Identifying the direct effects of ammonia on the brain.

Elevated concentrations of ammonia in the brain as a result of hyperammonemia leads to cerebral dysfunction involving a spectrum of neuropsychiatric and neurological symptoms (impaired memory, shortened attention span, sleep-wake inversions, brain edema, intracranial hypertension, seizures, ataxia and coma). Many studies have demonstrated ammonia as a major player involved in the neuropathophysiology associated with liver failure and inherited urea cycle enzyme disorders. Ammonia in solution is composed of a gas (NH(3)) and an ionic (NH(4) (+)) component which are both capable of crossing plasma membranes through diffusion, channels and transport mechanisms and as a result have a direct effect on pH. Furthermore, NH(4) (+) has similar properties as K(+) and, therefore, competes with K(+) on K(+) transporters and channels resulting in a direct effect on membrane potential. Ammonia is also a product as well as a substrate for many different biochemical reactions and consequently, an increase in brain ammonia accompanies disturbances in cerebral metabolism. These direct effects of elevated ammonia concentrations on the brain will lead to a cascade of secondary effects and encephalopathy.

Brain edema in acute liver failure and chronic liver disease: Similarities and differences.

Hepatic encephalopathy (HE) is a complex neuropsychiatric syndrome that typically develops as a result of acute liver failure or chronic liver disease. Brain edema is a common feature associated with HE. In acute liver failure, brain edema contributes to an increase in intracranial pressure, which can fatally lead to brain stem herniation. In chronic liver disease, intracranial hypertension is rarely observed, even though brain edema may be present. This discrepancy in the development of intracranial hypertension in acute liver failure versus chronic liver disease suggests that brain edema plays a different role in relation to the onset of HE. Furthermore, the pathophysiological mechanisms involved in the development of brain edema in acute liver failure and chronic liver disease are dissimilar. This review explores the types of brain edema, the cells, and pathogenic factors involved in its development, while emphasizing the differences in acute liver failure versus chronic liver disease. The implications of brain edema developing as a neuropathological consequence of HE, or as a cause of HE, are also discussed.

Soluciones hipertónicas en trauma craneoencefálico: revisión sistemática de la literatura

El traumatismo craneoencefálico, es la epidemia silenciosa de nuestra época, que genera gastos en salud, en países como Estados Unidos, cercanos a los 60 billones de dólares anuales, y cerca de 400 billones en rehabilitación de los discapacitados. El pilar del manejo médico del trauma craneoencefálico moderado o severo, es la osmoterapia, principalmente con sustancias como el manitol y las soluciones hipertónicas. Se realizó la revisión de 14 bases de datos, encontrando 4657754 artículos, quedando al final 40 artículos después de un análisis exhaustivo, que se relacionaban con el manejo de la hipertensión endocraneana y terapia osmótica. Resultados: Se compararon diferentes estudios, encontrando gran variabilidad estos, sin homogenización en los análisis estadísticos, y la poca rigurosidad no permitieron, la recolección de datos y la comparación entre los diferentes estudios, no permitió realizar el meta-análisis y por esto se decidió la realización de una revisión sistemática de la literatura. Se evidenció principalmente tres cosas: la primera es la poca rigurosidad con la que se realizan los estudios clínicos; la segunda, es que aún falta mucha más investigación principalmente, la presencia de estudios clínicos aleatorizados multicéntricos, que logren dar una sólida evidencia y que genere validez científica que se requiere, a pesar de la evidencia clara en la práctica clínica; la tercera es la seguridad para su uso, con poca presencia de complicaciones para las soluciones salinas hipertónicas.

Neurocisticercose e síndrome de lennox-gastaut: relato de caso

Relata-se o caso de uma menina que, aos 2 anos de idade, apresentou a forma epiléptica, hidrocefálica e encefalítica da neurocisticercose, diagnosticada por exame do líquido cefalorraqueano e tomografia computadorizada de crânio, evolução com crises polimórficas, episódios de descompensação da hipertensão intracraniana por obstrução do sistema de derivação ventriculoperitoneal, retardo no desenvolvimento neuropsicomotor e cegueira até que, aos 10 anos de idade, foi diagnosticada síndrome de Lennox-Gastaut. Atualmente, a paciente tem 16 anos, apresenta sequelas neurológicas e crises parciais complexas com automatismos, parcialmente controladas com o uso de clobazan e oxcarbazepina. A primeira associação de neurocisticercose e síndrome de Lennox-Gastaut foi descrita em 1973, por Frochtengarten & Scarante, em uma menina com quadro clínico semelhante ao do caso relatado.

