L'incidence annuelle du syndrome de Guillain-Barre est de 1,5/100000 habitants La mortalite actuelle est estimee a environ 5 % d'apres des essais therapeutiques recents, bien conduits Dix pour cent des malades gardent des sequelles motrices tres invalidantes un an apres le debut des premiers signes neurologiques La prise en charge de ces malades necessite des equipes entrainees, multidisciplinaires, pouvant pratiquer l'ensemble des therapeutiques specifiques La corticotherapie per os'ou par voie intraveineuse est inefficace Les echanges plasmatiques sont le premier traitement dont l'efficacite a ete demontree par rapport a un groupe controle Les indications sont maintenant mieux connues Les formes benignes (marche possible) beneficient de 2 echanges plasmatiques; 2 echanges supplementaires sont realises en cas d'aggravation Dans les formes intermediaires (marche impossible) et les formes severes (recours a la ventilation mecanique), 4 echanges plasmatiques sont conseilles Il n'est pas utile d'augmenter leur nombre dans les formes severes ou en cas d'absence d'amelioration De fortes doses d'immunoglobulines donnees par voie intraveineuse (lq IV) [0,4 g/kg/j pendant 5 jours] sont aussi efficaces que les echanges plasmatiques dans les formes intermediaires et severes Dans ces formes, le choix entre Ig IV et echanges plasmatiques depend des contre-indications respectives de ces traitements et de leur faisabilite Les travaux en cours ont comme objectif de mieux preciser les indications respectives des echanges plasmatiques et des lq IV dans des formes de gravite differente, leur morbidite comparee, la dose optimale des lq IV

[Guillain-Barré syndrome].

Background: This systematic literature review of the epidemiology of Guillain-Barre syndrome (GBS) identifies trends in incidence rates by age, study method and cause of disease. It

/pdf/the-epidemiology-of-guillain-barre-syndrome-worldwide-a-2l8y0557xv.pdf

The Epidemiology of Guillain-Barre Syndrome Worldwide A Systematic Literature Review

Kounis syndrome has been established as a hypersensitivity coronary disorder induced by various conditions, drugs, environmental exposures, foods and coronary stents. Allergic, hypersensitivity, anaphylactic and anaphylactoid reactions are associated with this syndrome. Vasospastic allergic angina, allergic myocardial infarction and stent thrombosis with occluding thrombus infiltrated by eosinophils and/or mast cells constitute are the three reported, so far, variants of this syndrome. Apart from coronary arteries, it affects the cerebral and mesenteric arteries. Its manifestations are broadening and its etiology is continuously increasing. Kounis syndrome is a ubiquitous disease which represents a magnificent natural paradigm and nature's own experiment in a final trigger pathway implicated in cases of coronary artery spasm and plaque rupture. Kounis syndrome seems to be not a rare disease but an infrequently diagnosed clinical entity which has revealed that the same mediators released from the same inflammatory cells are also present and in acute coronary events of non allergic etiology. These cells are not only present in the culprit region before plaque erosion or rupture but they release their contents just before an actual coronary event. Therefore, awareness of etiology, epidemiology, pathogenesis and clinical manifestations seems to be important for its prognosis, diagnosis, treatment, prevention.

https://www.degruyter.com/downloadpdf/j/cclm.2016.54.issue-10/cclm-2016-0010/cclm-2016-0010.xml

Kounis syndrome: an update on epidemiology, pathogenesis, diagnosis and therapeutic management

Coronary Hypersensitivity Disorder: The Kounis Syndrome

Background In 1991, the first world survey of neuromuscular disorders (NMDs) was published in the peer reviewed literature. Since then, diagnostics have been greatly improved through genetic confirmation and consensus on criteria. This prompted us to search the scientific literature since 1990 for the epidemiology of NMDs. Objectives To study occurrence rates, gender and age distribution. Methods Pubmed was searched for 'epidemiology', 'incidence' and 'prevalence' regarding thirty NMDs for peer reviewed literature from 1990-2014. Results We found incidence rates for ten disorders, ranging from 0.05 to 9 per 100,000/yr. Most NMDs showed prevalence rates between 1 and 10 per 100,000 population, except for multifocal motor neuropathy, Lambert-Eaton myasthenic syndrome, Emery-Dreifuss dystrophy, oculopharyngeal muscular dystrophy, and congenital muscular dystrophies, which were l1/100,000. Post-polio syndrome and Charcot-Marie-Tooth disease revealed prevalences g10/100,000. Information regarding incidence, prevalence, age distribution and gender was complete for eight disorders. No data were found for chronic inflammatory demyelinating polyneuropathy, neuralgic amyotrophy, progressive spinal muscular atrophy, McArdle's and Pompe's disease. For the 17 remaining disorders, information was partially available. Conclusions Compared to 1991, prevalence rates of Becker muscular dystrophy, facioscapulohumeral dystrophy, myotonic dystrophy and Charcot-Marie-Tooth disease showed increase, yet with highly overlapping ranges with the exception of myotonic dystrophy. The sum of the available prevalence rates comprises only the tip of the iceberg, but is already in range with the prevalence of Parkinson's disease. Although individual NMDs are rare, as a group they are not.

