Actualización en el tratamiento del síndrome de hipertensión intracraneana / Update on the management of intracranial hypertension syndrome

Elevated intracranial pressure (ICP) is a devastating complication, with great impact on neurological status and high morbidity and mortality. Intracranial hypertension (ICH) has multiple etiologies. The natural history of this condition can lead to brain death. The successful management of patients with elevated ICP (> 20-25 mmHg) requires fast and timely recognition, judicious use of invasive monitoring and therapies aimed to reversing its underlying cause. Therefore, it must be managed as a neurological emergency. The objective of this review is to present in a friendly way the diagnostic approach and the management of ICH, focused on general practitioners.

Psicosis depresiva como forma de presentación de un tumor cerebral / Depressive Psychosis as an Onset Symptom of a Brain Tumor

Introducción: Los tumores cerebrales a veces tienen una presentación clínica compleja y atípica en sus inicios, en dependencia de su localización, lo que puede confundirse con trastornos de la esfera psiquiátrica. Objetivo: Describir un caso clínico interpretado como una psicosis por su sintomatología cuyo diagnóstico resultó un glioblastoma multiforme de alta malignidad. Caso clínico: Mujer de 35 años que comienza desde hace varios meses con trastornos de la conducta, síntomas depresivos, irritabilidad y conflictos en el hogar. Se instala un cuadro de depresión profunda que no mejora con tratamiento farmacológico impuesto por psiquiatría y se decide tratamiento electroconvulsivo, después del cual cae en estado de coma con elementos de hipertensión intracraneal y focalización neurológica. La tomografía de urgencia evidencia un tumor intracraneal, con áreas de necrosis, infiltración y desplazamiento de estructuras adyacentes y signos de hipertensión intracraneal. Al realizar intervención microquirúrgica fallece. La necropsia reveló glioblastoma multiforme de alta malignidad con focos de necrosis y de hemorragias. Conclusiones: La depresión y manifestaciones de trastornos mentales pueden tener causa orgánica, un hecho que debe considerarse en el contexto clínico. La historia clínica bien obtenida, la evaluación psicopatológica y las técnicas de imágenes en la actualidad son herramientas fundamentales en la precisión diagnóstica(AU)

Introduction: Brain tumors sometimes have a complex and atypical clinical presentation at the beginning, depending on their location, which can be mistaken for psychiatric disorders. Objective: To describe a clinical case interpreted as a psychosis for its symptoms and whose diagnosis was a highly malignant glioblastoma multiforme. Clinical case: This is the case of a 35-year-old woman who, several months ago, started to present behavioral disorders, depressive symptoms, irritability, and conflicts at home. Clinical signs of deep depression onset, which does not improve with pharmacological treatment prescribed by the psychiatry specialist; electroconvulsive treatment is decided, after which she falls into a coma with elements of intracranial hypertension and a neurological focus. The emergency tomography shows an intracranial tumor, with necrotic areas, infiltration, and displacement of adjacent structures, as well as signs of intracranial hypertension. When microsurgical intervention is performed, she died. The necropsy revealed highly malignant glioblastoma multiforme with necrosis focuses and hemorrhage. Conclusions: Depression and manifestations of mental disorders may have an organic cause, a fact that must be considered in the clinical setting. A well-obtained clinical history, psychopathological evaluation, and imaging techniques are currently essential tools for an accurate diagnosis(AU)

Descripción clínico-radiológica de un angiosarcoma cardiaco con metástasis cerebral que simula un quiste hidatídico / A radiological and clinical description of metastatic angiosarcoma simulating a hydatid cyst

RESUMEN Los angiosarcomas son sarcomas malignos que se originan en las células endoteliales vasculares. Su diagnóstico diferencial es muy amplio debido a su parecido con otras enfermedades, como las parasitarias, y usualmente es un diagnóstico por exclusión. La neurocisticercosis y la hidatidosis cerebral son parasitosis intestinales que pueden comprometer el sistema nervioso central y tienen mayor incidencia en los países suramericanos. El diagnóstico se establece a partir del perfil epidemiológico, el estudio parasitológico, la apariencia radiológica de las lesiones y el estudio de histopatología del espécimen. Se presenta el caso de una adolescente con factores de riesgo para parasitosis y neuroimágenes sugestivas de hidatidosis cerebral, cuyo diagnóstico definitivo fue angiosarcoma cardiaco metastásico.

