Cognitive Testing Battery: differences among groups with dementia, Huntington's disease, and controls

This study evaluated a new test for dementia, the Cognitive Testing Battery, used for over six years in outpatient clinics for aging-related disorders. This battery was developed to be brief, easy to administer, and to provide useful feedback as a cognitive profile for clinicians, patients, and caregivers. 489 participants were tested during a 5-yr. period. Multidisicplinary teams diagnosed these patients with dementia, depression, or Huntington's Disease. A control group was also included. Groups were then compared on the 10 key subscales that subserve dementia populations. Analysis showed there were significant differences among the groups on all measures and that selected scales differentiated the groups. The total score maximally differentiated the groups, and the dementia group was most different from the other groups.     

Perceived trauma: its etiology and treatment

Female adolescents in treatment for emotional disturbances are often diagnosed as suffering from borderline personality disorder. The usual treatment plan for such a diagnosis is based upon limit setting and boundary reinforcement. When this fails to produce results, the diagnosis comes into question. The present paper investigated 85 female adolescents who were being treated for problems associated with differentiation and emancipation in an inpatient setting. A treatment variation was introduced which suggested that these females were more likely to be suffering from a variety of post-traumatic stress disorder (PTSD). When their ritualistic behavioral repetitions were addressed by cognitive behavioral approaches, the behavior decreased. The struggle with boundary issues and references to the historical context of misery and deprivation became less frequent when the focus shifted to current behavior. Two groups emerged through the analysis of data. No difference in behavior or response to treatment could be established, but one group lacked a specific troubling experience which is essential for a diagnosis of PTSD. Maladaptive social behavior (e.g., splitting and projection) were accepted as confounding factors associated with cognitive style rather than "borderline" symptoms. With the new approach, recidivism decreased 14% and the number of outbursts diminished 10% to 12%. The altered treatment approach confirmed the hypothesis that these adolescents were experiencing the effects of trauma (actual or perceived) rather than some disabling personality disorder.     

Cognitive functions and corticostriatal circuits: insights from Huntington's disease

The basic mechanisms of information processing by corticostriatal circuits are currently a matter of intense debate amongst cognitive scientists. Huntington's disease, an autosomal-dominant neurogenetic disorder characterized clinically by a triad of motor, cognitive, and affective disturbance, is associated with neuronal loss within corticostriatal circuits, and as such provides a valuable model for understanding the role of these circuits in normal behaviour, and their disruption in disease. We review findings from our studies of the breakdown of cognition in Huntington's disease, with a particular emphasis on executive functions and visual recognition memory. We show that Huntington's disease patients exhibit a neuropsychological profile that shows a discernible pattern of progression with advancing disease, and appears to result from a breakdown in the mechanisms of response selection. These findings are consistent with recent computational models that suggest that corticostriatal circuits compute the patterns of sensory input and response output which are of behavioural significance within a particular environmental context.     

Destination and source memory in Huntington's disease

Destination memory refers to the recall of the destination of previously relayed information, and source memory refers to the recollection of the origin of received information. We compared both memory systems in Huntington's disease (HD) participants. For this, HD participants and healthy adults had to put 12 items in a black or a white box (destination task), and to extract another 12 items from a blue or a red box (source task). Afterwards, they had to decide in which box each item had previously been deposited (destination memory), and from which box each item had previously been extracted (source memory). HD participants showed poorer source as well as destination recall performance than healthy adults in the proposed tasks. Correlation analysis showed that destination recall was significantly correlated with episodic recall in HD participants. Destination memory impairment in HD participants seems to be considerably influenced by their episodic memory performance.     

Depression and Suicidal Ideation After Predictive Testing for Huntington's Disease: A Two-Year Follow-up Study

The present study reports a two-year follow-up of psychological effects of predictive testing for Huntington's disease. Questionnaires assessing depression, general health, well-being, self injurious behavior, life satisfaction, and lifestyle were completed by 35 carriers and 58 non-carriers before the predictive test, and 2, 6, 12, and 24 months afterwards. Both carriers and non-carriers showed high suicidal ideation before the predictive testing. Depression scores and frequency of suicidal thoughts increased for carriers, compared to non-carriers, over time. There were no differences regarding life satisfaction or life style between carriers and non-carriers. Predictive testing was beneficial in reducing overall ill-health symptoms and increasing well-being for those initially expressing concern about Huntington's disease. The importance of assessing suicidal ideation and of continuing to provide adequate support is emphasized.     

Craving,longing, denial,and the dangers of change: clinical manifestations of greed

Greed is the unrelenting and unrealistic search for all the good an object has to offer and, via identification, all the good one can produce and provide. In phantasy, and sometimes in the patient's early developmental environment, the object and the ego demand more from each other than either have to give. Some patients cannot contain their urge to possess all and to be all, so it becomes a part of the interpersonal and psychological relationship with the analyst rather quickly. These patients feel something is owed to them, and they demand to be fed immediately. Other patients try and hide these greedy phantasies by being the opposite of greedy. They strive to be independent and charitable, while having great conflict over deeper desires to be dependent and in possession of an idealized giving object, an all-providing breast. Case material was used to explore these ideas.     

