Diagnóstico de encefalite límbica não paraneoplásica de etiologia imunomediada: estudo de caso e revisão de literatura / Diagnosis of immune-mediated non-paraneoplastic limbic encephalitis: case report and literature review

A encefalite límbica vem sendo descrita como um distúrbio neurológico raro, que afeta seletivamente as estruturas do sistema límbico. Clinicamente, é caracterizada como uma desordem neurológica debilitante, que se desenvolve como encefalopatia rapidamente progressiva, causada por inflamação encefálica. Objetivamos aqui relatar um caso de encefalite do sistema límbico de provável etiologia autoimune para melhor conhecimento da comunidade médica, bem como averiguar métodos diagnósticos deste quadro. Paciente do sexo masculino, 59 anos, admitido em nosso serviço com queixa de confusão mental. O exame clínico evidenciou desorientação, disartria, paresia e parestesia no hemicorpo esquerdo, dificuldade de marcha, desvio de rima e histórico de epilepsia há 2 anos. No estudo por ressonância magnética do crânio, foram observadas extensas lesões que acometiam a região mesial do lobo temporal direito, todo o hipocampo e giro para-hipocampal direito, estendendo-se pelo fórnix até a porção posterior do hipocampo esquerdo, substância branca do lobo frontal bilateral. Mediante os resultados da investigação complementar, o paciente foi tratado com pulsoterapia de metilpredinisolona por 5 dias, resultando na regressão parcial dos sintomas. Atualmente, o paciente se encontra em seguimento ambulatorial para acompanhamento. A encefalite límbica é uma doença rara, porém muito importante de ser investigada e diagnosticada precocemente, uma vez que a progressão da doença pode causar incapacidade e sequelas irreversíveis.

Limbic encephalitis has been described as a rare neurological disorder affecting the limbic system structures selectively. Clinically, it is characterized as a debilitating neurological syndrome that develops as a quickly progressive encephalopathy caused by brain inflammation. This paper reports a case of limbic encephalitis, probably of autoimmune etiology, aiming to improve the knowledge of the medical community, and to promote a debate on diagnosis methods for this pathology. The patient is male, 59 years old, and was admitted at our service complaining of mental confusion. The clinical examination showed disorientation, dysarthria, left hemiparesis and paresthesia, gait difficulties, light asymmetrical smile, and history of epilepsy 2 years ago. The magnetic resonance imaging of skull showed extensive lesions affecting the mesial region of the right temporal lobe, the entire hippocampus, and right parahippocampal gyrus, extending through the fornix to the posterior portion of the left hippocampus, white matter of bilateral frontal lobe. Based on the complementary investigation results, the patient was treated with intravenous methylprednisolone for five days. Currently, he is being followed in the outpatient's department. Although being rare, limbic encephalitis shall be investigated and diagnosed early because its progression can lead to disability and irreversible sequelae

Shunt porto-sistémico congénito en hígado no cirrótico como causante de síndrome confusional agudo reiterado: report of one case / Portosystemic shunt in a normal liver causing confusional states

Portosystemic shunts are rare vascularization disorders, and an uncommon cause of confusional states. We report an 87-year-old male with a previously normal cognitive status who was repeatedly admitted for sudden symptoms of disorientation and functional limitation. The patient had high ammonium levels which lead to the suspicion of the presence a portosystemic shunt, even in the absence of pre-existing liver disease. A contrast enhanced computed tomography of the abdomen confirmed the presence an abnormal communication of the right portal vein with the suprahepatic veins. The communication was embolized and the confusional states of the patient subsided.

Onset of psychosis at age 81? With regard to frontal lobe syndromes / Psicose inaugural aos 81 anos? A propósito das síndromes do lobo frontal

When the frontal lobe of the brain is affected important behavioral changes may occur mainly at the level of executive functioning, i.e., planning, decision-making, judgment and self-perception. However, the behavioral changes may be of different nature with marked indifference and apathy. We report a clinical case of an 81-year-old patient with sudden onset of behavioral changes that were initially interpreted as an acute confusional episode of infectious etiology, but actually they were due to an ischemic lesion in the frontal lobe.

