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Acute Intermittent Porphyria Presenting as Acute Pancreatitis and Posterior Reversible Encephalopathy Syndrome
Acta Neurol Taiwan 17:177-183, Shen, F.,et al, 2008
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Article Abstract
Acute intermittent porphyria (AIP) is an inherited metabolic disease that can affect the autonomic, peripheral and central nervous systems. Pancreatic diseases associated with AIP is rarely reported. We report here a 60-year-old non-alcoholic male who had typical manifestations of AIP, including abdonimal pain, constipation, tachycardia, hypertension, mental disturbances, psychiatric manifestations, seizures, peripheral neuropathy, and excessive excretion of porphyrin precursors in urine. Increases of serum amylase and lipase, as well as mild pancreatic edema on ultrasonography, were noted during the acute attack of AIP, suggesting concomitant acute pancreatitis. In this patient, brain magnetic resonance imaging revealed reversible multifocal cerebral lesions resembling a posterior reversible encephalopathy syndrome (PRES) during the acute attack of AIP. Because the clinical manifestations of acute pancreatitis could be present with an acute attack of AIP, early confirmation of diagnosis is mandatory to effectively manage the attack and avoid inappropriate treatment.
 
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acute intermittant porphyria
agitation
autonomic dysfunction
confusion
encephalopathy
hyperamylasemia
hypertension
ileus,paralytic
metabolic disorder,primary
MRI,abnormal
nausea and vomiting
nerve conduction studies
neuropathy
pain,abdominal
pancreatitis
porphyria
posterior leukoencephalopathy syndrome
psychiatric problems in neurologic disorders
psychosis
quadriplegia
respiratory failure
seizure
serum lipase,elevated
tachycardia
weakness
weakness,acute
weakness,generalized

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