Síndrome de Schnitzler: descripción de 3 casos / Schnitzler syndrome: report of 3 cases

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Mechanism-based treatment in complex regional pain syndromes.

Complex regional pain syndromes (CRPS) are multifactorial disorders with complex aetiology and pathogenesis. Management of CRPS is challenging, partly because of a lack of clinical data regarding the efficacy of the various therapies, and partly because successful treatment of CRPS requires a multidisciplinary, patient-tailored approach. The pain in CRPS is often described as typical 'burning' neuropathic pain, and is accompanied by a variety of sensory, motor and autonomic signs and symptoms. Because research into therapies specifically in CRPS has been scarce, treatment for these syndromes has been largely based on therapeutic strategies adapted from neuropathic pain states; however, increased understanding of the pathogenesis of CRPS has provided the opportunity to develop mechanism-based treatments. The interactions between the multiple pathophysiological mechanisms that contribute to the development, progression and maintenance of CRPS remain poorly understood. This Review describes the challenges in linking the current theories and knowledge of pathophysiological mechanisms to the mode of actions of the different treatment approaches. We discuss the current treatment strategies for CRPS, including pharmacotherapy, sympathetic ganglion block interventions, psychological support, physiotherapy and occupational therapy, and establish the concept of mechanism-based treatment for CRPS.

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Non-surgical therapy for bilateral glossopharyngeal neuralgia caused by Eagle's syndrome, diagnosed by three-dimensional computed tomography: a case report.

Eagle's syndrome is an uncommon sequela of elongation of the styloid process. Symptoms include recurrent throat pain and anterolateral neck pain, with referred pain to the ear. We report a 65-year-old man who presented with bilateral glossopharyngeal neuralgia. We performed three-dimensional computed tomography which revealed that the right styloid process was 35.1 mm and the left process was 29.6 mm, leading to diagnosis of an elongated styloid process, i.e. Eagle's syndrome. Because the patient refused surgical treatment, conservative therapy was selected. Oral gabapentin, stellate ganglion block, and 8 % lidocaine spray on the tonsillar branches of the glossopharyngeal nerve resulted in complete resolution of the paroxysms of pain in approximately 3 weeks.

Placebo-controlled pilot trial of mecamylamine for treatment of autism spectrum disorders.

OBJECTIVE: To explore possible benefits of a nicotinic acetylcholine receptor (nAChR) agent for autistic symptoms based on postmortem observation of nAChR abnormalities (deficient α4ß2 nAChRs, excess α7 nAChRs) in brains of patients with autism. METHOD: Mecamylamine, because of its safety record in children with other disorders, was chosen for this first exploration. Twenty children with autism spectrum disorder age 4-12 years were randomly assigned for 14 weeks to placebo (n=8) or mecamylamine (n=12) in ascending fixed doses: 0.5 mg/day for 6 weeks, 2.5 mg for 2 weeks, then 5 mg/day for 6 weeks. Improvement was rated by a blinded independent evaluator. Because of small sample, data analysis was descriptive. RESULTS: Eighteen participants (10 mecamylamine, 8 placebo) completed the study. All doses were well tolerated; the only side effect of note was constipation (50% compared with 25% of placebo group). Three children had clinically nonsignificant electrocardiographic QT prolongation. Both groups showed modest to moderate improvement, but differences between groups were negligible. On the primary outcome measure, the Ohio Autism Clinical Impressions Scale, 90% of the active treatment group showed improvement at some point (but only 40% sustained it), compared with 62% on placebo. Of the four in active treatment that sustained improvement, three had a maximum dose of 0.13-0.15 mg/kg/day, while those who regressed had doses ≥0.18 mg/kg/day. Graphed means suggested better outcome with lower mg/kg and longer medication duration. Four parents spontaneously reported reduced hyperactivity and irritability and better verbalization and continued mecamylamine at their own expense. CONCLUSION: Mecamylamine appeared to be safe, but not very effective in autism. The suggestion of better results at lower doses and longer exposure warrants consideration for future trials. The next step would be exploration of a more specific α4ß2 nAChR agonist, such as varenicline.

