Acute intermittent porphyria in pregnancy

Area of Science:

• Neurology
• Toxicology
• Obstetrics

Background:

• Hyperemesis gravidarum can lead to complications requiring medical intervention.
• Acute intermittent porphyria (AIP) is a rare metabolic disorder that can be triggered by various factors.
• Pregnancy and certain medications can pose risks for individuals with porphyria.

Observation:

• A 27-year-old pregnant woman presented with hyperemesis gravidarum, treated with IV fluids and metoclopramide.
• Following treatment, she developed a severe neuropsychiatric syndrome: acute asymmetrical axonal motor-sensory polyneuropathy, anxiety, depression, irritability, and cognitive deficits.
• Elevated urinary porphyrin precursors were detected, indicative of porphyria.

Findings:

• The patient's symptoms were consistent with an acute porphyria attack.
• Pregnancy, starvation, and metoclopramide were identified as potential precipitating factors.
• Other conditions such as thiamine deficiency, Guillain-Barré syndrome, and obstetric complications were ruled out.

Implications:

• This case highlights the rare but serious association between acute porphyria and pregnancy.
• It underscores the importance of considering medication side effects and metabolic disorders in pregnant patients with unexplained neurological and psychiatric symptoms.
• Prompt diagnosis and management, including drug withdrawal and supportive care, are crucial for maternal and fetal outcomes.