Huib de Jong,
Charles C. Obihara
This supplementary information is presented as submitted by the corresponding author. It has not been copy-edited by NTvG.
Posterior reversible encephalopathy syndrome (PRES) or reversible posterior leukoencephalopathy syndrome (RPLS) is a rare complication of a post-streptococcal glomerulonefritis (PSGN) in children.
Its clinical features include headache, nausea, vomiting, decreased state of arousal, confusion, abnormality of visual perception, seizures and even coma. Neuro-imaging is characterized by cerebral edema, often localized in the parieto-occipital region, although other regions of the brain may be involved. We describe the case of an eight-year-old boy who diagnosed with PRES due to a PSGN, and discuss the diagnostic and treatment methods.
An eight-year-old boy is brought into the hospital emergency department with seizures following acute headache, nausea and vomiting. He had been treated in the preceding week with orale antibiotics for a streptococcal tonsillitis. On physical examination he had a normal body temperature, and was hypertensive (blood pressure was 155/117 mmHg). A cerebral CT scan revealed bilateral hypodense lesions in the parieto-occipital lobes. A cerebral MRI scan showed vasogenic cerebral edema in the bilateral parieto-occipital and left frontal lobes. Cerebrospinal fluid chemistry, gram stain, culture and PCR were all normal. Urinanalysis showed proteinuria. Blood levels of urea are elevated. Both serum antistreptolysine titer and anti-DNase B are elevated and complement factor, C3, decreased. Lupus erythematosus antibodies were negative. The seizure was successfully treated with midazolam and valproatic acid, and the hypertension with a calcium channel blocker (amlodipine). Both the blood pressure and intracerebral lesions on MRI-scan normalised within 8 weeks following presentation.
PRES is a rare complication of a PSGN in children. The major symptoms are headache, nausea, vomiting and neurological abnormalities including seizures and coma. The major aim of the treatment is a controlled rapid controlled normalization of the blood pressure. Early recognition of signs and symptoms, rapid diagnosis and adequate treatment of PRES are important for preventing permanent neurological damage or mortality.
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