[Subacute encephalopathy associated with relapsing polychondritis. Report of one case]. / Encefalopatía subaguda en una paciente con policondritis recidivante. Caso clínico.

Relapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.

Electroencefalograma cuantitativo en la predicción precoz de la isquemia cerebral tardía en hemorragia subaracnoidea aneurismática: reporte de un caso / Quantitative electroencephalogram for prediction of delayed ischemia in subarachnoid haemorrhage: report of one case

Subarachnoid hemorrhage (SAH) is a devastating disease, with a mortality rate of 35%. Among patients who survive the initial bleeding, the leading cause of morbidity and mortality is delayed cerebral ischemia (DCI). Electroencephalography (EEG) can detect cerebral ischemia in the early stages. We report a 66-year-old female patient who consulted for ictal headache and impaired consciousness. On admission, she was confused, dysarthric, and with meningeal signs. Brain angio-CT showed SAH FISHER IV and an aneurysm of the left posterior cerebral artery. After excluding the aneurysm (by coiling), the patient recovered the altered consciousness. Continuous EEG monitoring was initiated. On the sixth day of follow up, she had a transient headache and apathy. The brain MRI showed low cerebral blood flow in the left frontotemporal area, without ischemic lesions. On the seventh day, she presented expression aphasia and right facial-brachial paresis. Angiography confirmed severe vasospasm in M1 and M2 segments bilaterally. Pharmacological angioplasty with nimodipine was performed, with an excellent radiological response, although not clinical. A second MRI was carried out on the eighth day, which showed a left insular infarction and generalized vasospasm. A second therapeutic angiography was performed; the patient persisted with aphasia and left central facial paresis. The quantitative EEG analysis performed retrospectively showed a generalized reduction in the spectral edge frequency 95 (SEF95; meaning slowing in the EEG signal) at the fourth day of follow up, three days earlier than the clinical and imaging diagnosis of DCI was established.

Encefalopatía subaguda en una paciente con policondritis recidivante: caso clínico / Subacute encephalopathy associated with relapsing polychondritis: report of one case

Relapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.

[Quantitative electroencephalogram for prediction of delayed ischemia in subarachnoid haemorrhage. Report of one case]. / Electroencefalograma cuantitativo en la predicción precoz de la isquemia cerebral tardía en hemorragia subaracnoidea aneurismática.Reporte de un caso.

Subarachnoid hemorrhage (SAH) is a devastating disease, with a mortality rate of 35%. Among patients who survive the initial bleeding, the leading cause of morbidity and mortality is delayed cerebral ischemia (DCI). Electroencephalography (EEG) can detect cerebral ischemia in the early stages. We report a 66-year-old female patient who consulted for ictal headache and impaired consciousness. On admission, she was confused, dysarthric, and with meningeal signs. Brain angio-CT showed SAH FISHER IV and an aneurysm of the left posterior cerebral artery. After excluding the aneurysm (by coiling), the patient recovered the altered consciousness. Continuous EEG monitoring was initiated. On the sixth day of follow up, she had a transient headache and apathy. The brain MRI showed low cerebral blood flow in the left frontotemporal area, without ischemic lesions. On the seventh day, she presented expression aphasia and right facial-brachial paresis. Angiography confirmed severe vasospasm in M1 and M2 segments bilaterally. Pharmacological angioplasty with nimodipine was performed, with an excellent radiological response, although not clinical. A second MRI was carried out on the eighth day, which showed a left insular infarction and generalized vasospasm. A second therapeutic angiography was performed; the patient persisted with aphasia and left central facial paresis. The quantitative EEG analysis performed retrospectively showed a generalized reduction in the spectral edge frequency 95 (SEF95; meaning slowing in the EEG signal) at the fourth day of follow up, three days earlier than the clinical and imaging diagnosis of DCI was established.

Relationship between infarct size and serum uric acid levels during the acute phase of stroke.

