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Acute intermittent porphyria (AIP) in a patient with celiac disease

Sebastian Nunnemann1, Christoph Uibel1, Petra Budig1

  • 1Department of Neurology, Klinikum Würzburg Mitte gGmbH, Standort Juliusspital, 97070 Würzburg, Germany.

Neurological Research and Practice
|December 16, 2020
PubMed

Insights

Malnutrition in celiac disease can trigger acute intermittent porphyria (AIP), a rare genetic disorder. Early treatment of severe AIP symptoms, including neurological issues, led to significant recovery in this case.

Area of Science:

  • Biochemistry
  • Genetics
  • Internal Medicine

Background:

  • Celiac disease is an autoimmune disorder affecting the small intestine.
  • Acute intermittent porphyria (AIP) is a rare genetic metabolic disorder.
  • AIP attacks can manifest with severe neuropsychiatric and motor symptoms.

Purpose of the Study:

  • To report a unique case of AIP triggered by malnutrition in a patient with celiac disease.
  • To highlight the importance of prompt and comprehensive treatment for severe AIP.
  • To discuss the interplay between celiac disease, malnutrition, and AIP exacerbation.

Main Methods:

  • Case report of an 18-year-old female with celiac disease.
  • Documentation of a severe AIP attack with tetraparesis and respiratory compromise.
  • Treatment regimen including heme arginate, high-dose glucose, and rehabilitation.

Main Results:

  • The patient experienced a severe first attack of AIP.
  • Treatment led to a slow but nearly complete recovery of motor function.
  • This represents the first reported case of AIP triggered by malnutrition secondary to celiac disease.

Conclusions:

  • Malnutrition associated with celiac disease can precipitate severe AIP.
  • Aggressive and timely symptomatic management is crucial for favorable outcomes in severe neurological AIP.
  • This case underscores the need for vigilance in patients with celiac disease at risk for nutritional deficiencies and AIP.

Keywords:
Acute intermittent porphyriaCeliac diseaseNeurological rehabilitationPolyneuropathy

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