Asymptomatic cerebral small vessel disease in adults with low cardiovascular risk.

Introduction: Cerebral small vessel disease (CSVD) frequently occurs in individuals with vascular risk factors. This condition might go unrecognised or result in only mild functional deficits. Objective: To evaluate the relationship between cardiovascular (CV) risk calculated with the HEARTS app and CSVD burden in a population without cardio-cerebrovascular diseases, and to estimate the prevalence of CSVD in low risk (LR) individuals. Methods: Asymptomatic subjects with vascular risk factors were included from primary health areas in Havana. The WHO's revised CV disease risk prediction chart (HEARTS app) was applied to all individuals, who were classified into two groups: LR and moderate/high risk (M/HR). Brain MRI was performed in all subjects. Results: 170 patients were included: 43 (25.3%) classified as low CV risk and 127 (74.7%) had M/HR CV risk. Half of the neurologically healthy individuals included displayed cerebral small vessel involvement (51.2%). White matter hyperintensities (WMH) and enlarged perivascular spaces were the most frequent lesions observed in both groups. WMH were more severe and more severe global score for CSVD were more frequent in the M/HR group (57.5%). It was noteworthy that 32.6% of LR-patients also exhibited more severe CSVD. The multivariate regression analysis revealed an independent association of arterial hypertension and age with the severity of CSVD. Conclusions: CV risk stratification through the HEARTS app has limited utility for predicting brain health in individuals with low CV risk. Identifying silent CSVD in individuals with apparently low CV risk is important, especially if they suffer from arterial hypertension.

Impacto psicosocial del desarrollo científico-técnico en el estudio de la epilepsia

Introducción: Se ha planteado que no existe otra enfermedad asociada a tantos problemas sociales como lo es la epilepsia. Objetivo: Describir el impacto social del desarrollo científico-técnico en el estudio de la epilepsia. Método: Se realizó una revisión narrativa a partir del estudio documental de varias fuentes bibliográficas encontradas en base de datos electrónicas. Los principales criterios de búsqueda fueron: artículos publicados en los últimos 10 años sobre impacto psicosocial del desarrollo científico-técnico en el estudio de la epilepsia. Desarrollo: El efecto sobrenatural atribuido a la epilepsia, con su consecuente repercusión social, es resultado de especulaciones provocadas por siglos de falsas creencias sobre esta enfermedad. El impacto social de la enfermedad es negativo, genera problemas financieros, de aislamiento, de exclusión social y discriminación. Se plantea que la epilepsia tiene gran influencia en todos los niveles de calidad de vida. Las anomalías detectadas por las novedosas técnicas de estudio por neuroimagen en la epilepsia se han relacionado con el deterioro cognitivo, refractariedad de la enfermedad y otros hallazgos que pueden estar relacionados indirectamente con las alteraciones psicosociales de los pacientes. Consideraciones finales: La epilepsia, además del daño orgánico, genera consecuencias psicosociales negativas que limitan el desempeño saludable de los enfermos. En los últimos años los adelantos científico-técnicos han limitado de forma parcial los efectos sociales negativos de la enfermedad con la incorporación de novedosas tecnologías para su estudio y tratamiento.

Introduction: Views has been expressed that there is no disease more linked with social problems than epilepsy. Objective: To describe the social impact of scientific and technological development on the study of epilepsy. Method: A narrative review was carried out supported on the documentary research of several bibliographic sources found in electronic databases. The main search criteria were as follow: articles published in the last 10 years, which had relation with aspects concerning the psychosocial impact of scientific and technological development on the study of epilepsy. Development: The supernatural effect attributes to epilepsy, including its social repercussions, is the result of centuries of speculative theories and false beliefs about this disease. Epilepsy has negative impact on social well-being, causing serious economic problems, isolation, social exclusion and discrimination. Epilepsy is described as a disease with a great influence on all levels of quality of life. The abnormalities detected using novel neuroimaging techniques referred to the presence of cognitive impairment, refractory period and other aspects which may be indirectly related to psychosocial alterations in patients. Final considerations: Epilepsy, in addition to its traumatic effects, has negative psychosocial consequences that affect the healthy performance of patients. In recent years, the scientific and technological advancements have partially limited the negative social effects causes by this disease with the use of new technologies for its study and treatment.

