Guillain-Barre syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.

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Diagnosis and management of Guillain–Barré syndrome in ten steps

Since the beginning of this century, humanity has been facing a new emerging, or re-emerging, virus threat almost every year: West Nile, Influenza A, avian flu, dengue, Chikungunya, SARS, MERS, Ebola, and now Zika, the latest newcomer. Zika virus (ZIKV), a flavivirus transmitted by Aedes mosquitoes, was identified in 1947 in a sentinel monkey in Uganda, and later on in humans in Nigeria. The virus was mainly confined to the African continent until it was detected in south-east Asia the 1980's, then in the Micronesia in 2007 and, more recently in the Americas in 2014, where it has displayed an explosive spread, as advised by the World Health Organization, which resulted in the infection of hundreds of thousands of people. ZIKV infection was characterized by causing a mild disease presented with fever, headache, rash, arthralgia, and conjunctivitis, with exceptional reports of an association with Guillain-Barre syndrome (GBS) and microcephaly. However, since the end of 2015, an increase in the number of GBS associated cases and an astonishing number of microcephaly in fetus and new-borns in Brazil have been related to ZIKV infection, raising serious worldwide public health concerns. Clarifying such worrisome relationships is, thus, a current unavoidable goal. Here, we extensively review what is currently known about ZIKV, from molecular biology, transmission routes, ecology, and epidemiology, to clinical manifestations, pathogenesis, diagnosis, prophylaxis, and public health.

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Zika Virus: the Latest Newcomer.

Intracerebral haemorrhage (ICH) is a common and serious disease. About 1 to 2 out of 10 patients with stroke have an ICH. The mortality of ICH is higher than that of ischaemic stroke. Only 31% are functionally independent at 3 months. Only 38% of the patients survive the 1(st) year. The cost of ICH is high. Hypertension is the major risk factor, increasing the risk of ICH about 4x. Up to half of hypertensive patients who suffer a ICH are either unaware of their hypertension, non-compliant with the medication or fail to control periodically their blood pressure levels Microbleeds and white matter changes are MRI markers of the risk of ICH. ICH has 3 main pathophysiological phases: arterial rupture and haematoma formation, haematoma enlargement and peri-haematoma oedema. Up to 40% of the haematomas grow in the first hours post-rupture. ICH growth is associated with early clinical deterioration. Two randomised clinical trials (RCTs) demonstrated that treatment with rFVIIa limited haematoma growth and improved outcome, but was associated with a increase in thromboembolic complications. Ventricular drainage with thrombolytics might improve outcome for patients with intraventricular bleeding. A large RCT and meta-analysis failed to show a benefit of surgery over conservative treatment in acute ICH.

Update on intracerebral haemorrhage.

Guillain-Barre syndrome (GBS) is characterized by acute onset and progressive course, and is usually associated with a good prognosis. However, there are forms of poor prognosis, needing ventilatory support and major deficits at discharge. With this study we try to identify the factors associated with a worse outcome. 106 cases of GBS admitted in our hospital between years 2000–2010 were reviewed. Epidemiological, clinical, therapeutical and evolutionary data were collected. At admission 45% had severe deficits, percentage which improves throughout the evolution of the illness, with full recovery or minor deficits in the 87% of patients at the first year review. Ages greater than 55 years, severity at admission (p < 0.001), injured cranial nerves (p = 0.008) and the needing of ventilator support (p = 0.003) were associated with greater sequels at the discharge and at the posterior reviews in the following months. 17% required mechanical ventilation (MV). Values < 250 L/min in the Peak Flow-test are associated with an increased likelihood of requiring MV (p < 0.001). Older age, severe deficits at onset, injured cranial nerves, requiring MV, and axonal lesion patterns in the NCS were demonstrated as poor prognostic factors. Peak Flow-test is a useful predictive factor of respiratory failure by its easy management.

https://link.springer.com/content/pdf/10.1186%2F1471-2377-13-95.pdf

Guillain-Barré syndrome: natural history and prognostic factors: a retrospective review of 106 cases.

