Trastornos prolongados de conciencia postinjuria cerebral aguda / Prolonged disorders of consciousness after an acute brain injury

Only a fraction of patients in coma secondary to a primary acute brain injury develop a vegetative state (VS). At least 20% of patients show late transitions to a minimally conscious states (MCS). They are particularly common in young adults with traumatic brain injury. The main problems faced by clinicians are the diagnostic accuracy of VS and MCS as well as the usefulness of sophisticated paraclinical investigations. Specific therapies are of limited effectiveness. This population is vulnerable to misdiagnosis and limited access to medical care and rehabilitation, thus generating ethical problems.

Disorders of Consciousness: Practical Management in an Emergency Room

Lowering of the level of consciousness is a very common presentation at the emergency room, often without any history that helps finding an etiology. This emergency requires quick empirical measures to reduce neuronal mortality, with additional protection against sequelae. According to the Advanced Cardiac Life Support (ACLS) guidelines, there are current emergency neurological support protocols, such as the Emergency Neurological Life Support (ENLS) created by the Neurocritical Care Society. The present paper shows how to approach unconscious patients, highlighting possible etiologies and proposed treatments.

Coma mixedematoso e trombo de veia cava superior. Relato de caso e revisão da literatura / Myxedema coma and superior vena cava thrombus: case report and literature review

O coma mixedematoso é uma emergência endocrinológica rara e consiste na máxima expressão do hipotireoidismo, com alta mortalidade por suas complicações hemodinâmicas e ventilatórias, podendo ser agravadas por distúrbios da coagulação. Relatamos o caso de uma paciente diagnosticada com coma mixedematoso e trombo de veia cava superior. Buscamos salientar os distúrbios de coagulação frequentes no hipotireoidismo grave, que contribuem para o aumento da mortalidade deste grupo de pacientes. O diagnóstico e o tratamento precoce do coma mixedematoso, aliados à instituição imediata da terapia para o fenômeno trombótico encontrado, permitiram a evolução favorável do quadro. O relato, juntamente da bibliografia pesquisada, orientou o raciocínio sobre a relação dos distúrbios de coagulação, que ocorrem no hipotireoidismo descompensado. Apesar de poucos relatos, estes distúrbios podem ser frequentes e devem ser pesquisados, pois contribuem com o aumento da mortalidade.(AU)

Myxedema coma is a rare endocrinological emergency, consisting of the highest expression of hypothyroidism with high mortality due to hemodynamic and ventilatory complications, which may be aggravated by coagulation disorders. We report the case of a patient diagnosed with myxedema coma and superior vena cava thrombus. We sought to emphasize the frequent coagulation disorders in severe hypothyroidism, which contribute to increased mortality in this group of patients. The diagnosis and early treatment of myxedema coma, together with the immediate institution of therapy for the thrombotic phenomenon found, allowed the favorable evolution of the condition. The report, together with the literature, has guided the rationale for the influence of coagulation disorders that occur in decompensated hypothyroidism. Despite the few number of reports, these disorders can be frequent and should be investigated because they contribute to the increase in mortality.(AU)

Coma mixedematoso / Myxedema coma

El hipotiroidismo es una enfermedad frecuente, de diagnóstico y tratamiento simples. Si no es detectada a tiempo puede progresar a la forma más grave conocida como coma mixedematoso. El término "coma mixedematoso" es considerado generalmente engañoso, ya que la mayoría de los pacientes no se presenta inicialmente en estado de coma. La progresión típica es la letargia, evolucionando al estupor y eventualmente al coma, con insuficiencia respiratoria e hipotermia. Es relativamente infrecuente, afecta fundamentalmente a mujeres ancianas, y a menudo ocurre en invierno. Esta entidad debe ser considerada una forma de hipotiroidismo descompensado, desencadenada a partir de una variedad de enfermedades o condiciones no tiroideas que provocan un compromiso sistémico generalizado de extrema gravedad, con desenlace fatal de no mediar un diagnóstico precoz y un tratamiento intensivo.

