Erythropoietin in the serum and cerebrospinal fluid of epileptic children

Recent years' research has revealed a specific, neuroprotective enythropoietin [EPO] system in the central and peripheral nervous system, that is upregulated by neuronal damage due to brain hypoxia. Few studies have investigated the endogenous production of EPO in human nervous system. The presence, origin and clinical importance of EPO in epileptic children are investigated for the first time. Forty-five children divided into 3 groups were studied. Group I included 15 recently diagnosed epileptics, not receiving antiepileptic drugs [AEDs], aged 3.8-15 years. Group II consisted of 15 refractory epileptics on AEDs for more than 1 year, aged 3.5-14.7 years. Fifteen healthy children of matched age, sex and socioeconomic status represented groups III [control group]. All children were suffering no hematological, neurological or renal diseases. They were subjected to detailed history, thorough clinical examination an routine investigations [CBC, urea, creatinine, ESR, CRP]. CT brain and EEG were done for epileptic children. Serum levels of albumin [by a colorimetric reaction] and serum prealbumin levels and CSF levels of albumin and prealbumin [by nephelometry] were measured for all children. Serum and CSF levels of EPO were assessed 12 hours after epileptic fits and on admission of control children [using erythropoietin ELISA kit]. Family history was positive for epilepsy in 16.67% of our epileptic children. Their most common types of convulsions were generalized tonic-clonic [GTC], generalized tonic [GT], myoclonic then focal seizures. CT brain was normal in the majority. A minority showed brain atrophy, calcification, hemorrhage, and infarction. EEG showed focal [FEA], generalized [GEA] and multifiocal epileptic activities among our recent epileptics while diffuse slowing [DS] and burst suppression were additional EGG findings among refractory epileptics. The serum and CSF levels of albumin, and prealbumin were normal just as control levels with no correlation with other demographic, clinical and laboratory studied variables. Q albumin was normal in epileptic children indicating the integrity of the blood brain barrier [BBB]. Q prealbumin was as expected markedly higher than Q albumin among epileptic and control children as it has a well known CNS synthesis. The serum levels of EPO were not significantly different in epileptic children, while its CSF levels were significantly higher compared to control children. The degree of elevation of the CSF levels of EPO among refractory epileptics was significantly lesser than that observed among recent epileptics. The CSF levels of EPO in recent epileptics were directly proportionate to the duration of the epileptic fits, while they were inversely proportionate to it in refractory epileptics; a disturbed and/or exhausted neuroprotective role of EPO among prolonged and refractory epileptics may be an explanation. So far; as the Q EPO is much higher [as Q prealbumin] than Q albumin, as there is no significant correlation between CSF and serum levels of EPO among epileptics, as there is no significant correlation between CSF levels EPO and Q albumin and as there is no significant correlation between Q EPO and Q albumin among epileptics and control children; it is concluded that the origin of this CSF erythropoietin is the brain; as a neuroprotective cytokine against neuronal damage caused by the epileptic fits, with the duration of the fit as a determinant factor. As commercially available forms of genetically engineered EPO are safely used for several indications in pediatrics; it is concluded that EPO is an ideal compound to study and it should be thoroughly evaluated in epileptic children, specially the refractory epilepsies and those with prolonged epileptic fits considering a possible therapeutic potential for EPO. It is also concluded that EPO in the CSF of epileptic children is a marker of epileptic fits and has its clinical indications in prognosis and therapeutic intervention

IgG2 immunodeficiency: association to pediatric patients with bacterial meningoencephalitis

An IgG subclass deficiency is often associated with bacterial infections. We studied four pediatric patients suffering from meningoencephalitis, two of them due to Streptococcus pneumoniae and two due to Haemophilus influenzae type b. Simultaneous diagnostic serum and cerebrospinal fluid samples were taken during income. The four subclasses of IgG and albumin were quantified in both biologic fluids by radial immunodiffusion. Very low levels of seric IgG2 with non detectable cerebrospinal fluid IgG2 were found in the patients. No intrathecal IgG subclass synthesis was found in two patients. One patient with S. pneumoniae had IgG3 intrathecal synthesis. Intrathecal IgG1, IgG3 and IgG4 synthesis was found in one patient suffering from H. influenzae according with reibergrams. Substitutive therapy with intravenous gammaglobulin was given to the patients as part of the treatment.

