Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jan 2018
ReviewControversy of posterior reversible encephalopathy syndrome: what have we learnt in the last 20 years?
Over two decades have passed since posterior reversible encephalopathy syndrome (PRES) was first described in 1996. It has becoming increasingly recognised because of improved and more readily available imaging modality. The exact pathophysiological mechanism is not completely understood and remains controversial at present. ⋯ We emphasise the importance of recognising its diagnostic criteria and biomarker, which would be of great relevance to either outcome evaluation or study design. PRES has a favourable prognosis generally, but neurological sequelae and even fatalities can occur, especially in severe forms that might cause substantial morbidity and even mortality, particularly when the syndrome is complicated by intracranial haemorrhage or brain infarction. In this review, the pathophysiology, approach to diagnosis, some controversies as to the prognosis, as well as the future research direction of PRES are described.
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J. Neurol. Neurosurg. Psychiatr. · Jan 2018
Longitudinal diffusion imaging across the C9orf72 clinical spectrum.
Discrepancies between diffusion tensor imaging (DTI) findings and functional rating scales in amyotrophic lateral sclerosis (ALS) may be due to symptom heterogeneity, particularly coexisting cognitive-behavioural dysfunction affecting non-motor regions of the brain. Carriers of expansion mutations in the C9orf72 gene, whose motor and cognitive-behavioural symptoms span a range from ALS to frontotemporal dementia, present an opportunity to evaluate the relationship between symptom heterogeneity and DTI changes. ⋯ Discrepancies between DTI findings and clinical measures of disease severity in ALS may partly be accounted for by cognitive-behavioural deficits affecting extramotor white matter tracts. Both ALSFRS-R and King's stage correlated with corticospinal DTI measures. Group-level DTI changes could be detected over 6 months.