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BACKGROUND AND OBJECTIVES: Structured and systematised checklists have been shown to prevent complications and improve patient care. We evaluated the implementation of systematic safety checklists in our neurocritical care unit (NCCU) and assessed its effect on patient outcomes. DESIGN/METHODS: This quality improvement project followed a Plan-Do-Study-Act (PDSA) methodology. A checklist for medication reconciliation, thromboembolic prophylaxis, glycaemic control, daily spontaneous awakening, breathing trial, diet, catheter/lines duration monitoring and antibiotics de-escalation was implemented during daily patient rounds. Main outcomes included the rate of new infections, mortality and NCCU-length of stay (LOS). Intervened patients were compared with historical controls after propensity score and Euclidean distance matching to balance baseline covariates. RESULTS: After several PDSA iterations, we applied checklists to 411 patients; the overall average age was 61.34 (17.39). The main reason for admission included tumour resection (31.39%), ischaemic stroke (26.76%) and intracerebral haemorrhage (10.95%); the mean Sequential Organ Failure Assessment (SOFA) score was 2.58 (2.68). At the end of the study, the checklist compliance rate throughout the full NCCU stays reached 97.11%. After controlling for SOFA score, age, sex and primary admitting diagnosis, the implementation of systematic checklists significantly correlated with a reduced LOS (ß=-0.15, 95% CI -0.24 to -0.06), reduced rate of any new infections (OR 0.59, 95% CI 0.40 to 0.87) and reduced urinary tract infections (UTIs) (OR 0.23, 95% CI 0.09 to 0.55). Propensity score and Euclidean distance matching yielded 382 and 338 pairs with excellent covariate balance. After matching, outcomes remained significant. DISCUSSION: The implementation of safety checklists in the NCCU proved feasible, easy to incorporate into the NCCU workflow, and a helpful tool to improve adherence to practice guidelines and quality of care measurements. Furthermore, our intervention resulted in a reduced NCCU-LOS, rate of new infections and rate of UTIs compared with propensity score and Euclidean distance matched historical controls.

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BACKGROUND: Hyponatremia is a common complication after aneurysmal subarachnoid hemorrhage (aSAH). Previous studies have reported an association between hyponatremia and vasospasm, however whether hyponatremia directly contributes to the pathogenesis of cerebral vasospasm (CVS), or is a by-product is still unclear. The aim of this study was to explore an association between hyponatremia and CVS after aSAH, and evaluating the temporality of these 2 events. METHODS: A retrospective study of consecutive patients with aSAH admitted to the Baylor St. Luke's Medical center between January 2008 and December 2012 was conducted. Demographics, baseline characteristics, serum sodium levels, and evidence of vasospasm detected by transcranial Doppler, CT Angiogram, MR angiogram, and digital subtracted angiography were collected. Patients were dichotomized into a hyponatremic and a normonatremic group. CVS incidence and clinical outcome was compared between groups. Timing of CVS after initial hyponatremia episodes was recorded Results: One hundred and sixty 4 patients with aSAH were included. Hyponatremia was identified in 66 patients (40.2%) and CVS occurred in 71 subjects (43.2%). The incidence of CVS was higher in the hyponatremic group compared to the normonatremic group, 65.1 % versus 28.5%, respectively (P < .001). Hyponatremia preceded CVS by median 1.5 days suggesting a temporal trend. CONCLUSIONS: Our study shows a significant association between hyponatremia and CVS, with hyponatremia preceding CVS events. This retrospective finding denotes the need for larger prospective studies, aiming to clarify the temporal relationship of serum sodium levels and CVS.

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Weight loss dietary supplements are used with some frequency by an increasingly overweight population. Some products are not adequately regulated and may pose potential health risks. We report two new cases of acute toxic leukoencephalopathy (ATL) due to the use of a supplement marketed as a thermogenic weight loss aid. ATL is a heterogeneous clinic-radiological entity that has been associated with various compounds, such as chemotherapeutic drugs and immunomodulators. It is characterized by an often reversible periventricular and infratentorial demyelination. The commercialization of non-regulated weight loss products continues to be a health risk in our population.