ABSTRACT Angiosarcoma is the most malignant sarcoma originating in endothelial vascular cells. It has a wide differential diagnosis due to its similarities with other entities, such as parasitic diseases. More often, angiosarcoma is diagnosed by exclusion. Neurocysticercosis and hydatid disease, or echinococcosis, are parasitic infections that may involve the central nervous system and their incidence is higher in South American countries. Diagnosis is established based on the epidemiological profile, the parasitological examination, the radiological appearance of the lesions, and the histopathology analysis of specimens. We present the case of a female adolescent with parasitosis risk factors whose neuroimages suggested cerebral hydatid cysts and who was finally diagnosed with cardiac metastatic angiosarcoma.

Diagnóstico y tratamiento de la hipertensión intracraneal / Diagnosis and Treatment of Intracranial Hypertension

La presión intracraneal elevada es una complicación devastadora de la lesión neurológica, que puede complicar el trauma, los tumores del sistema nervioso central, la hidrocefalia, la encefalopatía hepática y el flujo venoso del SNC alterado. El adecuado tratamiento consta de un rápido reconocimiento, utilizar material de monitoreo neurológico invasivo y su manejo para reducir la hipertensión intracraneal y sus múltiples causas subyacente. A continuación presentamos una revisión de sus principales características y principios de abordaje diagnóstico-terapéutico

Intracranial pressure is a devastating complication of neurological damage, which can complicate trauma, central nervous system disorders, hydrocephalus, hepatic encephalopathy, and altered CNS venous flow. The appropriate treatment consists of a rapid recognition, the use of an invasive neurological system and its management to reduce intracranial hypertension and its multiple underlying causes. Below we present a review of its main characteristics and principles of diagnostic-therapeutic approach.

Pseudotumor cerebral asociado a hipovitaminosis A, B6 y D: A propósito de dos casos / Pseudotumor cerebri associated with hypovitaminosis A, B6 and D: About two cases

La hipertensión endocraneana idiopàtica se asocia infrecuentemente con la hipovitaminosis A y D. Se presenta el caso de una paciente femenina de 8 años con visión borrosa de 24 horas y papiledema bilateral. Resonancia magnética nuclear normal. Presión de apertura de líquido cefalorraquídeo: 260 mm^O. Presentó déficit de vitamina A y D, e inició un tratamiento sustitutivo. El segundo caso corresponde a un paciente masculino de 12 años con fiebre y odinofagia de 3 días. Con antecedente de glomerulonefritis y sobrepeso. Presentaba edema bipalpebral y papiledema. Tomografia axial computada de la órbita: aumento de líquido en la vaina de ambos nervios ópticos. Resonancia magnética nuclear: aracnoidocele intraselar. Presión de apertura de líquido cefalorraquídeo: 400 mm^O. Presentó déficit de vitamina D y B6, e inició el tratamiento sustitutivo. La elevación de la presión intracraneal desencadena mecanismos de compensación que, al fallar, pueden comprometer la vida o provocar graves discapacidades neurológicas. Reconocer la causa para un enfoque terapéutico preciso es clave para disminuir la morbimortalidad asociada a esta patología.

Idiopathic endocranial hypertension is infrequently associated with hypovitaminosis A and D. The case of an 8-year-old female with 24-hour blurred vision and bilateral papilledema is presented. Nuclear magnetic resonance was normal. Opening pressure of cerebrospinal fluid: 260 mm^O. She presented vitamin A and D deficiency and started replacement therapy. The second case corresponds to a 12-year-old male with fever and odynophagia of 3 days. History of glomerulonephritis and overweight. He had bipalpebral edema and papilledema. Computed tomography scan of the orbit: increase of fluid in the sheath of both optic nerves. Nuclear magnetic resonance: intrasellar arachnoidocele. Opening pressure of cerebrospinal fluid: 400 mmH2O. He presented vitamin D and B6 deficiency and started replacement treatment. The elevation of intracranial pressure triggers compensation mechanisms that, when they fail, can compromise life or cause serious neurological disabilities. Recognizing the cause for an accurate therapeutic approach is key to reduce the morbidity and mortality associated with this pathology.

