A new paradigm: Diagnosis and management of HSCT-associated thrombotic microangiopathy as multi-system endothelial injury

Posterior reversible encephalopathy syndrome [PRES (also known as reversible posterior leukoencephalopathy syndrome)] presents with rapid onset of symptoms including headache, seizures, altered consciousness, and visual disturbance (1,2). It is often—but by no means always—associated with acute hypertension (1,2). If promptly recognized and treated, the clinical syndrome usually resolves within a week (2,3), and the changes seen in magnetic resonance imaging (MRI) resolve over days to weeks (2–4). Chronic kidney disease and acute kidney injury are both commonly present in patients with PRES (4), and PRES is strongly associated with conditions that co-exist in patients with renal disease, such as hypertension, vascular and autoimmune diseases, exposure to immunosuppressive drugs, and organ transplantation. It is therefore important to consider PRES in the differential diagnosis of patients with renal disease and rapidly progressive neurologic symptoms. Posterior reversible encephalopathy syndrome is an increasingly recognized disorder, with a wide clinical spectrum of both symptoms and triggers, and yet it remains poorly understood.

/pdf/posterior-reversible-encephalopathy-syndrome-a-truly-3r99nt4alx.pdf

Posterior reversible encephalopathy syndrome: a truly treatable neurologic illness

A fifth of all strokes and transient ischaemic attacks occur in the posterior circulation arterial territory. Diagnosis can be challenging, in part because of substantial overlap in symptoms and signs with ischaemia in the anterior circulation. Improved methods of non-invasive imaging of the vertebrobasilar arterial tree have been used in recent prospective follow-up studies, which have shown a high risk of early recurrent stroke, particularly when there is associated vertebrobasilar stenosis. This finding emphasises the importance of urgent secondary prevention, and the role of stenting for vertebral stenosis is being investigated.

https://www.thelancet.com/pdfs/journals/laneur/PIIS1474-4422(13)70211-4.pdf

Posterior circulation ischaemic stroke and transient ischaemic attack: diagnosis, investigation, and secondary prevention

Posterior reversible encephalopathy syndrome--Insight into pathogenesis, clinical variants and treatment approaches.

Summary Acute neurological symptoms in pregnant and post-partum women could be caused by exacerbation of a pre-existing neurological condition, the initial presentation of a non-pregnancy-related problem, or a new acute-onset neurological problem that is either unique to or occurs with increased frequency during or just after pregnancy. Pregnant and postpartum patients with headache and neurological symptoms are often diagnosed with pre-eclampsia; however, a range of other causes must also be considered, such as cerebral venous sinus thrombosis and reversible cerebral vasoconstriction syndrome. Precise diagnosis is essential to guide subsequent management. Our ability to differentiate between the specific causes of acute neurological symptoms in pregnant and post-partum patients is likely to improve as we learn more about the pathogenesis of these disorders.

https://www.thelancet.com/pdfs/journals/laneur/PIIS1474-4422(12)70306-X.pdf

Diagnosis of acute neurological emergencies in pregnant and post-partum women

The posterior reversible encephalopathy syndrome is an increasingly recognised disorder. Most patients have several symptoms; seizures are the most frequent, often multiple or status epilepticus. A combination of seizures, visual disturbance and/or headache, in particular, should lead to an early brain MRI to reveal the typical pattern of bilateral hyperintensities on fluid attenuated inversion recovery imaging, predominantly in the parieto-occipital region. There seem to be many possible triggers, including abrupt arterial hypertension, impaired renal function, pregnancy, immunosuppressive therapies and various inflammatory conditions. The clinical outcome is excellent, with recovery within a few days, while the MRI abnormalities resolve much more slowly. Little is known about the best management. Seizures do not normally progress to chronic epilepsy so antiepileptic drugs should be discontinued after about 3 months.

C Roth

Papers

The posterior reversible encephalopathy syndrome: what's certain, what's new?