Publicación Oficial de la Sociedad Ecuatoriana de Neurología, de la Liga Ecuatoriana Contra la Epilepsia y de la Sociedad Iberoamericana de Enfermedad Cerebrovascular

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Funcionalidad de la Marcha en la Calidad de Vida Relacionada con la Salud en Adultos con Enfermedad Cerebro Vascular: Revisión Sistemática – Metaanálisis. Functionality Of The Gait In The Quality Of Life Related To Health In Adults With Stroke: Systematic Review – Metaanalysis.

Introduction: Stroke causes deficiencies that affect movement; these include deficit in motor function, reflex integrity, sensory integrity, and gait.

Objective: determine the effect of gait functionality on health-related quality of life in adults with Stroke.

Method: databases PUBMED / MEDLINE, EMBASE, LILACS, SCIELO, Central Cochrane Registry of Controlled Trials (CENTRAL) were consulted between November 2016 and February 2017. Randomized clinical trials were selected. For the analysis, the methodological quality was evaluated using the Physiotherapy Evidence Database, PEDro score and the Risk of bias with the Review Manger 5.3 (RevMan) criterias, also RevMan was being used for analysis and data extraction according to the eligibility criteria.

Results: Lokomat as a rehabilitation strategy for gait functionality has a positive effect on improving the quality of life in people with stroke. The analysis of the included studies demonstrated a low level of statistical heterogeneity based on I2 and Chi2, for the global scale of quality of life. The results obtained for quality of life related to health, when the proposed intervention is carried out with the use of Lokomat and measured with the SF-36 scale, an increase of 1.83 points.

Conclusions: The rehabilitation of gait functionality through different protocols and intervention strategies does not present differences, due to its variability in terms of technique, application method, clinical involvement of stroke and the duration of its application. However, clinical changes that favor quality of life related to health are observed.

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Palabras clave: Accidente Cerebrovascular, Adulto, Anciano, Balance postural, calidad de vida, marcha, Persona de mediana edad,

Keywords: Adult, Aged, gait, Middle aged, Postural balance, quality of life, stroke,

Síndrome de Sturge-Weber: Reporte de un Caso y Revisión de la Literatura. Sturge-Weber Syndrome: A Case Report And Literature Review.

Sturge-Weber Syndrome is a rare developmental neuroectodermical disorder. It is characterized by a facial port-wine stain and a leptomeningeal angiomata, frequently localized ipsilateral to the facial port-wine stain. This syndrome predisposes either to brain atrophy, calcifications and refractory seizures.

In this paper a Sturge-Weber Syndrome literature review was made and a 18 month aged child case with this diagnosis is reported. He was admitted to the emergency department of a local hospital with a history of three days of fever and tonic-clonic seizures localized on the right side and refractory to conventional treatment.

This review highlights the importance of an early diagnosis and an appropriate follow up. To carry out this review a search in PubMed, Science Direct and Scielo databases was done, confirming that there are some issues about this disorder that are still unknown. However, with the GNAQ somatic mutation discovery, there is an open field for new researches. It is very important in medical practice not to understimate a facial port-wine stain over trigeminal territory in newborns in order to make an early diagnosis and try to achieve a better future neurodevelopment.

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Palabras clave: angioma, Convulsión, leptomeninges, nevus, Sturge-weber,

Keywords: angiomata, facial stain, leptomeningeal, seizure, Sturge-weber,

Síndrome de Fahr Secundario a Hiperparatiroidismo Primario e Isquemia Cerebral: A Propósito de un Caso. Fahr Syndrome Secondary To Primary Hyperparathyroidism And Cerebral Ischemia: A Case Report.

Fahr’s syndrome (FS) is a rare neurodegenerative condition, characterized by cerebral calcifications mainly in the basal ganglia. Although its most common presentation are the disorders of movement, cognition, behavior and epilepsy, in recent years cases of cerebrovascular disease (CVD) related to this entity have been appearing.

We report a 59-year-old male patient who presented 2 transitory ischemic attacks (TIAs), the 1st of 5 minutes, of speech alteration what happened unnoticed, and the second of 45 minutes, 2 weeks later, of speech alteration, loss of muscle strength and tingling sensation in the left side of the body. Brain computed tomography and magnetic resonance imaging revealed calcifications suggestive of FS and a treatable cause was found (primary hyperparathyroidism with hypovitaminosis D). The patient was treated with aspirin, atorvastatin and colecalciferol without vascular recurrence and the levels of vitamin D and PTH normalized. Although the association between CVD in young people and SF has not yet been determined, the occurrence of these cases leads us to suspect that ischemic CVD could be part of the natural history of this entity. Being the prevalence of FS unknown, we alert clinicians to keep CVD in mind as a form of presentation of this condition. We review the association between FS and ischemic CVD (without in clusion of aneurysmal disease).