Early experiences of vasodilators and hypotensive anesthesia in children.

The physiological application of OHMS LAW explains the basis of hypotensive anesthesia. V = IR translates into: Pressure = Flow × Resistance or Blood pressure = Cardiac Output × Peripheral Resistance. If peripheral resistance is reduced by a vasodilator such as sodium nitroprusside (a short acting, vascular smooth muscle relaxant) or phenoxybenzamine (a long acting α adrenoreceptor antagonist), blood pressure will fall and vasoconstriction and bleeding will be reduced. A less desirable alternative to lowering blood pressure could be to reduce cardiac output by suppressing myocardial contractility using a ß(1) adrenoceptor antagonist or an inhalational agent such as isoflurane.

[Selected interventional methods for the treatment of chronic pain: Part 1: peripheral nerve block and sympathetic block]. / Ausgewählte interventionelle Verfahren zur Behandlung chronischer Schmerzen : Teil 1: periphere Nervenblockade und Sympathikusblockade.

Approximately 5-8 million people in Germany suffer from chronic pain. Some patients can obtain relief from specific interventional techniques. In detail these are blocks of the sympathetic chain and peripheral nerve blocks, regional anesthetic techniques close to the spinal cord and neuromodulation. Part 1 of this article presents peripheral nerve blocks using the example of intercostal blocks and blocks of the sympathetic chain. Peripheral nerve blocks are important for postoperative pain treatment. Only a few methods are used for chronic pain and this applies primarily to the intercostal block which is used for the treatment of pain occurring after thoracotomy, intercostal neuralgia and pain associated with infiltration of cancer. Blocks of the vegetative nervous system are accomplished on the ganglions of the head and the sympathetic chain and are therefore most commonly applied to treat headache, neuropathic and sympathetic pain in the area of abdomen and the extremities.

Novel mechanism of action hypothesized for stellate ganglion block related to melatonin.

The mechanism of action of stellate ganglion block has generally been explained by vasodilation within its sphere of innervation. However, the success of treatment cannot always be explained by just one mechanism of action, because its clinical indications in Japan extend to many diseases, including systemic diseases. We propose a new mechanism of action for stellate ganglion block that is based on correction of melatonin rhythm disorder resulting from increased sympathetic nerve tone and does not involve vasodilation.

[Ganglionic blockers in abdominal surgery: facts and hypotheses. I. Benzohexonium and motor function of the gastrointestinal system in the early postoperative period]. / Ganglioblokatory v abdominal'noi khirurgii: fakty i gipotezy. Soobshchenie I. Benzogeksonii i motornaia funktsiia zheludochno-kishechnogo trakta v rannem posleoperatsionnom periode.

The method of registration of the intraluminal pressure was used to study the function of the stomach, small intestine and sigmoid colon under conditions of partial ganglionic blockade in the first days after truncal vagotomy and resection of the stomach. It was found that benzohexonium in doses 0.1-0.3 mg/kg failed to substantially decrease the frequency of early functional motor-evacuation disorders of the "operated" stomach, but the results of using N-cholinolytic was better after truncal vagotomy than after resection of the stomach. Benzohexonium in doses 0.1-0.2 mg/kg failed to considerably stimulate the motor function of the small intestine while the doses of 0.3-0.4 mg/kg resulted in a decrease of its contractile activity. No reliable changes in the qualitative and quantitative parameters of the sigmoid colon motor function were found against the background of ganglionic blockade. So, for prevention and correction of early postoperative motor-evacuation disorders of the gastrointestinal tract the ganglionic blockade with N-cholinolytics should not be taken as a method of choice or a variant of monotherapy.

[Ganglionic blockers in abdominal surgery: facts and hypotheses. 2.Clinical, pathophysiological and pharmacological aspects of ganglionic blocker use]. / Ganglioblokatory v abdominal'noi khirurgii: fakty i gipotezy. Soobshchenie 2. Klinicheskie, patofiziologicheskie i farmakologicheskie aspekty primeneniia ganglioblokatorov.

