Epidemiology of ataxia and hereditary spastic paraplegia in Spain: A cross-sectional study.

INTRODUCTION: Ataxia and hereditary spastic paraplegia are rare neurodegenerative syndromes. We aimed to determine the prevalence of these disorders in Spain in 2019. PATIENTS AND METHODS: We conducted a cross-sectional, multicentre, retrospective, descriptive study of patients with ataxia and hereditary spastic paraplegia in Spain between March 2018 and December 2019. RESULTS: We gathered data from a total of 1933 patients from 11 autonomous communities, provided by 47 neurologists or geneticists. Mean (SD) age in our sample was 53.64 (20.51) years; 938 patients were men (48.5%) and 995 were women (51.5%). The genetic defect was unidentified in 920 patients (47.6%). A total of 1371 patients (70.9%) had ataxia and 562 (29.1%) had hereditary spastic paraplegia. Prevalence rates for ataxia and hereditary spastic paraplegia were estimated at 5.48 and 2.24 cases per 100 000 population, respectively. The most frequent type of dominant ataxia in our sample was SCA3, and the most frequent recessive ataxia was Friedreich ataxia. The most frequent type of dominant hereditary spastic paraplegia in our sample was SPG4, and the most frequent recessive type was SPG7. CONCLUSIONS: In our sample, the estimated prevalence of ataxia and hereditary spastic paraplegia was 7.73 cases per 100 000 population. This rate is similar to those reported for other countries. Genetic diagnosis was not available in 47.6% of cases. Despite these limitations, our study provides useful data for estimating the necessary healthcare resources for these patients, raising awareness of these diseases, determining the most frequent causal mutations for local screening programmes, and promoting the development of clinical trials.

Recurrent bilateral asynchronous cellulitis: Report of two cases and a literature review.

Bilateral recurrent periorbital cellulitis is a very rare condition, with only five cases having been reported. Two cases are presented of recurrent bilateral asynchronous eyelid oedema in two paediatric patients. It was associated with respiratory infections, with no systemic disease. The patients had recurrent episodes (three times) since nine months old. They received oral and intravenous antibiotics, with a good response. There were no complications and no surgery was needed.

Celulitis recurrente bilateral asincrónica: a propósito de 2 casos y revisión de la bibliografía / Recurrent bilateral asynchronous cellulitis: Report of two cases and a literature review

La celulitis recurrente bilateral es una enfermedad muy poco frecuente, con tan solo 5 casos descritos en la literatura. Se describen 2 casos de edema palpebral recurrente bilateral asincrónico en 2 pacientes pediátricos asociados a infección respiratoria de vías altas, sin otra enfermedad asociada. Desde los 9 meses de edad padecieron recurrencias de la celulitis, hasta 3 veces, en ambos ojos. La antibioterapia oral e intravenosa resolvió cada proceso, sin desarrollar complicaciones ni necesidad de cirugía.

Bilateral recurrent periorbital cellulitis is a very rare condition, with only five cases having been reported. Two cases are presented of recurrent bilateral asynchronous eyelid oedema in two paediatric patients. It was associated with respiratory infections, with no systemic disease. The patients had recurrent episodes (three times) since nine months old. They received oral and intravenous antibiotics, with a good response. There were no complications and no surgery was needed.

Clinical and molecular characterization of hereditary spastic paraplegia in a spanish Southern region.

INTRODUCTION: Spastic paraplegia (SPG) is a syndrome characterised by lower limb spasticity, occurring alone or in association with other neurological manifestations. Despite of the new molecular technologies, many patients remain yet undiagnosed. OBJECTIVE: The purpose of this study was to describe the clinical presentation and molecular characteristics of a cohort of 27 patients from 18 different families with SPG in the south of Spain. METHODS: We used a targeted next-generation sequencing (NGS) approach to study a proband from each family. RESULTS: Variants in SPG11 gene were the most common cause of SPG in our area. We made a genetic diagnosis in 52% of cases, identified 3 novel variants and reclassified one uncertain variant in SPG11 gene as pathogenic variant. We identified a patient with two truncanting mutations in SPG11 gene and late onset disease and report another missense mutation outside of motor domain of KIF1A gene in a family with pure SPG. CONCLUSION: Our study contributes to enhance the scientific knowledge of SPG. It is important to note the large group of cases (48%) that were not genetically diagnosed in our cohort. Therefore NGS approach is an efficient diagnostic tool, but it still large the number of non-diagnosed subjects, suggesting further genetic heterogeneity.

