[Treatment of connective tissue disorder-related acral syndromes using UVA-1 phototherapy. An open study of 11 cases]. / Traitement des acrosyndromes des connectivites par photothérapie UVA-1. Etude ouverte de 11 cas.

BACKGROUND: UVA-1 phototherapy is used to treat various connective tissues disorders including systemic lupus erythematosus (SLE) and systemic sclerosis (SS). We conducted an open study to investigate the efficacy of this therapy on connective tissue disorder-related acrosyndrome. MATERIAL AND METHODS: Eleven patients with Raynaud's phenomenon refractory to the standard therapy (six SLE and five SS) were treated with UVA-1 in an open study. Whole-body phototherapy was given in seven cases but was restricted to the hands in four cases. The results were evaluated by comparing status before and after treatment using semi-quantitative tools in terms of daily frequency and intensity of spastic phenomena, pain, overall patient satisfaction, improvement of cutaneous lesions, trophic disorders and distal cutaneous flexibility for patients with ScS. RESULTS: After treatment, Raynaud's phenomenon improved in terms of both frequency and severity in 9/11 patients (82%: 4/6 SS and 5/5 SLE). Likewise, pain decreased in 8/11 cases (73%: 3/6 SS and 5/5 SLE). 7/12 patients felt their condition had improved (64%: 3/6 SS and 4/5 SLE). Cutaneous lesions improved in 5/11 patients (45%: 2/6 SS and 3/5 SLE), especially for lupus-related lesions including chilblains and in idiopathic chilblains that totally subsided within one month. Digital ulcers improved in all cases, with complete healing in 3/4 patients (75%). In SS, cutaneous flexibility significantly improved in 2/6 cases (33%). No major adverse effects were seen in patients treated with hand-only phototherapy but a slight and spontaneously reversible cutaneous rash, reminiscent of lupus lesions, occurred in one female patient receiving whole-body phototherapy. DISCUSSION: This study is the first to provide a precise evaluation of the efficiency of UVA-1 phototherapy on connective tissue disorder-related acrosyndrome. This therapeutic effect is not necessarily restricted to the laboratory effects of UVA-1 since the favourable impact of infrared radiation and a placebo effect cannot be ruled out. Although its methodological limitations are obvious, our study also confirms dare data in the recent literature data by demonstrating significant improvement in cutaneous lesions, trophic ulcers and Raynaud's phenomenon in patients presenting connective tissue disorders, including SS, without any major adverse effects. CONCLUSION: Although these preliminary results remain to be confirmed by large-scale, randomized studies, UVA-1 phototherapy clearly offers a new and valuable therapeutic option in connective tissue disorders associated with acral manifestations and/or lesions, including SLE and SS.

[Interstitial granulomatous dermatitis with arthritis: four cases]. / Dermatite granulomateuse interstitielle avec arthrites: quatre cas.

BACKGROUND: Interstitial granulomatous dermatitis with arthritis, or Ackerman's syndrome, is characterized by inflammatory articular flares of potentially destructive outcome and cutaneous lesions of varied patterns: cord-like lesions on the flanks or violaceous plaques on the trunk and limbs. Histologically, the main features are histiocytes laminated between collagen fibres, sometimes organized in rosettes comprising tiny granulomas surrounding fibres of homogeneous aspect. CASE REPORTS: Four patients fitting this description were investigated. Cutaneous lesions displayed various patterns: cord-like lesions (one patient), brown plaques on the flanks edged with papules (one patient), grossly annular violaceous plaques on the back (one patient), and infiltrated plaques on the palms and thighs (one patient). Joint symptoms were present in all cases (one seropositive rheumatoid arthritis and one seronegative rheumatoid arthritis, both already known; acute rheumatoid arthritis in two cases). The histological pattern was quite similar in all cases, with laminated histiocytes in palisading layers mixed with swollen collagen fibres; "rosette" figures were present in two cases. Autoimmune response was seen with antinuclear antibodies (two cases) and/or rheumatoid factor (two cases). The short-term outcome of cutaneous lesions was rapidly favourable in most cases with systemic steroids (one case) or Non Steroidal Anti-Inflammatory Drugs (one case), and spontaneously in one case. COMMENTS: This entity, defined by the presence of inflammatory arthritis and cutaneous lesions of various clinical types, either more specific but infrequent (cord-like), or less specific but more frequent (plaque-like), and featuring a peculiar histological pattern, chiefly affects women aged between 30 and 80 years. Pathophysiological mechanisms and nosological borders are debatable and authors consider this condition as either an autonomous disease or else a mere subset of vasculitis with palisading granulomas in collagen vascular disorders, despite the rarity of authentic vasculitis. A setting of autoimmunity is frequently present. Furthermore, a fairly similar histological pattern is sometimes seen in some lesions forming a subset of cutaneous side-effects of drugs. Articular outcome may be unfavourable with joint destruction in more than half of patients, whether or not in a setting of rheumatoid arthritis. Spontaneous resorption can occur. Treatment has not yet been codified and is based primarily on anti-inflammatory drugs.

