A los diecisiete años desaparecen del EEG las polipuntas y polipunta ondas: a propósito del síndrome de Lance-Adams / At the seventeen years they disappear from the EEG The little and little waves: About the Lance-Adams syndrome

Objetivo: la inesperada ausencia del signo de polipunta o de polipunta onda (PPO) en el electroencefalograma (EEG) de dos casos, de 29 y 51 años, respectivamente, del Síndrome Lance Adams (SLA), que hemos visto y publicado con anterioridad, nos motivó a investigar la edad cuando este signo tiende a extinguirse, dejando de ser obligatorio para diagnosticar SLA a pacientes de edad madura. Métodos: de una muestra de 7137 trazados se incluyeron 6939, tras excluir 198 por referimientos no identificables. Estos EEG del Centro de Rehabilitación y el Centro Médico de la Universidad Central del Este (UCE), fueron realizados con electroencefalógrafos Nervus y Cadwell, de manera respectiva. Se revisaron buscando la presencia de PPO para estudiar la edad, la patología sospechada en cada indicación y el género de los pacientes. Resultados: PPO fue encontrada en 293 casos: 4.22 % de la muestra total. En 272 habría ocurrido antes de los 17 años, con la gráfica mostrando una elevación inicial máxima a las nueve. En cambio, de los 18 a los 65 solo apareció la PPO en 18 casos. 14 pacientes mostraron supresión de paroxismo o patrones de electro depresión sin PPO. Conclusión: la polipunta/polipunta onda prácticamente desaparece a los 17 años, a mayor edad, por lo tanto, el signo PPO deja de ser obligatorio para el diagnóstico del SLA en pacientes mayores. Es más frecuente en epilépticos y en varones

Objective: Unexpected absence of polyspike/polyspike & wave sign (PPW) in the EEG of our 29 and 51-year old published cases of Lance Adams Syndrome (LAS), prompted us to investigate the age when this sign may nearly disappear. This in order of considering it not obligatory for late LAS diagnosis. Methods: Inclusion consisted of a sample of 7137 recordings performed at the Rehabilitation Center and the Universidad Central del Este's (UCE) teaching clinic (Nervus and Cadwell respectively). Excluded were 198 for unidentifiable indications. PPW was searched in order to study patient's age, suspected pathology plus gender. Results: PPW was found in 293cases: 4.22% of the total sample: in 275.it occurred before the age of 17 with curve showing an initial tip at nine while between 18 and 65 only 18 cases showed it. Incidentally 14 patients showed Burst suppression or electro-decrement patterns without PPW. The most common indication pathology was Epilepsy followed by learning disability. Finally it was more frequent in 176(63.9%) male vs. 113(36.1%) in female cases: ratio 1.56 to 1. however not significant for the gender ratio in the total EEG sample is 1.55 to 1. Conclusion: PPW nearly disappears after the age of seventeen, perhaps explaining rarity in advanced aged SLA. It is more frequent in Epilepsy EEG indications and in the male

Estado de coma y patrón electroencefalográfico de brote-supresión por sobredosis de Baclofeno / Coma state and the electroencephalogram burst-suppression pattern related to baclofen overdose

El baclofeno es un análogo estructural del ácido gamma-aminobutírico empleado principalmente como relajante del músculo esquelético para el tratamiento de espasticidad y distonías. Las manifestaciones clínicas de intoxicación por este fármaco, se deben a alteración del nivel de conciencia que produce somnolencia, estado de coma e incluso la muerte. Desde el punto de vista electroencefalográfico, se ha descrito en estos casos, el llamado patrón de brote- supresión. Se presenta el caso de una mujer que súbitamente presentó dificultad para la bipedestación y mutismo acinético y, posteriormente, evolucionó a un estado de coma sin causa aparente. Una evaluación minuciosa en el Servicio de Emergencia, incluyó electroencefalograma urgente que resultó compatible con un patrón continuo de brote-supresión. Ante la sospecha de una intoxicación por baclofeno, la paciente fue tratada con diálisis, la cual revirtió el cuadro clínico y normalizó la actividad electroencefalográfica en un periodo de cinco días.