Introdução: Tem sido sugerido que não há outra doença associada a tantos problemas sociais quanto a epilepsia. Objetivo: Descrever o impacto social do desenvolvimento científico-técnico no estudo da epilepsia. Método: Realizou-se revisão narrativa a partir do estudo documental de diversas fontes bibliográficas encontradas em bases de dados eletrônicas. Os principais critérios de busca foram: artigos publicados nos últimos 10 anos sobre o impacto psicossocial do desenvolvimento científico-técnico no estudo da epilepsia. Desenvolvimento: O efeito sobrenatural atribuído à epilepsia, com sua consequente repercussão social, é fruto de especulações causadas por séculos de falsas crenças sobre essa doença. O impacto social da doença é negativo, gera problemas financeiros, isolamento, exclusão social e discriminação. Sugere-se que a epilepsia tenha grande influência em todos os níveis da qualidade de vida. As anormalidades detectadas pelas novas técnicas de estudo de neuroimagem na epilepsia têm sido relacionadas à deterioração cognitiva, refratariedade da doença e outros achados que podem estar indiretamente relacionados às alterações psicossociais dos pacientes. Considerações finais: A epilepsia, além dos danos orgânicos, gera consequências psicossociais negativas que limitam o desempenho saudável dos pacientes. Nos últimos anos, os avanços técnico-científicos limitaram parcialmente os efeitos sociais negativos da doença com a incorporação de novas tecnologias para seu estudo e tratamento.

Surgical Outcome in Extratemporal Epilepsies Based on Multimodal Pre-Surgical Evaluation and Sequential Intraoperative Electrocorticography.

OBJECTIVE: to present the postsurgical outcome of extratemporal epilepsy (ExTLE) patients submitted to preoperative multimodal evaluation and intraoperative sequential electrocorticography (ECoG). SUBJECTS AND METHODS: thirty-four pharmaco-resistant patients with lesional and non-lesional ExTLE underwent comprehensive pre-surgical evaluation including multimodal neuroimaging such as ictal and interictal perfusion single photon emission computed tomography (SPECT) scans, subtraction of ictal and interictal SPECT co-registered with magnetic resonance imaging (SISCOM) and electroencephalography (EEG) source imaging (ESI) of ictal epileptic activity. Surgical procedures were tailored by sequential intraoperative ECoG, and absolute spike frequency (ASF) was calculated in the pre- and post-resection ECoG. Postoperative clinical outcome assessment for each patient was carried out one year after surgery using Engel scores. RESULTS: frontal and occipital resection were the most common surgical techniques applied. In addition, surgical resection encroaching upon eloquent cortex was accomplished in 41% of the ExTLE patients. Pre-surgical magnetic resonance imaging (MRI) did not indicate a distinct lesion in 47% of the cases. In the latter number of subjects, SISCOM and ESI of ictal epileptic activity made it possible to estimate the epileptogenic zone. After one- year follow up, 55.8% of the patients was categorized as Engel class I-II. In this study, there was no difference in the clinical outcome between lesional and non lesional ExTLE patients. About 43.7% of patients without lesion were also seizure- free, p = 0.15 (Fischer exact test). Patients with satisfactory seizure outcome showed lower absolute spike frequency in the pre-resection intraoperative ECoG than those with unsatisfactory seizure outcome, (Mann- Whitney U test, p = 0.005). CONCLUSIONS: this study has shown that multimodal pre-surgical evaluation based, particularly, on data from SISCOM and ESI alongside sequential intraoperative ECoG, allow seizure control to be achieved in patients with pharmacoresistant ExTLE epilepsy.

CSF oligoclonal band frequency in a Cuban cohort of patients with multiple sclerosis. comparison with Latin American countries and association with latitude.