Epidemiology of ataxia and hereditary spastic paraplegia in Spain: a cross-sectional study. / Mapa epidemiológico transversal de las ataxias y paraparesias espásticas hereditarias en España.

INTRODUCTION: Ataxia and hereditary spastic paraplegia are rare neurodegenerative syndromes. We aimed to determine the prevalence of these disorders in Spain in 2019. PATIENTS AND METHODS: We conducted a cross-sectional, multicentre, retrospective, descriptive study of patients with ataxia and hereditary spastic paraplegia in Spain between March 2018 and December 2019. RESULTS: We gathered data from a total of 1.809 patients from 11 autonomous communities, provided by 47 neurologists or geneticists. Mean (SD) age in our sample was 53.64 (20.51) years; 920 patients were men (50.8%) and 889 were women (49.2%). The genetic defect was unidentified in 920 patients (47.6%). A total of 1371 patients (70.9%) had ataxia and 562 (29.1%) had hereditary spastic paraplegia. Prevalence rates for ataxia and hereditary spastic paraplegia were estimated at 5.48 and 2.24 cases per 100 000 population, respectively. The most frequent type of dominant ataxia in our sample was SCA3, and the most frequent recessive ataxia was Friedreich ataxia. The most frequent type of dominant hereditary spastic paraplegia in our sample was SPG4, and the most frequent recessive type was SPG7. CONCLUSIONS: In our sample, the estimated prevalence of ataxia and hereditary spastic paraplegia was 7.73 cases per 100 000 population. This rate is similar to those reported for other countries. Genetic diagnosis was not available in 47.6% of cases. Despite these limitations, our study provides useful data for estimating the necessary healthcare resources for these patients, raising awareness of these diseases, determining the most frequent causal mutations for local screening programmes, and promoting the development of clinical trials.

Recurrent bilateral asynchronous cellulitis: Report of two cases and a literature review. / Celulitis recurrente bilateral asincrónica: a propósito de 2 casos y revisión de la bibliografía.

Bilateral recurrent periorbital cellulitis is a very rare condition, with only five cases having been reported. Two cases are presented of recurrent bilateral asynchronous eyelid oedema in two paediatric patients. It was associated with respiratory infections, with no systemic disease. The patients had recurrent episodes (three times) since nine months old. They received oral and intravenous antibiotics, with a good response. There were no complications and no surgery was needed.

Repercusión de la introducción de neurología en un hospital comarcal de Andalucía / Impact of introducing neurology into a local hospital in Andalusia

Introducción: En Andalucía la mortalidad por ictus duplica la de otras autonomías lo que podría relacionarse con que, a diferencia del resto de CC. AA., sólo 3 de 17 hospitales comarcales cuentan con neurología. El objetivo de este estudio fue evaluar la repercusión de la introducción de un neurólogo en un hospital comarcal de Andalucía en la evaluación y evolución de pacientes con ictus.Métodos: Se realizó un estudio observacional de los ictus ingresados en el primer trimestre de 2006. Se analizaron indicadores de calidad, mortalidad y dependencia comparando los pacientes atendidos por neurología o medicina general. Resultados: En el primer trimestre de 2006 ingresaron 116 pacientes con ictus. Se detectaron diferencias significativas en la realización de las pruebas complementarias necesarias para el diagnóstico (Doppler, ecocardio). La estancia media fue significativamente menor en neurología. Se detectó una reducción absoluta de 39,1% de mortalidad y un incremento absoluto del 35,7% de pacientes independientes al final del seguimiento. Los únicos factores que reducen de forma independiente la estancia y la mortalidad son la atención por neurología durante el ingreso y una menor discapacidad al ingreso.Conclusión: La atención especializada por neurología es eficaz y coste-efectiva por la reducción en estancia hospitalaria, mortalidad y dependencia. La implantación de neurología en los hospitales comarcales de Andalucía es prioritaria para garantizar la equidad en el territorio nacional. Este objetivo debería incluirse en el Plan Andaluz de Atención al Ictus como paso previo para constituir una red de equipos/unidades de ictus (AU)