Edema cerebral crônico na neurocisticercose

Neste estudo retrospectivo, relatam-se as características clínicas do edema cerebral crônico (ECCr) em 34 pacientes com neurocisticercose (NCC), que apresentavam edema cerebral difuso, à tomografia computadorizada (TC), como característica comum. Todos foram tratados com dextroclorofeniramina e, 32 deles, com albendazol. O ECCr predominou no sexo feminino (73,5%) na faixa etária dos 11 - 40 anos (92,3%). A cefaléia ocorreu em 94,1% dos pacientes, náuseas/vômitos em 47,1%, crises epilépticas em 41,1% e distúrbios psíquicos em 38,2%. A hiperreflexia ocorreu em 82,3% e o papiledema em 58,8% e o exame neurológico normal em 11,8%. Na TC, o edema esteve associado a calcificações em 61,8% dos casos. As pressões liquóricas foram mais elevadas (p< 0,05) antes do tratamento. Atualmente, estão assintomáticos, ou com melhora clínica, 79,4% dos pacientes (57,1% deles sem medicação). Discute-se a possibilidade do ECCr, na NCC, ser uma manifestação antigênica, sem a presença concomitante de cistos parasitários, e poder representar mais uma condição clínica associada à hipertensão intracraniana benigna.

Trigone ventricular meningiomas surgical approaches

Objective: Report our experience with trigone ventricular meningiomas and review the surgical approaches to the trigone. Method: From 1989 to 2006, six patients with meningiomas of the trigone of the lateral ventricles underwent microsurgical resection. Their clinical features, image, follow up, and surgical approaches were retrospectively analyzed. Results: Five patients presented with large and one with small volume meningioma. Unspecific symptoms occurred in three patients; intracranial hypertension detected in three patients; homonymous hemianopsy in three; and motor deficit present in one patient. Three patients were operated by transparietal transcortical approach, two by middle temporal gyrus approach, and one by parieto-occipital interhemispheric precuneus approach. Total resection was achieved in all patients without additional deficits. Conclusion: Judicious preoperative plan, adequate knowledge of anatomy, and use of correct microsurgical techniques are fundamental in achieving complete resection of trigone meningioma with low morbidity.

Aspectos clínico-epidemiológicos da neurocisticercose no Brasil: análise crítica

Com o objetivo de mostrar as características da neurocisticercose (NCC) no Brasil, realizou-se análise critica da literatura nacional que mostrou incidência de 1,5% nas necropsias e de 3,0% nos estudos clínicos, correspondendo a 0,3% das admissões em hospitais gerais. em estudos soroepidemiológicos, a positividade para cisticercose foi de 2,3%. O paciente brasileiro com NCC pode apresentar um perfil clínico-epidemiológico geral (homem, 31-50 anos, procedência rural, manifestações epilépticas parciais complexas, LCR normal ou hiperproteinorraquia, calcificações ao exame de TC, constituindo a expressão da forma inativa da NCC) e outro de gravidade (mulher, 21-40 anos, procedência urbana, manifestações de cefaléia vascular e HIC, típica síndrome do LCR ou alteração de dois ou mais parâmetros, vesículas associadas ou não a calcificações ao exame de TC, constituindo a expressão da forma ativa da NCC). Os coeficientes de prevalência nacionais são muito subestimados, embora em duas cidades do interior de São Paulo tenham sido verificados os valores de 72:100.000 e 96:100.000/habitantes. Discutem-se aspectos relacionados à subestimação da prevalência desta neuroparasitose no Brasil.