Effects of streptozotocin and dietary fructose on delta-6 desaturation in spontaneously hypertensive rat liver.

We have investigated the effects of hypertension associated with diabetes mellitus on polyunsaturated fatty acid biosynthesis. For this purpose, two rat models for these pathologies have been established: a type 1 diabetic hypertensive model obtained by streptozotocin injection to spontaneously hypertensive rat (SHR), followed or not by insulin treatment (experiment 1); a type 2 diabetic hypertensive model by feeding SHR with a fructose enriched diet (experiment 2). Liver gene expression of delta-6 desaturase (D6D), microsomal D6D activities and fatty acid composition of total lipids were estimated. In experiment 1, an increase of linoleic acid (18:2 n-6) level was observed in the streptozotocin group. D6D gene expression appeared depressed in both experimental groups. Insulin did not reverse the streptozotocin effect in SHR, as it does in insulin-dependent diabetic rats. In experiment 2, the results showed a decrease of 18:2 n-6 and of long chain products of desaturation in rats fed on fructose diet. Delta-6 n-3 desaturase activity was significantly increased, whereas gene expression tended to decrease. Feeding fructose induced a significant increase in delta-9 desaturated products, suggesting a stimulation of stearoyl-CoA desaturase. These changes in monounsaturated fatty acids strongly differ from those observed in the streptozotocin experiment, indicating that the effects on lipogenesis of hypertension linked to diabetes differ according to the type of diabetes. Then, these results indicate that the liver steatosis observed during genetic hypertension was reinforced by fructose feeding. All together, the present results showed that hypertension associated to type 1 or type 2 diabetes exacerbated the damage caused by diabetes or hypertension alone on liver lipid metabolism. The metabolic effects induced by fructose being very similar to those found in human NIDDM, SHR fed a fructose-rich diet appears to be an appropriate model for studying the consequences of the combination of hypertension and NIDDM in the metabolic syndrome diseases.

[Unopened colostomy, a protective procedure for high risk colorectal anastomosis]. / La colostomie non ouverte, procédé de protection des anastomoses colo-rectales à haut risque.

The classical "protective colostomy" upstream of a high risk colo-rectal anastomosis is not fully effective and requires subsequent reconstructive surgery. For these reasons, it is little used to date. Unopened colostomy provides complete obturation above the anastomosis and therefore effective protection. When no anastomotic fistula develops, the colostomy loop is re-entered on the 8th postoperative day. In case of fistula, it is opened and becomes functional. The procedure is easy to perform and well accepted by the patient. It increases surgical safety and shortens the duration of stay in hospital.

Characteristics and origin of frontal paroxysmal responses induced by light stimulation in the Papio papio under allylglycine.

In the Papio papio, curarized and rendered photosensitive by injection of a subconvulsant dose of DL-allylglycine, single flashes induce frontal paroxysmal evoked responses on condition that they be preceded by trains of intermittent light stimulation (ILS). The characteristics of these responses have been compared to those of non-paroxysmal responses induced in the same cortical area by isolated flashes (not preceded by trains of ILS). The paroxysmal responses resemble spikes and waves and consist of one or two positive spikes followed by a slow negative wave. The intracortical distribution of these responses has been studied in the motor cortex. The non-paroxysmal responses are probably not generated at this level. On the other hand, observations made during paroxysmal responses show the existence of two cortical responses; this demonstration follows from the existence of an inversion in some response components, linked to a negativity and a local cellular activation. A generator, situated in the pyramidal cell layer, is active during the positive surface spikes; the other generator, situated in the more superficial cortical layers, is active at the beginning of the slow negative surface wave. The cortical and subcortical afferents likely to bring these generators into play are discussed.