The authors discuss facts and hypotheses on the effects of benzohexonium upon the motor activity of the intestine and the significance of N-cholinolytics for prophylactics and treatment of postoperative pareses of the gastrointestinal tract. The ganglioblockers possess antistress effect, reduce the degree of pathological vegetative reactions and facilitate realization of the mechanisms of selfregulation of functions of the small and large intestine. Using benzohexonium during operation and in the first days after it makes the intestinal pareses less frequent. N-cholinolytics however do not have a considerable stimulating influence on the contracting activity of the gastrointestinal tract that accounts for their not high effectiveness in treatment of early functional motor evacuatory disorders. The points of action of gangliolytics, those at the level of the intestinal wall included, can not be considered to be completely established, as well as the mechanisms of their indirect effect. The ganglionic blockade should be considered as the basic method of prophylactics of the postoperative paresis of the intestine.

Role of autonomic nervous system for development and suppression of motion sickness in Suncus murinus.

To clarify the role of autonomic nervous function in motion sickness, the effect of agents that act on the autonomic nervous system on the motion stimuli-induced emesis was studied in two strains of Suncus murinus (Jic:SUN-Her and Jic:SUN-Ler) with congenitally different sensitivity to veratrine sulfate. We demonstrated significant differences between the two strains in sensitivity to motion stimuli. Isoproterenol (2.5 mg kg(-1), s.c.) significantly prolonged the latency to the first emetic episode induced by motion stimuli and significantly decreased the number of emetic episodes in Jic:SUN-Her suncus. Hexamethoium (2.0 mg kg(-1), s.c.) tended to shorten the latency in Jic:SUN-Ler. Acetylcholine (1.2 mg kg(-1), s.c.) enhanced the emetic response in Jic:SUN-Ler, but atropine (4.0 mg kg(-1), s.c.) suppressed motion stimuli-induced emetic response in Jic:SUN-Her. These results suggest that the predominance of parasympathetic nervous activity is relevant to the enhancement of motion stimuli-induced emetic response, whereas the predominance of sympathetic nervous activity suppresses motion stimuli-induced emetic response. Norepinephrine (0.8 mg kg(-1), s.c.) enhanced motion stimuli-induced emesis contrary to isoproterenol in Jic:SUN-Ler although both drugs are adrenergic agents. However, atropine pretreatment (4.0 mg kg(-1), s.c.) inhibits norepinephrine-induced emetic response. It was considered that norepinephrine-induced emetic response might be dependent on a secondary increase of parasympathetic nervous activity due to bororeflex. Moreover, the different emetic response in Jic:SUN-Her and Jic:SUN-Ler suncus to motion stimuli and drug administration mentioned above indicated that different participation of autonomic nervous activity and/or afferent information from the baroreceptor in the emetic response may exist between these animal groups.

Differential effects of right versus left stellate ganglion block on left ventricular function in humans: an echocardiographic analysis.

STUDY OBJECTIVES: To evaluate the effects of unilateral stellate ganglion blockade on left ventricular function. DESIGN: Prospective cohort of patients with chronic regional pain syndrome type I and II of the upper extremity requiring therapeutic stellate ganglion blockade. SETTING: University-affiliated hospital. PATIENTS: Fifteen adult ASA physical status I and II patients with the diagnosis of chronic regional pain syndrome type I and II of the arm were studied. Right stellate ganglion block was performed in nine subjects and a left in six. INTERVENTIONS: Stellate ganglion block was performed with 10 mL of 1% plain Xylocaine. Transthoracic echocardiograms were performed immediately prior and 30 min following the block. MEASUREMENTS: Heart rate and blood pressure were monitored at regular intervals. Global systolic function was determined by calculating ejection fraction. Regional systolic motion was evaluated on the short axis and four-chamber views using the American Society of Echocardiography criteria. Diastolic function was assessed with pulsed-wave Doppler of the left ventricular outflow tract and the mitral valve. Data collected included isovolumic relaxation time and early and atrial velocity patterns. MAIN RESULTS: A successful stellate ganglion block was achieved in all patients. Blood pressure and heart rate were not significantly different during data collection. Patients who underwent a right stellate ganglion block showed no significant differences in systolic or diastolic function. Following a left stellate ganglion block, global and regional systolic function remained unchanged. Isovolumic relaxation time was increased but did not reach statistical significance (80 +/- 13 ms to 88 +/- 9 ms; p = 0.09). Left ventricular end-diastolic (LVEDV) and end-systolic volumes (LVESV) were significantly increased (LVEDV from 73 +/- 9 mL to 100 +/- 9 mL, p < 0.02; LVESV from 31 +/- 4 mL to 37 +/- 4 mL, p < 0.03). CONCLUSIONS: In patients without cardiovascular disease, unilateral denervation of the left ventricle after stellate ganglion block produces no clinical deleterious effects on left ventricular function.