Introduction: The stroke mortality rate in Andalusia is twice that of other autonomous communities. This could be associated with the absence of neurologists in most local hospitals in this community, unlike in the rest of Spain. The objective of this study was to evaluate the impact of incorporating a neurologist to evaluate and monitor stroke patients in a local hospital in Andalusia. Methods: An observational study was conducted on stroke cases admitted in the first quarter of 2006. Quality indicators, mortality rates, and incapacity rates at follow-up were analysed, comparing groups with and without neurological care. Results: A total of 116 stroke patients were admitted. There were significant differences in tests performed to diagnose patients (Doppler and echocardiography). The mean hospital stay was significantly lower with neurology care. There was a 39.1% absolute decrease in mortality and a 35.7% absolute increase in capacity for daily life activities was also observed. Neurological care and a lower incapacity level at admission were the only two factors independently associated with a decrease in length of hospital stay and mortality.Conclusion: Specialised care by a neurologist is effective in reducing length of hospital stay, mortality and incapacity. The incorporation of neurologists in local hospitals in Andalusia should be a priority to guarantee equal care in all autonomous communities in Spain. This objective should be included in the Plan Andaluz de Atención al Ictus as a first step in forming a network of stroke units and teams (AU)

Fiabilidad del diagnóstico de ictus en urgencias / Reliability of stroke diagnosis in emergency departments

Introducción: Tanto en el Plan de atención sanitaria al ictus como en la Estrategia en ictus del Sistema Nacional de Salud se establece que solo se realizará fibrinólisis en los hospitales con unidad de ictus. Sin embargo, en Andalucía el Plan andaluz de atención al ictus incluye dentro de la cartera de servicios del equipo de ictus en los hospitales comarcales la realización de fibrinólisis prescindiendo de la figura del neurólogo en el tratamiento agudo del ictus. El objetivo de este estudio es evaluar si los médicos de urgencias realizan adecuadamente el diagnóstico de ictus. Métodos: Se recogieron los diagnósticos realizados en urgencias y al alta de hospitalización de todos los pacientes ingresados por motivos neurológicos en el hospital comarcal Infanta Elena durante el año 2006. Se analizó la validez del diagnóstico realizado en urgencias. Resultados: El número de ingresos por patología neurológica fue de 655 pacientes en 2006, representando el ictus un 76%. Aunque la sensibilidad del diagnóstico de ictus en urgencias resultó alta (95%), la especificidad del diagnóstico era muy baja (52%) y el valor predictivo positivo de un 75%. Conclusiones: Aplicar la fibrinólisis sobre la base del diagnóstico de ictus en urgencias implica someter a un riesgo no justificado de hemorragia cerebral a un número importante de pacientes diagnosticados erróneamente. Este riesgo hace cuestionable éticamente la administración de dicho tratamiento en los hospitales comarcales en las condiciones actuales. No solo es importante que el tratamiento fibrinolítico sea accesible a la población sino que dicho tratamiento se realice de forma segura por neurólogos en unidades de ictus (AU)