[Modification of frontal and occipital cortical excitability provoked by trains of flashes in Papio papio (author's transl)]. / Modifications de l'excitabilite corticale (frontale et occipitale) par des trains d'eclairs chez le singe Papio papio.

The experimental conditions necessary for obtaining an evoked paroxysmal response from the frontal cortex were studied in the baboon Papio papio. The trigger stimulus was comprised of an isolated flash preceded by a train of intermittent light stimulation (SLI). Two conditions were necessary for the appearance of paroxysmal responses: a subconvulsant dose of DL-allylglycine had to be injected at least 3 h previous to recording, and a sufficient number of SLI trains had to be presented to the animal. The paroxysmal responses disappeared as soon as SLI trains were stopped. At the same time, modifications in the evoked occipital potential continue, although these do not become paroxysmal. These modifications appear either simultaneously with or previous to the paroxysmal frontal response.

Absence of seizure activity following focal cerebral injection of enkephalins in a primate.

Baboons (Papio papio) with photosensitive have been chronically prepared with guide cannulae and deep electrodes to study the effects of focal injections of opioids. In the hippocampus, amygdala and thalamus (centre median) 50--100 micrograms morphine, 20--100 micrograms Met-enkephalin or 2--10 micrograms FK 33,824 do not induce local or general electrographic or motor signs of epilepsy. The acute epileptogenic effect of morphine and enkephalins observed in rats is not a general phenomenon whereas the anticonvulsant action of opioids acting on mu-receptors is seen in rodents and primates.

Cortical unit activity during intermittent photic stimulation in Papio papio. Relationship with paroxysmal fronto-rolandic activity.

Extracellular records have been made in the fronto-rolandic (FR), parietal and occipital cortical areas in Papio papio injected with allylglycine and paralysed with a synthetic curarizing agent. The organization of the unit discharges in the absence of intermittent light stimulation (ILS) is normal. During ILS, unit discharges in the FR cortex are organized in bursts of high frequency that are synchronous with the spikes of the EEG paroxysmal discharges (PD) in the same territory; this burst-organized FR activity is reversible, and bursts disappear when the ILS stops. In addition, in the FR cortex, triple or single flashes induced paroxysmal visual evoked potentials (PVEP) whose spikes were accompanied by bursts identical to the preceding ones. The slow waves which constituted the PD and the PVEP corresponded to a transitory inhibition of the FR neuronal activity. From the unit discharge patterns, no difference was observed between the two EEG paroxysmal activities recorded. No pattern of discharge in bursts was ever observed in the parietal and occipital cortex. The synchronizing role of the light stimulation in the FR cortex in Papio papio under allylglycine is discussed and the results are used to compare the experimental model with the naturally highly photosensitive animal. The particular reactivity of the FR cortex with respect to other regions is also discussed. Finally, the results bring forth new information in favour of a similarity between PD and PVEP.

Sustained limbic seizures induced by intraamygdaloid kainic acid in the baboon: Symptomatology and neuropathological consequences.

In Papio papio baboons chronically prepared for cortical and deep electroencephalographic recording, injection of kainic acid into the amygdala (7 animals) or temporal pole (2 animals) gave rise to focal epileptic discharges lasting 15 to 150 hours. Electrographically, the seizure activity spread ipsilaterally and contralaterally within the limbic system but did not become generalized. The principal associated motor signs were arrest of movement and oral automatisms. Histological examination after two to ten days demonstrated lesions (neuron loss and gliosis) at the injection site that varied according to the amount of kainic acid injected (2 to 68 microgram). "Remote" lesions occurred in the ipsilateral hippocampus (end-folium and Sommer sector) and neocortex (occipital and frontal regions). The hippocampal lesions were comparable to those previously described as consequent to status epilepticus.