INTRODUCTION: Uric acid has gained considerable attention as a potential neuroprotective agent in stroke during the last decades, however, its role in the pathophysiology of ischemic stroke remains poorly understood. A serial evaluation of uric acid levels during the acute phase of stroke and its association with infarct size on magnetic resonance imaging is lacking. METHODS: We present a cohort study of 31 patients with ischemic stroke who were not candidates for thrombolysis according to current criteria at the time. We performed daily measurements of serum uric acid and total antioxidant capacity of plasma during the first week after symptoms onset and 30 days after. Infarct size was determined in the acute phase by a DWI sequence and the final infarct size with a control MRI (FLAIR) at day 30. RESULTS: Uric acid significantly decreases between days 2 to 6 compared to day 1, after adjustment by sex, age and DWI at diagnosis, with a nadir value at 72h. A mixed model analysis showed a negative association between DWI at diagnosis and uric acid evolution during the first week after stroke. Moreover, multivariable linear regression of uric acid values during follow up on DWI volumes demonstrated that DWI volume at diagnosis is negatively associated with uric acid levels at day 3 and 4. There were no significant associations between total antioxidant capacity of plasma and DWI at diagnosis, or FLAIR at any point. DISCUSSION: Patients with larger infarcts exhibited a significant decrease in serum uric acid levels, accounting for a more prominent reactive oxygen species scavenging activity with subsequent consumption and decay of this antioxidant. The different kinetics of total antioxidant capacity of plasma and serum uric acid levels suggests a specific role of uric acid in the antioxidant response in ischemic stroke.

[Emergency endovascular management of ischemic stroke. Experience in 104 patients]. / Experiencia en el tratamiento endovascular del accidente cerebrovascular isquémico agudo en un centro chileno.

BACKGROUND: Recently, five randomized controlled trials confirmed the efficacy and safety of endovascular treatment with or without intravenous thrombolysis in acute ischemic stroke with large-vessel occlusion. AIM: To report patients with ischemic stroke treated with endovascular methods. MATERIAL AND METHODS: Retrospective analysis of 104 patients aged 61 ± 15 years (54% males) with ischemic stroke who received endovascular treatment at a single medical center between 2009 and 2017. RESULTS: Sixty one percent were treated with intravenous thrombolysis plus endovascular procedures and 39% with endovascular procedures alone. The median door-to needle time was 61 minutes and door-to femoral puncture was 135 minutes. The median National Institute of Health Stroke Scale (NIHSS) scores on admission, 24 hours later and at discharge were 12,4 and 1 points, respectively. Middle cerebral artery was occluded in 60% of cases. Other frequent localizations where distal carotid artery in 17% and vertebro-basilar artery in 14%. Thrombolysis in cerebral infarction (TICI) scale flow score after the procedure was 3 or 2b in 58% of cases and significantly correlated with NIHSS scores at 24 hours and discharge. Fifty percent of patients had a mRankin score < = 1 and ten patients died (9.6%). Eight percent had a symptomatic intracerebral hemorrhage. CONCLUSIONS: The clinical improvement of these patients 24 hours after the procedure and at discharge demonstrate the effectiveness of endovascular treatment in ischemic stroke. The presence neurologists able to interpret multimodal images at the emergency room, the use of local guidelines, the availability of an experienced neuro-interventional team engaged with the workflow and the use of stent retrievers are strongly associated with good outcomes.

