Familial aggregation in giant cell arteritis and polymyalgia rheumatica: a comprehensive literature review including 4 new families.

OBJECTIVE: To review personal and published observations of giant cell (temporal) arteritis (GCA) or polymyal-gia rheumatica (PMR) with familial or conjugal aggregation and emphasise on epidemiological, clinical and genetic features of such cases. METHODS: We pooled data obtained from all cases of GCA or PMR with familial aggregation recruited in the department since 1976 and those from reports of familial or conjugal GCA or PMR published in the French-English literature since 1970. RESULTS: During the study period, we diagnosed 460 patients (128 with isolated PMR, 227 with isolated GCA, 105 with PMR/CGA). No conjugal couples were observed in the whole series. No familial cases were identified among PMR patients, whereas the prevalence of familial GCA was 1 in 83 (1 in 250 to 500 expected by chance), as we identified 4 patients (brother-brother, sister with history of affected sister, and daughter with priory affected mother). An additional pair of sisters with TA, recruited several months after diagnosis, is also presented. Pooling data from 85 patients (74 with GCA) including our patients, representing 32 families and 8 conjugal pairs, enabled us to draw the following observations: 1) partial or full agreement in the clinical picture (GCA, PMR, or GCA/PMR) was observed in 96% of the siblings pairs, suggesting a common pathogenic mechanism; 2) five kindred were described in whom at least three members were affected; 3) the lag between manifested diseases in familial or conjugal pairs averaged 5.7 years, with synchronous or close disease occurrence in only 26% of the pairs; 4) 18 of 32 assessed patients (56%) carried the DR4 antigen. CONCLUSION: Our survey on familial aggregation of GCA and PMR accumulated data pointing to a genetic predisposition. However, environmental contagious factors could have trigger synchronous disease onset in up to one-fourth of the cases.

Three-dimensional reconstruction of the temporal bone: Comparison of in situ, CT, and CBCT measurements.

OBJECTIVE: To evaluate the accuracy of three-dimensional (3D) Cone Beam Computed Tomography (CBCT) and Computed Tomography (CT) reconstructions of human temporal bones compared with in situ measurements. MATERIAL AND METHODS: Experimental anatomical study of 10 human temporal bones. Wilcoxon's test was used to compare 8 distances on each temporal bone measured in situ and then on 3D CT and CBCT reconstructions. Six landmarks were used: external auditory canal (EAC), tip of the mastoid process, tip of the occiput, zygoma, a point situated 1cm above the tip of the mastoid process (T0) (open technique: lower limit of the mastoidectomy), head of stapes. RESULTS: No significant difference was observed between the 3 measuring techniques for any of the distances studied (P>0.05). DISCUSSION: This study demonstrates the equivalence of CBCT and CT for temporal bone measurements. CONCLUSION: CBCT is a new imaging modality providing 3D reconstructions of the temporal bone that are as reliable as those obtained by CT. As a result of better spatial resolution compared to CT, CBCT is associated with a significantly lower radiation dose. This technique constitutes a morphological progress, as CBCT is comparable to CT, allowing investigation of pathological ears with a lower radiation dose.

Cervical cord and dorsal medullary infarction presenting with retro-orbital pain.

A 36-year-old woman experienced an excruciating right retro-orbital paroxysmal headache after a stroke of the anterior two-thirds of the right hemicord at the C1 level (anterior spinal artery territory) and the dorsal medulla (posterior spinal artery territory). A right vertebral artery dissection was demonstrated. This unusual infarct mimicked a cluster headache attack or paroxysmal hemicrania.

Ion-selective electrode for dodecyldimethylamine oxide: a "twice-Nernstian" slope for the determination of a neutral component

An electrode originally sensitive to dodecyltrimethylammonium (DTA+) was proven to be sensitive to dodecyldimethylamine oxide (DDAO), a surfactant with acidobasic properties. The response of the electrode was tested from pH 2 to 9.3. Its slope is Nernstian when the surfactant is entirely protonated. At a pH where the molecule is mainly under the neutral form, the electrode responds with a "twice-Nernstian" slope around 120 mV/decade. The validity of this electrode for measurements was checked by confronting the evolution of the critical micelle concentration of DDAO vs pH with data already published and by determining the complexation constant of DDAO and beta-cyclodextrin. A possible explanation of the "twice-Nernstian" slope, using a dimer of DDAO is proposed.