Noninvasive intracranial pressure monitoring for HIV-associated cryptococcal meningitis

Mortality and adverse neurologic sequelae from HIV-associated cryptococcal meningitis (HIV-CM) remains high due to raised intracranial pressure (ICP) complications. Cerebrospinal fluid (CSF) high opening pressure occurs in more than 50% of HIV-CM patients. Repeated lumbar puncture with CSF drainage and external lumbar drainage might be required in the management of these patients. Usually, there is a high grade of uncertainty and the basis for clinical decisions regarding ICP hypertension tends to be from clinical findings (headache, nausea and vomiting), a low Glasgow coma scale score, and/or fundoscopic papilledema. Significant neurological decline can occur if elevated CSF pressures are inadequately managed. Various treatment strategies to address intracranial hypertension in this setting have been described, including: medical management, serial lumbar punctures, external lumbar and ventricular drain placement, and either ventricular or lumbar shunting. This study aims to evaluate the role of a non-invasive intracranial pressure (ICP-NI) monitoring in a critically ill HIV-CM patient.

Avaliação e reconhecimento da síndrome de hipertensão intracraniana / Evaluation and recognition of intracranial hypertension syndrome

O aumento da pressão intracraniana resulta de etiologias distintas que afetam o encéfalo. O reconhecimento e o manejo precoces dessas complicações impedem o surgimento de lesões neuronais devastadoras, muitas delas irreversíveis. O objetivo desse artigo de revisão é abordar o diagnóstico precoce para se instituir o ideal manejo para a hipertensão intracraniana.

Increased intracranial pressure results from different etiologies that affect the brain. Recognition and early management of these complications prevent the emergence of devastating neuronal damage, many of them irreversible. The aim of this review article is to discuss early diagnosis to establish the optimal management for intracranial hypertension.

Noninvasive evaluation of intracranial hypertension? Is there a gold standard? / Avaliação não invasiva da hipertensão intracraniana? Existe um padrão-ouro?

Computed tomography is essential in head injuried patients for the detection of structural damage to the brain. However, the ability of CT scanning to predict the presence or absence of intracranial hypertension has been debated in the literature. Since the optic nerve is part of the central nervous system and in case of raised pressure in the cerebrospinal fluid its sheath inflates. Based in this hypothesis the authors reviewed the role of the optic nerve sheat diameter in diagnosis intracranial hypertension after traumatic brain injury. This non-invasive method is useful to predict the risk of intracranial hypertension and select patients to ICP monitoring, especially in those with normal CT scans.

A tomografia de crânio é um exame fundamental para avaliação estrutural dos pacientes com traumatismo craniano. Entretanto, a capacidade da tomografia em predizer a ausência ou presença de hipertensão intracraniana é controversa. Como o nervo óptico é parte do sistema nervoso central, em situações de hipertensão intracraniana há aumento da bainha neural. Com base nessas hipóteses, os autores revisaram o papel da análise do diâmetro da bainha do nervo óptico no diagnóstico de hipertensão intracraniana pós-traumatismo. Esse método não invasivo é útil para predizer os riscos de hipertensão intracraniana e selecionar os pacientes para monitorização da pressão intracraniana, principalmente naqueles com tomografia de crânio normal.

Management of intracranial hypertension.

Raised intracranial pressure (ICP) is a life threatening condition that is common to many neurological and non-neurological illnesses. Unless recognized and treated early it may cause secondary brain injury due to reduced cerebral perfusion pressure (CPP), and progress to brain herniation and death. Management of raised ICP includes care of airway, ventilation and oxygenation, adequate sedation and analgesia, neutral neck position, head end elevation by 200 -300, and short-term hyperventilation (to achieve PCO2 32- 35 mm Hg) and hyperosmolar therapy (mannitol or hypertonic saline) in critically raised ICP. Barbiturate coma, moderate hypothermia and surgical decompression may be helpful in refractory cases. Therapies aimed directly at keeping ICP <20 mmHg have resulted in improved survival and neurological outcome. Emerging evidence suggests that cerebral perfusion pressure targeted therapy may offer better outcome than ICP targeted therapies.