Introduction: According to the Spanish Stroke Health Care Plan and the Spanish Health National Service Stroke Strategy, thrombolysis should only be performed in hospitals with Stroke Units. However, the Andalusian Stroke Health Care Plan includes, within the list of services of the Stroke Team, the need to have a neurologist present for the performing of thrombolysis in local hospitals. The objective of this study is to evaluate whether emergency doctors are able to achieve a reliable diagnosis of stroke in order to safely perform thrombolysis. Methods: The diagnoses on hospital admission and discharge of all patients admitted for neurological reasons in 2006 in the community Hospital Infanta Elena (Huelva, Andalusia) were collected. The reliability of diagnosis performed by emergency doctors was analysed.Results: A total of 655 patients were admitted to the hospital for neurological reasons, and 76% of them were diagnosed as strokes. The sensitivity of stroke diagnosis made by emergency doctors was very high (97%), but specificity and positive predictive value of that diagnosis was low (52% and 75%, respectively). Conclusions: To apply thrombolysis based of the diagnosis of a stroke by emergency doctors may subject a significant number of erroneously diagnosed patients to an unnecessary risk of brain haemorrhage. This risk makes performing thrombolysis in community hospitals ethically questionable in these circumstances. Although it is important to have thrombolytic treatment available to everyone, this treatment must be performed safely by neurologists Stroke Units (AU)

Impact of introducing neurology into a local hospital in Andalusia.

INTRODUCTION: The stroke mortality rate in Andalusia is twice that of other autonomous communities. This could be associated with the absence of neurologists in most local hospitals in this community, unlike in the rest of Spain. The objective of this study was to evaluate the impact of incorporating a neurologist to evaluate and monitor stroke patients in a local hospital in Andalusia. METHODS: An observational study was conducted on stroke cases admitted in the first quarter of 2006. Quality indicators, mortality rates, and incapacity rates at follow-up were analysed, comparing groups with and without neurological care. RESULTS: A total of 116 stroke patients were admitted. There were significant differences in tests performed to diagnose patients (Doppler and echocardiography). The mean hospital stay was significantly lower with neurology care. There was a 39.1% absolute decrease in mortality and a 35.7% absolute increase in capacity for daily life activities was also observed. Neurological care and a lower incapacity level at admission were the only two factors independently associated with a decrease in length of hospital stay and mortality CONCLUSION: Specialised care by a neurologist is effective in reducing length of hospital stay, mortality and incapacity. The incorporation of neurologists in local hospitals in Andalusia should be a priority to guarantee equal care in all autonomous communities in Spain. This objective should be included in the Plan Andaluz de Atención al Ictus as a first step in forming a network of stroke units and teams.

[Reliability of stroke diagnosis in emergency departments]. / Fiabilidad del diagnóstico de ictus en urgencias.

INTRODUCTION: According to the Spanish Stroke Health Care Plan and the Spanish Health National Service Stroke Strategy, thrombolysis should only be performed in hospitals with Stroke Units. However, the Andalusian Stroke Health Care Plan includes, within the list of services of the Stroke Team, the need to have a neurologist present for the performing of thrombolysis in local hospitals. The objective of this study is to evaluate whether emergency doctors are able to achieve a reliable diagnosis of stroke in order to safely perform thrombolysis. METHODS: The diagnoses on hospital admission and discharge of all patients admitted for neurological reasons in 2006 in the community Hospital Infanta Elena (Huelva, Andalusia) were collected. The reliability of diagnosis performed by emergency doctors was analysed. RESULTS: A total of 655 patients were admitted to the hospital for neurological reasons, and 76% of them were diagnosed as strokes. The sensitivity of stroke diagnosis made by emergency doctors was very high (97%), but specificity and positive predictive value of that diagnosis was low (52% and 75%, respectively). CONCLUSIONS: To apply thrombolysis based of the diagnosis of a stroke by emergency doctors may subject a significant number of erroneously diagnosed patients to an unnecessary risk of brain haemorrhage. This risk makes performing thrombolysis in community hospitals ethically questionable in these circumstances. Although it is important to have thrombolytic treatment available to everyone, this treatment must be performed safely by neurologists Stroke Units.

