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Posterior reversible encephalopathy syndrome in acute intermittent porphyria

Bi Zhao1, QianQian Wei1, YunHan Wang2

  • 1Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China.

Pediatric Neurology
|July 19, 2014
PubMed

Insights

Acute intermittent porphyria (AIP) can present with symptoms mimicking posterior reversible encephalopathy syndrome (PRES). This case highlights AIP

Area of Science:

  • Pediatric Neurology
  • Metabolic Disorders
  • Neuroimaging

Background:

  • Acute intermittent porphyria (AIP) is a rare inherited metabolic disorder affecting the nervous system.
  • Posterior reversible encephalopathy syndrome (PRES) is a neurological condition with characteristic imaging findings.
  • AIP is seldom diagnosed in children, and its association with PRES is rarely reported.

Observation:

  • A 9-year-old girl presented with symptoms including abdominal pain, confusion, seizures, and visual disturbances post-appendectomy.
  • Clinical presentation included hypertension, tachycardia, nausea, vomiting, constipation, and dark urine.

Findings:

  • Brain MRI revealed parieto-occipital lobe lesions consistent with PRES.
  • Diagnosis of AIP confirmed by elevated urinary porphyrin precursors.
  • Symptoms and MRI lesions resolved with intravenous glucose and supportive care.

Implications:

  • AIP should be considered in children presenting with PRES-like symptoms, especially with abdominal pain and autonomic dysfunction.
  • Early diagnosis and treatment of AIP are crucial for neurological recovery.
  • This case expands the understanding of AIP's neurological manifestations.
Abstract

Keywords:
abdominal painacute intermittent porphyriamagnetic resonance imagingposterior reversible encephalopathy syndrome

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