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Amyotrophic lateral sclerosis (ALS) is described as a neurodegenerative disorder. However, neuroinflammation and chemokine expression are prominent pathological finding at sites of injury. Adipsin and adiponectin are molecules that are implicated in the pathogenesis of neurodegenerative and neuroimmune disorders. Adipsin and adiponectin concentrations were determined in the CSF of ALS patients and controls and the relationship of these chemokines with clinical severity and disease duration in ALS was determined. Seventy-seven ALS patients (mean age 49.5 ± 10.4 years) (mean body mass index 23.5 ± 4.5) were included. Twenty patients had bulbar, 53 spinal, and four bulbospinal onset ALS. Median adipsin CSF level was 12,650.94 pg/ml in ALS patients and 3290.98 pg/ml in controls (p < 0.001). Median adiponectin CSF level was 4608 pg/ml in ALS patients and 3453 pg/ml in controls (p = 0.1). No differences were observed in disease duration, progression rate or disease severity. There was a significant positive correlation between adipsin and adiponectin concentrations (r = 0.379, p = 0.01). No correlation with age, body mass index or ALFRS-R score was found. Adipsin was significantly elevated in CSF, suggesting that this chemokine might have a role in ALS pathogenesis. Adiponectin showed a trend towards higher concentrations, but failed to reach statistical significance. Due to the clinical heterogeneity in our cohort, these chemokines do not appear to be associated with disease duration or severity.

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INTRODUCTION: Increased cytokine expression is a prominent finding in amyotrophic lateral sclerosis (ALS). Due to their interdependence and pleiotropism, interpretation of CSF concentrations of a single cytokine is challenging. We describe a cytokine analysis in ALS patients using a pathway-based statistical method to identify changes in the whole cytokine network. METHODS: We analyzed 19 cytokines in CSF of ALS patients and controls. An equality of concentration matrices was conducted that allowed us to evaluate disturbances in the relationship of cytokines between controls and ALS patients with less and more than 12months of disease length. MANOVA assessed differences in cytokines and interdependence was compared by partial correlations among specific pairs of cytokines. RESULTS: Seventy-seven ALS patients and 13 control subjects were included. Significant differences were identified in the cytokine pathway between controls and ALS patients with less (p<0.0001) and more than 12months of disease length (p<0.0001), and between ALS patients with less than 12months and those with more than 12months (p=0.0058). In ALS patients with a shorter disease length, IL4 and IL6 were negatively correlated (-0.3571), whereas in ALS>12months, a positive correlation was detected (0.4080). CONCLUSIONS: The pathway-based statistical method revealed remarkable variations in the whole cytokine pathway in ALS patients and controls. Cytokine-network changes and the positive correlation between IL4 and IL6 were related to disease length; these variations might explain the deleterious immunological effects on motor neurons. A further analysis using this method is needed to confirm the exact interaction among cytokines in ALS.

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ResumenINTRODUCCIÓN: La retinosis pigmentaria es la forma hereditaria y crónica más común de distrofia retiniana. Esta condición se caracteriza inicialmente por la afectación progresiva de los fotorreceptores y, posteriormente, de otras capas de la retina. En la exploración ocular esta situación se traduce como palidez del disco óptico, disminución vascular y depósitos de pigmento en la retina.CASO CLÍNICO: Se presenta el caso de un paciente masculino de 15 años de edad con una historia de 6 meses de evolución caracterizada por ceguera nocturna y disminución de la visión lateral temporal superior en ambos ojos.CONCLUSIONES: Este tipo de padecimiento ocular distrófico, genético y progresivo comienza durante la adolescencia y condiciona una discapacidad visual.

AbstractBACKGROUND: Retinitis pigmentosa is the most common chronic and inherited condition of retinal dystrophy. The progressive involvement of retinal photoreceptors and other layers characterize this condition. This situation results in optic disc pallor and retinal pigment deposition vascular attenuation.CASE REPORT: We present the case of a 15-year-old male with a history of 6 months evolution characterized by night blindness and bilateral impairment of superior temporal vision.CONCLUSIONS: This type of dystrophy is a genetic and progressive eye condition that begins during adolescence and produces visual impairment.

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The branchio-oculo-facial syndrome is a dominant autosomic condition with variable expressivity that affects particularly the facial and neck structures by an inadequate development of the first and second branchial arch. It is characterized by malformations of eyes and ears, with distinct facial characteristics. It is associated with alterations in TFAP2A gene. We present a patient with 9 years of age with phenotype of the branchio-oculo-facial syndrome and the presence of 2 new oral manifestations, the bifid uvula and the tongue with partial central cleft, not yet described in this clinical condition.

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El síndrome branquio-óculo-facial es una condición autosómica dominante con expresividad variable y que afecta particularmente las estructuras de la cara y cuello por un desarrollo inadecuado del primero y segundo arco branquial; presenta malformaciones de los ojos y oídos, con características faciales distintivas. Está asociado con alteraciones en el gen TFAP2A. Se presenta una paciente de 9 años con fenotipo de síndrome branquio-óculo-facial y la presencia de dos nuevas manifestaciones orales, la úvula bifida y la lengua con hendidura central parcial, no descritas hasta ahora en esta condición clínica.(AU)