O lobo frontal, quando afetado, pode provocar alterações importantes do comportamento, principalmente na função executiva: planejamento, tomada de decisões, juízo e autopercepção. Contudo, as alterações podem ser de outra natureza, caracterizando-se por marcada indiferença e apatia. É relatado aqui o caso de uma paciente de 81 anos, com alterações súbitas do comportamento, que foram interpretadas inicialmente como um quadro confusional agudo de natureza infecciosa, mas que se mostraram provocadas por uma lesão isquêmica na região frontal.

Acute computed tomography findings in patients with acute confusion of non-traumatic aetiology / Hallazgos agudos con tomografía computarizada en pacientes con confusión aguda de etiología no traumática

BACKGROUND: A retrospective review was undertaken of all patients referred for computed tomography (CT) scans of the head for acute onset of confusion, not consequent on head trauma, during the period June 1, 2004 to May 31, 2007. METHOD: Data were obtained by Microsoft Word search of the reports of the Radiology Department of the University Hospital of the West Indies, Kingston, Jamaica. Two hundred and twenty-one patients were reviewed: 103 men and 118 women. The mean age of the sample was 64 years; 168 patients (76%) were 50 years old or older. RESULT: Computed tomography scans were reported normal in 170 (76.9%) patients; 45 patients (20.4%) had definite acute intracranial CT findings. Findings were equivocal in three patients (1.4%) and unavailable for three (1.4%); 23.2% and 15.6% of patients above and below the age of 50 years respectively showed acute abnormalities on CT. The most common acute finding on CT scan was an ischaemic infarct (68%). Other abnormalities included intracerebral haemorrhage and metastases 6.2% each, toxoplasmosis and primary brain tumour 4.2% each and subdural haematoma and meningitis 2.1% each. The diagnoses of toxoplas-mosis were made based on appearances typical of toxoplasmosis on CT scans in patients whose request stated that they were HIV positive. CONCLUSION: In the sample reviewed, most patients who presented with acute confusion were above the age of 50 years. Overall, 20.4% of patients from all age groups had acute abnormalities on CT with a relative higher proportion, 23.2% versus 15.6% of those over 50 years, having acute pathology. The most common abnormality was an ischaemic infarct. This finding is similar to that in developed countries and unlike that seen in other developing countries where infectious aetiologies predominate.

ANTECEDENTES: Durante el período de junio, 2004 a mayo 31, 2007, se llevó a cabo una revisión retrospectiva de todos los pacientes remitidos para escaneos mediante tomografía computarizada (TC escáner) de la cabeza, en casos de ataques de confusión aguda, no originados por traumas en la cabeza. MÉTODO: Los datos fueron obtenidos mediante búsqueda con Microsoft Word de los informes del Departamento de Radiología del Hospital Universitario de West Indies, Kingston, Jamaica. Se revisaron doscientos veintiún pacientes: 103 hombres y 118 mujeres. La edad promedio de la muestra fue 64 años; 168 pacientes (76%) tenían 50 años de edad o más. RESULTADO: Los escaneos mediante tomografía computarizada fueron reportados como un procedimiento normal en 170 (76.9%) pacientes; 45 pacientes (20.4%) tuvieron hallazgos agudos intracraneales definidos por TC. Los hallazgos fueron equívocos en tres pacientes (1.4%) y no disponibles en tres (1.4%); 23.2% y 15.6% de los pacientes por encima y por debajo de la edad de 50 años respectivamente, mostraron anormalidades agudas en la TC. El hallazgo agudo más común con el escaneo de TC fue el infarto isquémico (68%). Otras anormalidades incluyeron hemorragias intracerebrales y metástasis, 6.2% respectivamente; toxoplasmosis y tumor primario del cerebro, 4.2% respectivamente, y hematoma subdural y meningitis, 2.1% respectivamente. CONCLUSIÓN: En la muestra revisada, la mayoría de los pacientes que presentaron confusión aguda se hallaban por encima de 50 años de edad. En general, 20.4% de los pacientes de todos los grupos etarios presentaban anormalidades agudas en la TC en una proporción relativamente más alta, siendo el caso que el 23.2% frente al 15.6% de aquéllos por encima de 50 años, presentaban patologías agudas. La anormalidad más común fue el infarto isquémico. Este hallazgo es similar al de los países desarrollados, y diferente al que se observa en otros países en vías de desarrollo, dónde predominan las etiologías infecciosas.