Intracranial hypertension syndrome in systemic lupus erythematosus: clinical analysis and review of the literature / 华中科技大学学报（医学）（英德文版）

In order to better understand the clinical manifestation of systemic lupus erythematosus (SLE) with intracranial hypertension syndrome (IHS), we analyzed the clinical features and treatment of a typical SLE patient with IHS. SLE is one of the most unpredictable autoimmune diseases involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. IHS is an uncommon manifestation of neuropsychiatric SLE (NPSLE) and is characterized by an elevated intracranial pressure, papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. IHS has been reported in a few sporadic cases in patients with SLE worldwide, but rarely has been reported in China. In this study, a 34-year-old female SLE patient with IHS was reported and pertinent literature reviewed. The clinical presentation, image logical features, and investigatory findings were discussed.

Spontaneous acute subdural hematoma contralateral to an arachnoid cyst

Arachnoid cysts (AC) are extra-cerebral cerebrospinal fluid collections of unknown origin. They correspond to 1 percent of all intracranial nontraumatic space-occupying lesions and appear more frequently in the middle fossa (50 percent). More than 25 percent of these cysts are incidental findings and the majority of patients are asymptomatic. Seizures, intracranial hypertension signs, neurological deficits, macrocrania, developmental delay and bulging of the skull are the main signs and symptoms of the lesion. AC rupture and bleeding are rare, usually occurring in young adults and associated with trauma. The risk of hemorrhage does not exceed 0.04 percent / year. We describe the case of a ten-year-old boy who presented with acute signs of intracranial hypertension secondary to a spontaneous acute subdural hematoma, contralateral to an AC of the middle fossa. Three factors were significant in this case: signs and symptoms occurred spontaneously; the presence of an acute subdural hematoma exclusively contralateral to the AC; successful outcome of the conservative treatment.

Os cistos aracnóideos (CA) são coleções liquóricas extra-cerebrais e intra-aracnóideas de origem desconhecida. Correspondem a 1 por cento de todas as lesões expansivas intracranianas não traumáticas e têm nítido predomínio na fossa média (50 por cento). Até 25 por cento destes cistos são achados incidentais sendo que a maioria dos pacientes é assintomática. Crises epilépticas, sinais de hipertensão intracraniana, déficits neurológicos focais, macrocrania, atraso no desenvolvimento e abaulamento da calota craniana são os principais sinais e sintomas da lesão. A ruptura dos CA, assim como seu sangramento, são situações raras, geralmente associadas a traumas e acometem adultos jovens. O risco de hemorragia em pacientes com CA não excede 0,04 por cento ao ano. É descrito caso de paciente de dez anos de idade que subitamente apresentou sinais de hipertensão intracraniana secundários a hematoma subdural agudo espontâneo, contralateral a volumoso CA de fossa média. Três fatores foram de relevância neste paciente: a ocorrência de sinais e sintomas espontaneamente, especialmente em um menino; presença de hematoma subdural agudo exclusivamente contralateral ao CA; e a boa evolução com o tratamento conservador.

Aspectos clínicos e evolutivos da hidrocefalia na neurocisticercose / Clinical and evolutive aspects of hydrocephalus in neurocysticercosis

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 por cento eram homens, entre 21 e 50 anos. A hipertensão intracraniana (HIC) ocorreu em todos os pacientes, cefaléia (CEF) em 89,4 por cento, meningoencefalite (ME) em 80,8 por cento e distúrbios psíquicos (PSI) em 74,5 por cento. A síndrome liquórica da NCC foi detectada em 65,9 por cento pacientes. Além da HDC, as tomografias computadorizadas de crânio (TC) mostraram lesões císticas e edema cerebral difuso em 59,6 por cento cada, calcificações em 55,3 por cento. Dos 41 pacientes (87,2 por cento) com derivação ventriculoperitoneal (DVP), em 22 (53,7 por cento) deles foram necessárias uma a sete revisões/paciente (média=3). A evolução foi satisfatória em 51,1 por cento e fatal em 31,9 por cento. 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.