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Palabras clave: Hiperparatiroidismo primario, Isquemia Cerebral, Síndrome de Fahr,

Keywords: Cerebral ischemia, Fahr's syndrome, Primary hyperparathyroidism,

Revistas Predatorias, Que Son y Como Evitarlas. Predatory Journals: What They Are And How To Avoid Them.

The progress of humanity goes hand in hand with the production of new scientific knowledge. To carry out an investigation has as its ultimate purpose to fill an information gap in the endless and dynamic universe of knowledge. It is said that unpublished information is non-existent information and this assertion is the one that provokes a frenzy within the researchers to look for opportunities to publish their results. This growing demand becomes an opportunity for those who seek to profit from the needs of teachers, researchers, students and professionals in general who try to publish their results every day.

It is currently estimated that there are more than 10,000 journals that seek to publish results in exchange for money, with a non-existent or a very brief peer review, known as predatory journals. They multiply exponentially and endanger the credibility of the science worldwide. In this brief editorial we seek to explain what predatory journals are, how to identify them and how to avoid them within the context of scientific production in the medical field.

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Factores Asociados a la Mortalidad de la Hemorragia Cerebral Intraparenquimatosa Espontánea en Pacientes Mayores de 50 Años de Edad que Acudieron al Hospital Teodoro Maldonado Carbo Durante el Año 2017. Factors Associated With Mortality Of Spontaneous Intraparenchymal Cerebral Hemorrhage In Patients Over 50 Years Old Who Attended The Teodoro Maldonado Carbo Hospital During 2017.

Objective. To determine the factors associated with the mortality of spontaneous intraparenchymal cerebral hemorrhage in patients over 50 years old who attended the Teodoro Maldonado Carbo Hospital during 2017.

Methods: A retrospective analytical observational study of 92 patients of diagnosis of spontaneous primary intraparenchymal hemorrhage, 30-day mortality was evaluated according to demographic characteristics, risk factors and poor prognostic factors. The Intracerebral Hemorrhage Grading Scale (ICH-GS) scale was applied in our population to evaluate the correlation of the scores obtained with the 30-day mortality.

Results: From 92 patients (mean age: 69 years, mean Glasgow Coma Scale [GCS] on admission: 10, mean supratentorial and infratentorial volume, respectively 36.63 and 13.92 ml, most common hematoma location: thalamus (21.74%). at 30 days it was [31.40%]). In a univariate analysis, GCS (odds ratio [OR] = 2.20, 95% confidence interval [CI] = 1.04- 4.65, p <0.04), infratentorial volume (OR) = 3.74 per ml, 95% CI = 1.25 to 11,120, p <0.02) and the ventricular extension was (OR = 5.43, 95% CI = 1.40-22.35, P = 0.02) were significant predictors for 30-day mortality The Pearson correlation showed correlations of 0.6556 between the IC-GS score and the 30-day mortality (P <0.001).

Conclusions: The GCS score at admission together with infratentorial volume and intraventricular extension are significant predictors of 30-day mortality in patients with primary spontaneous Intracerebral Hemorrhage (ICH) being useful for identifying high-risk patients in the short term.

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Palabras clave: escala de ICH-GS, Hemorragia cerebral; pronóstico; factores de riesgo,

Keywords: Cerebral haemorrhage, ICH-GS scale, Prognosis, Risk factors,

Ausencia de Asociación Entre la Posición de la Lengua Tipo IV de Friedman y Apnea Obstructiva de Sueño en Adultos Mayores con Ancestro Amerindio. Lack Of Association Between The Friedman’s Tongue Position Type IV And Obstructive Sleep Apnea In Older Adults Of Amerindian Ancestry.

Background: The burden of obstructive sleep apnea (OSA) in rural settings is unknown. In these regions, devices needed for OSA diagnosis are not available, and mass screening with field instruments may be complicated due to cross-cultural factors and illiteracy. The association between the Friedman’s tongue position (FTP) and OSA has been assessed in people from different ethnic groups but not in Amerindians.

Objective: We aimed to assess whether a FTP type IV is associated with OSA severity and with the apnea-hypopnea index (AHI) in community-dwelling older adults of Amerindian ancestry living in rural Ecuador.

Methods: A total of 201 Atahualpa residents aged ≥60 years, who underwent tongue position assessment, brain MRI, and polysomnography were included. After adjusting for relevant confounders, ordinal logistic regression models were fitted to assess the association between the presence of a FTP type IV and OSA categories (none, mild, and moderate-to-severe), and generalized linear models with a Gaussian link were fitted to assess the association between the presence of a FTP type IV and the continuous AHI.