Hypothyroidism is a frequently diagnosed and simply treated disease. If not recognised, however, in time it may develop into the most severe manifestation of hypothyroidism known as myxedema coma. The term "myxedema coma" is generally seen as misleading since most patients do not initially present in a coma. The typical progression is lethargy evolving into stupor and, eventually, into coma with respiratory failure and hypothermia. It mainly affects elderly women, often occurring in winter and is relatively rare. It can be considered a form of decompensated hypothyroidism often triggered by a variety of non-thyroid conditions or diseases provoking an extremely severe condition of multiple system failure with lethal consequences unless an early diagnosis is made and an aggressive treatment is administered.

Encefalopatía de Wernicke y polineuropatía asociada a déficit de complejo B después de una cirugía bariátrica / Wernicke´s encephalopathy and polyneuropathy associated with vitamin B complex deficiency after a bariatric surgery

La cirugía bariátrica es un tratamiento que garantiza una pérdida de peso sustancial y duradera, y beneficios tangibles respecto a condiciones médicas asociadas a la obesidad. El aumento del número de cirugías bariátricas ha llevado también a un aumento de las complicaciones relacionadas con ella, incluyendo la encefalopatía de Wernicke y la polineuropatía por deficiencia de vitaminas del complejo B. En este artículo se reporta un caso de encefalopatía de Wernicke siete semanas después de la cirugía, enfatizando en la importancia de reconocer el espectro de la sintomatología para hacer un diagnóstico temprano, que permita intervenir en la fase reversible de esta enfermedad potencialmente letal.

Bariatric surgery is a treatment that guarantees a substantial and lasting weight loss in addition to the tangible benefits relating to obesity-associated medical conditions. The increasing number of bariatric surgeries has revealed an increasing number of complications related to this procedure, including Wernicke´s encephalopathy and vitamin B deficiency polyneuropathies. Herein, a 7-week post-surgery case of Wernicke´s encephalopathy is presented that emphasizes the importance of an early recognition of these symptoms so as to initiate intervention during the reversible phase of these potentially lethal pathologies.

Familial Hemiplegic Migraine with Prolonged Coma and Cerebellar Atrophy: CACNA1A T666M Mutation in a Korean Family

We report the first Korean patient with familial hemiplegic migraine type 1, with clinical and multimodal imaging findings. A 43-yr-old man was admitted for right hemianopia and aphasia, followed by coma. MRI showed only cerebellar atrophy. CT angiography showed mild vasodilation of intracranial blood vessels and increased vascularity in the left hemisphere and perfusion-weighted imaging showed elevated cerebral blood flow. Gene analysis of the patient and his mother led to the identification of a heterozygous point mutation (1997C-->T, T666M) in exon 16 of the CACNA1A gene. Familial hemiplegic migraine should be considered in patients with episodic neurological dysfunction with cerebellar atrophy.

Coma scales: a historical review / Escalas de coma: uma revisão histórica

OBJECTIVE: To describe the most important coma scales developed in the last fifty years. METHOD: A review of the literature between 1969 and 2009 in the Medline and Scielo databases was carried out using the following keywords: coma scales, coma, disorders of consciousness, coma score and levels of coma. RESULTS: Five main scales were found in chronological order: the Jouvet coma scale, the Moscow coma scale, the Glasgow coma scale (GCS), the Bozza-Marrubini scale and the FOUR score (Full Outline of UnResponsiveness), as well as other scales that have had less impact and are rarely used outside their country of origin. DISCUSSION: Of the five main scales, the GCS is by far the most widely used. It is easy to apply and very suitable for cases of traumatic brain injury (TBI). However, it has shortcomings, such as the fact that the speech component in intubated patients cannot be tested. While the Jouvet scale is quite sensitive, particularly for levels of consciousness closer to normal levels, it is difficult to use. The Moscow scale has good predictive value but is little used by the medical community. The FOUR score is easy to apply and provides more neurological details than the Glasgow scale.

