[Abdominal pain in progressive encephalomyelitis with rigidity]. / Encéphalomyélite progressive avec rigidité révélée par une douleur abdominale.

INTRODUCTION: Stiff-person syndrome is rare neurological disease, associating trunk rigidity and painful muscular spasms. A clinical variant of stiff person syndrome is the progressive encephalomyelitis with rigidity and myoclonus (PERM), which includes neurological cognitive disturbances. CASE REPORT: We report a 73-year-old woman initially addressed for abdominal pain, anorexia and severe weight-loss, for whom diagnosis of PERM was made. CONCLUSION: Because of its various clinical presentations, sometimes without evidence for neurological disease, the diagnosis of PERM is delayed. The presence of antineuropile antibodies associated with muscular spasms at electromyogram are strong evidence for this diagnosis.

[MR cholangiography in biliary complications after liver transplantation in children]. / Apport de la cholangiographie-IRM dans les complications biliaires de la transplantation hépatique chez l'enfant.

PURPOSE: To evaluate the role of MR Cholangiography in a pediatric population with biliary complications after liver transplantation and particularly with anastomotic stenosis. PATIENTS AND METHODS: Ten MR cholangiography studies were performed in 10 children with liver transplant who were suspected of having biliary complications between December 1996 and April 1998. The findings on MR were correlated with the results from liver biopsy, liver ultrasound, liver function tests and with clinical information when available. RESULTS: MR cholangiography identified 9 children with biliary tree dilatation, 4 with anastomotic stenosis, 5 with multiple bile ducts stenosis, 2 with stones in the intra-hepatic biliary tree and 2 with abnormalities suspicious for acute cholangitis. Three of 4 anastomotic stenoses were confirmed and treated by percutaneous cholangiography. There was no correlation between the different exams in 6 children but MR cholangiography confirmed the final diagnosis. CONCLUSION: In children with liver transplantation, MR cholangiography may be useful to evaluate and to confirm a diagnosis of bile duct complications and it is helpful in the absence of correlation between liver biopsy, ultrasound and liver function test.

MR cholangiography in neonates and infants: feasibility and preliminary applications.

OBJECTIVE: The objective of the study was to evaluate the accuracy of MR cholangiography in showing the biliary tree in neonates and infants and to show preliminary applications in a pediatric population. SUBJECTS AND METHODS: MR cholangiography was performed with T2-weighted turbo spin-echo sequences (8000/300 [TR/TE], six or eight excitations). The study included seven patients with suspected bile duct disorders and 10 control individuals. RESULTS: In the control group, the extrahepatic bile ducts (EHBDs) were revealed by MR cholangiography in all patients. In one of the five patients with neonatal cholestasis, biliary atresia was excluded by identifying the entire EHBD on MR cholangiograms. In the four other patients with neonatal cholestasis, biliary atresia was suspected because the EHBD could not be seen on MR cholangiography. The diagnosis was confirmed in three of these four patients, in whom the MR finding was associated with significant periportal thickening. In the remaining patient, in whom periportal thickening was not seen, sclerosing cholangitis was diagnosed on biopsy. In the sixth patient with neonatal cholestasis, who had received a liver transplant, MR cholangiography showed biliary dilatation above the biliary-digestive anastomosis. In the seventh patient, a neonate with a prenatal diagnosis of hepatic cyst, MR cholangiography showed a choledochal cyst. CONCLUSION: MR cholangiography may become an important diagnostic tool in the detection of biliary atresia, congenital choledochal dilatation, and biliary complications in hepatic transplantation for pediatric patients.