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Wilson disease in two consecutive generations: an exceptional family

organ, it is not feasible in most cases. Drugs are a relatively uncommon cause of pancreatitis, reported only infrequently in the English language literature (5). The association of riluzole with pancreatitis in this case appears probable. The patient had clinical, laboratory, and radiological evidence of acute pancreatitis. There was a temporal relationship between the use of riluzole and the onset of symptoms, and the picture resolved after the drug was stopped. Other likely causes of pancreatitis were not present. Although the patient was diagnosed with peptic ulcer he had been symptom free for years when the pancreatitis occurred. Rechallenge was not attempted, and therefore evidence for a definite association is failing. The absence of systemic hypersensitivity manifestations and the appearance of injury months after exposure suggest that the damage may be produced through the accumulation of a toxic metabolite. The mechanism of drug-induced damage in the pancreas is still not completely understood (6). Although acute pancreatitis is a serious medical condition with considerable morbidity and mortality, drug-induced acute pancreatitis usually runs a benign course (5). A wide variety of different drugs ( .85) have consistently been associated with acute pancreatitis (7), but few data exist about the incidence of drug-induced pancreatitis in the general population. Acute pancreatitis has previously been reported with the use of riluzole (8). Overall, 11 cases of pancreatitis possibly associated with riluzole have been reported up to December, 1997, resulting in an estimated incidence of about 4.5/10,000 treated patients, according to data reported to Rhone-Poulenc-Rourer (9). Therefore, riluzole should be added to the list of medications that potentially cause acute pancreatitis. Although this is not consistent with the product information, physicians should be made aware of this risk. In view of the modest improvements in survival rate that have been demonstrated in this fatal progressive degenerative disorder with riluzole and the concerns about its side effect profile, only patients for whom evidence of efficacy is provided by well-designed clinical trials should be treated (10), so that the benefit of the agent outweighs potential risk and therefore it could be used with an acceptable benefit to risk ratio.