Perceived stress in prodromal Huntington disease

This study examines perceived stress and its relationship to depressive symptoms, life changes and functional capacity in a large sample of individuals who are positive for the Huntington disease (HD) gene expansion but not yet diagnosed. Participants were classified by estimated proximity to HD diagnosis (far, mid, near) and compared with a non-gene-expanded comparison group. Persons in the mid group had the highest stress scores. A significant interaction between age and time since HD genetic testing was also found. Secondary analyses using data from a different data collection point and including a diagnosed group showed the highest stress scores in the diagnosed group. Possible explanations and implications are discussed.     

Category knowledge in Alzheimer's disease: normal organization and a general retrieval deficit.

Three hypotheses that could account for deficits in the retrieval of category information in Alzheimer's disease (AD) were evaluated: abnormal organization, class- or category-specific vulnerability, and limitation by general factors, such as decreased processing speed. Relative to 18 elderly control subjects, 18 patients with AD produced fewer items in a category fluency task and had longer reaction times in a category decision task. The pattern of performance across categories on both tasks was normal in the AD group: The same categories elicited the most (or fastest) responses in both the control group and the AD group. AD patients showed normal performance in ranking of category exemplars by typicality. There was no evidence for differential accessibility by category or by class of information (animate vs. inanimate). The authors conclude that a general factor or factors limit(s) retrievability equally across all categories.     

Biological markers of cognition in prodromal Huntington's disease: a review

Huntington’s disease (HD), an autosomal-dominant genetic disorder, has historically been viewed as a degenerative movement disorder but it also includes psychiatric symptoms and progressive cognitive decline. There has been a lack of consensus in the literature about whether or not cognitive signs can be detected in carriers before clinical (motor) onset of the disease, i.e., prodromal HD. However, recently validated mathematical formulas to estimate age of clinical onset, refined over the past 5–7 years, have allowed researchers to overcome the methodological limitation of treating all prodromal carriers as a homogenous high-risk group (i.e., whether they may be 2 or 15 years from diagnosis). Here we review 23 articles on the HD prodrome, all of which related cognition to a biological marker of disease burden (i.e., genetic load, neuroimaging). All studies found at least one cognitive domain was associated with disease burden in prodromal HD participants. There was greater variability in both the detection and cognitive domain affected in those farther from onset (or those with less pathology) while most studies reliably found declines in visuomotor performance and working memory in those closer to onset. These findings indicate that cognitive signs can be reliably detected in the HD prodrome when comparing cognition to additional disease markers, however, there continues to be significant variability on cognitive findings among large and methodologically rigorous studies. This may reflect true heterogeneity in the prodromal HD phenotype which must be further explored by analyzing intra-individual variance, determining demographic risk factors associated with decline/protection, and examining if particular HD families exhibit distinct cognitive profiles. These and additional future directions are discussed.     

Confabulation in Alzheimer's disease.

Confabulation is present when memory is disturbed, but does it occur in Alzheimer's disease (AD) where memory abnormalities are often severe? In this study, confabulation was assessed prospectively in 26 AD patients and 15 normal elderly (NE) controls using a neuropsychological battery specifically designed to assess different types of memory errors. The results indicated that the AD group made significantly more verbal intrusions than the NE group. These findings provide evidence for the feasibility of eliciting confabulatory-type behavior during clinical assessment and support previous reports which have documented verbal intrusions as a common accompaniment of the memory impairment manifested by AD patients.     

Posture recognition in Alzheimer's disease

BACKGROUND: Apraxia is neurologically induced deficit in the ability perform purposeful skilled movements. One of the most common forms is ideomotor apraxia (IMA) where spatial and temporal production errors are most prevalent. IMA can be associated Alzheimer's disease (AD), even early in its course, but is often not identified possibly because the evaluation of IMA by inexperienced judges using performance tests is unreliable. The purpose of this study, therefore, is to learn if the Postural Knowledge Test (PKT), a praxis discrimination test that assesses knowledge of transitive (PKT-T subtest) and intransitive (PKT-I subtest) postures and does not require extensive training, is as sensitive and specific as the praxis performance tests. METHODS: We studied 15 subjects with probable AD as well as 18 age-matched controls by having them perform transitive and intransitive gestures to command and imitation, as well as having them discriminate between correct and incorrect transitive and intransitive postures. RESULTS: Overall on all tests, the control subjects performed better than those with AD. In addition all subjects had more trouble with transitive than intransitive gestures. Using a stepwise discriminative analysis, 81.8% of the subjects could be classified according to Group (94.4% of Controls, 66.7% of AD subjects). In this analysis, the PKT-T (transitive posture subtest) was the only measure that contributed to the discrimination of subjects. CONCLUSION: We found that having subjects select the correct transitive hand postures in this "booklet test" was more sensitive than grading their praxis performances even when using judges with extensive training. This suggests that this discrimination test might be an excellent means for diagnosing and screening patients for AD. The reason why recognition of transitive postures is relatively more difficult for our AD subjects is not known. Two possibilities are that the representations for intransitive movements are stronger than those for transitive movements, and hence, more resistant to degradation, or that intransitive acts are stored in parts of the brain not affected by AD.     

