Long-term study of the cognitive profile of Moyamoya Disease in adults.

Moyamoya Disease (MMD) is a rare cerebrovascular disorder which can have significant cognitive consequences. The aim of the current study was to describe comprehensively the domain-specific cognitive profile of adult patients with MMD and to assess whether this changes in the absence of recurrent stroke over long-term follow-up. Comprehensive neuropsychological assessment covering seven cognitive domains was conducted on 61 adult patients with MMD at baseline and then at up to 3 further time points during follow up (median=2.

Lifespan normative data (18-89 years) for Raven's Advanced Progressive Matrices Set I.

Raven's Advanced Progressive Matrices (APM) Set I is a validated and brief test of fluid intelligence, ideal for use in busy clinical settings. However, there is a dearth of normative data allowing an accurate interpretation of APM scores. To address this, we present normative data from across the adult lifespan (18-89 years) for the APM Set I.

Primary progressive aphasia: ReADing the clinical GRANularity.

Primary progressive aphasia remains a diagnostic challenge despite (or even because of) the increasing availability of ancillary tests and biomarkers. We present a 67-year-old man with apparently sporadic logopenic aphasia and positive Alzheimer biomarkers who was subsequently found also to have a pathogenic mutation in the progranulin gene. This was signalled by early atypical features (mild expressive agrammatism and behavioural change, rapid clinical deterioration) around the core logopenic aphasia syndrome.

Neuropsychological and neuroimaging characteristics of classical superficial siderosis.

Objective: To define the neuropsychological and neuroimaging characteristics of classical infratentorial superficial siderosis (iSS), a rare but disabling disorder defined by hemosiderin deposition affecting the superficial layers of the cerebellum, brainstem and spinal cord, usually associated with a slowly progressive neurological syndrome of deafness, ataxia and myelopathy.

Methods: We present the detailed neuropsychological and neuroimaging findings in 16 patients with iSS (mean age 57 years; 6 female).

Results: Cognitive impairment was present in 8/16 (50%) of patients: executive dysfunction was the most prevalent (44%), followed by impairment of visual recognition memory (27%); other cognitive domains were largely spared.

Comfort Always: The Importance of Providing Psychological Support to Neurology Staff, Patients, and Families During COVID-19.

Background: Although the impact of COVID-19 disruption on healthcare staff is increasingly understood, there has been no discussion of how it affects neurological patients and their families. This study sought to understand the impact of COVID-19 on staff, patients and families.

Methods: The Department of Neuropsychology at the National Hospital for Neurology and Neurosurgery established three new support services for staff, patients and families.

Factors contributing to the distress, concerns, and needs of UK Neuroscience health care workers during the COVID-19 pandemic.

COVID-19 research from China suggests health care workers are at risk of distress, have specific concerns, and need support. It remains unknown whether findings are applicable to UK health care staff and whether psychological support based on generic approaches is effective. We administered an online survey at a leading neuroscience hospital in the UK to examine how individual staff characteristics contribute to distress, concerns, and interventions most valued during the COVID-19 pandemic.

Cognitive estimation: Performance of patients with focal frontal and posterior lesions.

The Cognitive Estimation Test (CET) is a widely used test to investigate estimation abilities requiring complex processes such as reasoning, the development and application of appropriate strategies, response plausibility checking as well as general knowledge and numeracy (e.g., Shallice and Evans, 1978; MacPherson et al.

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.

Are multiplication facts implemented by the left supramarginal and angular gyri?

A patient with presumed cerebral vasculitis showed preserved single digit multiplication facts and impaired single digit subtraction facts. Her ability to comprehend and manipulate numerical quantities was intact. Detailed analysis of her MRI-scan revealed a lesion involving the left parietal lobe including the supramarginal gyrus up to the intraparietal sulcus and extending posteriorly to involve part of the angular gyrus.