The branchio-oculo-facial syndrome is a dominant autosomic condition with variable expressivity that affects particularly the facial and neck structures by an inadequate development of the first and second branchial arch. It is characterized by malformations of eyes and ears, with distinct facial characteristics. It is associated with alterations in TFAP2A gene. We present a patient with 9 years of age with phenotype of the branchio-oculo-facial syndrome and the presence of 2 new oral manifestations, the bifid uvula and the tongue with partial central cleft, not yet described in this clinical condition.(AU)

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El síndrome branquio-óculo-facial es una condición autosómica dominante con expresividad variable y que afecta particularmente las estructuras de la cara y cuello por un desarrollo inadecuado del primero y segundo arco branquial; presenta malformaciones de los ojos y oídos, con características faciales distintivas. Está asociado con alteraciones en el gen TFAP2A. Se presenta una paciente de 9 años con fenotipo de síndrome branquio-óculo-facial y la presencia de dos nuevas manifestaciones orales, la úvula bifida y la lengua con hendidura central parcial, no descritas hasta ahora en esta condición clínica.

The branchio-oculo-facial syndrome is a dominant autosomic condition with variable expressivity that affects particularly the facial and neck structures by an inadequate development of the first and second branchial arch. It is characterized by malformations of eyes and ears, with distinct facial characteristics. It is associated with alterations in TFAP2A gene. We present a patient with 9 years of age with phenotype of the branchio-oculo-facial syndrome and the presence of 2 new oral manifestations, the bifid uvula and the tongue with partial central cleft, not yet described in this clinical condition.

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El síndrome branquio-óculo-facial es una condición autosómica dominante con expresividad variable y que afecta particularmente las estructuras de la cara y cuello por un desarrollo inadecuado del primero y segundo arco branquial; presenta malformaciones de los ojos y oídos, con características faciales distintivas. Está asociado con alteraciones en el gen TFAP2A. Se presenta una paciente de 9 años con fenotipo de síndrome branquio-óculo-facial y la presencia de dos nuevas manifestaciones orales, la úvula bifida y la lengua con hendidura central parcial, no descritas hasta ahora en esta condición clínica.(AU)

The branchio-oculo-facial syndrome is a dominant autosomic condition with variable expressivity that affects particularly the facial and neck structures by an inadequate development of the first and second branchial arch. It is characterized by malformations of eyes and ears, with distinct facial characteristics. It is associated with alterations in TFAP2A gene. We present a patient with 9 years of age with phenotype of the branchio-oculo-facial syndrome and the presence of 2 new oral manifestations, the bifid uvula and the tongue with partial central cleft, not yet described in this clinical condition.(AU)

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BACKGROUND: Retinitis pigmentosa is the most common chronic and inherited condition of retinal dystrophy. The progressive involvement of retinal photoreceptors and other layers characterize this condition. This situation results in optic disc pallor and retinal pigment deposition vascular attenuation. CASE REPORT: We present the case of a 15-year-old male with a history of 6 months evolution characterized by night blindness and bilateral impairment of superior temporal vision. CONCLUSIONS: This type of dystrophy is a genetic and progressive eye condition that begins during adolescence and produces visual impairment.

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The branchio-oculo-facial syndrome is a dominant autosomic condition with variable expressivity that affects particularly the facial and neck structures by an inadequate development of the first and second branchial arch. It is characterized by malformations of eyes and ears, with distinct facial characteristics. It is associated with alterations in TFAP2A gene. We present a patient with 9 years of age with phenotype of the branchio-oculo-facial syndrome and the presence of 2 new oral manifestations, the bifid uvula and the tongue with partial central cleft, not yet described in this clinical condition.

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BACKGROUND AIMS: Amyotrophic lateral sclerosis (ALS) is rare in pregnant patients. Stem cell therapy has been proposed as a potential therapeutic strategy for ALS. METHODS: We describe a young woman with sporadic ALS that started during the second trimester of pregnancy with a rapid progression after delivery and severe motor impairment. Several drugs and stem cell injection by lumbar puncture were performed without changes before the patient was referred to our institution. RESULTS: After bilateral autologous stem cell transplantation into the frontal motor cortices, we observed stabilization in ALS functional rating scale, significant delay of ALS progression and an extension in her life expectancy. CONCLUSIONS: Stem cell transplantation may alter the clinical course of ALS and improve quality of life in pre-menopausal women.

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The vestibular schwannoma is a benign intracranial tumor of the myelin-forming cells of the vestibulocochlear nerve or cranial nerve VIII. It comprises 8-10% of all intracranial neoplasms in adults. It originates in the vestibular portion of the cranial nerve VIII and it is located in the cerebellopontine angle. This disorder is characterized by ipsilateral hearing loss, tinnitus, disturbed sense of balance and altered gait, facial numbness, muscle weakness or ipsilateral paralysis. This report presents the magnetic resonance imaging of a patient with this rare condition.

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