Posterior Reversible Encephalopathy Syndrome Unmasking Acute Glomerulonephritis

Abstract

A 9-year-old girl presented with an acute history of headache, vomiting, abdominal pain, right focal seizures with secondary generalization and altered sensorium. There was a history of fever for a day, at the onset of symptoms. Seizures were focal, involving the right upper limb and angle of the mouth. She had not experienced any visual aura/ hallucination or any other visual problem. On admission, she was found to be drowsy and afebrile, with a blood pressure of 180/140 mmHg. There were no meningeal signs or neurological deficits. Rest of the examination was essentially normal, except for a few papular lesions over her arms and legs, which were ostensibly caused by insect bite allergy. There was no history of oliguria, haematuria or oedema, which proved to be a red herring, and the child was managed as a case of viral meningoencephalitis with raised intracranial tension. She was treated with anti oedema measures, anticonvulsants and supportive measures. The child regained normal sensorium within 12 hours of admission, though the hypertension persisted.At this point, an MRI brain was done [Table/ Fig-1] and it revealed extensive vasogenic oedema involving the parietal, occipital, frontal and temporal areas of the cerebrum. These findings were suggestive of PRES and so, the child was evaluated for hypertension. On probing, we obtained a history of a secondary infection on the skin lesions, two weeks prior to her presentation. Urine microscopy revealed microscopic haematuria and serum C3 levels were significantly low (10 mg/dl, normal range 90 -180), though ASLO was normal. Platelet count and peripheral smears were normal. We also did a lupus work up (ANA, DsDNA), which was negative. Renal artery Doppler, VMA levels and ESR were normal. CSF analysis was not done.The hypertension resolved within 2 days of starting oral nifedipine and frusemide. A repeat MRI which was done after two weeks, [Table/ Fig-2] showed a complete resolution of the oedema. At discharge, the child was off anti hypertensives and she was symptom free. C3 levels normalized after four weeks, further validating the diagnosis of post infectious glomerulonephritis. DisCussionPosterior reversible encephalopathy syndrome (PRES) has been associated with a typical pattern of vasogenic oedema, which involving the white matter and is seen mainly in the parieto occipital areas of the cerebrum. It had been originally described in association with ecclampsia and immunosuppressive therapy. Though this condition has been described earlier under other names, Hinchey et al renamed it as PRES [1].The pathogenesis is still an area of debate, with two diametrically opposite hypotheses. The newer and currently accepted one suggests that when the auto regulatory limit of the blood vessels in the brain are exceeded, as in severe hypertension, then the blood brain barrier breaks down, leading to vasogenic oedema [2,3]. In this case, the significantly high blood pressure (>99th percentile) may have contributed to the extensive vasogenic oedema, which in itself is a poo...

Posterior reversible encephalopathy in an adult patient with poststreptococcal glomerulonephritis

“…Although poststreptococcal glomerulonephritis has been described previously as a very rare association with PRES in children, 3 to our knowledge this is the first published case with PRES clearly associated with a poststreptococcal syndrome in an adult. ► PRES should be part of the differential diagnosis in any patient with new onset of mental status change, blurred vision or seizures regardless of the blood pressure at symptom onset.…”

“…PRES is likely a consequence of an abnormal cerebral autoregulation, with hypertension or relative blood pressure increase, leading to a damage of the blood-brain barrier allowing leakage of blood and fluid into the brain parenchyma. [1][2][3] It predominantly affects the posterior cerebral circulation where the endothelium is believed to be less resistant to high blood pressure. ► PRES is associated with severe high blood pressure, autoimmune diseases, immunosuppressive medications, eclampsia and renal failure.…”

“…Regardless of the aetiology, treatment consists of blood pressure control and aetiology-specific therapy. [1][2][3] …”