Young-onset dementia with Lewy Bodies presenting with apathy and alexithymia.

A diagnosis of young-onset dementia can pose a significant challenge for the clinician. We present a young patient with a very unusual presentation of Dementia with Lewy Bodies. The lack of motor symptoms and his marked apathy delayed his diagnosis. His symptoms were thought to be due to depression based on normal structural imaging and the psychiatric nature of his presentation. An extensive work-up was performed. Evidence of a structural neurodegenerative process was provided by the HMPAO-SPECT. Cardiac MIBG confirmed the diagnosis.

Past, present, and prospects: Reflections 40 years on from the selective impairment of semantic memory (Warrington, 1975).

We summarize the main findings and conclusions of Warrington's (1975) paper, The Selective Impairment of Semantic memory, a neuropsychological paper that described three cases with degenerative neurological conditions [Warrington, E. K. (1975). The selective impairment of semantic memory. The Quarterly Journal of Experimental Psychology, 27, 635-657]. We consider the developments that have followed from its publication and give a selective overview of the field in 2014. The initial impact of the paper was on neuropsychological investigations of semantic loss followed some 14 years later by the identification of Semantic Dementia (the condition shown by the original cases) as a distinctive form of degenerative disease with unique clinical and pathological characteristics. We discuss the distinction between disorders of semantic storage and refractory semantic access, the evidence for category- and modality-specific impairments of semantics, and the light that has been shed on the structure and organization of semantic memory. Finally we consider the relationship between semantic memory and the skills of reading and writing, phonological processing, and autobiographical memory.

The role of configurality in the Thatcher illusion: an ERP study.

The Thatcher illusion (Thompson in Perception, 9, 483-484, 1980) is often explained as resulting from recognising a distortion of configural information when 'Thatcherised' faces are upright but not when inverted. However, recent behavioural studies suggest that there is an absence of perceptual configurality in upright Thatcherised faces (Donnelly et al. in Attention, Perception & Psychophysics, 74, 1475-1487, 2012) and both perceptual and decisional sources of configurality in behavioural tasks with Thatcherised stimuli (Mestry, Menneer et al. in Frontiers in Psychology, 3, 456, 2012). To examine sources linked to the behavioural experience of the illusion, we studied inversion and Thatcherisation of faces (comparing across conditions in which no features, the eyes, the mouth, or both features were Thatcherised) on a set of event-related potential (ERP) components. Effects of inversion were found at the N170, P2 and P3b. Effects of eye condition were restricted to the N170 generated in the right hemisphere. Critically, an interaction of orientation and eye Thatcherisation was found for the P3b amplitude. Results from an individual with acquired prosopagnosia who can discriminate Thatcherised from typical faces but cannot categorise them or perceive the illusion (Mestry, Donnelly et al. in Neuropsychologia, 50, 3410-3418, 2012) only differed from typical participants at the P3b component. Findings suggest the P3b links most directly to the experience of the illusion. Overall, the study showed evidence consistent with both perceptual and decisional sources and the need to consider both in relation to configurality.

Discriminating Thatcherised from typical faces in a case of prosopagnosia.

We report data from a prosopagnosic patient (PHD), and aged-matched control participants, from experiments where participants categorised individually presented emotional faces (experiment 1) and Thatcherised (from typical) faces (experiment 2). In experiment 2 participants also discriminated between simultaneously presented Thatcherised and typical faces. PHD was at chance categorising Thatcherised from typical faces. He was, however, able to discriminate between Thatcherised and typical faces, and partially able to categorise emotional faces. The results are discussed in terms of a loss of configural processing but preserved feature processing in PHD. The loss of configural processing impacts his categorisation of Thatcherised and typical faces, and his emotion processing, while his preserved feature processing supports his ability to categorise some emotional faces and his ability to discriminate between Thatcherised and typical faces.

Bilingual aphasia due to spontaneous acute subdural haematoma from a ruptured intracranial infectious aneurysm.

We report a case of spontaneous subdural haematoma due to ruptured intracranial infectious aneurysm, presenting with bilingual aphasia and illustrating differential language recovery. A 62-year-old right-handed bilingual gentleman, with a diagnosis of infective endocarditis, developed headache and became expressively aphasic in the English language. Three days later he was receptively and expressively aphasic in both English and Arabic. Cranial MRI scans showed a left-sided acute subdural haematoma with mass effect and midline shift. Contrast CT brain scans showed an enhancing speck adjacent to the clot and cerebral angiogram confirmed a distal middle cerebral artery aneurysm. He underwent image-guided craniotomy, evacuation of the subdural haematoma and excision of the aneurysm. Histopathological examination was consistent with an infectious intracranial aneurysm. Postoperatively his aphasia did not improve immediately. He had widened pulse pressure due to severe aortic regurgitation, confirmed on echocardiography. He underwent aortic valve replacement and mitral valve repair, following which his aphasia recovered gradually. Initially the recovery of his language was limited to Arabic. About a week later he recovered his English language as well. At 3-year follow-up he is doing well and has no neurological deficits. His aphasia has recovered completely. The present case is unique because of (a) presence of pure subdural haematoma, and (b) the differential susceptibility and recovery of native (L1) and acquired language (L2) in presence of a common pathology. The neurology of language in a bilingual is analysed and possible mechanisms discussed.

Form completion across a hemianopic boundary: behindsight?