[Outcome of intraarterial procedures in acute ischemic stroke]. / Resultados del intervencionismo en el ictus isquémico agudo.

INTRODUCTION: In addition to the thrombolytic agents, mechanical devices are being used in intraarterial procedures in acute stroke patients. The intervention protocol and outcomes of intraarterial procedures performed in the Hospital Virgen del Rocío in 2003-2004 are described. METHODS: The results in each arterial occlusion site and case-control study of the middle cerebral artery (MCA), the placebo group in PROACT II study (Prolyse in Acute Cerebral Thromboembolism II) as historic control group, are described. RESULTS: A total of 32 procedures were performed: 18 MCA occlusions, 8 basilar thrombosis and 6 intracranial carotid occlusions. Urokinase, abciximab and mechanical devices were used in 60%, 26% and 60% of the cases, respectively. MCA occlusions: 60% of the cases improved in 24 hours (25% remained asymptomatic). Only 25% controls remained independent versus 55% of the treated patients (p=0.001). Recanalization and complete recanalization rates were 95 % and 45 %, respectively (18% and 2% in the control group). Symptomatic intracranial hemorrhage (SICH) rate was 18% vs 4% in cases and controls. Basilar thrombosis: only one patient died and 57% of the remaining patients were independent. Carotid occlusions: no complete recanalization was achieved and only one patient was independent. Evolution time in SICH patients was longer (p=0.04), with increased international normalized ratio (INR) for thromboplastin time (p=0.06) and old ischemic lesions in brain CT scan (p=0.02) and they were more dependent at three months (p=0.01). CONCLUSIONS: Intraarterial procedures in MCA occlusions achieve high rates of recanalization and better clinical outcomes than those described in isolated intraarterial thrombolysis. Long-term outcomes in basilar thrombosis were also excellent. Nevertheless, the natural evolution of carotid occlusions was not modified.

[Financial analysis of intraarterial procedures in acute stroke]. / Análisis económico del intervencionismo en el ictus agudo.

INTRODUCTION: Since the most decisive factor related to the cost of stroke is disability, any acute phase intervention to reduce disability will probably be cost-effective. The present acute stroke phase analysis is the first cost-benefit study on intraarterial procedures in acute stroke phase. METHOD: Case-control study focusing on the cost of stroke including acute stroke patients subjected to interventionism in Hospital Virgen del Rocío in 2003-2004 was conducted. The data obtained was subsequently extrapolated to the number of patients who would have been treated if intraarterial procedures could be performed on a 24 hour-day basis. RESULTS: 32 patients were treated in 2003-2004. Direct cost (devices and human resources) of each treated patient was 10,502 euros and global saving in hospital stay and rehabilitation was 11,069 euros, resulting in 567 euros net saving per patient. Nevertheless, 92 patients would have been treated if intraarterial procedures could have been performed on a 24 hour-day basis, resulting in better financial results with 5,792 saving for each treated patient. CONCLUSIONS: Intracraneal procedures in acute stroke has been shown to be cost-effective since cost of material and human resources is greatly exceeded by the saving in hospitalization and rehabilitation as a result of reduction in patient disability.

Análisis económico del intervencionismo en el ictus agudo / Financial analysis of intraarterial procedures in acute stroke

