Jahi McMath, a New Disorder of Consciousness / Jahi McMath, un nuevo trastorno de la conciencia / Jahi McMath, um novo transtorno da consciencia

Abstract: In this paper, I review the case of Jahi McMath, who was diagnosed with brain death (BD). Nonetheless, ancillary tests performed nine months after the initial brain insult showed conservation of intracranial structures, EEG activity, and autonomic reactivity to the "Mother Talks" stimulus. She was clinically in an unarousable and unresponsive state, without evidence of self-awareness or awareness of the environment. However, the total absence of brainstem reflexes and partial responsiveness rejected the possibility of a coma. Jahi did not have UWS because she was not in a wakefulness state and showed partial responsiveness. She could not be classified as a LIS patient either because LIS patients are wakeful and aware, and although quadriplegic, they fully or partially preserve brainstem reflexes, vertical eye movements or blinking, and respire on their own. She was not in an MCS because she did not preserve arousal and preserved awareness only partially. The CRS-R resulted in a very low score, incompatible with MCS patients. MCS patients fully or partially preserve brainstem reflexes and usually breathe on their own. MCS has always been described as a transitional state between a coma and UWS but never reported in a patient with all clinical BD findings. This case does not contradict the concept of BD but brings again the need to use ancillary tests in BD up for discussion. I concluded that Jahi represented a new disorder of consciousness, non-previously described, which I have termed "reponsive unawakefulness syndrome" (RUS).

Resumen: En este artículo, revisó el caso de Jahi McMath, quién fue diagnosticada con muerte encefálica (ME). No obstante, exámenes complementarios realizados nueve meses después de la lesión cerebral inicial mostraron conservación de las estructuras intracraneales, actividad en electroencefalografía EEG, y reactividad autonómica a estímulos llamados "Conversación de Madre". Ella estaba clínicamente en un estado sin respuesta a los estímulos, sin evidencia de autoconciencia o conciencia del ambiente. Sin embargo, la ausencia total de reflejos del tronco encefálico y la capacidad de respuesta parcial rechazaron la posibilidad de un coma. Jahi no tenía síndrome de vigilia sin respuesta SVSR porque no estaba en un estado de vigilia y mostró una capacidad de respuesta parcial. Tampoco pudo ser clasificada como paciente LIS porque los pacientes LIS están despiertos y conscientes, y aunque tetrapléjicos, conservan total o parcialmente los reflejos del tronco encefálico, los movimientos oculares verticales u el parpadeo, y respiran por sí mismos. Ella no estaba en un EMC porque no preservaba la excitación y preservaba la conciencia solo parcialmente. La CRS-R dio una puntuación muy baja, incompatible con pacientes de EMC. Los pacientes de EMC preservan total o parcialmente los reflejos del tronco encefálico y, por lo general, respirar por sí solos. El EMC siempre se ha descrito como un estado de transición entre un coma y SVSR pero nunca se ha reportado en paciente con todos los hallazgos clínicos de ME. Este caso no contradice el concepto de ME pero vuelve a plantear la discusión acerca de la necesidad de utilizar exámenes complementarios en ME. Llegué a la conclusión de que Jahi representaba un nuevo trastorno de la conciencia, no descrito anteriormente, que he denominado "síndrome de no despertar con respuesta" (SNDR).

Resumo: Neste artigo, foi revisado o caso Jahi McMath, que foi diagnosticada com morte encefálica (ME). Contudo, exames complementares realizados nove meses depois da lesão cerebral inicial mostraram conservação das estruturas intracranianas, atividade em eletroencefalografia (EEG) e reatividade autonômica a estímulos chamados "Conversación de Madre". Ela estava clinicamente em um estado sem resposta aos estímulos, sem evidência de autoconsciência ou consciência do ambiente. Contudo, a ausência total de reflexos do tronco encefálico e a capacidade de resposta parcial rejeitaram a possibilidade de um coma. Jahi não tinha síndrome de vigia sem resposta (SVSR), porque não estava em um estado de vigia e mostrou uma capacidade de resposta parcial. Também nao pode ser classificada como paciente LIS, porque estes estão acordados e conscientes, e ainda que tetraplégicos, conservam total ou parcialmente os reflexos do tronco encefálicos, os movimentos oculares verticais ou cintilação, e respiram por si próprios. Ela não estava em um EMC porque não preservava a excitação e preservava a consciencia somente parcialmente. A CRS-R deu uma pontuação muito baixa, incompatível com pacientes de EMC. Os pacientes de EMC preservam total ou parcialmente os reflexos do tronco encefálico e, em geral, respirar por si só. O EMC sempre foi descrito como um estado de transição entre coma e SVSR, mas nunca foi relatado em paciente com todos os achados clínicos de ME. Esse caso não contradiz o conceito de ME, mas volta a colocar a discussão sobre a necessidade de utilizar exames complementares em ME. Cheguei a conclusão de que Jahi representava um novo transtorno da consciencia, nao descrito anteriormente, que denominei "síndrome de resposta sem vigília" (SRSV)