BACKGROUND: The diagnostic sensitivity of CSF specific oligoclonal bands (OCBs) in multiple sclerosis (MS), using state of the art methods, has been clearly established to be over 95% in patients with a predominantly Caucasian background. This is not the case for other geographical regions, where reports of OCB prevalence can be much lower, and a relationship between OCB frequency and latitude has been suggested. OBJECTIVE: The aim of the present study was to assess the frequency of OCBs in a cohort of MS patients evaluated at the Institute of Neurology and Neurosurgery (Havana, Cuba), and to review the scientific literature in order to investigate the possible relationship between OCB status and latitude in the region of Latin America. METHODS: Fifty-three patients (47 with definite MS and 6 with clinically isolated syndrome - CIS) were included. Isoelectric focusing (IEF) with IgG immunoblotting for OCB analyses, was performed placing paired CSF and serum samples in the same analytical run. PubMed, Scielo and Google Scholar were searched for papers containing information concerning CSF OCB status (employing isoelectric focusing with IgG immunoblotting) in patients with definite MS in Latin America and the Caribbean. RESULTS: In Cuban patients with definite MS, an OCB prevalence of 87% was observed, while the frequency in CIS patients was lower (67%). The prevailing pattern was that of OCBs restricted to the CSF (type 2), which was observed in 71% of definite MS patients and in all CIS patients with intrathecal IgG synthesis. OCB prevalence was slightly lower, but very close to that reported in Caucasian populations. Comparison with other Latin American countries revealed a significant correlation between OCB prevalence and latitude. CONCLUSIONS: A prevalence of CSF restricted OCBs of 87% was observed in definite MS patients, a frequency which was slightly lower, but similar to that reported in Caucasian populations. The analysis of OCB frequency in Latin American countries revealed a possible relationship between OCB prevalence and latitude, but this must be further investigated in more countries and larger samples of patients.

Cerebral Small Vessel Disease Associated with Subclinical Vascular Damage Indicators in Asymptomatic Hypertensive Patients.

Background: Cerebral small vessel disease (CSVD) is frequent in patients with cardiovascular risk factors including arterial hypertension, and it is associated with vascular damage in other organs and the risk of stroke, cognitive impairment, and dementia. Early diagnosis of CSVD could prevent deleterious consequences. Objective: To characterize CSVD associated with indicators of subclinical vascular damage in asymptomatic hypertensive patients. Materials and Methods: Participants were hypertensive (HT) and non-hypertensive (non-HT) individuals; without signs of cerebrovascular disease, dementia, and chronic renal failure. For CSVD, white matter hyperintensities (WMH), enlarged Virchow-Robin perivascular spaces (EVRPS), lacunar infarcts, and microbleeds were investigated. Subclinical vascular damage was evaluated (hypertensive retinopathy, microalbuminuria, and extracranial carotid morphology: intima media thickness (IMT) and atheroma plaque). Results: CSVD MRI findings were more frequent in HT; as well as greater intimal thickening. The IMT + plaque was significantly associated with all MRI variables; but retinopathy was correlated with EVRPS and lacunar infarcts. Only microalbuminuria was related to the greater severity of WMH in HT. Multivariate analysis evidenced that CSVD was independently associated with the combination of indicators of vascular damage and systolic blood pressure. Conclusions: Combining indicators of subclinical vascular damage, such as carotid morphological variables, microalbuminuria, and hypertensive retinopathy for early detection of CSVD in asymptomatic hypertensive patients could prove to be useful to take actions for the prevention of irreversible brain damage, which could lead to cognitive impairment, dementia and stroke.

Spontaneous Subdural Hematoma and Behavioral Changes Due to a Dural Arteriovenous Fistula. A Case Report and Literature Review.

Dural arteriovenous fistulas (DAVFs) represent 10-15% of intracranial arteriovenous malformations. Of these, only 12-29% cause intracranial hemorrhage. The presentation of DAVF as a subdural hematoma (SDH) and intraparenchymal hemorrhage (IPH) is infrequent; additionally, behavioral changes are not common among these patients. We report, for the first time in our country, the case of a 23-year-old man with no history of head injury, in which a brain computed tomography (CT) scan revealed SDH and IPH with behavioral disturbances. The angiotomography showed ecstatic venous vessels, indicating the presence of a DAVF, which was later confirmed by cerebral angiography. Endovascular therapy, which followed the clinical diagnosis, resulted in satisfactory evolution two years after treatment. A review of the literature concerning cases with DAVF and behavioral disturbances is presented. DAVF may lead to cognitive impairment, behavioral changes, and dementia as a result of diffuse white matter and thalamus modifications related to venous ischemia, and it should be considered as a reversible cause of vascular dementia.

Serum neuron specific enolase could predict subclinical brain damage and the subsequent occurrence of brain related vascular events during follow up in essential hypertension.