A comparative study of the clinico-aetiological profile of hyponatremia at presentation with that developing in the hospital

Background & objectives: Hyponatremia is a common problem encountered in patients presenting with nonspecific symptoms. We undertook this study to investigate the clinical profile of patients with hyponatremia, the precipitating factors, the response to therapy and to compare, using these parameters, hyponatremia at presentation to that developing in the hospital. Methods: Seventy consecutive patients with serum sodium less than or equal to 125 mmol/l at presentation or at any time during hospital admission were identified and studied using a proforma. The severity of hyponatremia, therapy given and time taken for recovery were analysed. Results: The mean age of patients was 48.1 ± 16.1 yr. The mean serum sodium was 117.8 ± 6.4 mmol/l. Confusion, headache and malaise were the most common symptoms, two patients had seizures, and 20.0 per cent patients showed no clinical manifestations. Nausea was significantly (P<0.05) more common in patients presenting with hyponatremia. 22 patients (31.4%) developed hyponatremia during their stay in the hospital. 3 patients (4.3%) presented with hyponatremia which got worse during the admission period. Most had multiple precipitating factors, decreased intake being the most common (82.9%), followed by increased losses (65.7%) and miscellaneous factors (70.0%). Drugs, fluid overload and inappropriate Ryle's tube feeds more commonly precipitated hyponatremia in in-hospital patients. Time taken for recovery showed negative correlation with the serum sodium. Patients with in-hospital hyponatremia took significantly longer time to recover (P<0.05). Interpretation & conclusions: Decreased intake was found to be the commonest cause of hyponatremia, thus, ensuring adequate oral intake, especially in patients on liquid diet and in manual labourers, and correction of hyponatremia as soon as an abnormality is detected is important.

Síndrome confusional agudo como forma de presentación de un absceso espinal / Acute confusional syndrome presenting as a spinal abscess

Fundamento: La alteración del nivel de conciencia puede ser producida por múltiples causas, es un síndrome que requiere de una actuación ordenada, rápida y eficaz; ya que se trata de una urgencia médica en la que el tiempo siempre va en contra del paciente. La aproximación al paciente geriátrico con alteración del nivel de conciencia debe ir encaminada al tratamiento de su causa y prevención de sus complicaciones. Objetivo. Presentar un caso poco frecuente con un absceso espinal que debutó con un síndrome confusional agudo. Caso Clínico: Paciente de 62 años de edad que inicia de forma aguda con cambio en su comportamiento, insomnio, abandono de hábitos cotidianos, se le olvida donde deja las cosas y está muy irritable. Al examen físico. Se encuentra: Trastornos en la perceptividad y esfera cognitiva, dolor a la percusión, movilización de las espinosas dorsales I Y II (DI-DII). Se practicó una Resonancia magnética nuclear (RMN) de región dorsal, la cual demostró la presencia de una lesión en el espacio epidural que se extendía desde el segundo al sexto segmento (DII a DVI) .Se llevó de forma urgente al salón de operaciones, se realizó abordaje posterior al raquis dorsal, a través del cual se evacuó un absceso epidural. En un período de 12 días las manifestaciones neuropsiquiatrías desaparecieron. Conclusiones. El síndrome confusional agudo es una patología que se puede ver entre el 10-15 por ciento de los pacientes con una enfermedad médico-quirúrgico, incidencia que se eleva hasta el 30 por ciento en los ancianos, y puede ser la forma de presentación poco frecuente de una infección intrarraquídea. El diagnóstico oportuno y tratamiento eficaz impidieron la progresión de las manifestaciones neurológicas y revirtieron la sintomatología.

