0000000000012592
AUTHOR
Peter Rudge
The cognitive profile of prion disease: a prospective clinical and imaging study
Objectives Prion diseases are dementing illnesses with poorly defined neuropsychological features. This is probably because the most common form, sporadic Creutzfeldt-Jakob disease, is often rapidly progressive with pervasive cognitive decline making detailed neuropsychological investigation difficult. This study, which includes patients with inherited, acquired (iatrogenic and variant) and sporadic forms of the disease, is the only large-scale neuropsychological investigation of this patient group ever undertaken and aimed to define a neuropsychological profile of human prion diseases. Methods A tailored short cognitive examination of all of the patients (n = 81), with detailed neuropsycho…
Recollection and familiarity in dense hippocampal amnesia: A case study
In the amnesia literature, disagreement exists over whether anterograde amnesia involves recollective-based recognition processes and/or familiarity-based ones depending on whether the anatomical damage is restricted to the hippocampus or also involves adjacent areas, particularly the entorhinal and perirhinal cortices. So far, few patients with well documented anatomical lesions and detailed assessment of recollective and recognition performance have been described. We report a comprehensive neuroanatomical assessment and detailed investigation of the anterograde memory functions of a previously described severe amnesic patient (VC). The results of four previously published neuroradiologic…
Cognitive and social impairments in patients with superficial siderosis
Superficial siderosis of the CNS is a rare condition, caused by deposition of haemosiderin in the superficial layers of the CNS due to repeated chronic subarachnoid or intraventricular haemorrhage. Typically, the hindbrain structures, especially the cerebellum, are most affected. There is a surprising lack of studies investigating in detail the behavioural functioning of patients with such a condition. In this study, we document for the first time the cognitive, social and emotional processing of six patients with a confirmed clinical diagnosis of superficial siderosis. They were aged between 40 and 62 years, with a mean age of 50.2 years; four were male. We administered a comprehensive bat…
Distinct neuropsychological profiles correspond to distribution of cortical thinning in inherited prion disease caused by insertional mutation
Background The human prion diseases are a group of universally fatal neurodegenerative disorders associated with the auto-catalytic misfolding of the normal cell surface prion protein (PrP). Mutations causative of inherited human prion disease (IPD) include an insertion of six additional octapeptide repeats (6-OPRI) and a missense mutation (P102L) with large families segregating for each mutation residing in southern England. Here we report for the first time the neuropsychological and clinical assessments in these two groups. Method The cognitive profiles addressing all major domains were obtained for 26 patients (18 6-OPRI, 8 P102L) and the cortical thickness determined using 1.5T MRI in …
Does the left inferior parietal lobule contribute to multiplication facts?
We report a single case, who presents with a selective and severe impairment for multiplication and division facts. His ability to retrieve subtraction and addition facts was entirely normal. His brain lesion affected the left superior temporal and to lesser extent in the left middle temporal gyri and the left precentral gyrus extending inferiorly to the pars opercularis of the left frontal lobe. Interestingly, the left supramarginal and angular gyri (SMG/AG) were spared. This finding realised a double dissociation with a previously reported patient, who despite lesions in the SMG/AG did not have a multiplication impairment (van Harskamp et al., 2002). The previously suggested crucial role …