Experiencia en el tratamiento endovascular del accidente cerebrovascular isquémico agudo en un centro chileno / Emergency endovascular management of ischemic stroke: experience in 104 patients

Background: Recently, five randomized controlled trials confirmed the efficacy and safety of endovascular treatment with or without intravenous thrombolysis in acute ischemic stroke with large-vessel occlusion. Aim: To report patients with ischemic stroke treated with endovascular methods. Material and Methods: Retrospective analysis of 104 patients aged 61 ± 15 years (54% males) with ischemic stroke who received endovascular treatment at a single medical center between 2009 and 2017. Results: Sixty one percent were treated with intravenous thrombolysis plus endovascular procedures and 39% with endovascular procedures alone. The median door-to needle time was 61 minutes and door-to femoral puncture was 135 minutes. The median National Institute of Health Stroke Scale (NIHSS) scores on admission, 24 hours later and at discharge were 12,4 and 1 points, respectively. Middle cerebral artery was occluded in 60% of cases. Other frequent localizations where distal carotid artery in 17% and vertebro-basilar artery in 14%. Thrombolysis in cerebral infarction (TICI) scale flow score after the procedure was 3 or 2b in 58% of cases and significantly correlated with NIHSS scores at 24 hours and discharge. Fifty percent of patients had a mRankin score < = 1 and ten patients died (9.6%). Eight percent had a symptomatic intracerebral hemorrhage. Conclusions: The clinical improvement of these patients 24 hours after the procedure and at discharge demonstrate the effectiveness of endovascular treatment in ischemic stroke. The presence neurologists able to interpret multimodal images at the emergency room, the use of local guidelines, the availability of an experienced neuro-interventional team engaged with the workflow and the use of stent retrievers are strongly associated with good outcomes.

Oxidative stress and pathophysiology of ischemic stroke: novel therapeutic opportunities.

Stroke is the second leading cause of death, after ischemic heart disease, and accounts for 9% of deaths worldwide. According to the World Health Organization [WHO], 15 million people suffer stroke worldwide each year. Of these, more than 6 million die and another 5 million are permanently disabled. Reactive oxygen species [ROS] have been implicated in brain injury after ischemic stroke. There is evidence that a rapid increase in the production of ROS immediately after acute ischemic stroke rapidly overwhelm antioxidant defences, causing further tissue damage. These ROS can damage cellular macromolecules leading to autophagy, apoptosis, and necrosis. Moreover, the rapid restoration of blood flow increases the level of tissue oxygenation and accountsfor a second burst of ROS generation, which leads to reperfusion injury. Current measures to protect the brain against severe stroke damage are insufficient. Thus, it is critical to investigate antioxidant strategies that lead to the diminution of oxidative injury. The antioxidant vitamins C and E, the polyphenol resveratrol, the xanthine oxidase [XO] inhibitor allopurinol, and other antioxidant strategies have been reviewed in the setting of strokes. This review focuses on the mechanisms involved in ROS generation, the role of oxidative stress in the pathogenesis of ischemic stroke, and the novel therapeutic strategies to be tested to reduce the cerebral damage related to both ischemia and reperfusion.

Delayed recurrent stroke in a young patient with acute posterior multifocal placoid pigment epitheliopathy.

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a chorioretinal disease that causes acute binocular visual disturbance with characteristic funduscopic lesions at the level of the retinal pigment epithelium. APMPPE has been associated with multiple neurologic complications, including cerebrovascular diseases. We report a 15-year-old patient who had bilateral APMPPE, which was successfully treated with corticosteroids. One year later he presented with transient dysarthria and right hemiparesis. Brain magnetic resonance imaging (MRI) showed bilateral ischemic lesions in both lenticular nuclei and corona radiata. Brain MRI performed 3 months later revealed a new asymptomatic ischemic lesion. Cerebral angiography showed diffuse multifocal segmental vessel narrowing. The cerebrospinal fluid showed mononuclear pleocytosis in keeping with vasculitis. We started corticosteroid treatment, which lasted 10 months. Currently, after 2 years of clinical and neuroradiologic follow-up, the patient is asymptomatic and shows no worsening of the cerebrovascular lesions.

Incidence of lobar and non-lobar spontaneous intracerebral haemorrhage in a predominantly Hispanic-Mestizo population--the PISCIS stroke project: a community-based prospective study in Iquique, Chile.

