Clinicopathological co-occurrence of Fahr’s disease and dementia with Lewy bodies

We present the clinicopathological findings of a case of combined Fahr’s disease (FD) and dementia with Lewy bodies (DLB), associated with a novel pathogenic mutation. The patient presented with visual hallucinations, fluctuating confusion and parkinsonism, leading to a presumptive diagnosis of DLB. CT scan showed extensive bilateral parenchymal calcifications, suggestive of FD. DNA sequencing identified a novel missense variant (c.92A>T p.(Asn31Ile)) in the SLC20A2 gene, a gene known to be associated with FD. This change has not been previously recorded in genetic repositories, and in silico analyses classified it as likely to be disease-causing. The patient died aged 77, four years after symptom onset. Neuropathological examination revealed, macroscopically and microscopically, extensive calcification in the striatum, globus and cerebellar white matter. There was also neuronal loss in the substantia nigra and residual neurones contained alpha-synuclein-positive Lewy bodies. The neuropathology was therefore consistent with DLB and FD. A literature review identified 3 other cases of co-existing Fahr’s and Lewy body pathology, thus the frequency of dual pathology (44%) is higher than expected by random association. Further studies are needed to determine whether alpha-synucleinopathy is linked mechanistically to FD and/or represents a phenotypic subtype

Recollection-based memory in frontotemporal dementia: Implications for theories of long-term memory

It has been convincingly demonstrated that patients with semantic dementia (the temporal variant of frontotemporal dementia) can show intact recognition memory for pictorial stimuli. As yet, the contribution made by recollective processes to this ability and the status of associated neural regions have not been investigated in the disease. Here, we used both a source monitoring paradigm and an associative memory test to evaluate the ability of patients with semantic dementia to use recollection‐based memory processes, and a volumetric MRI technique to assess the extent of atrophy in the hippocampus. Although some patients showed impaired source and associative memory, many performed as well as control participants. Importantly, status of semantic knowledge, as measured by tests of comprehension and production, did not predict recollection‐based memory ability. There was no significant positive correlation between recollection and volume of the hippocampus; instead, both source discrimination and associative memory correlated highly with performance on a battery of frontal lobe tests. Consistent with the view that damage to the prefrontal cortex might influence recollection performance, patients with the frontal variant of frontotemporal dementia, with atrophy largely confined to the frontal lobes, all performed at floor level on source discrimination. These results provide further compelling evidence in favour of the multiple input model of long‐term memory and highlight the role of frontal lobe systems in recollection‐based memory

Listening to your heart: How interoception shapes emotion experience and intuitive decision making

Theories proposing that how one thinks and feels is influenced by feedback from the body remain controversial. A central but untested prediction of many of these proposals is that how well individuals can perceive subtle bodily changes (interoception) determines the strength of the relationship between bodily reactions and cognitive-affective processing. In Study 1, we demonstrated that the more accurately participants could track their heartbeat, the stronger the observed link between their heart rate reactions and their subjective arousal (but not valence) ratings of emotional images. In Study 2, we found that increasing interoception ability either helped or hindered adaptive intuitive decision making, depending on whether the anticipatory bodily signals generated favored advantageous or disadvantageous choices. These findings identify both the generation and the perception of bodily responses as pivotal sources of variability in emotion experience and intuition, and offer strong supporting evidence for bodily feedback theories, suggesting that cognitive-affective processing does in significant part relate to “following the heart.