Baclofen is a structural analog of gamma-aminobutyric acid, primarily used as a skeletal muscle relaxant for treatment of spasticity and dystonia. The clinical picture of baclofen intoxication includes reduction or loss of consciousness and manifestations such as sleepness, coma or even death. Electroencephalograms performed in these cases have consistently shown the so-called burst-suppression pattern. The case of a female patient who suddenly presented difficulty to still-standing and mutism and later evolved into coma, is presented. A detailed study was performed in the emergency department, including an electroencephalogram compatible with a continuous burst-suppression pattern. The patient was treated with dialysis in the face of a possible baclofen intoxication and, in a period of five days, the clinical symptoms reverted and the electroencephalographic activity was normalized.

Caracterización clínica, bioquímica e imagenológica en una cohorte de pacientes diagnosticados con hiperglicinemia no cetósica clásica: estudio ambispectivo 2000-2014, Medellín, Colombia / Clinical, biochemical and imaging characterization in a cohort of patients diagnosed with classical nonketotic hyperglycinemia: ambispective study 2000-2014, Medellin-Colombia

Introducción: La hiperglicinemia no cetósica (HGNC) es un error innato del metabolismo del grupo de las aminoacidopatías, de carácter autosómico recesivo, causado por un defecto en el sistema de clivaje de la glicina. Es una entidad rara y no se conoce su incidencia en Colombia. Objetivo: Describir características clínicas, bioquímica e imagenológicas en una cohorte de pacientes diagnosticados con hiperglicinemia no cetósica clásica Materiales y métodos: Estudio de tipo descriptivo, ambispectivo, en el periodo enero 2000-2014, en varios centros de Medellín. Resultados: Se incluyeron 20 pacientes que cumplían criterios de inclusión, de los 35 pacientes que cumplían con el criterio de búsqueda, en su mayoría de sexo femenino y con un Apgar adecuado al nacer. El promedio de inicio de los síntomas fue de 2,6 días; somnolencia, hipoactividad, apnea, convulsiones y singulto fueron los principales síntomas, y las convulsiones de tipo focal las más frecuentes. La relación glicina LCR/plasma en promedio fue 0,42. El patrón estallido-supresión en el electroencefalograma y la ausencia o retraso en la mielinización de la sustancia blanca supratentorial en la resonancia magnética fueron hallazgos comunes. Conclusión: La HGNC es frecuente en nuestro medio, por lo cual es necesario que se disponga de pruebas bioquímicas y moleculares necesarias para diagnóstico oportuno, manejo integral y asesoría genética.

Introduction: Nonketotic Hyperglycinemia is an inborn error of metabolism in a group of aminoacidopathies, autosomal recessive, caused by a defect in the system of the glycine cleavage. It is rare, and the incidence is unknown in Colombia. Objective:To describe clinical, biochemical and imaging characteristics in a cohort of patients diagnosed with classical nonketotic hyperglycinemia. Materials and methods: This is a descriptive-ambispective study during the period January 2000 - 2014 in some centers of Medellin. Results: There were 35 patients who met the search criteria and finally 20 patients who met inclusion criteria. We found in this cohort more girls than boys, and most of them with a good APGAR. The average onset of symptoms was 2.6 days, with drowsiness, hypoactivity, apnea, seizures and singultus the main symptoms. The focal seizures were the most frequent type. The average value of CSF glycine to plasma glycine ratio was 0.42. The burst suppression pattern in the EEG and the absence or delayed myelination in the supratentorial white matter on MRI were common findings. All patients received dextromethorphan as part of their treatment and the vast majority of sodium benzoate. Conclusion: HGNC is common in our environment. It´s necessary to have available biochemical and molecular evidence for timely diagnosis, comprehensive management and genetic counseling.