BACKGROUND: The incidence of intracerebral haemorrhage (ICH) in Hispanics is high, especially of non-lobar ICH. Our aim was to ascertain prospectively the incidence of first-ever spontaneous ICH (SICH) stratified by localisation in a Hispanic-Mestizo population of the north of Chile. METHODS: Between July 2000 and June 2002 all possible cases of ICH were ascertained from multiple overlapping sources. The cases were allocated according to localisation. Those with vascular malformations or non-identifiable localisations were excluded. RESULTS: We identified a total of 69 cases of first-ever ICH. Of these, 64 (92.7%) had SICH, of which we allocated 58 cases (84%) to non-lobar or lobar localisation. The mean age was 57.3 +/- 17 years, and 62.3% of the subjects were male. The age-adjusted incidence rates were 13.8 (non-lobar) and 4.9 (lobar) per 100,000 person-years. Non-lobar SICH was more frequent in young males and lobar SICH in older women. The non-lobar-to-lobar ratio was similar to previous findings in Hispanics. Hypertension was more frequent in non-lobar SICH and in diabetes, heavy drinking and antithrombotic use in lobar SICH, but in none significantly. There was no association between localisation and prognosis. CONCLUSIONS: The incidence of non-lobar SICH was high, but lower than in most non-white populations. This lower incidence could be due to a lower population prevalence of risk factors, a higher socioeconomic level in this population, or chance.

Incidence, case-fatality rate, and prognosis of ischaemic stroke subtypes in a predominantly Hispanic-Mestizo population in Iquique, Chile (PISCIS project): a community-based incidence study.

BACKGROUND: Incidence of ischaemic stroke subtypes, classified by cause, seems to vary between communities. We aimed to prospectively ascertain the incidence of first-ever ischaemic stroke in a predominantly Hispanic-Mestizo population in the northern desertic region of Chile. METHODS: Between July, 2000, and June, 2002, all patients with possible stroke or transient ischaemic attacks were identified from multiple overlapping sources and were rapidly assessed by two field neurologists. All identified patients were diagnosed by at least two stroke neurologists according to Trial of Org 10172 in Acute Stroke Treatment (TOAST) definitions and were followed up at 6 months. Annual incidence rates were age adjusted to WHO, European, and US populations by the direct method to allow comparisons. FINDINGS: A total of 239 ischaemic strokes were identified, of which 185 (77%) were first-ever cases. 151 (82%) patients were hospitalised, of whom only 70 (38%) were assessed within 6 h of symptom onset. The mean age of patients was 66.4 years (SD 14.9) and 56% were men. The crude annual incidence rates (per 100 000) according to stroke subtype were: cardioembolic, 9.3; large-artery disease, 2.0; small-vessel disease, 15.8; other determined cause, 0.2; and undetermined cause, 17.4. Hypertension was the most common cardiovascular risk factor in all subtypes and atrial fibrillation was the most common cause of cardioembolic stroke. Case fatality at 30 days was highest in cardioembolic strokes (28%) and lowest in small-vessel disease (0%). Dependency or death at 6 months was also highest in cardioembolic strokes (62%) and lowest in small-vessel disease (21%). INTERPRETATION: Incidence and prognosis of small vessel and cardioembolic infarction was similar to that in other populations and incidence of large-artery atherothrombotic infarction was lower than in most previous reports. Hypertension and atrial fibrillation were the most common risk factor and cause, respectively, of ischemic stroke in this population. These findings should help the national stroke programme in the prevention of cardioembolic stroke, increase access to specialists and acute brain imaging and vascular studies, and improve stroke care.

