[Incomplete spontaneous rupture of the supravalvular aorta complicated by pericarditis and aortic insufficiency]. / Rupture spontanée incomplète de l'aorte sus-sigmoïdienne compliquée de péricardite et d'insuffisance aortique.

The natural history of an incomplete spontaneous rupture of the ascending aorta over a 4-year period is reported. The initial presentation was subacute pericarditis which regressed spontaneously. Aortic regurgitation developed on the 100th day of the disease and was perfectly well tolerated haemodynamically for 4 years. This favourable course was interrupted by type I aortic dissection. The post-mortem examination provided an explanation for the peculiar development and course of the aortic regurgitation: it was due not to the usual prolapse of the cups but to displacement of the left posterior commissure by the retractile fibrosis arising from the lower lip of the spontaneous rupture.

[Pericarditis and pulmonary embolism. A difficult differential diagnosis and a confusing association]. / Péricardite et embolie pulmonaire. Un diagnostic différentiel difficile, une association déroutante.

The association of pericarditis and pulmonary embolism may be the source of diagnostic error and delay in the administration of anticoagulant therapy. Two cases are reported. Pericarditis occurred late in patients with severe, chronic pulmonary embolism with electrocardiographic changes of acute cor pulmonale. Two physiopathological mechanisms for this association have been proposed. The first, haemodynamic, suggests friction between the pericardium and distended right ventricle and pulmonary artery. The second, an immunological hypothesis, compares the association of pericarditis and pulmonary embolism to that of the Dressler syndrome after myocardial infarction. This assimilation would imply the constitution of an anatomical pulmonary infarction. It is not justifiable to accept this pathogenesis on the evidence of transient pulmonary opacities resulting from intra-alveolar haemorrhage or of linear opacities of pulmonary atelectasis secondary to hypocapnic pneumoconstriction which are radiological signs of anatomo-physiological stages of pre-infarction.

[A paraneoplastic acrosyndrome]. / Un acrosyndrome paranéoplasique.

BACKGROUND: Paraneoplastic digital ischemia is an uncommon complication of metastatic adenocarcinomas. CASE REPORT: Two years after remission of an uterine adenocarcinoma, the patient developed an acrosyndrome involving all four limbs with digital ischemia. Recurrent carcinoma was evidenced by a very high antinuclear antibody titer. Chemotherapy improved the acrosyndrome. DISCUSSION: Vasomotor disorders which developed in older subjects with no other signs of autoimmune disorders should suggest a neoplastic origin. Icshemia of the fingers would be caused by vasculitis. An elevated antinuclear antibody titer may be a supplementary argument suggesting a neoplastic etiology.

[Mixed corticomedullary tumor]. / Tumeur mixte corticomédullaire.

BACKGROUND: Pheochromocytoma and primary hyperaldosteronism rarely occur simultaneously. Few cases have been reported in the literature. CASE REPORT: A patient explored for hypertension was found to have hypokalemia related to primary hyperaldosteronism. Pathology examination of the ablated adrenal showed a co-existing pheochromocytoma suspected at history taking although urine catecholamines were normal. DISCUSSION: Different pathogenic hypothesis have been proposed. Such dual tumors could be a simple coincidence, occur in a particular genetic setting, be related to direct contact between cortical and medullary tissue leading to reactional cortical hyperplasia, pheochromocytoma produced factors stimulating aldosterone synthesis, or factor X, a substance produced by cortical adenomas and favoring growth of the pheochromocytoma.

[Hepatic adenoma and oral contraceptives (author's transl)]. / Adénome hépatique et contraceptifs oraux.

The authors report a new case of hepatic adenoma apparently related to oral contraception continued for more than 9 years with a pill containing mestranol. The clinical presentation suggested an attack of hepatic colic with swollen gall bladder. The preoperative diagnosis of a tumour of segment VI of the liver was made by radiological examination of neighbouring organs. Hepatic arteriography, hepatic radioisotope scan using technetium phytate and echotomography. Surgical removal of segment VI confirmed by histological study showed that it was an adenoma with multiple foci, associated with hepatic involvement. One of the tumours was the site of central included hemorrhage, responsible for the clinical syndrome. In connection with this case the authors review the possible relationship between hepatic adenoma and oral contraception.

