Acute intermittent porphyria after gastroplasty.

A 28-year-old female underwent gastroplasty for obesity in an uneventful procedure. This was followed within five days by persistent abdominal pain associated with weakness that progressed to generalized flaccid quadriparesis. She developed respiratory distress, and required intubation. Cerebrospinal fluid (CSF) revealed a normal cell count and elevated total protein. Her folate and vitamin B12 levels were normal. Electromyography (EMG) and nerve-conduction studies (NCS) showed acute generalized motor-sensory demyelinating neuropathy predominantly in the lower limbs. A diagnosis of Guillain-Barré syndrome was made, and a five-day course of immunoglobulin was begun, which did not produce any improvement. Three months later, she still had not improved and she came into our service looking for a diagnosis. The patient reported that seven relatives had lower limb weakness, and that five of them died due to respiratory failure after the onset of neurological manifestations. Neurological examination showed moderate quadriparesis, with proximal weakness greater than distal weakness. Muscle reflexes were absent, and no pathological reflexes were demonstrated. The patient’s sense of feeling was normal. The blood tests were normal. We repeated the EMG and NCS, which showed pure motor axonal polyradiculoneuropathy. The porphobilinogen (PBG) and δ-aminolevulinic acid (ALA) levels in urine were high: 32.4 mg/24h (normal range: 0-2 mg/24h) and 26.2 mg/g, (normal value: 4.5 mg/g) respectively, thus indicating the diagnosis of acute intermittent porphyria. Six months later, the patient returned complaining of a burning feeling in her lower abdomen and upper limbs. Two days after this, she became weak again and developed quadriparesis. She was then treated with intravenous glucose (300 g per day), with a slight improvement. Hematin was not used because it was not available at our service. Because the patient’s condition continued to worsen, we decided to reverse the gastric bypass, given that this could decrease the risk of further attacks. Within a couple days, she got better and her muscle strength gradually improved.