Critical evaluation of chemical neurolysis of the sympathetic axis for cancer pain.

BACKGROUND: Patients with pain caused by cancer frequently experience visceral pain. In addition to oral pharmacologic therapy to manage pain, neurolytic blocks of the sympathetic axis are also effective in controlling visceral cancer pain. METHODS: Four types of neurolytic blocks (interpleural phenol, celiac plexus, superior hypogastric plexus, and ganglion impar) used in the treatment of visceral cancer pain are reviewed. RESULTS: Several studies have documented the efficacy of neurolytic blocks in reducing pain intensity and opioid consumption. However, the narrow risk-benefit ratio associated with neurolysis techniques requires knowledge of the implications associated with the different neurolytic blocks to minimize undesirable effects. CONCLUSIONS: Neurolysis of the sympathetic axis has been shown to be an effective and safe approach to treat visceral pain in cancer patients and should be incorporated in the armamentarium of the pain specialist as a useful adjunct to oral pharmacologic therapy.

Thermographic assessment of stellate ganglion block effectiveness during cardiosurgical procedures.

UNLABELLED: The study present thermographic assessment of the effectiveness of temporary stellate blockade performed during cardiosurgical procedures. The assumption behind this method was the increase in the temperature of upper extremity on the side of blockade, due to the broadening of arterial bed. MATERIAL AND METHOD: The study was conducted on a group of 30 patients (21 men and 9 women) operated due to coronary disease involving three vessels. Mean age of the patients was 53 years. After introduction of anaesthesia blockade were performed with 2 ml 2% lignocainum and 8 ml 0.5% bupivacaine solution using peratracheal approach. Blockade effectiveness was assessed on the basis of images obtained in thermovisual camera, comparing the temperatures of upper extremity before and within 15 minutes after performing the blockade. Free blood outflow from radial artery, its diameter and length were also evaluated. The results obtained were subject to statistical analysis. RESULTS: Twenty-three patients (76.6%) displayed the increase in the temperature of upper extremity by 1-3 degrees C. Free blood outflow from radial artery was greater in this group than in the remaining patients. CONCLUSIONS: Thermography is a useful method for the assessment of stellate blockade effectiveness. Effective blockade results in the increased blood flow in radial artery.

Analysis of peak magnitude and duration of analgesia produced by local anesthetics injected into sympathetic ganglia of complex regional pain syndrome patients.

OBJECTIVE: Pain-relieving effects of lidocaine/bupivicaine local anesthetic (LA) and saline (S) block of sympathetic ganglia (stellate block, 4 patients; lumbar sympathetic block, 3 patients) were compared in 7 complex regional pain syndrome (CRPS) patients on a double-blind crossover basis to evaluate the diagnostic and therapeutic value of local anesthetic sympathetic blocks. DESIGN: Patients rated their pain on a visual analog scale before and after blocks and were tested for mechanical allodynia one-half hour after blocks. Thereafter, they rated their pain intensity in diaries four times a day for 7 days. Each patient received two blocks, S and LA, and served as his own control. RESULTS: Both S and LA injections of sympathetic ganglia produced large reductions in pain intensity in 6 of 7 patients 30 minutes after block. These large reductions were accompanied by the reversal of mechanical allodynia in both S and LA. The mean difference between initial peak reduction in pain intensity produced by saline (68.7%) and active local anesthetic (74.4%) did not approach statistical significance. In striking contrast, the mean duration of pain relief was reliably longer in the case of LA (3 days, 18 hours) as compared with S ( 19.9 hours), a difference that occurred in all 7 patients. In a larger sample of 41 CRPS patients, signs of sympathetic efferent blockade, including Homer' s syndrome or skin surface temperature change, were not predictive of initial peak magnitude of pain relief from sympathetic blockade but were predictive of duration of pain reduction. CONCLUSION: The combination of these results provides evidence that duration of pain relief is affected by injection of local anesthetics into sympathetic ganglia. These results indicate that both magnitude and duration of pain reduction should be closely monitored to provide optimal efficacy in procedures that use local anesthetics to treat CRPS.