Perindopril plus nifedipine versus perindopril plus hydrochlorothiazide in mild to severe hypertension: a double-blind multicentre study. The Multicentre Study Group on Treatment Association with Perindopril.

Thiazide diuretics are considered as the choice drug to combine with ACE inhibitors for the treatment of hypertension. However, there is much evidence showing that the combination of ACE inhibitors with a calcium channel blocker is effective and safe. We compared the safety and efficacy of perindopril 8 mg once daily plus nifedipine SR 10 mg twice daily with perindopril 8 mg once daily plus hydrochlorothiazide (HCTZ) 12.5 mg once daily in a two phase three month study. After a one month placebo run-in period, patients whose DBP averaged 95-125 mmHg received perindopril 4 mg once daily for the first open phase (n = 524). After one month those whose DBP remained > 90 mmHg were prescribed perindopril 8 mg once daily for a second month. Among them, those whose DBP were still > 90 mmHg entered the second phase for one month, in a double-blind fashion. Fifty-three patients received HCTZ (BP: 161.2/99.2 +/- 2.0/0.9 mmHg), 57 received nifedipine (BP: 161.4/98.7 +/- 2.2/0.7 mmHg). Five patients withdrew due to side-effects, three patients in the perindopril plus nifedipine group and two in the perindopril plus HCTZ group. After one month there was a significant drop in BP (P < 0.01) in both groups: perindopril plus HCTZ (-13.9/-11.9 mmHg) and perindopril plus nifedipine (-12.1/-10.8 mmHg). Heart rate was not significantly modified: perindopril plus HCTZ (-1.30 beats/min), perindopril plus nifedipine (+0.54 beats/min). There were no significant difference between the two combinations for BP reduction and heart rate. The incidence of adverse experiences was similar in both groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Nonconvulsive status epilepticus: the role of morphine and its antagonist.

A 77-year-old woman with no history of epilepsy presented a probable nonconvulsive status epilepticus while receiving continuous intravenous morphine for back pain relating to vertebral metastasis of a malignant lymphoma. A generalized tonic-clonic seizure occurred a few minutes after injection of the morphine antagonist naloxone. No cerebral lesion was detected. This observation supports the notion of a possible pro-epileptogenic effect of opioid substances in association with metabolic and other toxic factors. Various hypotheses as to the complex actions of morphine and its antagonist in epileptogenesis are discussed.

A potentiometric study of the adsorption of a cationic surfactant onto laponite in water-methanol and water-dimethylsulfoxide mixtures.

We studied the adsorption of a cationic surfactant, dodecyltrimethylammonium bromide (DTABr), onto laponite in water-methanol and water-DMSO mixtures at 298 K. Adsorption isotherms were determined by potentiometric methods using DTA(+) and Br(-)-selective electrodes. All of the isotherm shapes in the mixture were classical. Activity coefficients were determined for the transfer between water and hydroorganic mixtures. Comparison of the adsorption isotherms obtained for different surfactant chemical potentials showed that at higher concentrations of free surfactant, the aggregation state does not depend on the nature of the surrounding media. A study of the influence of the ionic strength showed that there is a common intersection point in each hydroorganic mixture. This point corresponds to the charge compensation point and depends on the nature of the surrounding medium.

[Orthotopic liver transplantation for metastases of bronchial carcinoid tumor]. / Transplantation orthotopique hépatique pour métastases d'une tumeur carcinoïde bronchique.

We report the case of a 42 year old man in whom orthotopic liver transplantation was performed successfully for unresectable hepatic metastases of a bronchial carcinoid tumor. Prior to transplantation, somatostatin therapy, pulmonary lobectomy, and systemic chemotherapy (streptozotocin and fluorouracil) were performed. After 9 months there were no signs of clinical or biological recurrence. Orthotopic liver transplantation might be indicated for unresectable and limited liver metastases of neuroendocrine tumor.

[Intermittent claudication of the jaw in temporal arteritis]. / La claudication intermittente de la mâchoire dans l'artérite temporale.