Evento cerebro vascular isquémico / Ischemic vascular brain event

Las enfermedades vasculares cerebrales son la tercera causa de muerte en el mundo occidental y el ictus isquémico sigue siendo una causa importante de morbilidad y mortalidad, pues representa un tercio de las muertes en Norteamérica. Los pacientes con mayor riesgo de desarrollar un Evento Cerebro Vascular Isquémico (ECVI) son aquellos que presentan fibrilación auricular, estenosis arterial sintomática de una de las arterias carótidas internas o vertebrales, amaurosis fugaz u otros eventos isquémicos. La tomografía computada de cerebro inicial resulta normal en un tercio de los pacientes. Existe evidencia de que el ingreso a unidades especializadas en el manejo del ictus disminuye su riesgo de muerte, incapacidad y cuidados crónicos institucionalizados. A pesar de todo, muchos pacientes fallecen en muerte cerebral. Aunque no existen estudios clínicos sobre la mejor forma de proteger la vía aérea en pacientes con ECVI, cuando hay compromiso de conciencia e incapacidad de protegerla se utiliza de preferencia la intubación endotraqueal. Se recomienda ser prudente en el manejo de la HTA en la fase aguda del ictus isquémico. La administración intravenosa o intraarterial de agentes trombolíticos puede lograr la re-permeabilización del vaso ocluido y mejorar el resultado neurológico de los pacientes con un infarto cerebral. Sólo el rt-PA tiene clara evidencia de su beneficio. Se revisan el tratamiento del EVCI y de sus complicaciones.

Brain vascular diseases are the third leading cause of death in the Western world, while ischemic ictus continues to be a major cause of morbimortality, accounting for one third of all deaths in North America. Patients with a higher risk of developing an Ischemic Cerebrovascular Event (ICE) are those presenting auricular fibrillation, symptomatic arterial stenosis in one of the vertebral or internal carotids, amaurosis fugax or other ischemic events. Initial brain computed tomography is found to be normal in a third of all patients. It has been evidenced that timely admission to Centers specialized in ictus treatment reduces death risk rates, handicaps, and residential health care for chronically ill patients. Nevertheless, many patients die from brain death. Despite the non-existence of clinical studies on how to best protect aerial pathway in ICE patients, when there is consciousness compromise and no possibility of protecting it, endotracheal intubation is performed preferably. During acute stage of ischemic ictus a sensible management of AHT is recommended. Intravenous or intraarterial administration of thrombolytic agents may produce repermeabilization of the occluded vessel and improve neurological outcome in patients with brain infarction. Only rt-PA has proved to be clearly beneficial. Management of ICE and its complications are revised.

Incidence, 30-day case-fatality rate, and prognosis of stroke in Iquique, Chile: a 2-year community-based prospective study (PISCIS project).

BACKGROUND: The epidemiology of stroke in Latin-American populations and variation of subtypes between communities are unclear. Our aim was to ascertain prospectively the incidence of first-ever stroke in the predominantly Hispanic-Mestizo population of Iquique, a city in the northern desert region of Chile. METHODS: We prospectively identified all possible cases of stroke and transient ischaemic attacks between July 1, 2000, and June 30, 2002, from several overlapping sources. Patients were rapidly assessed by two field neurologists. Standard definitions for incident cases, stroke, transient ischaemic attack, pathological type, and infarction subtype were used. All cases identified were adjudicated by at least two stroke neurologists and followed up at 6 months. Incidence rates of first-ever strokes were calculated from the population of Iquique (214 526) according to the national census of 2002. FINDINGS: Of 380 cases of stroke identified, 292 were incident. CT scans were done in 267 (91%) patients and the mean time to scan was 2.2 days. The hospital admission rate was 71% (207/292). The overall age-adjusted incidence rate of first-ever stroke was 140.1 per 100,000 (95% CI 124.0-156.2). The incidence rates per 100,000 according to pathological type were: infarcts 87.3, intracerebral haemorrhage 27.6, and subarachnoid haemorrhage 6.2. The 30 day and 6-month case-fatality rates were 23.3% and 33.0%, respectively. INTERPRETATION: Our results show incidence rates of stroke similar to those reported in other community studies. Although the proportion of intracerebral haemorrhages was higher than reported in previous studies, the overall incidence was not, which could indicate a slightly lower incidence of ischaemic strokes in this population than in other countries. The prognosis was similar to that found in other population-based studies.