Results: A FTP type IV was identified in 153 (76%) individuals, the mean AHI per hour was 11.9 ± 12.4, and 49 (24%) individuals had moderate-to-severe OSA, 88 (44%) had mild OSA, and the remaining 64 (32%) had no OSA. Fully-adjusted generalized linear models showed no independent association between the investigated exposure and the AHI (β: 0.09; 95% C.I.: -1.56 – 1.76; p=0.909). Likewise, ordinal logistic regression models showed no independent association between the investigated exposure and categories of OSA (β: 0.42; 95% C.I.: -0.47 – 1.31; p=0.357).

Conclusion: A FTP type IV is not associated with the AHI or the severity of OSA in this population of Amerindians. This lack of association could be related to phenotypic characteristics of people from this ethnic group (mostly their elliptic hard palate).

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Palabras clave: Apnea Obstructiva del Sueño, Índice apnea-hipopnea, posición de la lengua de acuerdo con Friedman,

Keywords: Amerindians, Apnea-hypopnea index, Friedman’s tongue position, Obstructive Sleep Apnea,

Adherencia al Tratamiento Antiepiléptico en Pacientes Pediátricos del Hospital Roberto Gilbert Elizalde en el Año 2014. Adherence To Antiepileptic Treatment In Pediatric Patients At Hospital Roberto Gilbert Elizalde During 2014.

Introduction: Epilepsy in the pediatric population is an entity that mostly affects underdeveloped countries. In Ecuador, hospital admissions due to poor control are increasing and the main cause is poor adherence to antiepileptic treatment.

Objective: To demonstrate the incidence of nonadherence to the antiepileptic regimen and determine the factors that contributes to it.

Materials and methods: A cross-sectional study was conducted in which 119 patients were included with ages between 6 months and 17 years. The parents or legal guardians were questioned regarding the degree of adherence to antiepileptic treatment using the Morisky questionnaire. A logistic regression model was used to measure the strength of association between variables.

Results: An incidence of non-adherence to antiepileptic treatment of 36.97% was found. The significantly associated variables were the mother’s schooling (x2=11.83, IC= 10.13 – 13.53, p=0.018), previous medical information (x2=9.35, IC= 7.95 – 10.75, p=0.02) and the failure to obtain medication due to lack of money (x2=5.98, IC= 5.29 – 6.67, p=0.01).

Conclusion: The incidence of nonadherence in this study was high; control over sociodemographic factors and related to treatment can have a great impact on these patients.

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Palabras clave: adherencia, Epilepsia, pediatría, Tratamiento,

Keywords: adherence, Epilepsy, pediatrics, Treatment,

Frecuencia de Casos Juveniles con Enfermedad de Huntington en Población Mexicana. Frequency Of Juvenile Huntington’s Disease In A Mexican Population.

Objective. The purpose of this study is to know the prevalence of juvenile cases in a sample of mexican subjects with confirmed Huntington Disease (HD).

Methods. Patients with clinical debut before 21 years of age were included who attended at movement disorders clinic of the National Institute of Neurology and Neurosurgery. The demographic and clinical information was obtained from the review of files.

Results. A total of 198 cases of patients diagnosed with HD were reviewed, of which 6.5% (n = .13) corresponded to juvenile forms. The mean age for the onset of symptoms was 17.8 ± 3.9 years. The mean score of the UHDRS-motor was 46.2 ± 17.4 points. The predominant motor symptom was chorea in (53.8%) of the cases. 84.6% of those affected presented at least one neuropsychiatric disorder.

Conclusion. It was detected that the dominant motor phenotype of these patients was chorea compared to the world reports until now, accordingly to that, our group of juvenile HD shows atypical motor clinical.

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Palabras clave: corea, enfermedad de Huntington, juvenil, México, Westphal,

Keywords: chorea, Huntington disease, juvenile, Mexico, Westphal.,

Fibrinólisis Farmacológica en el Ictus Isquémico Agudo. Experiencia en un Hospital Terciario del Ecuador. Pharmacological Thrombolysis In Ischemic Stroke. Experience In A Tertiary Hospital From Ecuador.

Introduction. Pharmacological thrombolysis in ischemic stroke is associated with a better recovery.

Objective. Describe the thrombolysis results after using r-Tpa applying an intrahospital stroke code, during one year.

Methods. A prospective, longitudinal study was performed in patients with cerebral infarction admitted to the stroke unit, with clinical follow-up up to 3 months after hospital discharge. The variables evaluated were compared in two groups of patients (only one group received the treatment).

Results. 107 patients were studied: 16 (14.9%) were thrombolyzed, 29 (27.1%) arrived in the therapeutic window period and 76 (71%) arrived after 4.5 hours. The average age was 68, 8 years and women predominated. The greatest impact of thrombolysis was on the difference in score between the initial assessment and the discharge on the NIHSS scale. At three months of evolution, the percentage of patients with mild disability (Rankin 0-2) was almost equal in the two groups. Mortality increased in patients with more severe disability (Rankin 3-5).