OBJETIVO: Apresentar as escalas de coma de maior relevância desenvolvidas nos últimos cinqüenta anos. MÉTODO: Foi realizado levantamento bibliográfico nos bancos de dados Medline e Scielo compreendendo o período de 1969 a 2009 de acordo com as palavras-chave: coma scales, coma, disorders of consciousness, coma score, levels of coma. RESULTADOS: Foram encontradas cinco escalas principais, em ordem cronológica: Escala de coma de Jouvet, Escala de coma de Moscou, Escala de coma de Glasgow (GCS), Escala de Bozza-Marrubini e Escala FOUR (Full Outline UnResponsiveness), além de outras com menor repercussão e raramente usadas fora do seu país de origem. DISCUSSÃO: Das cinco escalas principais, a GCS é, de longe, a mais usada. É de fácil aplicabilidade e bastante adequada para situações de trauma crânio encefálico (TCE), porém, apresenta falhas, como a impossibilidade de se testar o componente verbal em pacientes intubados, entre outras. A escala de Jouvet é bastante sensível, especialmente para níveis de consciência mais próximos do normal, no entanto, é de difícil execução. A escala de Moscou apresenta um bom valor preditivo, porém, é pouco usada pela comunidade médica. A escala FOUR é de fácil aplicação e fornece mais detalhes neurológicos se comparada à GCS.

Principal causes of coma in medical units of a tertiary care hospitals of Peshawar

To determine the principal causes of coma in patients admitted to the medical units of a tertiary care hospital of Peshawar. This cross sectional study was conducted at department of medicine, Khyber teaching hospital Peshawar, from July 2006 to August 2007. All patients were randomly selected. Relevant information's were recorded on a questionnaire prepared in accordance with the objectives of the study. A total of 124 patients with coma, 96[77.41%] males and 28[22.58%] females were included in the study. The age range of patients was from 12 years to 72 year with mean age of 50.5 years. The principal causes of coma were: cerebrovascular accident [CVA] 73.38% [n=91], epilepsy in 10.48% [n=13], injury head 5.64% [n=11], hepatic coma 2.41% [n=3], central nervous system [CNS] infections 2.41% [n=3], metabolic acidosis, drug abuse [1.61%] [n=2] each and cerebral malaria, hypoglycemia and uremia 0.8% [n=1] each. Risk factors for stroke recorded were hypertension in 46.2% [n=42/91], diabetes in 15.4% [n=14/91], ischemic heart disease 12.1% [n=11/91], smoking 5.5% [n=5/91], hyperlipedemia 3.3% [n=3/91] and atrial fibrillation 1.1% [n=1/91]. Scoring on Glasgow coma scale showed that 74.2% [n=92] patients scored 3-8, 25.8% [n=32] scored 9-12 and none of the patients scored 13-15. In our setup CVA is the most common cause of coma followed by epilepsy, and trauma head. Other minor causes recorded were metabolic acidosis, drug abuse, central nervous system infections, cerebral malaria, hypoglycemia and uremia

Facteurs de mauvais pronostic des comas non traumatiques (A propos de 449 cas observes dans le service de Reanimation Medicale du CHU.A/JRB

Justification: Le coma non traumatique traduit une alteration de la conscience survenant d'une facon spontanee; d'origine multifactorielle. C'est une urgence diagnostique et therapeutique. Le pronostic du patient comateux depend surtout de la precocite et de la qualite de la prise en charge initiale. Objectif : Cette etude essaie de determiner les facteurs de mauvais pronostic des comas non traumatiques chez 449 patients comateux; hospitalises dans le service de reanimation medicale du CHU Antananarivo/Hopital Joseph Raseta Befelatanana. Resultats : Les facteurs incrimines sont le sexe masculin; l'age avance; l'intersaison; les antecedents (alcoolisme; hypertension arterielle; accident vasculaire cerebral; broncho-pneumopathies chronique); la brutalite de l'installation du coma. D'autres parametres sont responsables des comas et aggravent le pronostic des patients : la deshydratation; l'hyperthermie superieure a 40oC; la pression arterielle tres elevee ou imprenable; la polypnee au-dela de 45cycles/min; la SpO2 basse; la tachycardie superieure a 140/min ou la bradycardie inferieure a 40/min. La decouverte de causes evidentes (hypoglycemie; pathologies vasculaires; coma ethylique complique; coma hepatique et infectieux) permet de cerner le diagnostic etiologique. Conclusion : La presence de ces facteurs de mauvais pronostic impose une prise en charge rapide et efficace afin de reduire le taux de morbi-mortalite

