Acute Intermittent Porphyria With Seizure and Paralysis in the Puerperium

Yi-Kong Keung, MD, Division of Hematology/Oncology, Temduang Chuahirun, MD, Department of Internal Medicine, and Everardo Cobos, MD, Division of Hematology/Oncology, Texas Tech University Health Sciences Center, Lubbock.

J Am Board Fam Med. 2000;13(1) 

In This Article

Introduction

Porphyrias are a group of metabolic disorders, usually genetic in origin, secondary to deficiencies of various enzymes involved in the heme biosynthetic pathways. They are usually classified into hepatic and erythropoietic types based on the major sites of the porphyrin production. It is more clinically useful, however, to classify this disorder into acute and nonacute types based on the clinical findings. Acute type includes the acute intermittent porphyria, variegate porphyria, and hereditary coproporphyria. The nonacute type includes the porphyria cutanea tarda and erythropoietic porphyrias.

Acute intermittent porphyria is the most severe form of the disease, with gastrointestinal and neuropsychiatric manifestations, such as abdominal pain, vomiting, peripheral neuropathy (motor, sensory, and autonomic), hypertension, seizures, depression, psychosis, and so on. Pregnancy complicating a known diagnosis of porphyria has been reported as early as in the 1950s. A first attack of acute porphyria with pregnancy is rare, however.[1,2,3,4,5,6,7,8,9,10,11] This disease can be a diagnostic challenge to obstetricians and family physicians. It is important that physicians are alert to porphyria so they can make this diagnosis promptly, because proper management will dramatically improve the outcome in this otherwise debilitating and potentially life-threatening disease.

We describe a very rare case of acute intermittent porphyria in which the patient had a generalized seizure and progressive paralysis in the puerperium.

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