Aspectos clínicos e evolutivos da hidrocefalia na neurocisticercose

Com o propósito de analisar os aspectos clínicos da hidrocefalia (HDC) na neurocisticercose (NCC), realizou-se o estudo retrospectivo de 47 prontuários de pacientes com HDC e NCC. Verificou-se que 70,2% eram homens, entre 21 e 50 anos. A hipertensão intracraniana (HIC) ocorreu em todos os pacientes, cefaléia (CEF) em 89,4%, meningoencefalite (ME) em 80,8% e distúrbios psíquicos (PSI) em 74,5%. A síndrome liquórica da NCC foi detectada em 65,9% pacientes. Além da HDC, as tomografias computadorizadas de crânio (TC) mostraram lesões císticas e edema cerebral difuso em 59,6% cada, calcificações em 55,3%. Dos 41 pacientes (87,2%) com derivação ventriculoperitoneal (DVP), em 22 (53,7%) deles foram necessárias uma a sete revisões/paciente (média=3). A evolução foi satisfatória em 51,1% e fatal em 31,9%. Conclui-se que a hidrocefalia é mais comum no sexo masculino em idade produtiva, tendo a HIC, CEF, MN e PSI como manifestações freqüentes e que, a necessidade de revisões de DVP, piora o prognóstico.

Cisto coloide no terceiro ventrículo e morte súbita em jovem

Morte súbita de jovem, sexo masculino, 23 anos, assintomático, suscitou verificação de óbito. Antes queixou-se de cefaleia excruciante, em sala de aula, caindo sobre o computador. Encéfalo apresentou edema e congestão vascular. Sem herniações. Cortes coronais evidenciaram dilatação dos ventrículos laterais e nódulo aderido ao teto do terceiro ventrículo. À microscopia o diagnóstico foi cisto coloide do terceiro ventrículo. A ameaça de morte súbita em portadores de cisto coloide é sério problema diagnóstico na emergência médica. A relevância deste caso está em lembrar aos médicos que esta entidade deve permanecer como diagnóstico diferencial nas cefaleias em crianças, adolescentes e adultos jovens.

Severe forms of neurocysticercosis: Treatment with albeimdazole

Study of 22 patients with the severe form of neurocysticercosis treated with albendazole (ABZ) administered in 6 different schedules ranging from 15 to 30 mg/kg/day for 21 to 60 days. Dextrochloropheniramine and ketoprofen were the adjuvant drugs Multiple symptoms were observed in 90 9% of patients. Intracranial hypertension was manifested in 90.9%. Hydrocephaly occurred in 86.4%. Evolution was satisfactory in 10 patients, 8 died and 4 had sequelae. Tomographic studies showed the appearance of an isolated IVth ventricle in 9 patients, after ventriculoperitoneal shunt, before ABZ treatment in 3 of them, during in 5 and after treatment in one. Median clinical follow-up duration was 10 months for the patients who died and 3-4 years for survivors. In 3 patients there was an increase in cyst size during the administration of the 15 mg/kg/day ABZ dose, which was not observed in any patient when the 30 mg/kg/day dose was used.

Polyradiculoneuropathy and neurocysticercosis: Case report

Report of a patient with the hydrocephalic and meningoencephalitic form of neurocysticercosis who simultaneously developed polyradiculoneuropathy and intracranial hypertension syndrome during the first week of treatment with albendazole. Etiologic agents associated with polyradiculoneuropathy related in the literature are cited. Some comments about the possible physiopathogeny of this entity in the presence of cysticercosis are also done. It is mentioned another case who presented polyradiculoneuropathy as the only manifestation of a probable cysticercosis of the nervous system. In this presented case, including the neurocysticercosis and even a mere coincidence of facts, some factors can have a relationship with the appearance of polyradiculoneuropathy as the surgical stress, the stress due to the severity of the clinical picture and the possible side effect of albendazole.

Epidemiology of neurocysticercosis in Brazil

A revision of literature was done with the objective of tracing an epidemiologic profile of neurocysticercosis (NCC) in Brazil. The prevalence was 0.12-9% in autopsies. The frequency was 0.03-7.5% in clinical series and 0.68-5.2% in seroepidemiological studies. The disease corresponds to 0.08-2.5% of admissions to general hospitals. Patient origin was rural in 30 63% of cases. The most involved age range (64-100%) was 11 to 60 years, with a predominance (22-67%) between 21 and 40 years. The male sex was the most affected (51-80%). In the severe forms there was a predominance of urban origin (53-62%) and of the female sex (53-75%). The period of hospitalization ranges from 1 to 254 days and 33 to 50% of patients suffer 1.7 ± 1.4 admissions. The clinical picture was variable, with a predominance of epileptic syndrome (22-92%) and intracranial hypertension (19-89%). Psychiatric manifestations were associated in 9-23% of patients. Lethality was 0.29% in terms of all diseases in general and 4.8-25.9% in terms of neurologic diseases. The asymptomatic form was detected in 6% of patients in clinical serie and in 48.5% of case from autopsies The racemose form and ventricular localization also was observed as asymptomatic form. Among the patients with cutaneous cysticercosis 65% of them showed neurologic manifestations.