Patients with homonymous hemianopia may report the completion of forms that overlap the vertical meridian of their field defects. While previous investigations of "hemianopic completion" have variously attributed to the disorder to inattention, residual vision or unstable fixation, we believe that our investigation has controlled for such potentially confounding factors. We report patient P.O.V. who experienced hemianopic completion in everyday life following a surgical lesion of his left occipital lobe. He showed normal spatial attention and normal spatial orienting: hemianopic completion can therefore occur in the absence of inattention. His completion was retinotopic and affected partial as well as complete forms: his hemianopic completion cannot be attributed to residual visual input or poor fixation. P.O.V.'s completion was also systematically affected by varying stimulus contrast and pattern masking. We argue that while other explanations may be appropriate for different cases, P.O.V.'s hemianopic completion reflects normal "constructive" visual processes and can be attributed to the unconstrained operation of visual routines that are normally involved in the perception of partially occluded forms. As such, this disorder has the potential to shed light on some of the most basic aspects of visual perception.

Probabilistic reasoning in schizophrenia: a comparison of the performance of deluded and nondeluded schizophrenic patients and exploration of possible cognitive underpinnings.

INTRODUCTION: A number of studies have suggested that deluded patients show a "jumping to conclusions" reasoning style on probabilistic reasoning tasks. In order to systematically explore the cognitive underpinnings of this task, we compared deluded and nondeluded patients on a number of experimental manipulations to investigate the role of memory and task pragmatics on performance. METHODS: In Study 1, the performance of deluded and nondeluded schizophrenia patient groups was compared to nonpsychiatric controls on a battery of probabilistic reasoning tests. In Study 2, two variants of the standard "beads in jars" task were compared in order to explore the possible role of working memory load on task performance. RESULTS: In Study 1, there were no significant differences between any of the groups on any of the probabilistic reasoning tasks. In Study 2, we found a significant difference between the two schizophrenic groups and the controls, but no difference in performance between deluded and nondeluded patient groups. The deluded group responded fastest in the memory intensive condition. CONCLUSIONS: Deluded and nondeluded schizophrenic patients perform similarly on probabilistic reasoning tasks and only show the "jumping to conclusions" response pattern under some conditions but not under others. Memory demands may influence the appearance of this pattern of responding in schizophrenia.

Remembering and forgetting of semantic knowledge in amnesia: a 16-year follow-up investigation of RFR.

We report our long-term follow-up investigations of RFR, a post-encephalitic case of very grave anterograde and retrograde amnesia. We also describe the results of quantitative neuroimaging of his brain injury that showed bilateral and severe reduction in the hippocampal formation and medial temporal structures with sparing of left lateral/posterior and right posterior temporal cortex. We established that RFR had a persistent severe anterograde and retrograde amnesia for personal and public events. His personal semantic knowledge was relatively spared for the retrograde period. There was a modest and global reduction in RFR's vocabulary for words acquired in adulthood before he became amnesic but there was no evidence of any retrograde gradient. His retrograde knowledge of people was also without any gradient. Remarkably, there had been no change in the extent of his semantic knowledge across a prolonged re-test interval indicating that the loss of semantic knowledge was stable and likely to have arisen at the time of his initial lesion. RFR also showed evidence of a limited but significant ability to acquire new word meanings and a more restricted capacity for learning about new celebrities. While he was able to demonstrate face and name familiarity for newly famous people, he was unable to provide much semantic detail. RFR's amnesia can be partially explained by contemporary theories that allow for parallel cortical and hippocampal memory systems but is difficult to reconcile in detail with any extant view.

2D but not 3D: pictorial-depth deficits in a case of visual agnosia.

Patients with visual agnosia exhibit acquired impairments in visual object recognition, that may or may not involve deficits in low-level perceptual abilities. Here we report a case (patient DM) who after head injury presented with object-recognition deficits. He still appears able to extract 2D information from the visual world in a relatively intact manner; but his ability to extract pictorial information about 3D object-structure is greatly compromised. His copying of line drawings is relatively good, and he is accurate and shows apparently normal mental rotation when matching or judging objects tilted in the picture-plane. But he performs poorly on a variety of tasks requiring 3D representations to be derived from 2D stimuli, including: performing mental rotation in depth, rather than in the picture-plane; judging the relative depth of two regions depicted in line-drawings of objects; and deciding whether a line-drawing represents an object that is 'impossible' in 3D. Interestingly, DM failed to show several visual illusions experienced by normals (Muller-Lyer and Ponzo), that some authors have attributed to pictorial depth cues. Taken together, these findings indicate a deficit in achieving 3D intepretations of objects from 2D pictorial cues, that may contribute to object-recognition problems in agnosia.

Evidence for a context-sensitive word retrieval disorder in a case of nonfluent aphasia.

We describe a patient (BM) with nonfluent aphasia who presents with sparse, fragmented spontaneous speech but normal or near-normal performance on standard naming tasks. However, more detailed investigation revealed some unusual features to BM's naming: On a task involving repeated naming of a small set of targets, his performance degenerated when the targets were semantically blocked, particularly at fast rates of presentation. This semantic blocking effect was not observed in an analogous wordpicture matching task. Also, it was not present on a task where a set of words had to be named repeatedly in a fixed, predictable sequence. Finally, a fluent aphasic patient who presented with a classic "output" anomia failed to show the semantic blocking and predictability effects. It is suggested that BM suffers from a context-sensitive word retrieval disorder. The disorder is attributed to a difficulty in modulating activation within the lexical network. Implications for nonfluent aphasia, as well as for models of lexical retrieval, are discussed.

Is the mind a cauliflower or an onion? British insights into cognitive organization from the study of abnormal function.

Clinical and normal psychology have had a long tradition of close interaction in British psychology. The roots of this interplay may predate the development of the British Psychological Society, but the Society has encouraged and supported this line of research since its inception. One fundamental British insight has been to consider the evidence from pathology as a potential constraint on theories of normal function. In turn, theories of normal function have been used to understand and illuminate cognitive pathology. This review discusses some of the areas in which clinical contributions to cognitive theory have been most substantial. As with other contributions to this volume, attempts are also made to read the runes and anticipate future developments.