The clinical overlap between the corticobasal degeneration syndrome and other diseases of the frontotemporal spectrum: three case reports

The corticobasal degeneration syndrome has been suggested to be part of a complex of conditions (including the different subtypes of frontotemporal dementia and progressive supranuclear palsy), which reflect a spectrum of pathological substrates. This concept is supported by the frequent clinical overlap that can be observed among patients diagnosed with these conditions. We report three clinical cases, characterized by the overlap of the clinical features of corticobasal degeneration syndrome with, respectively, nonfluent progressive aphasia, progressive supranuclear palsy and semantic dementia. Current diagnostic criteria emphasize differences in clinical presentation, which probably reflect the preferential location of pathology in the early stages of disease. However, with disease progression, a considerable clinical overlap can be expected among the different syndromes. This concept should be extended not only to the cognitive and behavioural features of the frontotemporal dementia subtypes, but also to the movement disorders of corticobasal degeneration and supranuclear palsy.     

Spatial neglect, Balint-Homes' and Gerstmann's syndrome, and other spatial disorders

Brain-damaged patients with lesion or dysfunction involving the parietal cortex may show a variety of neuropsychological impairments involving spatial cognition. The more frequent and disabling deficit is the syndrome of unilateral spatial neglect that, in a nutshell, consists in a bias of spatial representation and attention ipsilateral to of extrapersonal, personal (ie, the body) space, or both, toward the side of the hemispheric lesion. The deficit is more frequent and severe after damage to the right hemisphere, involving particularly the posterior-inferior parietal cortex at the temporo-parietal junction. Damage to these posterior parietal regions may also impair visuospatial short-term memory, which may be associated with and worsen spatial neglect. The neural network supporting spatial representation, attention and short-term memory is, however, more extensive, including the right premotor cortex. Also disorders of drawing and building objects (traditionally termed constructional apraxia) are a frequent indicator of posterior parietal damage in the left and in the right hemispheres. Other less frequent deficits, which, however, have a relevant localizing value, include optic ataxia (namely, the defective reaching of visual objects, in the absence of elementary visuo-motor impairments), which is typically brought about by damage to the superior parietal lobule. Optic ataxia, together with deficits of visual attention, of estimating distances and depth, and with apraxia of gaze, constitutes the severely disabling Balint-Holmes' syndrome, which is typically associated with bilateral posterior parietal and occipital damage. Finally, lesions of the posterior parietal lobule (angular gyrus) in the left hemisphere may bring about a tetrad of symptoms (left-right disorientation, acalculia, finger agnosia, and agraphia) termed Gerstmann's syndrome, that also exists in a developmental form.     

Switching between goals mediates the attentional blink effect

Humans are fundamentally limited in processing information from the outside world. This is particularly evident in the attentional blink (AB), the impaired ability to identify the second of two targets presented in close succession. We report findings from three experiments showing that the AB is significantly reduced when observers are set to achieve one single goal (reporting combinations of the two targets) instead of separate goals (reporting the two targets). This finding raises questions about the nature of AB, and suggests that processes involved in goal-switching must be taken into account by theories of the AB phenomenon.     

Facilitation and inhibition caused by the orienting of attention in propositional reasoning tasks

In an attempt to study the orienting of attention in reasoning, we developed a set of propositional reasoning tasks structurally similar to Posner's (1980) spatial cueing paradigm, widely used to study the orienting of attention in perceptual tasks. We cued the representation in working memory of a reasoning premise, observing whether inferences drawn using that premise or a different, uncued one were facilitated, hindered, or unaffected. The results of Experiments 1a, 1b, 1c, and 1d, using semantically (1a-1c) or statistically (1d) informative cues, showed a robust, long-lasting facilitation for drawing inferences from the cued rule. In Experiment 2, using uninformative cues, inferences from the cued rule were facilitated with a short stimulus onset asynchrony (SOA), whereas they were delayed when the SOA was longer, an effect that is similar to the “inhibition of return” (IOR) in perceptual tasks. Experiment 3 used uninformative cues, three different SOAs, and inferential rules with disjunctive antecedents, replicating the IOR-like effect with the long SOAs and, at the short SOA, finding evidence of a gradient-like behaviour of the facilitation effect. Our findings show qualitative similarities to some effects typically observed in the orienting of visual attention, although the tasks did not involve spatial orienting.     