Apert syndrome: Analysis of associated brain malformations and conformational changes determined by surgical treatment

Apert Syndrome, also called acrocephalosyndactylia type 1, is characterized by craniostenosis with early fusion of sutures of the vault and/ or cranial base, associated to mid-face hypoplasia, symmetric syndactylia of the hands and feet and other systemic malformations. CNS malformations and intracranial hypertension are frequently observed in these patients. Early surgical treatment aims to minimize the deleterious effects of intracranial hypertension. Fronto-orbital advancement, the usual surgical technique, increases the intracranial volume and improves the disposition of encephalic structures previously deformed by a short skull. This study analyzes CNS alterations revealed by magnetic resonance in 18 patients presenting Apert Syndrome, and the conformational alterations in the encephalic structures after surgical treatment. The patients' age in February 2001 ranged from 14 to 322 months (m=107). Image study included brain magnetic resonance showing ventricular enlargement in five cases (27.8%), corpus callosum hypoplasia in five cases (27.8%), septum pellucidum hypoplasia in five cases (27.8%), cavum vergae in two cases (11.1%) and, arachnoid cyst in the posterior fossa in two cases (11.1%). Absence of CNS alterations was noted in 44.4% of cases. A corpus callosum morphologic index was established by dividing its height by its length, which revealed values that ranged from 0.4409 to 1.0237. The values of this index were correlated to the occurrence or absence of surgical treatment (p=0.012; t=2.83). Data analysis allowed the conclusion that the corpus callosum morphologic measure quantified the conformational alterations of the cerebral structures determined by the surgical treatment.

Neurocysticercosis: The enigmatic disease

Neurocysticercosis (NCC) is an infection of the central nervous system (CNS) caused by the metacestode larval form of the parasite Taenia sp. Many factors can contribute to the endemic nature of cysticercosis. The inflammatory process that occurs in the tissue surrounding the parasite and/or distal from it can result from several associated mechanisms and may be disproportionate with the number of cysts. This discrepancy may lead to difficulty with the proper diagnosis in people from low endemic regions or regions that lack laboratory resources. In the CNS, the cysticerci have two basic forms, isolated cysts (Cysticercus cellulosae = CC) and racemose cysts (Cysticercus racemosus = CR), and may be meningeal, parenchymal, or ventricular or have a mixed location. The clinical manifestations are based on two fundamental syndromes that may occur in isolation or be associated: epilepsy and intracranial hypertension. They may be asymptomatic, symptomatic or fatal; have an acute, sub-acute or chronic picture; or may be in remission or exacerbated. The cerebrospinal fluid (CSF) may be normal, even in patients with viable cysticerci, until the patients begin to exhibit the classical syndrome of NCC in the CSF, or show changes in one or more routine analysed parameters. Computed tomography (CT) and magnetic resonance imaging (MRI) have allowed non-invasive diagnoses, but can lead to false negatives. Treatment is a highly controversial issue and is characterised by individualised therapy sessions. Two drugs are commonly used, praziquantel (PZQ) and albendazole (ABZ). The choice of anti-inflammatory drugs includes steroids and dextrochlorpheniramine (DCP). Hydrocephalus is a common secondary effect of NCC. Surgical cases of hydrocephalus must be submitted to ventricle-peritoneal shunt (VPS) immediately before cysticidal treatment, and surgical extirpation of the cyst may lead to an absence of the surrounding inflammatory process. The progression of NCC may be simple or complicated, have remission with or without treatment and may exhibit symptoms that can disappear for long periods of time or persist until death. Unknown, neglected and controversial aspects of NCC, such as the impaired fourth ventricle syndrome, the presence of chronic brain oedema and psychic complaints, in addition to the lack of detectable glucose in the CSF and re-infection are discussed. © 2011 Bentham Science Publishers.