Potencial de disparidad: [revisión] / Mismatch negativity: [revision]

El potencial de disparidad o mismatch negativity (MMN) corresponde a la respuesta eléctrica extraída del electroencefalograma que se produce frente a diversos cambios de las características del estímulo acústico. Se obtiene presentando al sujeto una secuencia de estímulos repetitivos de características acústicas similares (estímulo estándar) alternado en forma aleatoria con estímulos acústicos discrepantes que difieren del primero en alguno de sus atributos (estímulo discrepante). El MMN se originaría en la corteza auditiva primaria y se ha logrado registrar desde el nacimiento. No requiere la atención del sujeto, permitiendo evaluar en forma objetiva la discriminación de tonos y fonemas. En clínica se ha utilizado en evaluación del procesamiento auditivo central, pacientes con dislexia o trastorno específico del lenguaje, autismo, individuos con implante coclear e incluso en pacientes en coma. En el presente artículo se revisan las principales características, origen anatómico y utilidad clínica del MMN.

The mismatch negativity (MMN) is a specific component of the auditory event-related brain potentials. It is elicited by an infrequent, physically deviant sound (deviant-stimulus) occurring in a sequence of homogeneous repetitive sounds (standard-stimulus). MMN is probably generated in the primary auditory cortex and it has been successfully recorded in newborns. The MMN can be elicited even in the absence of attention and it can be used as an objective method to assess tone and phoneme discrimination. Some clinical applications of MMN are: evaluation of central auditory processing, patients with dyslexia or language specific disorders, autism, cochlear implant - users and even in prognosis of coma. In this article the main characteristics, origin and clinical applications of the MMN are reviewed.

Potencial evocado auditivo de tronco encefálico como auxílio diagnóstico de morte encefálica / Brainstem auditory evoked potentials as a method to assist the diagnosis of brain death

TEMA: Potencial Evocado Auditivo de Tronco Encefálico aplicado em morte encefálica. OBJETIVO: verificar a concordância entre o resultado do Potencial Evocado Auditivo de Tronco Encefálico e o desfecho do quadro clínico, em pacientes em coma Glasgow 3, por meio da análise do padrão de resposta elétrica do teste. MÉTODO: estudo tipo transversal realizado em 30 pacientes em coma Glasgow 3 submetidos ao Potencial Evocado Auditivo de Tronco Encefálico e acompanhados quanto ao desfecho clínico após o teste: recuperação ou óbito. O teste seria considerado positivo para morte encefálica ao registrar com reprodutibilidade ausência de todas as ondas ou presença de apenas a onda I, e considerado negativo na presença de duas ou mais ondas independentes de suas latências. RESULTADOS: entre os pacientes que apresentaram testes positivos para morte encefálica (86,66 por cento), todos foram a óbito e o único paciente que se recuperou apresentou teste negativo indicando especificidade de 100 por cento. Observou-se consistência interna dos dados, com coeficiente de correlação intraclasse de 0,562 por meio do Teste Alfa de Cronbach e concordância significativa entre o teste e o desfecho clínico por meio do Teste de Concordância de Kappa (K = 0,545; p = 0,015), com intervalo de confiança de 95 por cento. CONCLUSÃO: para o presente estudo o Potencial Evocado Auditivo de Tronco encefálico demonstrou ser um teste altamente específico na predição de óbito em pacientes em coma Glasgow 3, e possibilitou auxiliar o diagnóstico de morte encefálica.

