[Guillain-Barré syndrome and other autoimmune neurophaties: current therapy]. / Síndrome de Guillain-Barré y otras neuropatías autoinmunes: tratamiento actual.

Guillain-Barré syndrome (GBS) is characterized by rapidly progressive and generally ascending symmetrical muscle weakness, accompanied by decreased or absent osteotendinous reflexes. The inflammatory process may affect the myelin or the axon. There are 4 clinical forms of GBS: 1) acute inflammatory demyelinating polyradiculoneuropathy, 2) acute motor axonal neuropathy, 3) acute sensory and motor axonal neuropathy, and 4) the Miller-Fisher variant, which is characterized by ophthalmoplegia, ataxia and areflexia, with little muscle weakness. Diagnosis is based on the albumin-cytological dissociation observed at the end of the first week after the onset of symptoms and may persist until the third week, as well as on the specific neurophysiological alterations of each clinical form. The treatment of GBS will depend on the degree of severity, if the patient presents grade IV or less according to the Paradiso scale, it will be treated with Ig IV, if it presents grade V, the use of plasmapheresis and/or immunoadbosorption is recommended. In severe axonal cases, the use of corticosteroid bolus is recommended in initial stages. There is a clinical picture that overlaps GBS and chronic demyelinating polyneuropathy related to antibodies against neurophysin and contactin, in this case the appropriate therapy is rituximab.

El síndrome de Guillain-Barré (SGB) se caracteriza por debilidad muscular simétrica rápidamente progresiva y generalmente ascendente, acompañada de disminución o ausencia de reflejos osteotendinosos. El proceso inflamatorio puede afectar a la mielina o al axón. Existen 4 formas clínicas de SGB: 1) polirradiculoneuropatía desmielinizante inflamatoria aguda, 2) neuropatía axonal motora aguda, 3) neuropatía axonal sensitiva y motora aguda, y 4) la variante Miller-Fisher, que se caracteriza por oftalmoplejía, ataxia y arreflexia, con escasa debilidad muscular. El diagnóstico se basa en la disociación albúmino-citológica que se observa a final de la primera semana del inicio de los síntomas y puede persistir hasta la tercera semana, así como en las alteraciones neurofisiológicas específicas de cada forma clínica. El tratamiento el SGB, dependerá de la gravedad, si el paciente presenta grado IV o menor según la escala de Paradiso, se tratará con Ig IV, si presenta grado V, se recomienda el uso de plasmaféresis y/o inmunoadbosorción. En los casos axonales graves se recomienda el uso de bolus de corticoides en etapas iniciales. Existe un cuadro clínico que solapa SGB y polineuropatía desmielinizante crónica relacionado con anticuerpos contra neurofisina y contactina, en este caso la terapia adecuada es rituximab.

Prevalence of anti-DFS70 autoantibodies in a Latin American cohort of patients with systemic lupus erythematosus and without autoimmune diseases.

INTRODUCTION/OBJECTIVES: Anti-dense fine speckled 70 (DFS70) autoantibodies were reported to be more prevalent in healthy individuals than those with autoimmune diseases such as systemic lupus erythematosus (SLE). We determined anti-DFS70 autoantibody prevalence in a Latin American cohort of patients with SLE and healthy individuals. METHODS: This study included 127 individuals with anti-nuclear antibodies (ANAs; > 1:160) suggesting the presence of anti-DFS70, including 64 patients with SLE and 63 healthy controls. The anti-DFS70 autoantibodies were determined by immunoadsorption using NOVA Lite® HEp-2 Select kit with DAPI. Negative fluorescence after adsorption with the DFS70 antigen indicated anti-DFS70 autoantibody positivity. RESULTS: The presence of anti-DFS70 autoantibodies was confirmed by indirect immunofluorescence in 21 (33.3%) healthy controls and 8 (12.5%) patients with SLE (p = 0.005). Among the anti-DFS70-positive patients with SLE, the most frequent compromise was renal involvement in six cases (75%), 4 patients (37.5%) were positive for anti-Sm, which was the most frequently associated antibody, and one patient (12.5%) was positive for anti-DNA. CONCLUSIONS: Anti-DFS70 autoantibodies might be considered a biomarker to differentiate patients with SLE from ANA-positive individuals without autoimmune diseases. KEY POINTS: • In a Latin American cohort, the anti-DFS70 was higher in individuals without autoimmune diseases compared with that in patients with SLE.• The anti-DFS70 might have utility as a biomarker of exclusion in patients with non-specific clinical signs of AARDs.

