Case history
Case history
A 30-year-old woman has severe abdominal pain, nausea, vomiting, and diarrhoea, and is admitted to hospital for 2 weeks for a suspected intestinal infection. Evaluation, including upper and lower endoscopies, does not establish a definite cause for her symptoms. She gradually improves and is discharged after 2 weeks. Symptoms recur 2 years later, leading to multiple emergency department visits. She is admitted to a psychiatric unit with mental status changes and hallucinations, then transferred to the emergency department with abdominal pain, a grand mal seizure, and hyponatraemia. On admission to a medical unit her pulse is 120 bpm and BP is 174/114 mmHg. She is disoriented but has no focal neurological signs. Magnetic resonance imaging shows subcortical abnormalities, and the spinal fluid is normal. After cholecystectomy for a distended gallbladder, she is discharged and stays with a family member in another city because her symptoms worsen and she develops muscle weakness. She is admitted to another hospital and progresses to quadriparesis, respiratory failure, and aspiration pneumonia. Urinary porphobilinogen is reported as 44 mg/24 hours (reference range 0-4). Following treatment with intravenous glucose and stopping harmful drugs (including phenytoin), she improves gradually and is discharged for physiotherapy and rehabilitation. She recovers almost completely over a period of several months, although residual painful hyperaesthesia of the lower extremities, mild proximal muscle weakness, and impaired short-term memory are noted to persist. Several attacks occur in the next few years, and some are premenstrual.
Other presentations
Peripheral motor neuropathy is usually a late manifestation of an AIP attack, especially if the attack has not been diagnosed or treated.[5] Rarely, a rapidly progressive motor neuropathy can develop in the absence of more common symptoms, such as abdominal pain.
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