Delirium en ancianos hospitalizados: Seguimiento de 18 meses / Delirium in elderly inpatients: An 18 month follow-up

El objetivo de este trabajo fue describir la prevalencia de síndrome confusional agudo en ancianos hospitalizados, la evolución durante la internación y a los 18 meses. Se evaluó en forma prospectiva a pacientes de 70 años de edad o mayores, internados en el Servicio de Clínica Médica de nuestro hospital, entre septiembre de 2005 y mayo de 2006. Se utilizó una versión validada en español del Confussion Assessment Method para diagnosticar delirium. A los 18 meses se evaluó el estado vital, lugar de residencia, actividades de la vida diaria, dependencia de cuidadores y reinternaciones. Se evaluaron 194 pacientes y 74 fueron excluidos. De los 120 casos incluidos, 52 (43.3%) presentaron delirium. La edad media fue de 82.6 años (DS: 7.4) en el grupo de pacientes con síndrome confusional agudo y de 80.4 años (DS: 5.6) en el grupo de pacientes sin delirium. Al comparar estos dos grupos encontramos diferencias significativas en las características, siendo más frecuentes en el grupo con delirium la residencia previa en un centro de tercer nivel (17.3% vs. 1.5%; p < 0.002), la demencia (40.4% vs. 8.8%; p < 0.001), menor puntaje en la evaluación de las actividades de la vida diaria (5 vs. 6; p < 0.001), más días de internación (7 vs. 5; p = 0.04) y mayor mortalidad intrahospitalaria (21.2% vs. 1.5%; p < 0.001). A los 18 meses de seguimiento después del alta, el desarrollo de delirium durante la internación se asoció a mayor grado de dependencia evidenciado por el peor puntaje en las actividades de la vida diaria (mediana 1/6 vs. 5/6) y a menor sobrevida actuarial 35.3% (CI 95%: 24-49%) a los 569 días y 49% (CI 95%: 32.9-65.4%) a los 644 días (p=0.027).

Delirium usually hardens care during hospitalization and increases morbidity during hospital stay and after discharge. The objective of this study was to describe the prevalence of delirium in elderly inpatients in a Buenos Aires hospital, its morbidity and mortality during hospital stay and the next 18 month follow-up. Patients aged 70 or older admitted to internal medicine unit between September 2005 and May 2006 were enrolled. Delirium was assessed with the Spanish version of Confusion Assessment Method. Demographic data, cause of admition and length of stay, destination after discharge and mortality were registered. A new evaluation was made 18 months after discharge. We evaluated 194 patients and 74 were excluded. Of the 120 included, 52 (43.3%) presented delirium. We found significant differences between patients with and without delirium in previous placement in nursing home (17.3% vs. 1.5%; p < 0.002), dementia (40.4% vs. 8.8%; p < 0.001), median activity of daily living (5 vs. 6; p < 0.001), length-of-stay (7 vs. 5; p = 0.04) and mortality rate (21.2% vs. 1.5%; p < 0.001). Evaluation 18 months later showed differences between patients with and without delirium in median of activity of daily living (1/6 vs. 5/6), patients living in nursing homes (27.5% vs. 7.9%), estimated survival 35.3% (CI 95%: 24-49%) at day 569 and 49% (CI 95%: 32.9-65.4%) at day 644. The difference between survival curves was statistically significant (p = 0.027). Delirium increases morbidity and mortality during hospital stay. Elderly with delirium are at risk of worsening disability and of becoming dependent after discharge and it is a risk factor for higher mortality during the following months after discharge.

Topographical disorientation in Alzheimer's disease / Desorientação topográfica na doença de Alzheimer

Topographical disorientation (TD) has not been as extensively studied as other frequent manifestations of Alzheimer's disease (AD). OBJECTIVE: To verify the occurrence of TD and to identify the neuropsychological dysfunctions associated with TD in AD. METHOD: Thirty patients with probable AD, their caregivers and 30 subjects without dementia (controls) were interviewed with a questionnaire and evaluated with tests related to topographical orientation. RESULTS: AD patients, even those with mild dementia, differ from controls in the questionnaire on topographical orientation and in most neuropsychological tests except for tests of spatial working memory, point localization, three dimension and nonsense figure copy. When the performances in the neuropsychological tests of patients with mild or moderate dementia were compared, only landmark recognition and route description were more impaired in moderate dementia. CONCLUSION: TD occurs even in mild dementia of AD, a finding apparently not explained by the impairments of more elementary spatial functions.