Semantic memory in Alzheimer's disease

Patients with Alzheimer's disease have been suggested to have a semantic memory impairment not present in the normal old. This article reviews the performance of Alzheimer patients on tests of various aspects of semantic memory, including word finding, knowledge of the semantic attributes, and associates of concepts, as well as their category membership. The effect that semantic context has on cognitive processes such as lexical and semantic priming and memory encoding is also reviewed. Finally, the ability of theoretical constructs such as implicit memory and automaticity to explain intertask variability in Alzheimer patients' semantic performance is discussed.     

Maze learning in Alzheimer's disease

Alzheimer's disease (AD) patients and normal elderly controls performed a block of 10 trials on a finger maze, followed by a 45-min period during which verbal learning measures were administered. Subjects then performed an additional 10 trials on the original maze, followed by 10 trials on a new maze. The AD patients and a subset of control subjects decreased mean completion time across Blocks 1 and 2, suggesting skill acquisition. Mean time on Block 3 was significantly less than mean time on Block 1, suggesting skill generalization. A subset of controls did not show the above pattern. The results of this study suggest that AD patients are able to acquire and generalize a cognitively mediated perceptual-motor skill.     

Ideational action impairments in Alzheimer's disease

We report data from a group of patients with mild Alzheimer's disease on a range of tasks requiring either stored semantic knowledge about objects (e.g., naming object use) or the execution of action to objects (e.g., miming and using objects). We found that the patients were impaired at miming in response to objects, even when they could describe the object's function. On the other hand, copying gestures was not impaired relative to naming gestures, indicating that an ideomotor deficit in action execution, per se, was unlikely to explain the impairments in object use. We suggest instead that the patients had an impairment in stored motor programmes for action, over and above their deficits in semantic knowledge. Despite this, the patients were better at using than at miming to objects, consistent with the view that proprioceptive input (when using objects) can directly constrain selection of the appropriate motor programme for action.     

Pantomime recognition impairment in Alzheimer's disease

This study was designed to examine whether 10 AD patients with diffuse lesions and an early semantic deficit could comprehend the meaning of pantomimes and symbolic gestures. We especially wanted to determine the relationship between pantomime recognition and production. The tasks involved naming or showing an understanding (e.g., circumlocutions) of the pantomimes executed by the examiner and producing pantomimes to verbal command or to imitation. The results showed no significant relationship between pantomime comprehension and pantomime production. However, 60% of AD patients performed poorly compared to controls on the Gesture Recognition task. Gesture comprehension was highly correlated with a measure of global cognitive impairment (3MS). The data are consistent with the proposed separation of comprehension and action production systems in a cognitive model of praxis. Gesture comprehension may be diffusely distributed and thus preserved in the early stages of AD as in circumscribed lesions of the left parietal lobe.     

Syntactic comprehension deficits in Alzheimer's disease

Syntactic comprehension of German patients with dementia of the Alzheimer type was investigated and compared to healthy controls matched with respect to age, sex, and education. Special attention was directed at syntactic structures, which, in contrast to a language like English, are feasible in a grammatically rich language like German. In a sentence picture matching paradigm, only semantically reversible sentences were used. Syntactic complexity ranged from simple active voice sentences to more complex sentences like center-embedded object relative sentences. In comparison to their controls, patients showed a deficit in nearly all categories. Their performance was not influenced by age, but was heavily influenced by the degree of cognitive impairment. Patients with mild cognitive impairment, as defined by a MMSE score of 20 or higher, showed only slight difficulties in syntactic processing, whereas patients with moderate to severe impairment (MMSE < 20) did not perform above chance limits in most syntactic categories. It appears as though syntactic comprehension is only mildly affected in the early stages of Alzheimer's disease and is rather severely impaired in more advanced stages. In the present report, results are discussed in terms of working memory demands for syntactic processing.     

Visual aiming movements in Alzheimer's disease

This study examined whether AD affects the control of pointing movements. Sixteen older adults with probable AD and 10 age-matched healthy adults pointed at targets varying in size (3 and 7 mm in diameter) and located at three positions (at the midline and 33 degrees to the left and right). Results revealed the patients exhibited longer movement time, lower peak velocity and more time in deceleration, although they exhibited effects of target size and location comparable to the healthy controls. AD, then, would appear to slow down movement without affecting the motor system's ability to respond to task demands.     

Word association in early Alzheimer's disease

The hypothesis that Alzheimer's disease (AD) degrades semantic representations predicts that AD qualitatively alters spontaneous thoughts. In two experiments contrasting free associations to words with strong (e.g., bride-groom) versus weak (e.g., body-leg) associates participants with AD produced less common responses (e.g., bride-pretty) than normal controls but only for words with strong associations, and only on the first (but not on second or third) association response. Furthermore, all participants produced fewer semantically related responses to words with weak associates. Because strong associations should be retrieved more easily than weak associations these results are problematic for retrieval-based accounts of AD. Instead we propose that AD entails a semantic deficit, and that strong associations involve more semantic processing than weak associations (in all speakers).     

Long-term retention of a visually cued poison-based aversion in monkeys.

Retention of a poison-based aversion after a 3-yr. period by 8 vervet monkeys (Cercopithecus aethiops) was found. The color of the visual cue appeared to effect the retention of the aversion.