The object of this work was to explore if blood based biomarkers of brain damage could predict subclinical brain lesions and clinical outcome during follow-up in asymptomatic hypertensive patients. This was a cross-sectional study including 101 patients with essential hypertension and no clinical evidence of neurological disease and 53 healthy controls, followed by a longitudinal study of 62 hypertensive patients for an average of 33 months. Serum concentrations of two brain specific proteins (S100B and neuron specific enolase - NSE) were determined at inclusion. Fundoscopic exploration, brain MRI and echocardiographic studies were also performed. Clinical outcome at follow-up was registered: transient ischemic attack (TIA), stroke, vascular headache or migraine, cardiovascular events and death. Higher serum NSE and S100B concentrations were observed in hypertensive patients; and multiple regression analysis revealed independent associations of clinical variables and more severe white matter lesions only with NSE concentration. A panel combining two clinical variables (blood pressure>140/90 and years of hypertension>10) and serum NSE>13 µg/L predicted more severe white matter lesions with 80% sensitivity and 94.4% specificity. Higher NSE levels at inclusion were associated not only with the occurrence of vascular events related with the CNS (stroke, TIA and vascular headache), but also with an earlier presentation of these events during the follow-up period. Serum NSE concentration could be a useful biomarker to predict subclinical brain damage and future vascular events related with the CNS in hypertension. Blood based biomarkers could aid in filtering hypertensive patients with a higher risk of cerebrovascular disease for brain MRI scanning.

Enfermedad de hirayama en un adolescente / Hirayama disease observed in an adolescent

La Enfermedad de Hirayama -o amiotrofia monomélica- es una afección de baja frecuencia y escasamente reportada en la edad pediátrica. Se presenta el caso clínico de un adolescente de 15 años de edad con disminución de la fuerza muscular y pérdida de la masa muscular, que comenzó a los 10 años de edad por la mano izquierda, y le afectó posteriormente el antebrazo. Mantuvo un curso progresivo durante 3 años, para luego mantenerse estable. El electromiograma de aguja arrojó lesión de axones motores o motoneuronas dependientes de los miotomas C7-T1, y en menor grado, C5-C6. En la tomografía axial computarizada con contraste endovenoso en marcada flexión cervical, se observó desde C7-T2 una evidente ectasia venosa posmedular asimétrica, predominantemente del lado izquierdo, por congestión del plexo venoso vertebral posterior interno. En este paciente la enfermedad se detuvo espontáneamente, en otros casos es necesario limitar la motilidad de la columna con el uso de un collar cervical, y solo llegar a la cirugía en los casos más severos de evolución rápida.

Hirayama disease or monomelic amyotrophy is a low frequent, barely reported illness at pediatric ages. Here is the clinical case of 15 years-old boy that presented with reduced muscular strength and loss of muscle mass; this condition began at the age of 10 year in his left hand and then affected the forearm. The illness progressed for three years and then remained stable. The needle electromyogram showed a lesion in C7-T1 myotome-depending motor axons or motoneurons and to less extent in those C5-C6 depending ones. The venous contrast computed tomography on a marked cervical cord flexion position; it was observed an evident asymmetric postmedullary vein ectasia from the C7-T2 myotomes, mainly on the left side, caused by the internal posterior vertebral vein plexus congestion. There was spontaneous remission of the disease in this patient, but it is necessary in other cases to limit the cervical cord motility with the use of a collar and to only perform surgery in the most rapidly evolving and severe cases.

Association of status redox with demographic, clinical and imaging parameters in patients with Huntington's disease.

UNLABELLED: Huntington's disease (HD) is an autosomal dominant, progressive neurodegenerative disorder, caused by an expanded trinucleotide CAG sequence of the huntingtin (Htt) gene, which encodes a stretch of glutamines in the Htt protein. The mechanisms of neurodegeneration associated with the accumulation of Htt aggregates still remains unclear. OBJECTIVES: To determine oxidative stress biomarkers in HD patients and their relationship with clinical, demographic and neuroimaging parameters. DESIGN AND METHODS: Fourteen patients and 39 controls paired by age and sex participated in this study. Oxidative damage was assayed in blood by measuring malondialdehyde (MDA) and advanced oxidative protein products (AOPPs). Antioxidant status was determined by activities of superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPx), glutathione reductase (GR), reduced glutathione (GSH), protein thiols and total antioxidant capacity (FRAP). The Unified Huntington Disease Rating Scale (UHDRS) and neuroimaging studies were also employed. RESULTS: MDA, AOPP and GPx were significantly increased in HD patients with respect to the control group, while GR activity was decreased. FRAP correlated with age of disease onset, AOPP with motor severity (UHDRS score), age of patients and age of disease onset. Caudate atrophy was associated with lower plasma concentrations of GSH. CONCLUSIONS: These findings point to a redox imbalance in HD patients. GR activity could be a potential biomarker for symptom onset in asymptomatic gene carriers, while plasmatic GSH could be useful in monitoring the progression of neurodegeneration - as an expression of caudate atrophy - during the course of the disease.

