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Acute Intermittent Porphyria (AIP) is one of several disorders that arise from enzymatic derangements in the biosynthetic pathway of the heme molecule. AIP is the most common of the acute porphyrias worldwide, with an estimated prevalence of approximately 5 per 100,000 people [1]. Specifically, AIP results from a defective copy in one of the two genes for Porphobilinogen (PBG) deaminase, leading to the inadequate synthesis of the normal enzyme. Typically, patients will be symptom-free until a precipitating factor causes increased transit through the porphyrin metabolic pathway, resulting in accumulation in Aminolevulinic Acid (ALA) and Porphobilinogen (PBG) (Figure 1) [2-4]. AIP is the second most common porphyria but can have variable expression making diagnosis difficult [5]. We describe a case of acute intermittent porphyria that presented with non-specific lower back pain and progressive acute hyponatremia complicated by seizure, acute encephalopathy, tachycardia, and hypertension.

Severe Acute Hyponatremia as an Initial Presentation of Acute Intermittent Porphyria Triggered by a Subdermal Etonogestrel Implant