Conclusions. Treatment with r-Tpa shows benefits at hospital discharge. Further analysis is required with a greater number of cases.

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Palabras clave: Accidente Cerebrovascular, Ecuador, Fibrinólisis, infarto cerebral, Terapia Trombolítica,

Keywords: Cerebral infarction, Ecuador, fibrinolysis, stroke, Thrombolytic therapy,

Efectividad del Foto-Test Frente al MMSE, Para el Cribado del Deterioro Cognitivo en Población Peruana. Effectiveness Of The Photo-Test Front Of The MMSE, For The Screening Of Cognitive Deterioration In Peruvian Population.

The progressive increase of Alzheimer’s disease has generated interest in its early detection with cognitive screening tests being a useful tool, however, they need to be culturally adapted, objective and reliable. In Peru, this need is greater since the educational level of the elderly population is mostly low. The aim of the present study is to know the estimate sensitivity and specificity of FOTOTEST against MMSE for the screening/detection of cognitive impairment, analyzing the relationship of these cognitive tests with one of functional activity. 107 elderly people, aged 60-89 years, were evaluated. The Yesavage scale for geriatric depression was used, the functional activities questionnaire of Pfeffer, MMSE, and FOTOTEST. Pearson’s analysis showed a significant positive correlation between MMSE/FOTOTEST (Pearson 0.386, p <0.003), whereas only MMSE showed a significant negative correlation with PFAQ (Pearson -0.320, p <0.013). However, FOTOTEST did not show a significant correlation with PFAQ (Pearson -0.067, p <0.613). In addition, the percentage of effectiveness and specificity estimated for FOTOTEST was 100.00% and 92.68%, respectively, higher than the MMSE, with an estimated percentage of effectiveness being 83.33%, and specificity of 34.14%. We conclude that FOTOTEST would be a more useful test for the detection of cognitive impairment than MMSE.

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Palabras clave: Adultos Mayores, Cribado, Especificidad, Fototest, MMSE, Sensibilidad,

Keywords: elderly, MMSE, Phototest, Screening, Sensitivity, Specificity,

Las Curvas Para Medir Circunferencia Cefálica y las Potenciales Diferencias Antropométricas en Latinoamérica y el Mundo. Cephalic Circumference Curves And Potential Anthropometric Differences In Latin America And The World.

The cephalic circumference (CC) is a measurement that detects alterations in the proper growth of the brain. CC curves most used worldwide are those proposed by the CDC, NCHS and WHO. Despite criticism of the methodology used to create them, updates have been made to correct inconsistencies, and that has helped to detect and treat on time problems of cranial size at both the small (microcephaly) and large (macrocephaly) ends. Several authors have suggested that regional reference curves should be made, and others, like Kenton Holden and colleagues, have proposed new curves that consolidate different databases, aiming to reduce the risk of errors in the diagnosis of microcephaly or mild macrocephaly. It is necessary to study what the normal cranial growth pattern of Latin American children is for each region or country, as well as to determine the interethnic differences.

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Palabras clave: biometría, cefalometría, macrocefalia, microcefalia, perímetro craneal,

Keywords: biometrics, cephalometrics, cranial perimeter, macrocephaly, microcephaly,

La Actividad Embrionaria Espontánea de las Redes Neuronales y su Función en la Maduración Sináptica. Embryonic Spontaneous Network Activity And Its Role On Synaptic Development.

Embrionic neural networks exibit a temporary special form of electrical activity known as Spontaneous Network Activity (SNA). SNA occurs as soon as synaptic conections are stablished and consists on synchronized action potential firing for most of the neurons on the network, followed by long silents periods where network excitability is gradually recovered till a new SNA episode can happen. This kind of neural activity allows a high level of synchronization among neurons on developing networks, contributing to synaptic connection and maturation. SNA has been described in several regions of the developing nervous system due to conserved properties among developing neural networks: redundant intercellular connectivity and the fact that the neurotransmitter gamma-aminobutiric acid (GABA) is excitatory during early embryonic development (GABA is inhibitory in the adult nervous system).In this review we discuss the hypothesis that SNA contributes to synaptic strenght for glutamatergic and gabaergic synapsis while both of them are excitatory, by using the same synaptic plasticity mechanism known as homeostatic plasticity.

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Palabras clave: Actividad embrionaria espontánea, desarrollo embrionario, plasticidad homeostática,

Keywords: embryonic development, homeostatic plasticity, Spontaneous network activity,

Telesalud y Telemedicina para el Manejo de la Epilepsia. Telehealth and Telemedicine for the Management of Epilepsy.

Introduction. Epilepsy is one of the most frequent chronic neurological diseases, with figures close to 70 million cases worldwide; for which telehealth and telemedicine are framed as promising models that can bring multiple benefits.