/pdf/the-epidemiology-of-neuromuscular-disorders-a-comprehensive-2fmaypnniq.pdf

The Epidemiology of Neuromuscular Disorders: A Comprehensive Overview of the Literature.

Kounis syndrome is the concurrence of acute coronary syndromes with conditions associated with mast cell activation, such as allergies or hypersensitivity and anaphylactic or anaphylactoid insults that can involve other interrelated and interacting inflammatory cells behaving as a ‘ball of thread’. It is caused by inflammatory mediators such as neutral proteases including tryptase and chymase, arachidonic acid products, histamine, platelet activating factor and a variety of cytokines and chemokines released during the activation process. Platelets with FCeRI and FCeRII receptors also participate in the above cascade. Vasospastic allergic angina, allergic myocardial infarction and stent thrombosis with occluding thrombus infiltrated by eosinophils and/or mast cells constitute the three reported variants of this syndrome. Kounis syndrome is a ubiquitus disease that represents a magnificent natural paradigm and nature’s own experiment, in a final trigger pathway implicated in cases of coronary artery spasm a...

Kounis syndrome: a new twist on an old disease

https://www.internationaljournalofcardiology.com/article/S0167-5273(11)00465-7/pdf

Eosinophilic responses to stent implantation and the risk of Kounis hypersensitivity associated coronary syndrome.

Objective
We performed a systematic review and meta-analysis to evaluate the proposed association of restless legs syndrome (RLS) with cerebrovascular/cardiovascular outcomes.

Methods
We calculated the corresponding odds ratios on the prevalence of cerebrovascular/cardiovascular risk factors and standardized mean differences on the reported mean age at baseline between RLS patients and controls. We also calculated the corresponding risk ratios and adjusted for potential confounders hazard ratios (HRsadjusted) on the reported outcomes of interest between RLS patients and controls.

Results
We identified 8 eligible studies (644 506 patients, mean age: 60.2 years, 36.2% males; 3.3% with RLS). RLS patients were found to have significantly higher prevalence of hypertension (P = .002), diabetes (P = .003) and hyperlipidemia (P = .010) compared to controls. In the unadjusted analyses of prospective observational studies, RLS patients were found to have significantly higher risk for cerebrovascular ischaemia (P = .01) and all-cause mortality (P = .04) compared to controls during follow-up, while in the adjusted for potential confounders analyses RLS patients were only found to have a higher risk of all-cause mortality (HR adjusted=1.52, 95% CI: 1.17-1.97, P = .002).

Conclusions
The present report does not provide evidence for an increased risk of cerebrovascular and cardiovascular events in RLS patients, which highlights the vast presence of confounding factors.

Restless legs syndrome and cerebrovascular/cardiovascular events: Systematic review and meta-analysis.

Objective –  We present the epidemiological and clinical-laboratory features of Guillain-Barre syndrome (GBS) in northwest Greece over a 9.5-year period.



Materials and methods –  We studied all the patients with GBS who were admitted to our neurology inpatient service from January 1996 to May 2005 and compared them with previously published series.



Results –  Forty-six patients were hospitalized during this period. The average crude incidence rate was 1.22/100,000 populations per year, and males were more susceptible than females. There was a spring clustering, as 52.17% presented the syndrome during spring. The axonal type of GBS was recorded in 13.04% of the patients. The most frequent presenting symptom was dysesthetic numbness (52.17%). A large number of patients (56.52%) had up to three times the elevation of liver function values that resolved in a few weeks. Most patients had an excellent recovery and no deaths were recorded.



Conclusions –  In our series, there was no difference in the incidence rate and subtypes of GBS but there was a significant seasonality with spring clustering. A transient elevation of transaminases of undetermined etiology was noted in more than a half of our patients. Although seven patients (15.21%) had significant neurologic sequelae, no deaths occurred.

Guillain‐Barré syndrome in northwest Greece

Objective – The vascular protective effects of estrogens are mediated by their binding to the two known estrogen receptors. In this study, we examine the association of stroke with two common polymorphisms of the ESR1 gene in patients with metabolic syndrome.



Materials and methods –  DNA from 130 patients hospitalized for ischemic stroke and 240 healthy controls were genotyped for ESR1 PvuII and XbaI polymorphisms.



Results –  Comparing female and male patients, it was found that CCGG diplotype is more frequent in male patients (P = 0.03). In addition, the AA genotype is associated with the onset of stroke at a younger age in the male patient group (P < 0.05).



Conclusions –  These findings suggest that PvuII and XbaI polymorphisms may affect the age at onset of the first stroke and the probability of developing cerebrovascular disease.

Sotiris Giannopoulos

Papers

Kounis syndrome: a new twist on an old disease

Eosinophilic responses to stent implantation and the risk of Kounis hypersensitivity associated coronary syndrome.

Restless legs syndrome and cerebrovascular/cardiovascular events: Systematic review and meta-analysis.

Guillain‐Barré syndrome in northwest Greece

Association of estrogen receptor-alpha gene polymorphisms with stroke risk in patients with metabolic syndrome.