Neuropsychological tests in Alzheimer's disease.

The recent development of symptomatic pharmacological treatment for Alzheimer's disease (AD) and the probable introduction of new therapies in a near future make the assessment of dementia at its different stages an even greater scientific and public health challenge. Neuropsychological tests, together with clinical data, are at present the only in vivo non-invasive screening and diagnostic tools for AD and related disorders. This chapter reviews the application to AD of standard batteries and short screening tests. It also analyzes the tests to be applied to detect and assess the specific deficits of the disease, and discusses the advantages and flaws of current screening and diagnostic tests of dementia. Emphasis is placed on the need to devise and use tests developed in a rational manner, with high sensitivity and specificity, not only in the moderate stages of the disease, but also in the very early and even "preclinical" stages, as well as during the late stages (severe dementia). It is known that neuropsychological tests allow one to determine various patients' profiles. Future research should determine the possible predictive value of these profiles. This has important implications for therapeutic trials. The current implicit assumption that all patients with AD tend to evolve and decline in a similar fashion needs to be critically re-examined.

Safety and tolerability assessment of intrastriatal neural allografts in five patients with Huntington's disease.

This study describes issues related to the safety and tolerability of fetal striatal neural allografts as assessed in five patients with Huntington's disease. Huntington's disease (HD) is characterized by motor, cognitive, and behavioral disturbances. The latter include psychological disturbances and, as a consequence, we took particular care to analyze behavioral changes, in addition to the usual "safety" follow-up. We conducted multidisciplinary follow-up at least 2 years before and 1 year after grafting. Psychological care extended to close relatives. The grafting procedure itself was altogether safe and uneventful, and there were no apparent clinical deleterious effects for 1 year. The immunosuppressive treatment, however, was complicated by various problems (irregular compliance, errors of handling, side effects). Direct psychological consequences of the transplantation procedure were rare and not worrisome, although mood alteration requiring treatment was observed in one patient. Indirectly, however, the procedure required patients and relatives to accept constraints that tended to complicate familial situations already marred by aggressivity and depression. All patients and close relatives expressed major expectations, in spite of our strong and repeated cautioning. It is clearly important to be aware of these particular conditions since they may eventually translate into psychological difficulties in coping with the long-term clinical outcome of the procedure, if not beneficial. Despite an overall good tolerance, therefore, this follow-up calls for caution regarding the involvement of HD patients in experimental surgical protocols.

Confabulation following rupture of posterior communicating artery.

In this study we report a patient, MG, who following rupture of left posterior communicating artery exhibited an amnesic-confabulatory syndrome. Neuropsychological examination showed severe impairment on episodic memory tasks, which were marred by florid but plausible and semantically appropriate confabulation. Performance on tasks involving various kinds of semantic knowledge was normal or only mildly impaired. Performance on tasks traditionally considered sensitive to frontal dysfunction was severely impaired with the exception of Cognitive Estimates where MG's performance was completely normal. There was no evidence of structural (CT scan) or metabolic (SPECT) damage to the frontal lobe. It is argued that tasks traditionally considered sensitive to frontal dysfunction are not specifically implemented by cognitive resources based on frontal structures. MG's confabulation is discussed in terms of a possible disruption of cognitive functions involved in the control of the subjective experience of feeling of remembering.

Semantic and episodic memory in aphasia.

Within the framework of the distinction between episodic and semantic memory, it has been argued that these two memory Systems are organised in a hierarchical way. The hierarchical hypothesis assumes that episodic memory is a specific subsystem of semantic memory and therefore implies that episodic memory cannot exist without semantic memory. If this hypothesis is correct, it should be expected that (episodic) yes/no recognition performance would improve in patients with preserved semantic memory, following semantic encoding. In the present study we investigated the influence of semantic encoding on recognition memory performance in a population of 28 aphasic patients (AA) and 14 normal controls (NC). Experiment 1 considered recognition memory for semantically unrelated items, whereas Experiments 2 and 3 assessed recognition memory for semantically related items. In Experiment 3, but not in Experiment 2, subjects were explicitly instructed to make a semantic association between the items. AA were impaired, compared to NC, only on the recognition memory performance of Experiment 1. The ability to make a semantic association between two items was significantly and positively correlated to the ability to recognise, in a subsequent test, those same items. A further analysis showed that patients who were impaired on the semantic association task did significantly worse on the recognition task of Experiment 3 than NC and than patients who were unimpaired on the semantic association task. These findings are discussed in the context of memory deficits in aphasia and interpreted as giving support to the view that episodic memory for an item is affected by the level of semantic awareness of that same item.