation is reliably associated with selective regular past tense deficits in patients wi th subcortical cerebrovascular damage, Parkinson’s disease or Huntington’s dis ease.We focused on past tense morphology since this allows us to contrast the re gular past tense (jump-jumped), which is rulebased,with the irregular past tens e (sleep-slept), which is not We used elicitation and priming tasks to test pat ients’ability to comprehend and produce inflected forms. We found no evidence o f a consistent association between striatal dysfunction and selective impairment of regular past tensemorphology, suggesting that the basal ganglia are not esse ntial for processing the regular past tense as a sequence of morphemes, either i n comprehension or production, in contrast to the claims of the Declarative/Proc edural Model. All patient groups showed normal activation of semantic and morpho logical representations in comprehension, despite difficulties suppressing seman tically appropriate alternatives when trying to inflect novel verbs. This is con sistent with previous reports that striatal dysfunction spares automatic activat ion of linguistic information, but disrupts later language processes that requir e inhibition of competing alternatives.
文摘【目的】探讨8个家族性特发性基底节钙化（FIBGC）家系的临床特点及遗传规律。【方法】收集8个FIBGC家系，根据临床表现分为运动受损组和精神症状组，绘制家系图，分析先证者及家系其他患者的发病年龄、临床表现、基底节区钙化的体积，总结遗传规律。【结果】8个家系均呈常染色体显性遗传，先证者的性别比：男／女-4／4；患者的性别比：男／女-18／17，两组先证者人数构成比：运动受损／精神症状-4／4，运动受损组与精神症状组性别比（男／女）无显著性差异[（3／1）vs（1／3），P〉0．05]。两组先证者的发病年龄[（43．00±3．16）岁vs（29．50±6．95）岁]和基底节区钙化的体积[（1.526±0．679）cm3 vs （0．233±0．114）cm3]比较差异具有统计学意义（P〈0．05）。临床特点：患者均表现为一个系统损害的症状，运动系统受损或者精神症状。以运动受损的4个家系其他成员发病的症状也以运动受损为特点，精神症状为主的4个家系仅5人有精神症状，其他成员均没有临床症状。【结论]FIBGC临床表现具有明显的异质性，以运动受损的患者其病情严重程度与基底节区钙化病灶的大小相关，且发病年龄较晚，家系成员临床症状具有遗传性；以精神症状为主的患者其基底节区钙化病灶小，发病年龄早，家系成员临床症状遗传性不明显。
