To study presentations and outcome of posterior reversible encephalopathy syndrome in children, we retrospectively analyzed 14 patients admitted to our pediatric intensive care unit. We further assessed 94 additional pediatric cases from a systematic review. Our patients had a mean age of 11.6 years. Their precipitating factors were hypertension (100%), immunosuppressants (71%), antineoplastic agents (21%), and hemodialysis (14%). Initial neurologic manifestations included seizures (100%), mental change (100%), headache (79%), and visual disturbance (57%). After prompt diagnosis by magnetic resonance imaging (MRI) with intensive management, all patients had complete clinical recovery with subsequent radiologic resolution. Systemic literature review indicated that seizures (90%), hypertension (85%), and atypical neuroimaging findings (80%) are common presentations in childhood posterior reversible encephalopathy syndrome. We conclude that in children presenting with seizures and hypertension, a pediatric neurologist should consider posterior reversible encephalopathy syndrome within a comprehensive differential diagnosis of acute encephalopathy. Early recognition and intensive care are essential to ensure complete neurologic recovery in children with posterior reversible encephalopathy syndrome.
We characterized a cohort of patients with posterior reversible encephalopathy syndrome with spinal cord involvement. We encountered 2 children and identified an additional 19 patients from the internet databases. Of the 21 patients analyzed, 8 were children. The mean peak systolic blood pressure in adults was significantly higher than in children (221.8 ± 14.3 vs 191.4 ± 31.3 mm Hg; P < .01). Regardless of age, the most common clinical symptom was headache (90%) and the least common clinical symptom was seizures (28%). Atypical neuroimaging was more common in children (63%) than in adults (8%). Abnormal cerebrospinal fluid results were frequently found in children (83%). All children recovered uneventfully, but 3 adults had sequelae. A broader clinicoradiologic spectrum makes the diagnosis of children more complex than in adults. Awareness of the atypical features with a meticulous management of hypertension is imperative to avoid unnecessary invasive workups and to achieve an uneventful recovery.
Vascular wall injuries account for up to 80% of childhood strokes, excluding emboli of cardiac origin. Transient cerebral arteriopathy is a recently described entity that is increasingly recognized as an important cause. The cerebral arterial wall is thought to be affected by an inflammatory process related to certain infections. The authors report a 2.5-year-old girl with sudden left hemiplegia and aphasia. The neuroimaging showed occlusion of the right middle cerebral artery and ischemic damages. Laboratory revealed positive cytomegalovirus immunoglobulin M and G in cerebrospinal fluid and in early and late sera. Treatment with ganciclovir, anticytomegalovirus immunoglobulin, and prednisolone, followed by oral aspirin, resulted in clinical improvement. The follow-up neuroimaging showed stabilization of the arterial lesions without residual stenosis. To our knowledge, this is the first report of a cytomegalovirus-associated transient cerebral arteriopathy in an immunocompetent child. Our report demonstrates the propensity for cytomegalovirus to be involved in pediatric cerebral vascular disease.
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