BACKGROUND: brainstem auditory evoked potentials in brain death. AIM: to verify the agreement between the response in the auditory brainstem audiometry and the clinical outcome, analyzing the pattern of responses to electric stimulation. METHOD: a cross-sectional study performed in 30 patients with Glasgow coma score of 3, submitted to the auditory brainstem audiometry and followed up until their clinical outcome: recovery or death. The test was considered positive to brain death when there was no registry of waves or when there was only the registry of wave I; and negative when there were two or more waves, independently of their latencies. RESULTS: Among the patients who presented positive results for brain death (86.66 percent), all died; the only patient who recovered presented a negative result, indicating a specificity of 100 percent. Internal consistency of data was also observed, with an intraclass correlation coefficient of 0.562, obtained using the Cronbach´s test; and a significant agreement between the test and the clinical outcome using the Kappa´s test, with a confidence interval of 95 percent (K = 0.545; p = 0.015). CONCLUSION: in the present study, the brainstem auditory evoked potential demonstrated to be highly specific in death prediction of patients in Glasgow coma score of 3, and was useful in assisting the diagnosis of brain death.

Coma e outros estados de consciência / Coma and other consciousness disorders

As alterações do nível de consciência podem variar entre dois extremos, desde uma desorientação têmporo-espacial até um estado de coma profundo. Na prática clínica é extremamente comum o médico se deparar frente a um paciente...

The alterations of the level of conscience can vary among two ends, from a mental confusion to a state of deep coma. In clinical practice it is extremely common a patient with alteration of the level of conscience without diagnosis. In general hospitals, studies indicate that the alcoholism, the brain trauma and the cardiovascular diseases are the main causes of coma (82%). Its necessary know how to recognize the main causes consciousness of and their differential diagnosis, aimed to identify the severe ill patient and to treat the possible emergency states that take to a secondary neurological damage. In this revision, we emphasized the key points in the neurological exam to determine the different alterations of the level of consciousness...

Predictive value of electroencephalography and computed tomography in childhood non-traumatic coma.

OBJECTIVES: To study value of electroencephalogram (EEG) and computed tomography (CT Scan) in predicting outcome of non-traumatic coma in children. METHODS: 100 consecutive children, between 2 months to 12 years, with non-traumatic coma, (Glasgow Coma Scale score < 8). Demographic and clinical data was recorded at admission. EEG and CT scan were done within 24 hours of admission. Etiologic diagnosis was assigned on basis of clinical data and relevant laboratory investigations. The outcome was recorded as survived and died. Among survivors it was graded as no disability, or mild, moderate, or severe disability. Odds ratio and/or relative risk (RR) with 95% confidence interval (C I) were calculated. RESULTS: EEG could be done in 60 patients (43 survived; 7 were normal, 8 had mild, 17 moderate and 11 severe disability) CT scan in 93 patients (60 survived; 11 were normal, 14 had mild, 21 moderate and 14 severe disability). A normal/borderline EEG was associated with good outcome (P = 0.001); 11 of 12 survived and of survivors 55% had no or mild disability. Electrocerebral silence on EEG was a predictor of death (OR = 44 -sub .95% Cl - 1.5-7372; P = 0.01). An abnormal EEG was associated with significant increase in risk of disability among survivors (RR = 2.6, 95% Cl = 1.2-5.4, P = 0.03). Among CT abnormalities intracranial bleed suggested increased risk of death (RR = 2.1; 95% Cl - 0.8-5.3; P = 0.058), while, hydrocephalus was associated with better survival (RR = 0.7; 95% CI - 0.5 to 0.96; P = 0.029). However, hydrocephalus when compared with other abnormal CT scan findings, was associated with higher risk of moderate and severe disability among survivors (P = 0.046) CONCLUSION: A normal CT scan and EEG, and some of the specific findings could be helpful in predicting outcome in children with non-traumatic coma. EEG and CT scan should be done at admission in all patients with non-traumatic coma if feasible.