Isolated abducens nerve palsy treated by immunoadsorption in a patient with diarrhea-associated hemolytic uremic syndrome / Parálisis ocular aislada del sexto par craneal tratada con inmunoadsorción en un paciente con síndrome urémico hemolítico asociado a diarrea

Shiga toxin-producing Escherichia coli (STEC) that causes a prodromal hemorrhagic enteritis is the main cause of hemolytic uremic syndrome (HUS) particularly in pediatric patients. It is characterized by acute kidney injury with microangiopathic hemolytic anemia and thrombocytopenia. The kidney and brain are the two major target organs, and neurological involvement is the most frequent cause of mortality. The time delay between bloody diarrhea and neurological symptoms ranges from few days to a month. Neurological disorders include disturbances in cognitive functions, focal neurological signs, epileptic seizures, myoclonus and neuropsychiatric symptoms. Cerebral magnetic resonance imaging reveals various patterns of hyperintensities distributed through cerebral matter or may be totally normal even the patient has severe neurological involvement. Electroencephalography usually show generalized or focal slowing of the background activity, spikes or sharp waves despite being normal in around 20% of patients. We present here an adult male patient referred to our center with requirement of hemodialysis due to diarrhea-associated HUS complicated by acute kidney injury. Later during the course of plasma exchange therapy the patient developed an isolated abducens nerve palsy. Complete renal recovery was achieved by plasma exchange therapy but abducens palsy remedied rescue introduction of immunoglobulin G (IgG) depletion by immunoadsorption

Escherichia coli, productor de toxina Shiga (STEC), que causa una enteritis hemorrágica en fase prodrómica, es la principal causa del síndrome urémico hemolítico (SUH), particularmente, en pacientes pediátricos. Se caracteriza por una lesión renal aguda con anemia hemolítica microangiopática y trombocitopenia. El riñón y el cerebro son los dos órganos principales a los que ataca, y la afectación neurológica es la causa más frecuente de mortalidad. El tiempo que transcurre entre la aparición de diarrea sanguinolenta y los síntomas neurológicos varía entre pocos días y un mes. Los trastornos neurológicos incluyen alteraciones en las funciones cognitivas, signos neurológicos focales, ataques epilépticos, mioclonías y síntomas neuropsiquiátricos. La resonancia magnética de cerebro revela varios patrones de hiperintensidades distribuidas a través de la materia cerebral o puede ser totalmente normal incluso si el paciente tiene un compromiso neurológico severo. El electroencefalograma generalmente muestra una disminución generalizada o focal de la actividad de fondo, picos u ondas agudas, a pesar de ser normal en alrededor del 20% de los pacientes. Presentamos un paciente adulto de sexo masculino, derivado a nuestro centro para ser tratado con hemodiálisis debido a SUH asociado a diarrea, complicado por insuficiencia renal aguda. Luego, durante el transcurso de la terapia de intercambio de plasma, el paciente desarrolló una parálisis ocular aislada del sexto par craneal. Se logró una recuperación renal completa por medio de la terapia de intercambio plasmático; no obstante, la parálisis del nervio motor ocular externo remedió la disminución de la inmunoglobulina G (IgG) mediante el tratamiento de rescate de inmunoadsorción

Assessing the efficacy of co-inoculation of wheat seedlings with the associative bacteria Paenibacillus polymyxa 1465 and Azospirillum brasilense Sp245.

Co-inoculation of associative bacteria, which have high nitrogen-fixing activity, tolerance for environmental conditions, and the ability to compete with the natural microflora, is used widely to enhance the growth and yields of agricultural plants. We evaluated the ability of 2 co-inoculated plant-growth-promoting rhizobacteria, Paenibacillus polymyxa 1465 and Azospirillum brasilense Sp245, to colonize roots of wheat (Triticum aestivum L. 'Saratovskaya 29') seedlings, and we assessed the morphometric parameters of wheat early in its development. Analysis by ELISA with polyclonal antibodies raised against the exopolysaccharide of P. polymyxa 1465 and the lipopolysaccharide of A. brasilense Sp245 demonstrated that the root-colonizing activity of A. brasilense was higher when the bacterium was co-inoculated with P. polymyxa than when it was inoculated singly. Immunofluorescence microscopy with Alexa Fluor 532-labeled antibodies revealed sites of attachment of co-inoculated P. polymyxa and A. brasilense and showed that the 2 bacteria colonized similar regions of the roots. Co-inoculation exerted a negative effect on wheat seedling development, inhibiting root length by 17.6%, total root weight by 11%, and total shoot weight by 12%. Under certain conditions, dual inoculation of wheat may prove ineffective, apparently owing to the competition between the rhizobacteria for colonization sites on the plant roots. The findings from this study may aid in developing techniques for mixed bacterial inoculation of cultivated plants.