Síndrome de Ohtahara asociado con fístula traqueoesofágica en ''H'' / Ohtahara syndrome associated with H-type tracheoesophageal fistula

Introducción. El síndrome de Ohtahara es una encefalopatía epiléptica infantil temprana, caracterizada por espasmos tónicos frecuentes, crisis parciales y, ocasionalmente, mioclonías. El electroencefalograma interictal muestra un patrón característico de descargas de puntas que alternan con fases de supresión de la actividad eléctrica cerebral (brote-supresión). Las manifestaciones clínicas inician habitualmente antes de los 20 días de vida extrauterina. Los pocos casos reportados en la literatura no mencionan malformaciones congénitas asociadas. Caso clínico. Documentamos el primer caso clínico de un lactante masculino de 6 meses de edad con síndrome de Ohtahara asociado con una fístula traqueoesofágica tipo ''H''. Conclusiones. La asociación del síndrome de Ohtahara con la fístula traqueoesofágica pareciera deberse a una presentación fortuita y no con relación al síndrome neurológico.

Background. Ohtahara syndrome is an early infantile epileptic encephalopathy characterized by frequent tonic spasms, partial seizures and occasional myoclonus. Interictal EEG characteristically shows a pattern of burst of spikes alternating with phases of suppression of brain electrical activity (''burst-suppression''). Clinical manifestations usually begin before 20 days of life. The few cases reported in the literature do not mention associated congenital malformations. Case report. We report the first case of a 6-month-old male infant with Ohtahara syndrome associated with H-type tracheoesophageal fistula. Conclusions. The association between Ohtahara syndrome and tracheoesophageal fistula may be due to a fortuitous presentation without any relationship with the neurological syndrome.

Clinical Efficacy of Acute Monitoring Cortical Activity Using Subdural Strip Electrode after Decompressive Craniectomy / 대한신경손상학회지

OBJECTIVE: Decompressive craniectomy is widely used in cases of uncontrolled intracranial hypertension, including traumatic brain injury or acute stroke. Physiological monitorings, such as intracranial pressure or electroenecephalography (EEG) are critical for patients in the acute phase. We retrospectively reviewed our experience of continuous electrocorticography (ECoG) monitoring by subdural strip electrode in patients who performed decompressive craniectomy and assessed its clinical efficacy. METHODS: Patients who underwent decompressive craniectomy because of severe intracranial hypertension were included. 4 Channel strip electrodes were inserted on the frontal cortex before closure. 24-hour continuous monitoring of ECoG was done to identify abnormal electrical activity. The level of consciousness was assessed according to Glasgow Coma Scale (GCS). In patients with malignant intracranial hypertension, barbiturate coma therapy was considered. RESULTS: Fifteen patients (9 men and 6 women) were included and the mean age was 55.7 years (from 17 to 80). The initial mean GCS score was 7.9 (from 3 to 14). In six out of fifteen patients, abnormal spike activities were identified, and one of these six patients was diagnosed as nonconvulsive status epilepticus (NCSE). Cortical spreading depression (CSD) was suspected in five. Three patients underwent barbiturate coma therapy and ECoG monitoring of these patients showed typical burst suppression pattern, which was used for indicator of therapeutic level. The mean duration of strip electrode and ECoG monitoring was 3.5 days, and there was no complication. CONCLUSION: Continuous ECoG monitoring using subdural strip electrode was useful to detect abnormal brain activity in the acute period after decompressive craniectomy.

Reporte de casos de trastorno cognitivo postoperatorio en pacientes operados de prostatectomía radical laparoscópica / Report of cases of postoperative cognitive disorder in patients operated on laparoscopic radical prostatectomy

El presente reporte sugiere la relación entre el trastorno cognitivo postoperatorio y la tasa de supresión de electroencefalografía por índice biespectral en los pacientes operados de prostatectomia radical laparoscópica.