Introducción. El determinante más importante del coste del ictus es el estado de discapacidad, por lo que cualquier intervención en fase aguda que reduzca la discapacidad será probablemente coste-efectiva. El presente análisis es el primer estudio de coste-beneficio del intervencionismo en fase aguda del ictus. Métodos. Se ha realizado un estudio de casos y controles de costes de los pacientes con ictus agudo tratados mediante intervencionismo en el Hospital Virgen del Rocío en los años 2003-2004 y posteriormente se han extrapolado los datos obtenidos al supuesto del número real de pacientes que habrían sido tratados si hubiera estado organizado el intervencionismo como atención continua. Resultados. En los años 2003-2004 se han tratado a 32 pacientes. El coste directo (material fungible + recursos humanos) por cada paciente tratado fue de 10.502 euros y el ahorro global en estancia hospitalaria más rehabilitación resultó de 11.069 euros por paciente, resultando, por tanto, en un ahorro neto de 567 euros por paciente. Sin embargo, si el intervencionismo hubiese estado organizado de forma continua se habrían tratado a 92 pacientes, resultando la evaluación económica más favorable con un ahorro de 5.792 euros por cada paciente tratado. Conclusiones. El tratamiento intervencionista ha demostrado ser coste-efectivo, ya que el gasto derivado del material y los recursos humanos necesarios es superado con creces por el ahorro en tiempos de hospitalización y rehabilitación que se deriva de la reducción de la discapacidad de los pacientes

Introduction. Since the most decisive factor related to the cost of stroke is disability, any acute phase intervention to reduce disability will probably be cost-effective. The present acute stroke phase analysis is the first cost-benefit study on intraarterial procedures in acute stroke phase. Method. Case-control study focusing on the cost of stroke including acute stroke patients subjected to interventionism in Hospital Virgen del Rocío in 2003-2004 was conducted. The data obtained was subsequently extrapolated to the number of patients who would have been treated if intraarterial procedures could be performed on a 24 hour-day basis. Results. 32 patients were treated in 2003-2004. Direct cost (devices and human resources) of each treated patient was 10,502 euros and global saving in hospital stay and rehabilitation was 11,069 euros, resulting in 567 euros net saving per patient. Nevertheless, 92 patients would have been treated if intraarterial procedures could have been performed on a 24 hour-day basis, resulting in better financial results with 5,792 saving for each treated patient. Conclusions. Intracraneal procedures in acute stroke has been shown to be cost-effective since cost of material and human resources is greatly exceeded by the saving in hospitalization and rehabilitation as a result of reduction in patient disability

Resultados del intervencionismo en el ictus isquémico agudo / Outcome of intraarterial procedures in acute ischemic stroke

Introducción. Además de los agentes trombolíticos, se están empleando dispositivos mecánicos en el intervencionismo en pacientes con ictus agudo. Se describe el protocolo y resultados del intervencionismo en el Hospital Virgen del Rocío durante los años 2003-2004. Métodos. Descripción de resultados en cada tipo de oclusión y estudio caso-control de oclusiones de arteria cerebral media (ACM) tratadas empleando como control histórico el grupo placebo del estudio PROACT II (Prolyse in Acute Cerebral Thromboembolism II). Resultados. Se realizaron 32 procedimientos: 18 oclusiones ACM, 8 trombosis basilares y 6 carótidas intracraneales. Se empleó urocinasa, abciximab y disrupción mecánica en un 60, 26 y 60% de pacientes, respectivamente. Oclusiones ACM: el 60% mejoraron en 24 h (25% quedaron asintomáticos). Sólo el 25% de controles quedaron independientes frente a un 55 % de los tratados (p=0,001). La tasa de recanalización y recanalización completa fue del 95 y 45%, respectivamente (18 y 2% en el grupo control). La tasa de hemorragia intracraneal sintomática (HICS) fue del 18% en el grupo tratado y del 4% en el control. Trombosis basilares: un paciente falleció y el 57% de los restantes alcanzó la independencia. Oclusiones carotídeas: en ningún caso se consiguió la recanalización completa y sólo alcanzó la independencia un paciente. Los pacientes con HICS tenían un tiempo de evolución más prolongado (p=0,04), un international normalized ratio (INR) mayor para el tiempo de tromboplastina (p=0,06) e infartos antiguos en la tomografía computarizada cerebral (p=0,02) y quedaban más dependientes al tercer mes (p=0,01). Conclusiones. El intervencionismo en oclusiones de ACM consigue altas tasas de recanalización y mejores resultados clínicos que los descritos para la fibrinólisis intraarterial aislada. Los resultados a largo plazo en las trombosis basilares fueron también excelentes. Sin embargo, no se modificó la evolución natural de las oclusiones carotídeas