Clinical characteristics and prediction of Kawasaki disease unresponsive to initial dose of intravenous immunoglobulin / 中华实用儿科临床杂志

Objective To determine the prediction and clinical characteristics of intravenous immunoglobulin (IVIG) treated Kawasaki disease (KD) failure in initial dose.Methods Retrospective analysis was performed with the clinical data of 1 11 children with KD.The paticnts were divided into sensitive group and unresponsive group according to initial effect of IVIG.The clinical manifestations,laboratory examination and radiological features of the children were compared.Logistic regression analysis was performed in significant different indicators to determine independent correlation factors.In order to seek the reference indexes which indicate unresponsive to IVIG,a receiver operating characteristic (ROC) curve was made,of which the diagnostic cut-off was nine independent correlation factors while grouping was made according to patients' different responses to IVIG.Results (1)There were 90 cases (81.1％)in effective group and 21 cases (18.9％) in unresponsive group.(2) Compared with the sensitive group,hyperpyrexia cases[66.7％ (14/ 21 cases) vs 34.4％ (31/90 cases),x2 =7.334,P =0.007] and the chances of coronary artery lesions [47.6％ (10/ 21 cases) vs 23.3％ (21/90 cases),x2 =4.989,P =0.026] were significantly higher in the unresponsive group.(3)Compared with the children administered twice with gamma globulin,the children of single-dose treatment significantly reduced the unresponsive probability [12.5 ％ (9/72 cases) vs 30.8 ％ (12/39 cases),x2 =5.504,P =0.019],and there was no difference in the chances of coronary artery lesions[23.6％ (17/72 cases) vs 30.8％ (12/39 cases),x2 =0.672,P =0.412].(4)Comparing the sensitive group and the unresponsive group,the percentage of neutrophils count [(0.72 ± 0.06) vs (0.76 ± 0.04),t =-2.84,P =0.005],platelet counts [(352.38 ± 42.18) × 109/L vs (373.14 ±36.93) × 109/L,t =-2.076,P =0.040] and C-reactive protein (CRP) [(74.38 ± 12.92) mg/L vs (92.05 ± 11.17) mg/L,t =-5.780,P =0.000] were significantly higher in the unresponsive group,but the level of serum albumin[(34.09 ±3.19) g/L vs (31.61 ±2.03) g/L,t =4.442,P =0.000] was lower.(5) Multivariate Logistic regression analysis indicated that the percentage increase of neutrophils count (P =0.018),CRP (P =0.000) increase and serum albumin(P =0.040) decrease were independent risk factors for unresponsive treatment with gamma globulin.(6)After the area under the ROC curve was calculated,the percentage of neutrophils count,CRP and serum albumin could be effective predictors to IVIG treatment failure in initial dose,and the critical values were 0.72,78.5 mg/L and 33.11 g/L,respectively.Conclusions Treatment with IVIG for the first time may be ineffective in some situations such as the percentage of neutrophils count ≥0.72,CRP ≥78.5 mg/L or serum albumin≤33.11 g/L.

Salivary Cortisol Estimation to Assess Adrenal Status in Children with Fluid Unresponsive Septic Shock.

We evaluated the adrenal status by estimating baseline and ACTH stimulated salivary cortisol in 51 children with fluid unresponsive septic shock at 30 and 60 minutes, and basal salivary cortisol (9-11 am) in 79 healthy children. The baseline salivary cortisol (median,IQR) among patients (19.8, 7.2-42.4 nmol/L) was higher than healthy children (2.6, 1.3-7.6 nmol/L) (P=0.001). Non-survivors and those with catecholamine refractory shock had higher baseline cortisol level, though difference was statistically insignificant. Absolute adrenal insufficiency (baseline salivary cortisol <1.3nmol/L) was diagnosed in 8 (15.7%) patients. Relative adrenal insufficiency (rise in cortisol level above baseline value after stimulation <25nmol/L) was observed in 68.6% of all patients; 71.9% among non-survivors, and in 71.4% patients with catecholamine refractory shock. Salivary cortisol estimation appears to be feasible in children with septic shock. Relative adrenal insufficiency is common in these children.

Unresponsive cutaneous leishmaniasis and HIV co-infection: Report of three cases.

Cutaneous leishmaniasis (CL) is a vector borne disease caused by various species of Leishmania parasite. CL is endemic in the Thar desert of Rajasthan state and Himachal Pradesh in India. Immune suppression caused by human immunodeficiency virus (HIV) infection is associated with atypical clinical presentation of CL which responds poorly to the standard treatment and causes frequent relapses. We are reporting three cases of localized and disseminated CL due to Leishmania tropica which failed to respond to conventional intralesional/intramuscular sodium stibogluconate (SSG) injections. Initially, we did not think of HIV infection because CL is endemic in this region. When patients did not respond to SSG injections, we performed enzyme-linked immunosorbent assay (ELISA) tests for HIV and they turned out to be HIV positive. Our report showed that CL is emerging as an opportunistic infection associated with HIV/AIDS and may be the first manifestation in HIV positive patients in an endemic area.