Objective. To evaluate the initial schemes that have been proposed from telemedicine and telehealth in the management of the patient with epilepsy and the benefits that they have in the management.

Methods. A search of scientific literature was carried out in (PubMed, Cochrane library and LILACS). Different models of telehealth and telemedicine implemented in the management of patients with epilepsy are presented and the main benefits in terms of acceptability, costs and clinical results are described.

Conclusions. Current research of these models of care for the management of patients with epilepsy has the potential to effectively reduce the diagnostic and treatment gap, with the possibility of reducing costs in care for the institutions, the health system, patients and their families and harmoniously integrate with education in the self-control of the disease. Proving that there is a need for a more personalized approach to stimulate the use of these tools.

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Palabras clave: ensayos clínicos, Epilepsia, estudios observacionales, Telemedicina, teleneurología, telesalud,

Keywords: clinical trial, observational study, telehealth, telemedicine, teleneurology epilepsy,

Pruebas Terapéuticas con Fines Diagnósticos en Trastornos del Movimiento. Therapeutic Tests For The Diagnosis Of Movement Disorders.

El diagnóstico en medicina tiene su piedra angular en la obtención de la historia clínica y la exploración física; sin embargo, en algunas de las patologías el uso de pruebas diagnósticas es de gran utilidad como apoyo para confirmar o descartar una enfermedad. Las pruebas diagnósticas incluyen una amplia variedad de procedimientos y dentro de estas se incluyen las pruebas terapéuticas, también llamadas desafíos o retos, en los que se administra una sustancia activa para evaluar la respuesta y apoyar la impresión diagnóstica.  En el contexto de los trastornos del movimiento las pruebas terapéuticas más frecuentemente utilizadas son la de levodopa, apomorfina y etanol. En esta revisión se presenta, describe y discute el alcance y utilidad de estas.

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Palabras clave: apomorfina, etanol, levodopa, Pruebas terapéuticas, reto agudo,

Keywords: acute challenge, apomorphine, ethanol, levodopa, Therapeutic tests,

Estimulación Magnética Transcraneal “Theta-Burst Intermitente” en un Paciente con Trastorno del Espectro Autista: Reporte de un Caso. Transcranial Magnetic Stimulation “Theta-Burst Intermittent” In A Patient With Autism Spectrum Disorder: Case Report.

Background. Transcranial magnetic stimulation (TMS) is a potential treatment option for autism spectrum disorder (ASD) symptomatology.

Objectives. To determine the efficacy of the TMS intermittent theta-burst stimulation (iTBS) protocol over the left dorsolateral prefrontal cortex (DLPFC) in the management of pathological behaviors associated with ASD.

Patient/Methods. A 10-year-old male diagnosed with ASD by the Autism Diagnostic Observation Schedule (ADOS) presented with a family history of ASD with a younger sister diagnosed with the same disorder. Both magnetic resonance imaging (MRI) and functional MRI (fMRI) scans for emotional paradigms were performed before the iTBS treatment, immediately after the iTBS treatment, and after 6 months of iTBS treatment.

Results. The Autism Treatment Evaluation Checklist (ATEC) reports revealed improvement in communication, cognition, sociability, and behavior scales by 83%, 81%, 72%, and 52%, respectively. The overall score improved by 66% immediately after the treatment and by 55% lasting over a 6-month period. The fMRI revealed the activation of the frontal, parietal, and occipital cortex before iTBS treatment, and a better integration and activation of the frontal, temporal, and occipital cortex after iTBS treatment and persisted after 6 months.

Conclusions. iTBS is a well-tolerated, non-invasive neuromodulation technique that requires relatively less administration than the standard treatment.

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Palabras clave: corteza pre frontal dorso lateral izquierda, Estimulación magnética transcraneal, modalidad theta-burst intermitente, trastornos del espectro autista,

Keywords: Intermittent Theta Burst Stimulation (iTBS), Repetitive transcranial magnetic stimulation (rTMS),

Agenesia de la Arteria Carótida Interna: Presentación de dos Casos y Revisión de la Literatura. Agenesis Of The Internal Carotid Artery: Presentation Of Two Cases And Review Of The Literature.

Introduction: Two cases of patients with left internal carotid artery agenesis are presented, the first case with symptoms of open angle glaucoma and ipsilateral cataract and the second case of a patient with tension headache, as well as a review of the literature due to the Low frequency of this development anomaly.

Clinical cases: case 1, A 66-year-old woman with visual disorders associated with cataract and glaucoma in whom, due to suspicion of a carotid fistula, a left internal carotid agenesis was documented, with collaterality through the posterior system, case 2, patient A 58-year-old man with tension headache underwent an MRI where the agenesis of the left carotid artery, segments from C3 to C7, was documented.