Introduction. In addition to the thrombolytic agents, mechanical devices are being used in intraarterial procedures in acute stroke patients. The intervention protocol and outcomes of intraarterial procedures performed in the Hospital Virgen del Rocío in 2003-2004 are described. Methods. The results in each arterial occlusion site and case-control study of the middle cerebral artery (MCA), the placebo group in PROACT II study (Prolyse in Acute Cerebral Thromboembolism II) as historic control group, are described. Results. A total of 32 procedures were performed: 18 MCA occlusions, 8 basilar thrombosis and 6 intracranial carotid occlusions. Urokinase, abciximab and mechanical devices were used in 60%, 26% and 60% of the cases, respectively. MCA occlusions: 60% of the cases improved in 24 hours (25% remained asymptomatic). Only 25% controls remained independent versus 55% of the treated patients (p=0.001). Recanalization and complete recanalization rates were 95 % and 45 %, respectively (18% and 2% in the control group). Symptomatic intracranial hemorrhage (SICH) rate was 18% vs 4% in cases and controls. Basilar thrombosis: only one patient died and 57% of the remaining patients were independent. Carotid occlusions: no complete recanalization was achieved and only one patient was independent. Evolution time in SICH patients was longer (p=0.04), with increased international normalized ratio (INR) for thromboplastin time (p=0.06) and old ischemic lesions in brain CT scan (p=0.02) and they were more dependent at three months (p=0.01). Conclusions. Intraarterial procedures in MCA occlusions achieve high rates of recanalization and better clinical outcomes than those described in isolated intraarterial thrombolysis. Long-term outcomes in basilar thrombosis were also excellent. Nevertheless, the natural evolution of carotid occlusions was not modified

Clinical and electroencephalographic features of carotid sinus syncope induced by internal carotid artery angioplasty.

BACKGROUND AND PURPOSE: Carotid sinus syncope may occur acutely during internal carotid artery angioplasty (CA). We performed this study to investigate the clinical, electroencephalographic (EEG), and hemodynamic features of carotid sinus syncope induced by CA. MATERIALS AND METHODS: Between 1992 and 2003, clinical, EEG, and cardiovascular monitoring was performed in 359 consecutive patients undergoing CA. RESULTS: Carotid sinus reaction (CSR) and syncope occurred in 62.7% and 18.6% of the procedures, respectively. CSR and syncopal spells were classified into cardioinhibitory, vasodepressor, and mixed type. Syncope occurred more frequently in patients with cardioinhibitory CSR (P < .001). The odds ratios for the risk of syncope in patients with cardioinhibitory CSR and vasodepressor/mixed CSR were 6.9 and 1.4, respectively. Sixty-one patients had cardioinhibitory syncope; 7 had the vasodepressor/mixed type. Thirteen spells were not related to cardiovascular disturbances. This last syncope subtype was significantly associated with brain hemodynamic disturbances, including a decrease in cerebral vasoreactivity (P = .04) and the absence of function of both communicating arteries (P = .03). Convulsive movements resembling supplementary sensorimotor seizures occurred in 79% of patients who experienced syncopal spells. EEG changes were more prominent in patients with cardioinhibitory syncope. CONCLUSIONS: Syncope occurs frequently in patients undergoing CA and can be misdiagnosed as seizures. The most frequent mechanism was a cardioinhibitory response. Cerebral hemodynamic disturbances may play a crucial role in the pathophysiology of syncope with normal sinus rhythm and normotension. Moreover, direct depression of the CNS following carotid sinus distension is likely to be involved.

Disociación clinicorradiológica en la encefalopatía hipertensiva con afectación predomimanante de troncoencéfalo y cerebelo / Primary involvement of brainstem and cerebellum in hypertensive encephalopathy

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