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Ultrasound examination performed on a 36-year-old woman at 33 weeks of gestation showed the presence of isolated and bilateral ventriculomegaly in the fetus. Array-based comparative genomic hybridization (array-CGH) performed on uncultured amniocytes at 35 weeks of gestation revealed a 17q21.31 microdeletion. After genetic counseling, the pregnancy was terminated at 37 weeks of gestation. At autopsy, the fetus displayed facial dysmorphic features and triventricular ventriculomegaly. To our knowledge, this is the first case of a 17q21.31 microdeletion detected prenatally. Our report suggests that array-CGH should be performed when severe ventriculomegaly is observed in prenatal ultrasound examination.

The caffeic acid O-methyltransferase (COMT) gene is a gene candidate forimproving silage maize digestibility. Before assessing possible functionalconsequences of COMT gene polymorphism, the nucleotide sequence variability atthe COMT locus in 6 normal maize inbred lines was characterized and analyzed.Inaddition, the COMT bm3-3 mutation characterization was specified. Even if theCOMT gene seemed to evolve according to the predictions of the neutral model,estimates of nucleotide diversity indicated that polymorphism distributionwithin the sequence was not uniform and that nonsynonymous sites were the leastvariable. Linkage disequilibrium calculation and estimates of recombinationshowed that polymorphism was a dimorphic segregating distribution caused by alot of recombination events.

We present the neuropsychological study of a patient, I.R., who sustained bilateral damage to the temporal lobes and to the right frontal lobe as a result of successive brain surgeries that occurred ten years earlier. The patient is 40 years old and right-handed; she had no special training in music or in language, representing, therefore, the large majority of listeners. Her performance is compared to that of four neurologically intact subjects who are closely matched in terms of education, sex and age. In the present study, we report I.R.'s performance on various tests aiming at assessing her general cognitive functioning with a particular focus on auditory aspects. The results show that, despite extensive damage to her auditory cortex, I.R.'s speech abilities are essentially intact (see Tables 1 and 2). The only impairments that are detected in the language domain are related to a short-term memory deficit, to some abnormal sensitivity to retroactive interference in long-term memory (see Table 3) and to articulation. These difficulties do not, however, affect linguistic communication, which is obviously undisturbed I.R. is not aphasic). Similarly, I.R. does not experience any difficulty in the recognition and memorization of familiar sounds such as animal cries, traffic noises and the like (see Tables 5 and 7). In contrast, I.R. is severely impaired in most musical abilities: She can no longer discriminate nor identify melodies that were once highly familiar to her; she can no longer discriminate nor memorize novel melodies (see Table 4). Her pattern of musical losses is compatible with a basic and severe perceptual deficit that compromises access to and registration in memory systems. The observation that the auditory impairment affects music and spares language and environmental sounds refers to a neuropsychological condition that is known as music agnosia. I.R. represents, to our knowledge, the fourth case of music agnosia available in the literature (Peretz et al., 1994; Griffiths et al., 1997). The existence of such cases suggests that music processing is not mediated by a general-purpose auditory architecture but by specialized cortical subsystems. Not only does I.R. suffer from music agnosia, but she is also impaired in the discrimination and recognition of musical instruments and of human voices (see Table 5). These latter two deficits probably do not result from the music agnosic condition. Rather, they seem to reflect damage to adjacent brain areas that are specialized in timbre processing (see Peretz. et al., 1994, for the relevant discussion). It is also worth mentioning that I.R. appears to be impaired in musical expressive abilities as well: I.R. can no longer sing a single note. Thus, her losses are rather general in the musical domain, hence justifying the classification of her case as amusia. Cases of amusia without aphasia are relatively frequent in the neuropsychological literature. However, all of these reported cases are anecdotal. Thus, in the present study, special focus is given to the measurement and direct comparison of performance in the language and music domain; in both domains, task characteristics and materials were as similar as possible. To this aim, the lyrics and the tune of the same popular song excerpts were used. The musical and the spoken parts were presented separately in a primed familiarity decision task and in a memory recognition task. In both situations, I.R. performs at or close to chance when she has to deal with music, whereas she recognizes easily and performs normally on the spoken material (see Tables 6 and 7). These results clearly argue for the autonomy of music and language in the processing of auditory information.

Cell wall digestibility is an important determinant of forage quality, but the relationship between cell wall composition and digestibility is poorly understood. We analyzed the neutral detergent fiber (NDF) fraction of nine maize inbred lines and one brown midrib3 mutant with pyrolysis-gas chromatography-mass spectrometry (Py-GC-MS). Among 29 pyrolysis fragments that were quantified, two carbohydrate-derived and six lignin-derived fragments showed statistically significant genetic variation. The pyrolysis products 4-vinyl phenol and 2,6-dimethoxy-4-vinyl phenol were negatively correlated with digestibility, whereas furfural and 3-(4-hydroxyphenyl)-3-oxopropanal showed a positive correlation with digestibility. Linear discriminant analysis of the pyrolysis data resulted in the resolution of groups of inbred lines with different digestibility properties based on their chemical composition. These analyses reveal that digestibility is governed by complex interactions between different cell wall compounds, but that several pyrolysis fragments can be used as markers to distinguish between maize lines with different digestibility.