Miller Fisher syndrome (MFS), a variant of the Guillain-Barre syndrome (GBS), is characterized by ophthalmoplegia, ataxia, and areflexia. The annual incidence is around one patient per one million population. The antiganglioside anti-GQ1b IgG antibody has a role in the pathogenesis of the syndrome, especially of ophthalmoplegia. The presence of this antibody in the serum can be identified in over 80% of the patients, peaking in the first week, whereas albuminocytological dissociation in the cerebrospinal fluid (CSF) appears later. The most consistent electrophysiological findings in MFS are reduced sensory nerve action potentials and absent H reflexes. More variability is seen with F waves and various investigations involving cranial structures. Although there are usually no abnormalities in MFS by routine neuroimaging, in a few cases, contrast enhancement of nerve roots and signs of central nervous system involvement were described supporting the hypothesis of an anti-GQ1b-syndrome, a continuum involving GBS, MFS, and Bickerstaff's brainstem encephalitis. Owing to the lack of randomized trials, treatments used for GBS (intravenous immunoglobulin and plasmapheresis) are usually applied, although from retrospective analyses, the outcome was similar between treated and untreated subjects. The outcome of MFS is usually good with case fatality of < 5%. In the few autopsy cases, macroscopic abnormalities were generally not seen in the nervous system. Microscopic examination of the peripheral nervous system (including cranial nerves) showed segmental demyelination with minimal perivascular infiltration with normal spinal cord and brain stem.

Miller Fisher syndrome: brief overview and update with a focus on electrophysiological findings.

Diabetes Mellitus (DM) affects the cardiovascular response of patients. To study this effect, interbeat intervals (IBI) and beat-to-beat systolic blood pressure (SBP) variability of patients during supine, standing and controlled breathing tests were analyzed in the time domain. Simultaneous noninvasive measurements of IBI and SBP for 30 recently diagnosed and 15 long-standing DM patients were compared with the results for 30 rigorously screened healthy subjects (control). A statistically significant distinction between control and diabetic subjects was provided by the standard deviation and the higher moments of the distributions (skewness, and kurtosis) with respect to the median. To compare IBI and SBP for different populations, we define a parameter, α, that combines the variability of the heart rate and the blood pressure, as the ratio of the radius of the moments for IBI and the same radius for SBP. As diabetes evolves, α decreases, standard deviation of the IBI detrended signal diminishes (heart rate signal becomes more "rigid"), skewness with respect to the median approaches zero (signal fluctuations gain symmetry), and kurtosis increases (fluctuations concentrate around the median). Diabetes produces not only a rigid heart rate, but also increases symmetry and has leptokurtic distributions. SBP time series exhibit the most variable behavior for recently diagnosed DM with platykurtic distributions. Under controlled breathing, SBP has symmetric distributions for DM patients, while control subjects have non-zero skewness. This may be due to a progressive decrease of parasympathetic and sympathetic activity to the heart and blood vessels as diabetes evolves.

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Heart Rate and Systolic Blood Pressure Variability in the Time Domain in Patients with Recent and Long-Standing Diabetes Mellitus

Thalamic and Mesencephalic Hemorrhages after Multiple Honeybee Stings: A Life-Threatening Apitherapy Complication Jose Maria Remes-Troche a, Jose Francisco Tellez-Zenteno b, Jorge Rojas-Serrano a, Horacio Senties-Madridb, Felipe Vega-Boadab, Guillermo Garcia-Ramos b Departments of a Internal Medicine and b Neurology and Psychiatry, Instituto Nacional de Ciencias Medicas y Nutricion ‘Salvador Zubiran’, Mexico City, Mexico

Thalamic and mesencephalic hemorrhages after multiple honeybee stings: a life-threatening apitherapy complication.

Introduccion. El sindrome de Guillain-Barre (SGB) es una urgencia neurologica que constituye la principal causa de paralisis flacida en el mundo, y que afecta a todos los grupos etarios. Se desconocen notablemente las caracteristicas epidemiologicas esenciales del SGB en la mayor parte de los paises de America Latina. Objetivo. Determinar la mortalidad asociada con el diagnostico de SGB en altas hospitalarias efectuadas durante el ano 2010 en instituciones de la Secretaria de Salud, Mexico. Pacientes y metodos. Se analizo la base de datos de altas hospitalarias de instituciones que pertenecen a la Secretaria de Salud. Se identificaron los casos de SGB mediante el codigo G61.0 de la Clasificacion Internacional de Enfermedades, 10.a revision (CIE-10). Se excluyeron los registros de pacientes menores de 18 anos y los casos sin informacion demografica completa. Resultados. Durante el ano 2010 se registraron 2.634.339 altas de hospitales que pertenecen a la Secretaria de Salud. Se identifico un total de 467 hospitalizaciones por SGB en adultos (mediana de edad: 41 anos; 62,1% hombres) de 121 instituciones sanitarias de los 32 estados federados de Mexico. Durante el verano y otono se registro la mayor frecuencia de hospitalizaciones por SGB. La mediana de la estancia hospitalaria fue de 8 dias y la tasa de mortalidad hospitalaria, del 10,5%. La probabilidad de muerte se asocio directamente con la edad, sin una tendencia particular respecto al sexo, hospital de atencion o entidad federativa. Conclusiones. En 2010, la mortalidad hospitalaria por SGB en esta parte del sistema sanitario mexicano fue mas alta de la que se informa en estudios contemporaneos. Se observo una asociacion estacional con la frecuencia de hospitalizaciones por SGB.