Stiffness of the jaw was noted in the first descriptions of temporal arteritis. It was only in 1944 that Horton used the term intermittent claudication and related this sign to effort ischemia due to thrombosis of facial arteries. The introduction of ultrasound techniques has enabled the permeability of facial arteries to be confirmed in spite of induration and absence of pulsatility clinically. Anatomical studies have defined the preponderant role of the internal maxillary artery in the vascular supply of the masseter muscles and have enabled the localization of an appropriate and reliable site for ultrasound study: the pterygo-maxillary fossa. The velocimetric data thus collected confirm that the internal maxillary artery is affected and define the etiopathogenesis of intermittent jaw claudication during temporal arteritis. This sign is observed on average in one patient in three suffering from temporal arteritis. While several cases of intermittent jaw claudication have been described in severe atheromatous stenosis of the common carotid or external carotid arteries, or in relation to other causes (rheumatological, neoplastic, psychological ...), the observation of this syndrome in a suspicious clinical and paraclinical context constitutes an excellent orientation sign in favor of temporal arteritis.

[Prospective etiologic study of 128 patients with ambulatory dep vein thrombosis]. / Etude étiologique prospective de 128 patients avec une thrombose veineuse profonde survenue en ambulatoire.

Etiology of deep vein thrombosis in ambulant patients (DVTA = TVPA in text) varies: cancer, blood disease, infectious focus, dysimmunity syndrome, dysglobulinemia, extrinsic compression, metabolic disorder, anomaly of hemostasis. A prospective study was carried out between June 1988 and September 1989 by angiologists in 5 regions of France to evaluate the diagnostic rentability of an epidemiologic survey and to determine possible distinctive characters of DVTA. The survey was comprised of a questionnaire, a full clinical examination and screening tests: chest x-ray, abdominopelvic ultrasound imaging, a-uro/gynecologic examination, full blood count, serum iron, ferritin, uric acid, triglycerides, cholesterol, protein electrophoresis, antinuclear antibodies, circulating anticoagulant, hemostasis factors and liver function tests. The study included 128 patients, mean age 60 +/- 16 years with a DVTA developing without a previous immobilization. The usual predominance in the left leg was not observed. The etiology was identified in 33 cases, including 20 (15.6%) as a result of the screening tests: anomalies of hemostasis (8), blood diseases (3), dysimmunity syndromes (4), extrinsic compression (3), cancer metastasis (1) and hypertriglyceridemia in a diabetic (1). The screening procedure was of no greater value in the absence of a triggering or predisposing factor, on the contrary. An anomaly of hemostasis was detected more frequently in the presence of local or regional triggering factors in the men (4 out of 4) and in the women on the pill (4 out of 4). The number of cancers discovered following screening (2%) was smaller than that expected according to the literature (10%).(ABSTRACT TRUNCATED AT 250 WORDS)

Gay and bisexual male domestic violence victimization: challenges to feminist theory and responses to violence.

This article demonstrates how same-sex male battering challenges contemporary feminist domestic violence theory. The author shows current theory to be heterosexist and therefore insufficient to explain the phenomenon of battering among gay/bisexual men. Domestic violence theories that integrate a sociopolitical and a psychological analysis of battering are presented as more inclusive of same-sex domestic violence. Differences between battered gay/bisexual men and battered women are illustrated, focusing on how these men conceptualize and respond to violence against them. The author also examines the social context of homophobia in which same-sex battering occurs; the impact of AIDS on gay/bisexual men as it pertains to battering; the misconception of "mutual combat"; and the difficulty of seeking help. The article highlights the need for empirical research on same-sex male battering.

[Photodistribution of varicella in an adult]. / Photodistribution d'une varicelle chez un adulte.

INTRODUCTION: Exacerbation of viral exanthema has been described after different types of aggression such as sun exposure. Photoaggravation of varicella is unusual. CASE REPORT: We report the case of a 25 year-old woman who presented with varicella mainly distributed on the neckline and the top of the back. During the viral incubation she had been sunburnt in these areas. DISCUSSION: The clinical lesions and the evolution of a photoaggravated varicella differs from those of classical varicella. The vesicles are selectively distributed on areas previously sunburned or suntanned. The more tanned or more erythematous the area is, the greater the number of lesions. They are often at the same stage of development and the course can be shortened with only one or two progressive phases. Inflammatory modifications secondary to ultraviolet exposure induces in photoexposed areas an increase in vascular flow and also an increase in capillary permeability, which can promote diapedesis of the lymphocytes or the transit of viral particles. Therefore, sun exposure during the incubation of the virus can enhance the deposit of viral pArticles in the cutaneous areas submitted actinic stress. The amount of virus in sun exposed areas is greater than in the non-exposed ones.