Authenticity and the Analytic Process

In this paper we first make a differentiation between phenomena that can be defined as spontaneous and others that can be defined as authentic. We then attempt to present authenticity as a process rather than an outcome. Finally, we try to understand the location of authentic phenomena in the sensorial and pre-symbolic communicative register. We situate authentic phenomena in the register of sensorial and pre-symbolic communication. The authentic process becomes manifest, step by step in the analytic process (Borgogno, 1999), through the vivid iconic and sensorial elements that happen to cross the analytic field. Through two brief clinical vignettes, we seek to document the progression of the analytic process, in one case through the analyst's capacity for rêverie (Bion, 1962; Ogden, 1994, 1997; Ferro, 2002, 2007), and in the other through the sensorial elements with which analyst and patient are able to tune in to each other.     

Bilingual aphasia and language control: A follow-up fMRI and intrinsic connectivity study

In a world that is becoming more multilingual, bilingual aphasia is a clinical problem with a major clinical impact. However, at present we lack causal explanations of the many features of recovery patterns and there is no consensus about the language in which the patient should receive speech therapy. Further advance requires an understanding of the dynamics of recovery. In a novel longitudinal, single-case study, we combine fMRI and dynamic causal modeling to examine the effects of specific language treatment for picture naming on the representation and control of language areas during the course of recovery. Improved performance in the treated language was associated with increased activation in language areas. Consistent with theoretical expectations, causal modeling indicated increased connectedness of the control and language networks for the treated language. This functional approach holds great promise for investigating recovery patterns and the effects of specific language treatment in bilingual aphasic patients.     

Heart rate variability and drawing impairment in hypoxemic COPD

We studied 54 patients with hypoxemic chronic obstructive pulmonary disease (COPD). The Mini Mental State Examination and the Mental Deterioration Battery were used for neuropsychological assessment. Heart rate variability (HRV) was assessed based on 24-h Holter ECG recording. Mann–Whitney test was used to compare HRV parameters of patients performing normally or abnormally on individual neuropsychological tasks. Spearman’s rho was used to investigate the correlations between HRV parameters and neuropsychological scores, indexes of health status or COPD severity. Patients with defective performance at copying drawings with landmarks (CDL) test (N = 23) had lower very low frequency (VLF) power with respect to patients with normal performance (N = 31) (24 h: median 213; interquartile range 120–282 vs. 309; 188–431 ms², p = 0.043; daytime: 202; 111–292 vs. 342; 194–397 ms², p = 0.039). The CDL score correlated with the VLF power (24 h: ρ = 0.27, p = 0.049; daytime: ρ = 0.30, p = 0.028), and the normalized low frequency/high frequency (LF/HF) ratio (24 h: ρ = 0.27, p = 0.05; daytime: ρ = 0.33, p = 0.015). Sympathetic modulation decreased for increasing severity of COPD. In conclusion, drawing impairment correlates with depressed sympathetic modulation in patients with COPD, and both might be indexes of COPD severity.     

Alienating identifications and the psychoanalytic process1

The transmission of psychic life from one generation to the next can result in unconscious, alienating identifications when the parents have not been able to elaborate a process of mourning for their own childhoods. In this article, the author describes the nature of these identifications, constructed around insufficiently symbolized experiences, as revealed during the psychoanalytic process. These unconscious, alienating identifications raise some arduous technical problems for the psychoanalyst as they lead the patient to carry out complex enactments that erase the normal transference markers. The psychoanalyst may then be tempted to resort to pejorative theoretical concepts, such as the death drive. And yet, unknown to the analysand, the insufficiently symbolized psychic elements contain a potential for transformation that may lead to reconstructions and dis‐alienating interpretations. The author distinguishes between alienating identifications and fantasies of identification when the latter transiently appear during the psychoanalytic process. These identification fantasies symbolically register the emotional experience undergone during the analytic sessions and contribute to the integration of insufficiently symbolized psychic elements. These theoretical considerations are fully illustrated by the clinical report of some analytic sessions.