Conclusion: Abnormalities in the development of the carotid system occur early in embryogenesis. In relation to the moment in which the alteration occurs, different circulatory patterns have been described. It is possible that the presence of collateral circulation is the reason that the majority of cases are reported asymptomatic from the vascular neurological point of view. Despite being a rare entity, it must be known in order to differentiate it from other conditions and monitor the appearance of associated complications.

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Palabras clave: agenesia, carótida,

Keywords: agenesis, carotid, left,

Meningioma Intraventricular. Intraventricular Meningioma.

Intraventricular meningioma is an infrequent disorder. We report a case of a 20 years old woman with a clinical picture of headache, nausea, vomiting and gait disorder. Intraventricular meningioma was diagnosed with magnetic resonance and histopathology. A transcortical right parietal surgical approach was performed through ventricular trigone. The procedure was done without complications or sequelae.

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Palabras clave: intraventricular, meningioma, tumor,

Keywords: intraventricular, meningioma, tumor,

Temblor Mentoniano: Reporte de un Caso Aislado. Chin Tremor: Report of an Isolated Case.

Chin tremor or geniospasm is an involuntary, rhythmic, autosomal dominant hereditary movement disorder, triggered by stress or emotional situations. Registered for the first time in 1894, in Italy.

We report a 4-year-old girl with mental tremor from one year of age, with no previous inheritance, with a successful response to the use of Botulinum Toxin.

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Palabras clave: genioespasmo, Mentón, Temblor,

Keywords: Chin, Geniospasm, Tremor,

Hiperlisinemia Como Hallazgo Sugestivo de Acidemia Propiónica. Reporte de Caso. Hyperlisinemia As A Suggestive Finding Of Propionic Acidemia. Case Report.

Introduction. Propionic acidemia (AP) is an organic acidemia (AO) with clinical presentation of neonatal onset or late. Caused by deficiency of the enzyme propionil-CoA carboxilasa that causes accumulation of propionic acid and metabolites related to propionyl-CoA in tissues. Hyperglycinemia is characteristic, but hyperlysinemia may occur. This work describes a clinical case of AP of neonatal onset with fatal outcome and striking alteration of amino acids.

Clinical case. Female newborn (RN) admitted in the neonatal unit on the third day of life due to hypoactivity, vomiting and lethargy. Subsequent respiratory distress and cardiac arrest occurred, dying before a biochemical diagnosis was established. Initial paraclinics evidenced metabolic acidosis, leukopenia, hypoglycemia, later documented hyperglycinemia, hypercystinemia and severe hyperlysinemia. The organic acid chromatography in urine identified 3-hydroxy-propionic acid, methyl citrate and propionylglycine among other toxic metabolites, confirming the diagnosis.

Conclusions. AP is an inborn error of autosomal recessive metabolism of low incidence. The presence of severe metabolic acidosis, pancytopenia, hypoglycemia and family history should alert about this diagnosis. Additionally, although the definitive biochemical diagnosis is organic acids in urine, the presence of hyperammonemia, hyperglycinemia and hyperlysinemia can be highly suggestive of this disorder.

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Palabras clave: Acidemia orgánica, Acidemia propiónica, Error innato del metabolismo, Hiperglicininemia, Hiperlisininemia, Inicio neonatal,

Keywords: Hyperglycinemia, Hyperlysinemia, Inborn errors of metabolism, Neonatal onset, Organic acidemia, Propionic acidemia,

Insuficiencia Hepática Crónica y Lesiones Palidales Bilaterales. Chronic Liver Failure And Bilateral Pallidal Lesions.

A 66-year-old woman with history of cirrhosis of unknown etiology was evaluated one month after transient loss of consciousness followed by dystonic posturing, abnormal speech and shuffling gate.

She had been complaining of chronic fatigue, anorexia and weight loos for the past six months, and referred two episodes of non-traumatic nose bleeding in the past two weeks.

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Palabras clave: cirrosis hepática, degeneración hepatocerebral, globo pálido, Parkinsonismo pallidal,

Keywords: Globus pallidus, Hepatocerebral degeneration, Liver cirrhosis, Pallidal Parkinsonism,

Bioética, Neurociencia y Neurología. Bioethics, Neuroscience and Neurology.

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Palabras clave: Bioética, editorial, neurociencia, neurología,

Ictus: Estudios Latinoamericanos Sobre Grado De Conocimientos En La Población. Ictus: Latin American Studies On The Degree Of Knowledge In The Population.

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Palabras clave: carta al editor, Ictus, latinoamericanos.,

Publicar Desde América Latina. ¿En dónde estamos? Publishing From Latin America. Where we are?

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Palabras clave: america latina, carta al editor,

Esclerosis Múltiple, Un Enfoque Pronóstico. Multiple Sclerosis, A Prognostic Approach.

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Palabras clave: carta al editor, esclerosis múltiple,

Salud Cardiovascular en Población Migrante Ecuatoriana en Madrid (España). ¿Debemos Preocuparnos?* Cardiovascular Health in an Ecuadorian Migrant Population to Madrid (Spain). Should we worry?