alpha- and beta-adrenoceptor blockade fail to prevent high sodium diet-induced left ventricular hypertrophy.

High sodium diet (HS, 8% NaCl) induces left ventricular hypertrophy (LVH) in normotensive rats without an increase in pressure or volume load or in resting cardiac sympathetic activity. HS may affect LV adrenoceptors density or affinity or their postreceptor pathways, thereby causing LVH. We therefore assessed the effects of HS with and without blockade of alpha1- or beta-adrenoceptors by terazosin or nadolol, alone or in combination, on resting hemodynamics, LV and right ventricular (RV) weights, and LV dimensions of male WKY rats. HS increased LV weight by 14% to 17%, and the ratio of LV wall thickness to radius by 18% to 23%. Singly or in combination, the adrenoceptor antagonists did not prevent HS-induced LVH, but instead aggravated it. The increased ratio of LV wall thickness to radius and of LV to RV were attenuated by terazosin or nadolol alone. Neither the resting LV peak-systolic or end-diastolic pressures nor the right atrial pressure was changed by HS, either alone or in combination with the blockers. The failure of chronic alpha1- or beta-blockade to prevent HS-induced LVH suggests that adrenoceptor activation is not important in evoking the LVH. However, the blockers shifted the LVH from a concentric to an eccentric form, suggesting an involvement of additional trophic factors during adrenoceptor blockade.

Small-bowel obstruction and the effects of lidocaine, atropine and hexamethonium on inflammation and fluid losses.

BACKGROUND: The profuse fluid losses and morbidity of patients suffering from obstructive ileus are closely related to inflammatory changes in the obstructed bowel wall. Previous experimental studies have shown that use of steroids and NSAIDs can reduce fluid losses in obstructive ileus. In the present study, we investigated the effects of lidocaine on fluid losses since local anesthetics have been shown to possess wide and potent anti-inflammatory properties. Hexamethonium and atropine were used to study the importance of the autonomic nervous system in bowel obstruction. METHOD: Experiments were performed in rats in vivo. After 18 h of total obstruction of the jejunum by thread ligation, a segment of the obstructed jejunum was placed in a chamber with intact nervous and vascular supply and net fluid transport was continuously registered by a gravimetric technique. Extravasation of Evans blue albumin as marker of inflammation was quantified by spectrophotometry. RESULTS: Hexamethonium (10 mg.kg-1 i.v.) significantly inhibited net fluid secretion (P < .05), while atropine (0.25 mg.kg-1 i.v.) had no significant effect. Net fluid secretion was reversed into absorption following an intravenous bolus dose of lidocaine (2 mg.kg-1) (P < 0.01) and topical administration of lidocaine (20 mg) on the serosa of the obstructed gut (P < 0.01). Single topical administration of lidocaine (20 mg) immediately before ligation significantly reduced net fluid secretion (P < 0.05) and inflammation (P < 0.05) in the obstructed bowel 20 h post-ligation compared to obstructed controls. CONCLUSION: Lidocaine significantly inhibited or prevented fluid losses when administered intravenously or topically to the obstructed gut. Mechanisms of action could be inhibition of nerve reflexes involved in fluid secretion and by inhibition of inflammation in the bowel wall. The inhibition of fluid losses by hexamethonium further supports the importance of the autonomic nervous system in the pathophysiology of bowel obstruction.