[Von Recklinghausen's disease associated with pancreatic somatostatinoma]. / Maladie de Von Recklinghausen associée à un somatostatinome pancréatique.

INTRODUCTION: Pancreatic somatostatinoma is a rare entity and its association with Von Recklinghausen's disease has only been described on two occasions. We report a new observation, with clinical and evolving status differing from those described in isolated cases of somatostatinoma. OBSERVATION: A 28 year-old man presenting with familial Von Recklinghausen's disease, had suffered for 10 years from paroxysmic abdominal pain. He was hospitalized for intense pain. Imaging revealed a retroperineal tumoral formation. Following duodenopancreatectomy, somatostatinoma was diagnosed. Diffuse metastatic miliary was revealed and multiple glandular metastases. DISCUSSION: Other than the rarity of the morbid association (pancreatic somatostatinoma and Von Recklinghausen's disease) described, this case is particular in that the patient was very young, symptomatology was unapparent and tumoral evolution was minimal.

[Acute disulfiram intoxication with severe neurological sequaelae and presenting misleading psychiatric deficiency]. / Intoxication aiguë au disulfirame avec séquelles neurologiques graves et présentation psychiatrique déficitaire trompeuse.

Disulfiram self poisoning is exceptional. The authors report on the case of such an intoxication related to the ingestion of a potentially lethal dose of this drug (30 g) and draw the attention on the following points: 1) The initial signs may be misleading because they include both psychiatrics and neurological signs such as phonation abnormalities, myoclonias and tetraparesia. 2) The evolution is unforseeable with the possible occurrence of severe psychological and motricity sequaelae, associated with bilateral and symetric injuries of the putamen, the palladium and the basal nuclei on CT-scan (or MRI). The pathophysiologic al mechanisms of theses signs are discussed, and the need for disulfiram in the care of alcoholic patients seeking for withdrawal as well.

[Budd-Chiari syndrome with inferior vena cava stenosis]. / Un syndrome de Budd-Chiari par sténose cave inférieure.

BACKGROUND: Stenosis of the inferior vena cava is a rare cause of Budd-Chiari's syndrome. We report on such a case related to a specific cause with a dramatic improvement after endovascular treatment. CASE REPORT: A 53-year old woman was admitted to hospital because she demonstrated circulatory collapse, inferior limbs edema and post-prandial epigastralgia. These signs were related to the occurrence of a stenosis of the sus-hepatic segment of the inferior vena cava related to the presence of the distal part of a catheter. An angioplasty with the insertion of a stent was followed by a total recovery with a one-year survey. CONCLUSION: Angioplasty with the insertion of a stent should be considered in a Budd-Chiari syndrome related to the stenosis of the inferior vena cava. This option should be evaluated with reference to classic surgical techniques.