Tratamiento multimodal e individualizado a pacientes con metástasis encefálica / Multimodal and individualized treatment for patients with brain metastases

Introduction: The treatment of Brain Metastases is palliative and it is ignored the ideal therapeutic combination, constituting this a controversial topic. Objective: To determine the results of the treatment to patient with Brain Metastases according to different modalities. Method: It was carried out a prospective cohort study between april 2010 to april 2013 in the National Institute of Oncology and Radiobiology. It was designated as universe the total of patient with diagnosis of Brain Metastases of primary tumor assisted in consultation of Neurosurgery. The Fischer exact test was used to correlate qualitative variables and the Kaplan Meier curvesto estimate the survival. Results: It was obtained a sample of 42 patients and 84 lesions. The age average was of 53,1 years. The primary lesions prevailed in lung and breast. The used modalities were: Surgery-Whole Brain Radiation Therapy (20 lesions), Whole Brain Radiation Therapy alone (29 lesions), Radiosurgery alone (21 lesions) and Surgery alone (9 lesions). Conclusions: The combinations of Radiosurgery-Whole Brain Radiation Therapy and Surgery-Radiosurgery associated to a better local control. The treatment with Whole Brain Radiation Therapy didn't associate to a better distance control. The overall survival to the 6 months was of 91 percent and to the 12 months of 57 percent. A bigger survival was observed in patient with controlled primary illness and absence of extracraneal metastases. The Surgery presents the biggest number of complications.

Introducción: El tratamiento de la Metástasis Encefálica es paliativo y se desconoce la modalidad o combinación terapéutica óptima, constituyendo éste un tema controvertido. Objetivo: Determinar los resultados del tratamiento a pacientes con Metástasis Encefálica según diferentes modalidades. Método: Se realizó un estudio de cohorte prospectivo durante el período abril de 2010 a abril de 2013 en el Instituto Nacional de Oncología y Radiobiología. Se designó como universo el total de pacientes con diagnóstico de Metástasis Encefálica de neoplasia primaria conocida atendidos en consulta de Neurocirugía. Se utilizó el test exacto de Fischer para correlacionar variables cualitativas y las curvas de Kaplan Meier para estimar la supervivencia. Resultados: Se obtuvo una muestra de 42 pacientes y 84 lesiones. El promedio de edad fue de 53,1 años. Predominaron las lesiones primarias en pulmón y mama. Las modalidades más utilizadas fueron: Cirugía-Radioterapia Holocraneal (20 lesiones), Radioterapia Holocraneal sola (29 lesiones), Radiocirugía sola (21 lesiones) y la Cirugía sola (9 lesiones). Conclusiones: Las combinaciones de Radiocirugía-Radioterapia Holocraneal y Cirugía-Radiocirugía se asociaron a un mejor control local. El tratamiento con Radioterapia Holocraneal no se asoció a un mejor control a distancia. La supervivencia global a los 6 meses fue del 91 por ciento y a los 12 meses del 57 por ciento. Se observó una mayor supervivencia en pacientes con enfermedad primaria controlada y ausencia de metástasis extra craneal. La Cirugía presentó el mayor número de complicaciones.

Short-term prognostic value of serum neuron specific enolase and S100B in acute stroke patients.

OBJECTIVE: To explore the value of blood markers for brain injury as outcome predictors in acute stroke. DESIGN AND METHODS: The study included 61 patients with acute stroke (44 ischemic and 17 hemorrhagic) and a high risk control group (79 individuals with no known history of neurological disease). Serum neuron specific enolase (NSE) and S100B were determined by immunoassay (CanAg Diagnostics, Sweden). Outcome at 60 days was evaluated with clinical scales. RESULTS: Higher concentrations of NSE and S100B were measured in patients compared to high risk controls, but they were not related to stroke severity on admission. NSE was associated with functional neurological outcome at 60 days and to the degree of recovery, whereas S100B exhibited a strong correlation with depression symptoms at 60 days. CONCLUSIONS: The measurements of serum concentrations of NSE and S100B after acute stroke may be clinically relevant for predicting functional neurological outcome and post-stroke depression, respectively.