Mortalidad asociada al diagnóstico de síndrome de Guillain-Barré en adultos ingresados en instituciones del sistema sanitario mexicano

In the XIX century Claude Bernard discovered the action of the nervous system on the peripheral circulation. In the first half of the XX century Ewald Hering discovered the baro-receptor and the reflex control of the heart rate and blood pressure. Cowley and Guyton demonstrated that sino-aortic denervation induces persistent changes in the blood pressure in the dog. The autonomic nervous system is mainly responsible for the regulation of the circulation and blood pressure in the short term on a beat to beat basis. It controls the vasomotor tone, the heart rate and the cardiac output. With the advent of non invasive methods that measure the blood pressure on a beat to beat basis (Finapres) and with the methods of measurement of the variability of the blood pressure in the frequency domain (spectral analysis) we can currently measure many variables including heart rate, blood pressure, stroke volume, peripheral resistances and the baroreceptor sensitivity and make some inferences about their control mechanisms. These variables can be measured at rest in the supine position, standing up, during rhythmic breathing and during the Valsalva maneuver. In this article we present a review of the neural control of the blood pressure and heart rate.

http://www.scielo.org.mx/pdf/acm/v79s2/v79s2a20.pdf

Control neural de la circulación periférica y de la presión arterial

Introduccion: El sindrome de Guillain-Barre es la causa mas comun de paralisis generalizada aguda. La enfermedad usualmente es benigna y tiende a autolimitarse, pero en casos severos puede causar la muerte por insuficiencia respiratoria. Objetivo: Describir la experiencia obtenida en un centro de referencia, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, para pacientes con sindrome de Guillain-Barre (SGB). Material y metodos: Se realizo un analisis retrospectivo de 28 pacientes con el diagnostico de GB. Resultados: Se analizaron 28 pacientes durante un periodo de 10 anos. La edad promedio del grupo fue 37.6 anos ± 17.2. Trece pacientes eran del sexo masculino (46%) y 15 (54%) eran mujeres. Nueve pacientes (32%) tuvieron una infeccion de vias respiratorias superiores y cinco (18%) tuvieron un cuadro de gastroenteritis previo a los sintomas y 14 (50%) no tuvieron un factor precipitante. El tiempo de evolucion tuvo una mediana de siete dias (2-15). Veintiseis pacientes (93%) tuvieron una presentacion clinica tipica con una debilidad ascendente de extremidades y 18 (64%) tuvieron disestesias o parestesias. Dieciocho pacientes (64%) correspondieron a la variante clinica clasica PDIA (polirradiculopatia desmielinizante inflamatoria aguda), cinco (18%) correspondieron a la variante NASMA (neuropatia axonal sensitivo-motora aguda), tres (11%) NAMA (neuropatia axonal motora aguda), y dos (7%) presentaron el sindrome de Fisher-Miller. Veinticuatro pacientes (86%) tuvieron hiperproteinorraquia en el liquido cefalorraquideo. Quince pacientes (54%) requirieron ventilacion mecanica. Veinte pacientes (71%) tuvieron recuperacion total, seis (21%) tuvieron recuperacion parcial y dos (7%) pacientes no tuvieron ninguna respuesta. Discusion: Aunque la enfermedad tiende a autolimitarse en la mayoria de los casos, los cuadros mas severos son frecuentes en las unidades de tercer nivel como se muestra en nuestro estudio donde 54% requirieron ventilacion mecanica, sin embargo, la mayoria de estos casos tuvieron una buena respuesta (71%).

Horacio Sentíes-Madrid

Papers

Heart Rate and Systolic Blood Pressure Variability in the Time Domain in Patients with Recent and Long-Standing Diabetes Mellitus

Thalamic and mesencephalic hemorrhages after multiple honeybee stings: a life-threatening apitherapy complication.

Mortalidad asociada al diagnóstico de síndrome de Guillain-Barré en adultos ingresados en instituciones del sistema sanitario mexicano

Control neural de la circulación periférica y de la presión arterial

[Guillain-Barre syndrome. Experience in a third level hospital].