Non traumatic coma.

OBJECTIVE: To study the etiology and clinical profile of non-traumatic coma in children and to determine the clinical signs predictive of outcome. METHODS: 100 consecutive cases of non-traumatic coma between 2 months to 12 years. Clinical signs studied were temperature, pulse, heart rate, blood pressure, coma severity by Glasgow coma scale (GCS), respiratory pattern, pupillary and corneal reflex, extra ocular movements, motor patterns, seizure types and fundus picture. These were recoded at admission and after 48 hours of hospital stay. Etiology of coma was determined on basis of clinical history, examination and relevant laboratory investigations by the treating physician. The outcome was recorded as survived or died, and among those who survived as normal, mild, moderate, or severe disability. Chi-square test and logistic regression analysis were done to determine predictors of outcome. RESULTS: Etiology of coma in 60% cases was CNS infection (tubercular meningitis-19, encephalitis-18, bacterial meningitis-16, others-7); other causes were toxic-metabolic conditions (19%), status epilepticus (10%), intracranial bleed (7%), and miscellaneous (4%). 65 children survived, 11 were normal, 14 had mild disability, 21 had moderate disability and 14 were severely disabled and dependent. Survival was significantly better in patients with CNS infection (63%) as compared to those with toxic-metabolic causes (27%) and intracranial bleed (43%, P < 0.05). On bivariate analysis age < or = 3 years, poor pulse volume, abnormal respiratory pattern and apnoea, abnormal pupillary size and reaction, abnormal extra ocular movements, absent corneal reflex, abnormal motor muscle tone at admission or 48 hours correlated significantly with mortality. Survival was better with increasing GCS (Spearman rho = .32, P < 0.001). On logistic regression age < 3 years, poor pulse volume, absent extraocular movements and papilloedema at admission and 48 hours after admission were independent significant predictors of death. CONCLUSION: CNS infections were the most common cause of non-traumatic coma in childhood. Simple clinical signs were good predictors of outcome.

Long-term outcome in coma.

OBJECTIVE: To assess the relationship between Modified Glasgow Coma Scale, its components, brain stem reflexes and long term functional outcome in children with acute non-traumatic coma. METHOD: MGCS and brainstem reflexes were assessed at 6 hourly intervals for 72 hours from the time of admission. The children were followed up regularly and functional outcome was assessed at the end of 9 months. The lowest score of the MGCS and worst brain stem reflexes were used for the analysis. RESULTS: Higher total MGCS score and verbal response score had a significant positive correlation with better functional outcome as measured by GOS and intelligence quotient. There was no association between the language function and the initial MGCS, its components and brain stem reflexes. Lower verbal response (P = 0.005) was the only factor that was found to be individually associated with poorer long term GOS score and intelligence quotient by multivariate linear regression analysis. CONCLUSION: In the long term prediction of outcome in acute non-traumatic coma, MGCS is not useful. However, verbal response, a component of MGCS, correlates well with long term functional outcome and intelligence quotient.

Estado de coma en niños / Coma in children

El estado de coma (EC) es una urgencia médica frecuente que puede conducir a secuelas permanentes o a la muerte. El presente trabajo hace una revisión del tema, pretende facilitar el abordaje y la mejor comprensión del examen neurológico del niño en EC. El examen neurológico inicial puede realizarse con la escala de Glasgow o alguna otra similar, pero ésta tiene sus limitaciones, por lo que es conveniente desarrollar una valoración más completa. El EC puede originarse en trauma asociado a la cabeza y otros órganos o sistemas, en lesión traumática restringida a la cabeza, o no estar asociado a trauma. Así mismo debe establecerse si se trata de EC sin disfunción focal de tallo ni hemisférica; con disfunción focal hemisférica; con disfunción focal de tallo; o con disgunción focal tanto de tallo como hemisférica. Los estudios diagnósticos y medidas terapeuticas se realizarán de acuerdo a la patología probable y el criterio médico