Differential diagnosis of recurrent or bilateral peripheral facial palsy.

OBJECTIVE: To describe the differential diagnosis of recurrent or bilateral peripheral facial palsy. METHOD: Case report and literature review. RESULTS: Two patients with recurrent, alternating, peripheral facial palsy are described. In both patients, additional investigation was performed to search for a specific diagnosis. In the first patient, only a positive family history was found, indicating a possible familial susceptibility. In the other patient, diabetes mellitus and hypertension were identified as risk factors. CONCLUSION: There is an important and extensive differential diagnosis of recurrent or bilateral facial palsy. However, in a large proportion of patients the cause remains unknown.

SPES/SCOPA and MDS-UPDRS: formulas for converting scores of two motor scales in Parkinson's disease.

BACKGROUND: Motor impairment in Parkinson's disease (PD) can be evaluated with the Short Parkinson's Evaluation Scale/Scales for Outcomes in Parkinson's disease (SPES/SCOPA) and the Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS). The aim of this study was to determine equation models for the conversion of scores from one scale to the other. METHODS: 148 PD patients were evaluated with the SPES/SCOPA-motor and the MDS-UPDRS motor examination. Linear regression was used to develop equation models. RESULTS: Scores on both scales were highly correlated (r = 0.88). Linear regression revealed the following equation models (explained variance: 78%): CONCLUSION: With the equation models identified in this study, scores from SPES/SCOPA-motor can be converted to scores from MDS-UPDRS motor examination and vice versa.

[Cerebrovascular accident presenting as continuous partial epilepsy (author's transl)]. / Epilepsie partielle continue révélatrice d'un ramollissement cérébral.

Epileptic seizures due to cerebrovascular accidents are relative rare and only very exceptionally occur in the form of Kojewnikoff's continuous partial epilepsy. A 75-year-old man developed myoclonic spasms, limited to the face, following a left posterior cerebral ischaemic accident. The authors discuss the relationship between epilepsy and cerebrovascular accidents, and differentiate post-ischaemic epilepsy of deferred onset from epilepsy occurring simultaneously with a vascular accident, the latter sometimes presenting as fits. The relationship between myoclonic spasms and Kojewnikoff's continuous partial epilepsy are also discussed.

[Cryptic miliary tuberculosis. Two case-reports (author's transl)]. / Les tuberculoses miliaires cryptiques. A propos de deux observations.

The term cryptic miliary tuberculosis designates a particular form of hematogenous tuberculosis in which the usual diagnostic criteria, especially the roentgenographic ones, are lacking. Two cases are reported. Initial clinical manifestations were prolonged fever, poor general condition, low blood pressure, low serum sodium, mild hepatic biological disorders, and myocarditis. No other radiological or biological features, suggestive of tuberculosis were found. In the first patient, the various visceral tuberculous localizations occurred under specific treatment, although the latter was otherwise effective. In the second patient, they were identified by pathological examination. The incidence of these occult forms of tuberculosis, where diagnosis is often established too late, and sometimes only postmortem, is not negligible. This possibility must be borne in mind in severe infectious conditions without obvious etiology, particularly when the following features are present : inadequate secretion of ADH, which often indicates latent encephalitic and meningitic involvement where CSF anomalies may be incomplete or lacking ; leucopenia or pancytopenia ; biological hepatic anomalies ; exceptionally, such as in the two cases described, cardiac involvement. Among diagnostic procedures, liver biopsy is of the utmost importance. Mere diagnostic presumption is sufficient to warrant initiation of antituberculous therapy. This ensures survival and, paradoxically, allows delayed overt clinical manifestations to develop.

[Primary gastric plasmacytoma with IgG dysgammaglobulinemia and macrocytic anemia (author's transl)]. / Plasmocytome gastrique primitif avec dysglobulinémie à IgG et anémie macrocytaire.

The authors report a case of a woman aged 76 years with a single plasmocytoma of the stomach secreting large quantities of a paraprotein of the IgG type with Kappa light chains. A macrocytic anemia due to deficiency of gastric intrinsic factor was also present. This pathological association is extremely rare. About sixty cases of gastric plasmocytoma are been reported in the published literature and their principal characteristics are briefly outlined.