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Q1: What is the term used to describe the MRI brain appearance in fig 1 (see p 53)?
Reversible posterior leucoencephalopathy syndrome (RPLS).
Q2: Give the underlying cause of the brain appearance in this case
Q3: Give three other causes of this condition
(1) Drug induced reports of association with cisplatin, cyclosporin, tacrolimus, and intravenous immunoglobulin.
(2) Associated with eclampsia.
(3) Associated with thrombotic thrombocytopenic purpura.
Q4: What is the likely cause of the fundal appearance in fig 1 (see p 53)?
Q5: What other investigations would you order?
Urinary vanillylmandelic acid measurement and ultrasound of the abdomen to exclude renal artery stenosis or a renal/adrenal mass.
Reversible changes of the white matter on computed tomography of the brain in hypertensive encephalopathy has been recognised for some time,1 however the term RPLS was first coined by Hinchey and colleagues in 1996.2 The condition is also seen secondary to the toxaemia of pregnancy, thrombotic thrombocytopenic purpura, or after the use of some immunosuppressive or chemotherapeutic agents.2 The most common clinical symptoms recognised are headache, alteration of alertness, seizures, vomiting, and abnormalities of visual perception. The latter includes blurring of vision, hemianopia, visual neglect, and cortical blindness. Diagnosis in this case had been delayed as, being a life long migraineur, headache and visual loss (albeit temporary) were not unusual symptoms.
The commonest change on neuroimaging is oedema of the white matter primarily of the parieto-occipital lobes, although involvement of other areas of the brain and brainstem has also been reported.3 The pathogenesis is thought to involve areas of cerebral focal vasodilatation and vasoconstriction due to sudden elevation of arterial blood pressure exceeding the autoregulatory capacity of the brain vasculature. This ultimately results in breakdown of the blood-brain barrier and fluid transudation most marked in highly myelinated areas.
In the context of this case, it is worth noting the association of migraine with stroke. Migraine headache can be an independent risk factor for stroke (risk of ischaemic stroke can be increased threefold in migrainous European females of childbearing age4) or can occur in association with genetic syndromes causing stroke (for example, MELAS [mitochondrial encephalomyelopathy, lactic acidosis, and stroke-like episodes], familial hemiplegic migraine and CADASIL [cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy]). Migraine, as well as tension headache, can also occur secondary to ischaemic or haemorrhagic stroke (especially in the context of a posterior circulation event5). In the case history described here, the absence of a homonymous hemianopia type field defect and, in addition, neuroimaging evidence of bihemispheric involvement, would make either of these two migrainous entities unlikely.
This case illustrates the importance of fundoscopy and blood pressure measurement in all cases of headache, including those with a typical history of migraine, and furthermore highlights the complex association between migraine and intracerebral vascular events.
Reversible posterior leucoencephalopathy syndrome.