This report suggests the relationship between postoperative cognitive disorder and electroencephalography suppression rates by bispectral index in patients undergoing laparoscopic radical prostatectomy

Comparative Study of Brain Protection Effect between Thiopental and Etomidate Using Bispectral Index during Temporary Arterial Occlusion

OBJECTIVE: This study was conducted to compare the effect of etomidate with that of thiopental on brain protection during temporary vessel occlusion, which was measured by burst suppression rate (BSR) with the Bispectral Index (BIS) monitor. METHODS: Temporary parent artery occlusion was performed in forty one patients during cerebral aneurysm surgery. They were randomly assigned to one of two groups. General anesthesia was induced and maintained with 1.5-2.5 vol% sevoflurane and 50% N2O. The pharmacological burst suppression (BS) was induced by a bolus injection of thiopental (5 mg/kg, group T) or etomidate (0.3 mg/kg, group E) according to randomization prior to surgery. After administration of drugs, the hemodynamic variables, the onset time of BS, the numerical values of BIS and BSR were recorded at every minutes. RESULTS: There were no significant differences of the demographics, the BIS numbers and the hemodynamic variables prior to injection of drugs. The durations of burst suppression in group E (11.1+/-6.8 min) were not statistically different from that of group T (11.1+/-5.6 min) and nearly same pattern of burst suppression were shown in both groups. More phenylephrine was required to maintain normal blood pressure in the group T. CONCLUSION: Thiopental and etomidate have same duration and a similar magnitude of burst suppression with conventional doses during temporary arterial occlusion. These findings suggest that additional administration of either drug is needed to ensure the BS when the temporary occlusion time exceed more than 11 minutes. Etomidate can be a safer substitute for thiopental in aneurysm surgery.

The Etiology and Outcome Analysis of Neonatal Burst Suppression EEG / 华中科技大学学报（医学）（英德文版）

The neonatal burst suppression is a severe EEG pattern and always demonstrates serious damage of nerve system. But the outcome of these patients depends on the different etiology. A total of 256 cases of video EEG recordings were analyzed in order to summarize the etiology and outcome of burst suppression. The results showed that some patients in all 17 cases of burst suppression showed EEG improvement. The etiology was the dominant factor in long term outcome. It was sug-gested that effective video EEG monitoring is helpful for etiologic study and prognosis evaluation.

The Effect of Barbiturate in Brain Protection under Mild Hypothermia During Temporary Vessel Occlusion in Aneurysm Surgery

Temporary vessel occulusion is a useful technique which facilitates aneurysmal dissection and clipping, and reduces the risk of intraoperative aneurysal rupture; It may, however, result in postoperative stroke, and many authors have therefore used various agents for brain protection. Among these, barbiturate is well known. This study was undertaken to retrospectively review the effect of barbiturate on brain protection during short periods of ischemia. It involved patients with 146 anterior circulation aneurysms, who were divided into two groups: 43 who received intraoperative barbiturate burst suppression with sodium thiopental during temporary vessel occlusion and the remaining 103, who did not. Doses of thiopental 5mg/kg, usually singly or occasionally repeated at 10-mimute interval, were administered. All patients underwent general anesthesia using volatile isoflurane; mild hypothermia(32-34degreesC), mild hypocarbia(30-35mmHg), and normotension of 110-130mmHg were noted prior to permanent clipping, and mild hypertension(130-150mmHg) after clipping. In some cases, intraoperative scalp electroencep-halography was monitored. Clinical, radiological, and overall infarction were assessed postoperatively, and statistical analysis was perfomed. With regard to Hunt-Hess grades, number of episodes of occlusion, duration of temporary clip application lasting less than 13 minutes, and period of post-subarachnoid hemorrhage, no significant differences were seen between the two groups. Patients in whom barbiturate burst was suppressed showed a 4-hours delay in mean anesthetic recovery time, and after thiopental sodium was injected intravenously, systolic and mean arterial pressure(MAP), averaging 10-20mmHg, were lowered than in those without this suppression. In conclusion, we had found that under mild hypothermic anesthesia involving less than 13 minutes of temporary vessel occlusion, protection of the brain by barbiturate was not beneficial.