Desorientação topográfica (DT) não tem sido tão exaustivamente estudada quanto outros sintomas frequentes da doença de Alzheimer (DA). OBJETIVO: Verificar a ocorrência de DT e identificar as disfunções neuropsicológicas associadas com a DT na DA. Método: Trinta pacientes com DA provável, seus cuidadores e trinta sujeitos sem demência (controles) foram entrevistados com um questionário e testes relacionados à orientação topográfica. RESULTADOS: Pacientes com DA, mesmo aqueles com demência leve, diferiram dos controles no questionário de orientação topográfica e na maioria dos testes neuropsicológicos, exceto nos testes memória operacional espacial, localização de pontos, cópia de figuras sem sentido e de figura em três dimensões. Quando os desempenhos de pacientes com demência leve ou moderada foram comparados, apenas os testes de reconhecimento de marcos e descrição de rotas foram mais comprometidos na demência moderada. CONCLUSÃO: DT ocorre mesmo na demência leve da DA, um achado aparentemente não explicado pelo comprometimento das funções espaciais mais elementares.

Leucoencefalopatía hipóxico-isquémica retardada: caso clínico y revisión de la literatura / Hypoxic-ischemic delayed leukoencephalopathy: case report and review of the literature

Delayed Hypoxic-isquemic Leucoencephalopathy described in 1976 by Ginsberg is a brain white matter demyelinization phenomenon that occurred days or weeks after a hypoxic-isquemic injury followed by a complete recovery of the episode. The pathogenesis process remains unknown. We describe a 48 year old woman with cervico-uterine cancer in palliative treatment with opoids. She enters the emergency room with a respiratory depression, a prolonged hypotension and confusion, that it was recovered. At admission exhibits a recurrent pneumonia. Two weeks later, in conditions of discharge, initiates with agitation in context with rapidly progressive decline cognition, with concordant lesions of Leucoencephalopathy defined in the Magnetic Resonance (MR) study The metabolic profile, the cerebrospinal fluid and the electroencephalogram allowed dismissing other etiologic hypothesis. In front to the suspicious of Ginsberg syndrome, she had normal levels of Arylsulfatase. This acute post-hypoxic demyelinization process has been pathogenic interpreted as an arylsulfatase deficiency. Although numerous cases develop with normal arylsulfatase and the experimental studies of hypoxia, has support the hypothesis of a central hypoxic axonopathy due to failing in axonal transport as the base of the demyelinization phenomenal.

La leucoencefalopatía hipóxico-isquémica retardada (EHIR), descrita por Ginsberg en el año 1976, es un fenómeno desmielinizante de la sustancia blanca cerebral, que se origina días o semanas después de un daño hipóxico-isquémico que había sido seguido de una recuperación completa del episodio. La patogenia del proceso no está completamente establecida. Se presenta una mujer de 48 años portadora de cáncer cérvicouterino, en tratamiento paliativo con opiáceos. Ingresa al Servicio de Urgencia por una depresión respiratoria, hipotensión prolongada y compromiso de conciencia, donde es recuperada. Se hospitaliza por una neumopatía intercúrrente. Dos semanas más tarde, estando en condiciones de alta, se inicia agitación psicomotora en el contexto de un deterioro cognitivo rápidamente progresivo, que el estudio de Resonancia Magnética (RM) definió como lesiones concordantes con una leucoencefalopatía. El perfil metabólico, el líquido cefalorraquídeo y el electroencefalograma, permitieron descartar otras hipótesis etiológicas. Frente a la sospecha de un síndrome de Ginsberg, los niveles de arilsulfatasa fueron normales. Este proceso desmielinizante agudo post-hipóxico, ha sido interpretado patogénicamente como un déficit de arilsulfatasa. Sin embargo, la existencia de numerosos casos que cursan con arilsulfatasa normal y los estudios experimentales de hipoxia, ha avalado la hipótesis de una axonopatía central hipóxica, atribuible a fallas del transporte axoplásmico, como base para el fenómeno desmielinizante.

Neurological complications in Chikungunya fever.