With the purpose to verify clinical aspects of hydrocephalus (HC) in patients with neurocysticercosis (NCC), a retrospective study of 47 patients was performed. The majority of patients (70.2 percent) were men aging 21-50 years. Intracranial hypertension (ICH) occurred in all patients, headache (HA) in 42 (89.4 percent), meningoencephalitis (ME) in 38 (80.8 percent) and psychiatric disorders (PD) in 34 (72.3 percent). The cerebrospinal fluid syndrome of NCC was detected in 31 patients (65.9 percent). In addition to HC, computed tomography (CT) scans showed cystic lesions in 28 (59.6 percent) patients, diffuse brain edema also in 28 (59.6 percent), and calcifications in 26 (55.3 percent). Shunts were inserted in 41 (87.2 percent) patients and 22 (53.7 percent) of them were submitted to 1-7 surgical revision/patient (mean=3) that were higher (mean=4) in those who died than in survivors (mean=2). Evolution was satisfactory in 24 (51.1 percent) patients and fatal in 15 (31.9 percent). It is possible to conclude that, in patients with NCC, HC occurs predominantly in men in productive life with ICH, HA, ME and PD as common manifestations, and the need for shunt revision makes patient's prognosis worse.

Idiopathic intracranial hypertension. Atypical presentation

To describe the clinical features of 5 patients with rare atypical presentation of idiopathic intracranial hypertension [IIH], and propose the possible mechanism of this atypical presentation. We carried out a retrospective study of 5 patients admitted at King Khalid National Guard Hospital, Jeddah, Kingdom of Saudi Arabia with IIH during the period from January 2001 to December 2005. All were females with their age ranges from 24 to 40 years. The clinical presentations, and the laboratory and imaging studies were analyzed. The opening pressures of the lumbar puncture tests were documented. All patients were presented with headache. One had typical pain of trigeminal neuralgia, and one with neck pain and radiculopathy. Facial diplegia was present in one patient, and 2 patients had bilateral 6th cranial neuropathy. Papilledema was present in all patients except in one patient. Imaging study was normal in all patients, and they had a very high opening pressure during lumbar puncture, except in one patient. All patients achieved full recovery with medical therapy in 6 to 12 weeks with no relapse during the mean follow up of 2 years. Atypical findings in IIH are rare and require a high index of suspicion for early diagnosis

Hipertensão intracraniana / Intracanial hypertension

A hipertensão intracraniana (HIC) é um problema em pelo menos metade das lesões parenquimatosas e em um terço dos pacientes com lesão difusa. Metade dos pacientes que desenvolvem HIC morrem, a despeito da descompressão cirúrgica ou outras terapias. A clínica clássica de aumento da pressão intracraniana em adultos e em crianças consiste em cefaléia, náuseas e vômitos, além de vertigem, paralisias oculares, papiledema, turvação visual e amaurose fugaz. A mensuração da pressão intracraniana e o seu manejo adequado podem auxiliar o intensivista na otimização ds terapêuticas disponíveis atualmente para melhorar o prognóstico e a qualidade de vida dos pacientes severamente acometidos por essa condição.

Pulsatile tinnitus: Audiologic and MR imaging diagnostic lssues

Pulsatile tinnitus often presents a diagnostic and management dilemma to the neurologist. Correct diagnosis is imperative because in the majority of cases, there is a treatable underlying etiology. In addition, failure to make proper diagnosis may be disastrous because in some patients, a life-threatening intracranial disease may be present. This study was carried out aiming to investigating the feasibility of differentiating the possible causes of pulsatile tinnitus, particularly in patients with normal otoscopy, using clinical, audiological and radiological, tools. 29 patients complaining of pulsatile tinnitus were evaluated by history taking, clinical examination, laboratory investigations, otoscopy, basic audiological evaluation, MRI for petrous bone and brain and MRA. Glomus jugulare tumor was diagnosed in 3 patients. Benign intracranial hypertension [BIH] was diagnosed in 4 patients. Intraventricular neoplasm in 2 patients. Internal carotid artery stenosis in one patient. Post-traumatic pseudoaneurysm in one patient. Scalp AVM in one patient. Temporal bone metastasis in one patient. Severe anemia in one patient and no identifiable cause [idiopathic] in 15 patients. A unilateral mild low frequency pseudosensorineural hearing loss was identified in 10 patients with normal otoscopy. Hearing loss was normalized after elimination of tinnitus by applying a light digital pressure over the ipsilateral internal jugular vein