Background: The Ecuadorian community is the third largest foreign community in Spain. However, little is known about their cardiovascular (CV) health status and whether the effects of migration have caused a detriment in that state. Methods: With the aim of knowing the CV health in an Ecuadorian population of migrants in Madrid, a non-randomized, cross-sectional study was carried out, which included people of Ecuadorian nationality, older than 25 years, living in Madrid for a year or more. Surveys were conducted through the use of previously validated questionnaires, to determine the health status of the population, as well as the degree of neuro-physiological distress. Results: We included 165 participants (68.5% women), with an average age of 49 years. Of these, 86.1% presented poor cardiovascular health and 13.9% intermediate, without significant differences according to sex. There were no individuals that met the 7 variables consistent with and ideal CV status. Women had higher scores on the DASS-21 questionnaire compared to men (p <0.05). Conclusion: In the Ecuadorian migrant population living in Madrid, more than 85% had poor CV health, and none had an ideal CV health. Most prevalent risk factors in this population were poor physical activity, overweight/obesity and poor dietary habits. More studies are required to identify the real situation of CV risk in the Ecuadorian migrant population. Probably, in the medium-long term, it will be necessary to implement health policies especially addressed to migrants.

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Palabras clave: Ecuador, España, Madrid, Población migrante, salud cardiovascular,

Keywords: cardiovascular health, Ecuador, Madrid, Migrant population, Spain,

Intracranial Atherosclerosis And The Earlobe Crease (Frank’s Sign). A Population Study. Aterosclerosis Intracraneal y Pliegue Auricular (Signo De Frank). Estudio Poblacional.

Background: The earlobe crease (ELC) has been linked to coronary artery disease and other vascular conditions, but there is no information on its association with intracranial atherosclerosis. Objective: This study aimed to assess the association between high calcium content in the carotid siphons (as a surrogate of intracranial atherosclerosis) and ELC in communitydwelling adults living in rural Ecuador. Methods: Atahualpa residents aged ≥40 years underwent head CT to estimate calcium content in the carotid siphons, and visual inspection of both earlobes to evaluate the presence of ELC. The association between both variables was assessed by logistic regression models, after adjusting for demographics and cardiovascular risk factors. Results: Of 651 enrolled individuals (mean age: 59.7±12.8 years; 54% women), 225 (35%) had ELC, and 143 (22%) had high calcium content in the carotid siphons. Univariate logistic regression showed a borderline (non-significant) association between high calcium content in the carotid siphons and ELC presence (OR: 1.44; 95% C.I.: 0.99 – 2.12; p=0.057), which disappeared when age (OR: 0.98; 95% C.I.: 0.65 – 1.48; p=0.923) and other covariables (OR: 0.97; 95% C.I.: 0.63 – 1.49; p=0.890) were added to the model. Conclusion: This population study shows no association between high calcium content in the carotid siphons and ELC presence.

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Palabras clave: aterosclerosis intracraneal, calcificaciones de sifón carotídeo, estudio poblacional, Pliegue auricular, proyecto atahualpa,

Keywords: atahualpa project, carotid siphon calcifications, Earlobe crease, intracranial atherosclerosis, population-based study,

Rendimiento Diagnóstico de Minimental Frente Al DSM-5 en Trastorno Cognitivo: Experiencia de una Cohorte en Colombia. Diagnostic Performance Of Minimental Against DSM-5 In Cognitive Disorder. Experience Of A Cohort In Colombia.

Background: Cognitive screening tools are useful in the clinical and research setting. MiniMental (MMSE) is one of the most used instruments in Colombia, the objective of this work is to determine its performance against the new criteria for the diagnosis of cognitive disorder (DSM-5). Materials and methods: Diagnostic test study, assembled in a Colombian cohort, we evaluated a consecutive sample of 200 participants older than 50 years (66.5,+/-8.86) that represented the whole spectrum of the condition of interest, the index test (MMSE) was compared with the clinical reference standard (consensus diagnosis and classification by criteria DSM-5). Results: For mild cognitive impairment (MCI), the diagnostic performance of MMSE was: Sensitivity 45.3% (95% CI 33.7 to 57.4), specificity 96.9% (95% CI 91.4 to 99.0) and 76.9% accuracy (95% CI 69.5 to 82.4) at a cut-off point of 26 and, for major cognitive disorder (MCD) was: Sensitivity 76.32% (95% CI 60.8-87.0) and specificity 97.53% (95% CI 93.8-99.0) at the cut-off point of 24. Conclusions: MMSE is a valid alternative for the diagnosis of MCD, however it has limited validity for the detection of MCI, so new tools for the purpose of screening of MCI should be considered.