CT scan and neuropsychological correlates of Alzheimer's disease and Huntington's disease

"Cortical" and "subcortical" dementia syndromes differ in areas of primary neuropathology and clinical characteristics. Conventional CT scan interpretation, visual inspection of pictures, has not been useful in studying dementia. Recent studies of the digitally stored CT attenuation values have found systematic variations with normal aging and aphasia subtypes. In this study of numerical CT scan information in four Alzheimer's Disease and two Huntington's Disease patients, a double dissociation of frontal and temporal lobe density values was found, and a significant correlation was found between left temporal lobe density and verbal ability measures in the Alzheimer's Disease patients.     

Language deficits in pre-symptomatic Huntington's disease: evidence from Hungarian

A limited number of studies have investigated language in Huntington’s disease (HD). These have generally reported abnormalities in rule-governed (grammatical) aspects of language, in both syntax and morphology. Several studies of verbal inflectional morphology in English and French have reported evidence of over-active rule processing, such as over-suffixation errors (e.g., walkeded) and over-regularizations (e.g., digged). Here we extend the investigation to noun inflection in Hungarian, a Finno-Ugric agglutinative language with complex morphology, and to genetically proven pre-symptomatic Huntington’s disease (pre-HD). Although individuals with pre-HD have no clinical, motor or cognitive symptoms, the underlying pathology may already have begun, and thus sensitive behavioral measures might reveal already-present impairments. Indeed, in a Hungarian morphology production task, pre-HD patients made both over-suffixation and over-regularization errors. The findings suggest the generality of over-active rule processing in both HD and pre-HD, across languages from different families with different morphological systems, and for both verbal and noun inflection. Because the neuropathology in pre-HD appears to be largely restricted to the caudate nucleus and related structures, the findings further implicate these structures in language, and in rule-processing in particular. Finally, the need for effective treatments in HD, which will likely depend in part on the ability to sensitively measure early changes in the disease, suggests the possibility that inflectional morphology, and perhaps other language measures, may provide useful diagnostic, tracking, and therapeutic tools for assessing and treating early degeneration in pre-HD and HD.     

Associative encoding and retrieval in Alzheimer's and Huntington's disease

The associative encoding and retrieval abilities of patients with dementia of the Alzheimer type (DAT) and patients with Huntington's disease (HD) were investigated using an encoding specificity paradigm. When compared to age- and education-matched intact control subjects, both patient groups were severely (and equally) impaired on overall memory for word lists. However, the HD and DAT patients showed differential improvement in recall performance with the introduction of associated cues during stimulus presentation and recall. Although the HD patients, like intact subjects, were able to benefit from semantic retrieval cues (strong and weak) which were present during input, the performance of the patients with DAT improved only with the introduction of strong cues at output, regardless of whether the cues were present or absent during initial presentation. These findings suggest that patients with DAT failed to encode the semantic relationship between the to-be-recalled and cue words and simply generated free associations to the cue words during retrieval. Similarities between the performances of the patients with DAT and alcoholic Korsakoff patients are noted and discussed with regard to the neuropathological basis of the disorders.     

Neurolinguistic features of spontaneous language production dissociate three forms of neurodegenerative disease: Alzheimer's, Huntington's, and Parkinson's

An analysis of the temporal (prospective) form (silent and filled hesitations, repetitions, incomplete phrases, context-related comments, interjections), syntactic form, and lexical (retrospective) form (verbal deviations, open and closed class phrases) of spontaneous language production of early and middle stage Alzheimer's, Huntington's, and Parkinson's patients was made. Results showed that the language structure was disrupted in each disease, but in different ways. Temporal interruptions of varying types were frequent in the language of Alzheimer's and Huntington's Disease patients; only long-duration silent hesitations were frequent in Parkinson's language samples. Syntactic complexity was reduced in Huntington's Disease. Verbal paraphasias were found in both the language of Alzheimer's patients, as well as moderately advanced Huntington's patients. Closed class phrases were predominant in the language of Alzheimer's patients and Huntington's patients, and open class phrases in the language of Parkinson's patients. Taken together, the results suggest that (1) there is a unique neurolinguistic profile for spontaneous language production for each neurodegenerative disease, (2) pathology of the neostriatum disrupts syntactic organization, (3) adaptive strategies are used to cope with verbal and speech-motor difficulties, and (4) adaptive strategies fail to be effective with increasing disease severity.     

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.     

Pictorial priming and cued recall in Alzheimer's and Huntington's disease

A picture fragment test was used to compare the priming and cued recall performances of patients with Huntington's disease (HD), patients with dementia of the Alzheimer type (DAT), and neurologically intact normal control (NC) subjects. On the pictorial priming test, subjects were asked to say "the first thing you think of" when shown incompletely drawn pictures, half of which were previously exposed to the subject during an unrelated naming task. Normal controls and HD patients, but not DAT patients, demonstrated similar increases in their ability to identify fragmented versions of previously seen pictures relative to novel pictures. These results are consistent with the previously observed pattern of preserved and impaired verbal priming ability in HD and DAT patients, respectively. The NC subjects, as expected, also demonstrated better performance on the cued recall than on the priming version of the picture fragment test, whereas the HD patients evidenced the opposite relationship on these two tasks and DAT patients were found to be equally impaired on both tests. This finding provides further support for the notion that HD patients' memory impairment is characterized primarily by an inability to initiate systematic retrieval strategies.