Posterior reversible encephalopathy syndrome: a report of a case with atypical features

Abstract

SummaryWe report a case of a young woman presenting with profound depression of consciousness and intra-uterine death in the late stages of an unbooked pregnancy. She proceeded to develop features of cardiovascular, renal, hepatic and haematological failures. The patient was challenging to manage in view of uncertainty regarding the underlying cause, and required multidisciplinary consultation. A diagnosis was subsequently made of posterior reversible encephalopathy syndrome in the context of pre-eclampsia. We review the typical presentation and wide-ranging associations of this recently described clinico-neuroradiological syndrome, and look at how appropriate management may lead to rapid resolution of its often life-threatening features. We highlight the importance to anaesthetists and critical care physicians of recognising even atypical cases such as this one in view of key differences in management from similarly presenting conditions. Posterior reversible encephalopathy syndrome (PRES) presents with a variety of neurological features which, although devastating, are potentially reversible on prompt recognition and institution of appropriate treatment [1], but clinicians often fail to suspect it [2]. Delayed diagnosis is frequent and can lead to long-term neurological disability. We describe a case presenting as coma and intra-uterine death in the late stages of an unbooked pregnancy. This patient was challenging to manage in view of uncertainty regarding the diagnosis and particularly serious features, and required consultation and co-ordination between obstetricians, anaesthetists, intensive care physicians, radiologists, neurosurgeons and neurologists. Case reportA 20-year-old woman was brought to a maternity hospital by ambulance having been found unconscious by her flatmates. On arrival, she was unable to maintain her own airway. An oral airway was inserted. Her respiration was laboured with a rate of 10 breaths.min . The blood pressure was 220 ⁄ 120 mmHg. Capillary refill time measured over the sternum was prolonged at 5 s. There was marked generalised oedema. The Glasgow Coma Score was 4 out of 15 (Motor 1, Eyes 1, Verbal 2). Her pupils were 5 mm in diameter, equal, and reactive. Normal tone and tendon jerk reflexes were present in both upper and lower limbs. Inspection of the abdomen revealed a third trimester pregnancy (of which her friends were unaware), and contractions of early labour. Marks on her thigh were consistent with several hours lying on the floor. Vaginal examination showed that the cervix was dilated to 2 cm with fresh meconium draining. Core temperature was elevated at 38.5°C.Initial arterial blood gas analysis showed pH 7.21, P a CO 2 6.9 kPa, P a O 2 37.0 kPa and standard base excess )6.5 mmol.l . Ultrasonography revealed a fetus with no heartbeat. Urinalysis showed ‡ 20 g.dl )1 proteinuria. A working diagnosis was made of severe preeclampsia or eclampsia with an unwitnessed seizure. Possible aetiologies of the ongoing coma were considered

Unusual presentation of Takayasu′s arteritis as posterior reversible encephalopathy syndrome

“…[78] Most cases of PRES are described in conditions associated with an abrupt increase in blood pressure. [9–11] The seizures are usually of generalized tonic-clonic type and status epilepticus occurs in more than half of the cases. [910] They may be preceded by visual auras and visual hallucinations, consistent with occipital lobe seizures.…”

“…[9–11] The seizures are usually of generalized tonic-clonic type and status epilepticus occurs in more than half of the cases. [910] They may be preceded by visual auras and visual hallucinations, consistent with occipital lobe seizures. [10] TA is the third most common cause of childhood vasculitis in the world.…”

“…[1114] The relative paucity of sympathetic innervation in the posterior brain results in increased susceptibility to hyperperfusion and vasogenic edema during acute blood pressure elevations. [910] The lesions of PRES are best visualized with MR imaging with FLAIR sequence. T2 weighted MR images, at the height of symptoms, characteristically show diffuse hyperintensity selectively involving the parietooccipital white matter.…”

Posterior Reversible Encephalopathy Syndrome in an Eclamptic Patient After Cardiac Arrest; Case Report and Literature Review

“…Rapid diagnosis and treatment are important in the prevention of secondary complications such as intracranial bleeding, status epilepticus and cerebral infarction that may lead to mortality and morbidity (5).…”

Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

“…These, although potentially devastating, are reversible on prompt diagnosis and appropriate treatment. Delayed diagnosis can lead to long-term neurological disability [2]. A relatively symmetrical pattern of involvement, typically in the subcortical white matter and occasionally in the cortex of the posterior circulation area of the brain is evident on magnetic resonance imaging (MRI) studies [1].…”