OBJECTIVE: The present prospective study was undertaken to study the clinical manifestations and mainly neurological complication of an acute febrile illness termed chikungunya which has recently attacked india after 43 years. METHOD: This prospective study has been conducted in hospitalised patients admitted in government and private hospitals of Kota city from August 2006 to October 2006. Patients showing neurological involvement with typical clinical picture of chikungunya infection were studied in detail and followed up for improvement and any permanent damage or death. RESULTS: Apart from typical clinical triad of high grade fever, arthralgia and rash of chikungunya infection we have observed a spectrum of neurological abnormalities in terms of altered mental functions, seizures, focal neurological deficit with abnormal CT scan of head and altered CSF biochemistry. Permanent neurological sequelae and even death has occurred. CONCLUSION: Typical clinical history of chikungunya infection, neurological complications with associated CSF abnormalities, supportive laboratory evidences, positive chikungunya IgM card test, exclusion of other causes and known predilection of arboviruses for CNS infection allows us to conclude the diagnosis of study cases as Chikungunya Encephalitis.

Estado confusional agudo como manifestação de status epilepticus não-convulsivo em criança previamente hígida: relato de caso / Acute confusional state in a child with non-convulsive status epilepticus: case report

INTRODUÇÃO: O estado confusional em crianças pode ter várias causas. Entre elas está o estado de mal não-convulsivo. OBJETIVO: Relatamos o caso de um paciente que se apresentou ao serviço de emergência com confusão mental. Após investigação preliminar, o EEG mostrou tratar-se de estado de mal de ausência. RESULTADO: A administração de Diazepam endovenoso seguiu-se normalização do traçado eletroencefalográfico e melhora clínica do paciente. CONCLUSÃO: O EEG é fundamental para se diagnosticar e tratar quadros de estado de mal epiléptico não-convulsivo.

INTRODUCTION: There are many causes to acute confusional state in children. Non-convulsive status epilepticus is one of them. OBJECTIVE: We report the case of a patient who came to our emergency service with confusion. After preliminary investigation the EEG revealed abnormalities consistent with absence status. RESULTS: After treatment with diazepam intravenously occurred normalization of the EEG and, clinically, the patient was better. CONCLUSION: The EEG is crucial in the evaluation of patients with non-convulsive status epilepticus.

Series clínicas de Medicina de Urgencia: intoxicación por monóxido de carbono, el gran simulador / Clinical series of emergency medicine: carbon monoxide poisoning, the great simulator

El monóxido de carbono (CO) es un gas incoloro, inodoro e insípido que se produce durante la combustión incompleta de materiales orgánicos que contienen carbono. La intoxicación por este gas es una causa frecuente de muerte por intoxicación involuntaria durante el período de otoño-invierno. El cuadro clínico suele ser inespecífico, simulando cuadros banales como las virosis hasta episodios graves con compromiso neurológico y alteración de conciencia. El diagnóstico exige al clínico conocer los factores de riesgo epidemiológicos y las distintas variedades de presentación clínica. Se presenta el caso clínico de una paciente que consulta por segunda vez en el Servicio de Urgencia por compromiso de conciencia transitorio, con un estudio inicial negativo. El antecedente anamnéstico de una mascota con síntomas similares permitió buscar probables fuentes de monóxido de carbono en su hogar, confirmándose el diagnóstico por niveles de carboxihemoglobina elevados. El diagnóstico de esta intoxicación suele estar basado en la sospecha clínica considerando los antecedentes epidemiológicos, el cuadro clínico y los factores de riesgo del caso individual.

Posterior leukoencephalopathy in a girl with acute haemorrhagic leukoencephalitis.

In children, posterior leukoencephalopathy is frequently associated with hypertensive encephalopathy, anticancer chemotherapy, treatment with immunosuppressive drugs in patients with organ transplantation, transfusion or human immunodeficiency virus infection. Posterior leukoencephalopathy in these children appears as a complicating illness and resolves once precipitating factor (e.g. cancer chemotherapy) is removed. Here we are reporting a fatal case of acute haemorrhatic leukoencephalitis in a 13 year old girl, imaging abnormalities are also suggestive of posterior leukoencephalopathy. Posterior leukoencephalopathy in our patient possibly, is a part of post-infectious haemorrhagic leukoencephalitis, rather than because of ischaemia or cerebral oedema secondary either to abrupt increase in blood pressure or following administration of immunosuppressive drugs.