Eletroneuromiografia e potenciais evocados somatossensitivos na mielopatia pelo HTLV-I / Electroneuromyography and somatosensory evoked potentials in HTLV-I associated myelopathy

Tivemos como objetivo investigar por eletroneuromiografia (ENMG) e potenciais evocados somatossensitivos a possibilidade de acometimento do nervo periférico na mielopatia pelo HTLV-I (HAM), correlacionando os achados com os parâmetros clínicos e com a síntese intratecal de anticorpos anti HTLV-I. Os pacientes tinham sorologia negativa para HIV e apresentaram VDRL negativo. Outras causas para mielopatia ou neuropatia periférica foram excluídas. De 32 pacientes que realizaram ENMG, em 34,3 por cento ela foi considerada sugestiva de neuropatia periférica. Esta foi sobretudo assimétrica (82 por cento), sensitivo-motora (90 por cento), com padrao axonal (54,5 por cento) ou misto (45,4 por cento). Em 63,6 por cento dos casos de neuropatia periférica, havia sintomas correlatos. O potencial evocado auditivo foi anormal em apenas um caso. O potencial evocado visual foi normal em 28,5 por cento dos casos. Nao havia sinais ou sintomas auditivos ou visuais. Em 85,5 por cento dos casos obteve-se potencial evocado sensitivo alterado. Desses, 50 por cento tinham manifestaçao clínica compatível. Em 28 por cento dos pacientes com potencial evocado sensitivo anormal houve concomitância de ENMG sugestiva de neuropatia periférica.

Avaliaçäo da barreira hemato-encefálica no transplante de medula óssea / Blood-brain barrier evaluation in bone marrow transplantation

A barreira hemato-encefálica (BHE) contribui para o isolamento imunológico do sistema nervoso central (SNC). Sua avaliaçäo nunca foi realizada em pacientes submetidos a transplante de medula óssea (TMO). Neste estudo a integridade da BHE foi avaliada através das proteínas do LCR, de forma quantitativa, a fim de observar a incidência e entender a fisiopatologia da doença do enxerto contra o hospedeiro crônica (DECH-C) no SNC. Foram estudadas amostras pareadas de LCR e soro de 33 pacientes com leucemia mielóide crônica submetidos a TMO alogênico, de doador aparentado, HLA idêntico. As amostras foram coletadas nos períodos pré TMO, pós TMO e concomitante à DECH-C. Nao foi evidenciada quebra de BHE durante a DECH-C em enhum dos casos estudados.

Proteína C-reactiva en suero y líquido cefalorraquídeo en meningoencefalitis por Echo 4 y Neisseria meningitidis / C-reactive protein in serum and cerebrospinal fluid in meningoencephalitis due to echovirus type 4 and Neisseria meningitidis

Se comprueba la utilidad de la determinación de proteína C-reactiva en suero y líquido cefalorraquídeo durante un brote epidémico de meningoencefalitis. Se estudiaron 160 pacientes pediátricos con meningoencefalitis por Echo 4 y 76 por Neisseria meningitidis B. Se les cuantificó proteína C-reactiva y albúmina en suero y líquido cefalorraquídeo por inmunodifusión simple. Se encontraron diferencias significativas entre las concentraciones séricas de proteína C-reactiva de las meningoencefalitis bacterianas con los más altos niveles con respecto a las virales, con valores medios de 0,97 y 0,39 g/L respectivamente. En el líquido cefalorraquídeo la proteína C-reactiva dependió de las permeabilidad de la barrera hematoencefálica. No hubo variaciones en ambos líquidos biológicos con respecto a la edad. Las razones líquido cefalorraquídeo/suero de proteína C-reactiva poseen correlación significativa con la razón albúmina en la meningoencefalitis viral, no así en las bacterianas. Estas diferencias permiten que la proteína C-reactiva pueda ser utilizada para dar información etiológica auxiliar

Aplicación de una nueva fórmula en la síntesis intratecal de inmunoglobulinas / Implementations of a new formula in the intrathecal synthesis of immunoglobulins

Se aplicó la fórmula propuesta por Reiber y Felgenhauer a 462 pacientes pediátricos con meningoencefalitis. Se cuantificaron la IgA, IgM, IgG y la albúmina en suero y el líquido cefalorraquídeo por inmunodifusión radial. Hubo síntesis de IgA en el 58,87%, de IgM en el 57,79% y de IgC en el 23,8% de los pacientes estudiados. La cantidad de inmunoglobulinas sintetizadas con respecto al contenido total en el líquido cefalorraquídeo tuvo una alta variabilidad. En el caso de la IgM media local sintetizada fue mayor que en el resto de las inmunoglobulinas