Disorders of auditory identification in dementia of the Alzheimer type.

Fifteen patients with probable DAT and 18 matched controls were given tests that required the identification of verbal (phonemes and words) and non verbal (sounds and melodies) stimuli. In all tests, DAT patients made significantly more errors than controls. Errors predominated in non verbal tests in both groups. DAT patients (and, to a lesser degree, control subjects) made almost exclusively acoustic errors in word-identification, while errors in the identification of sounds and melodies could be either semantic or acoustic. Some categories of errors were observed predominantly in DAT patients. These results suggest that, in addition to their cognitive impairment, DAT patients have a specific deficiency of central auditory perception.

[Acute chondrolysis on primary protrusio acetabuli in children]. / Chondrolyse aiguë sur coxopathie protrusive primitive de l'enfant.

Two cases of acute chondrolysis of the upper femoral epiphysis associated with protrusio acetabuli are reported in two girls respectively 13 and 12 Y.O. The disease was unilateral in one patient and bilateral in other. Acute chondrolysis is characterized by onset of pain, restricted movements of the hip and evolution to an hip ankylosis within a few months. As usual no evidence of inflammatory disease could be shown at biology or at pathology of synovial membrane or of femoral head. CT and MR studies proved to be contributive in the first case. It's the author's opinion that in these 2 cases, chondrolysis appears as a complication of preexistent Protrusio Acetabuli.

[Radiographic aspects of galactoceles]. / Aspects radiographiques des galactocèles.

The authors report two cases of galactoceles with unusual radiographic patterns, different from those ordinarily described either (opacity or radiocrescent); that is an aspect with a clear superior crescent related to a level between milk and fat, included in a localized galactophoric dilatation.

Genetic control of the bypass of mono-adducts and of the repair of cross-links photoinduced by 8-methoxypsoralen in yeast.

A large UVA dose by itself induces lethal damage revealed in some repair-deficient strains of Saccharomyces cerevisiae. Following photoaddition of a monofunctional psoralen derivative, 3-carbethoxypsoralen, an extra killing effect is observed by applying a second high UVA dose, in conditions where a fraction of 8-methoxypsoralen (8-MOP) plus UVA-induced monoadducts are transformed into DNA cross-links. In an excision-repair-deficient context, the bypass of 8-MOP plus UVA-induced monoadducts is under the control of the RAD6+ gene product. However, when other steps of the mutagenic pathway are blocked by the rad18-2 or the pso1-1 mutations, bypass occurs. This is also true when in excision-deficient strains the recombinogenic pathway is blocked by the rad52-1 mutation. The recombinogenic pathway may be an alternative to the mutagenic pathway for bypass of monoadducts. The repair of the lesions induced by a second UVA dose applied after a first treatment by 8-MOP plus UVA [i.e. cross-links and other putative lesion(s)] is controlled by at least the RAD2+, RAD6+, RAD52+, PSO2+ and PSO1+ gene products. The role of the pathways involved is discussed according to the nature of the secondarily induced lesions.

Fate of photo-induced 8-methoxypsoralen mono-adducts in yeast. Evidence for bypass of these lesions in the absence of excision repair.

A fraction of UVA-induced 8-methoxypsoralen (8-MOP) mono-adducts can be transformed by a second UVA (365 nm) irradiation procedure into lethal cross-links in Saccharomyces cerevisiae. To follow the fate of cross-linkable mono-adducts, cells were incubated in complete medium between the two UVA doses and survival was measured. The killing effect of the second UVA dose decreases rapidly in haploid wild-type as well as in strains blocked in mutagenic (RAD6+ type) or in recombinogenic (RAD52+ type) repair pathways. This is also true in the pso1-1 and pso2-1 strains selected for sensitivity to 8-MOP plus UVA treatment. In contrast, persistence of mono-adducts is observed in strains blocked in the excision-resynthesis repair pathway. In other words, cross-linkable mono-adducts are repaired by the excision process. The use of the cell-cycle conditional mutant strain (cdc14-1) permitted us to apply the second dose at a specific cell-cycle stage (post-G2 phase) after a 'priming' UVA treatment on stationary (G1) phase cells. Such experiments showed a bypass of mono-adducts in an excision-deficient context for at least one round of DNA replication.

Mutagenesis induced by mono- and bi-functional alkylating agents in yeast mutants sensitive to photo-addition of furocoumarins (pso).

The inactivation and the induction of forward and reverse mutations by a mono- and a bifunctional nitrogen mustard in 3 pso mutants of Saccharomyces cerevisiae, initially selected for their sensitivity to psoralen photo-addition, were compared with that of the wild-type. The pso1-1 mutant was very sensitive to both alkylating agents, and the mutagenicity was abolished. This correlates with the defect in the error-prone repair capacity for lesions induced by psoralen photo-addition and radiations already observed for this mutant. Therefore it appears that the PSO1+ gene product acts on a spectrum of DNA lesions. The pso2-1 mutant was highly sensitive to the lethal effect of the bifunctional nitrogen mustard and was only slightly sensitive to the monofunctional one. For both agents a reduction in induced mutagenesis was seen. The same was true for mono- and bifunctional psoralen derivatives. The pso2-1 mutant having the same sensitivity as the wild-type to UV and ionizing radiations, it is suggested that the PSO2+ gene product is predominantly necessary for the repair of cross-links irrespective of their molecular nature. In contrast with psoralen photo-induced inactivation the pso3-1 mutant had the same sensitivity as the wild-type to alkylating agents. However, a reduction in induced mutagenesis was seen in both cases. This response was modulated according to dose and type of mutation. Consequently, it appeared that the PSO3+ gene product acts specifically on psoralen photo-induced sub-lethal lesions and on a fraction of premutagenic lesions independently of their structure.

The effects of three PSO genes on induced mutagenesis : a novel class of mutationally defective yeast.

Reverse and forward mutation, induced by photoaddition of 8-methoxypsoralen (8-MOP) and 3-carbethoxypsoralen (3-CPs) or ultraviolet light (UV), are reduced in three pso mutants of Saccharomyces cerevisiae. The pso1-1 strain exhibits a lower frequency of spontaneous reversion (anti-mutator) and is almost entirely unaffected by the three agents in both the haploid and diploid states. The pso2-1 strain demonstrates very reduced frequencies of 8-MOP and 3-CPs plus 365 nm radiation-induced mutations in haploid and diploid cells. UV-induced mutation are slightly reduced, whereas survival is almost normal. The pso3-1 strain is mutable by 8-MOP and 3-CPs photoaddition only in the low-dose range. After UV treatment, survival of pso3-1 is nearly normal, whereas the frequencies of induced mutants are diminished as compared to the normal PSO+. An analogue of adenine, 6-N-hydroxyaminopurine, is capable of inducing reversions in wild type, as well as in pso and rad6-1 mutant strains, indicating that this drug may act as a direct mutagen in yeast. The comparison of photoaddition of the bifunctional agent (8-MOP) to that of the monofunctional one (3-CPs) confirms that cross-links, as well as monoadditions, are mutagenic in S. cerevisiae. Repair, of the recombinational type, taking place in diploid cells or in haploid cells in G2 phase leads to higher survival, but appears to be error-free.