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Palabras clave: demencia, MiniMental, MMSE, prueba diagnóstica, Trastorno cognitivo, Trastorno cognitivo leve,

Keywords: Cognitive disorder, dementia, diagnostic test, Mild cognitive disorder, MiniMental, MMSE,

Tasa de Hospitalización Según Comorbilidades en Pacientes con Alzheimer en el Hospital Teodoro Maldonado Carbo. Hospitalization Rate According To Comorbidities In Alzheimer’s Patients At Hospital Teodoro Maldonado Carbo.

Introduction: According to World Health Organization (WHO) 2017 report there are 50 millions of people with dementia worldwide and 60% to 70% of cases belong to Alzheimer’s Disease. The WHO admit that dementia is a public health priority. Aims: Descriptive analysis of patients Alzheimer’s Disease in Guayaquil, Ecuador, focused hospitalization and mortality predictors. Methods: Cross-sectional study. Multivariate and univariate logistic regression analysis to indicate predictors of hospitalization by pneumonia and mortality. Results: 42.57% of the sample was men and the average age was 80.02 years. In a multivariate logistic regression analysis, the most associated comorbidity to increase the risk of pneumonia were Diabetes Mellitus combined with Arterial Hypertension (OR 5.62, 95% CI (1.17 – 26.96) p 0.031), statistically significant. Antipsychotic medication increase the pneumonia risk with a statistically significant meaning (OR 3.03, 95% CI (1.23 – 7.44) p 0.016). Conclusion: Future studies should focus on report the effect of medications and comorbidities on admissions in patients with Alzheimer. Guidelines to focus prevent common causes of hospitalization in these patients should be adapted.

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Palabras clave: Alzheimer; demencia, neumonía, readmisiones hospitalarias.,

Keywords: Alzheimer disease, dementia, patient readmission., pneumonia,

Evaluación de las Habilidades de la Corteza Prefrontal: La Escala Efeco II-VC y II-VR. Evaluation Of The Skills Of The Prefrontal Cortex: The Efeco II-VC And II-VR.

The pre-frontal cortex is the basis of the most complex mental abilities of human development. In its evaluation process, the EFECO scale provides an important contribution to assess its status. In previous investigations this scale has been studied with its configuration of 67 items, narrative focused on the deficit and evaluation of 8 executive functions. This research presents a new version of the scale, centered on its narrative in executive ability, proposed items to assess executive verification function and a summarized version of 42 items. The study included 118 healthy adults between 18 and 25 years of age (Mage = 20.72, SD = 1.65). In the results it was found that the EFECO II-VC scale (modified and complete version) obtained as internal consistency α = .96 and its sub-scales internal consistency between α = .64 and .81. The EFECO II-VR scale (modified and summarized version) obtained α = .94 and its sub-scales between α = .68 and .79. The internal consistency of the factors in which the executive functions are included were adequate: the supervisory system of cognition II-VC α = .93 and II-VR α = .70, while the supervisor system of behavior II- VC α = .93 and II-VR α = .81. The correlations between the executive functions assessed with both scales were between medium and large r = .36 and .94. The work is closed discussing the clinical and scientific contribution of the modification of the EFECO scale.

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Palabras clave: Corteza prefrontal, escala EFECO, evaluación neuropsicológica, Funciones Ejecutivas,

Keywords: EFECO scale, Executive functions, Neuropsychological Evaluation, Prefrontal cortex,

Conocimiento Acerca Del Ictus Isquémico En Ecuatorianos. Knowledge About Ischemic Stroke In Ecuadorian People.

Introduction. The arrival of patients with ischemic stroke to the hospital in the period of the therapeutic window, depends to a great extent on the identification of their clinical signs and the recognition that it is a medical emergency. Methods. A prospective, longitudinal, cross-sectional study was carried out, based on a structured interview with closed questions. The aim was to assess the degree of the population’s knowledge about the ischemic stroke. Results. A total of 135 subjects without a diagnosis of stroke were interviewed, randomly selected from the relatives of patients. The average age was 42.6 years, women predominated (92 / 68.1%), with an average level of education. 95.5% (129 subjects) admitted having little knowledge about stroke. Only 11.1% correctly indicated the clinical manifestations; The most recognized symptom was damping (59.3%) followed by speech disorders. 80.9% (109 respondents) identify stroke as a preventable condition. Less than half of the participants adequately named the risk factors (66 / 48.9%). 88.2% take a correct attitude to the symptoms. Conclusions. In the group of people interviewed there is a perception of risk regarding stroke, but knowledge about the manifestations and vascular risk factors is poor. It is necessary to carry out information campaigns aimed to improve knowledge of t he disease.

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Palabras clave: Conocimiento, Factores de riesgo, Ictus, infarto cerebral, Población.,

Keywords: ischemic stroke, Knowledge, Population., Risk factors, stroke,
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