Enfermedad cerebrovascular de pequeño vaso / Small vessel cerebrovascular disease

La enfermedad vascular de pequeño vaso representa un espectro de diferentes entidades que incluyen el infarto lacunar, la alteración de sustancia blanca y los microsangrados. La hipertensión es el principal factor de riesgo asociado, aunque la lesión aterotrombótica puede estar presente sobre todo en los infartos lacunares de gran tamaño y con otros factores de riesgo vascular. Los hallazgos en RM son característicos y las lesiones auténticos biomarcadores que permiten diferenciar el valor de los factores de riesgo y definir su valor pronóstico

Small vessel vascular disease is a spectrum of different conditions that includes lacunar infarction, alteration of deep white matter, or microbleeds. Hypertension is the main risk factor, although the atherothrombotic lesion may be present, particularly in large-sized lacunar infarctions along with other vascular risk factors. MRI findings are characteristic and the lesions authentic biomarkers that allow differentiating the value of risk factors and defining their prognostic value

[Small vessel cerebrovascular disease]. / Enfermedad cerebrovascular de pequeño vaso.

Small vessel vascular disease is a spectrum of different conditions that includes lacunar infarction, alteration of deep white matter, or microbleeds. Hypertension is the main risk factor, although the atherothrombotic lesion may be present, particularly in large-sized lacunar infarctions along with other vascular risk factors. MRI findings are characteristic and the lesions authentic biomarkers that allow differentiating the value of risk factors and defining their prognostic value.

Frequency and outcome of total anterior circulation strokes without intracranial large-vessel occlusion.

BACKGROUND AND PURPOSE: The percentage of patients with clinical total anterior circulation infarct (TACI) syndrome treated with reperfusion therapies in the absence of intracranial large-vessel occlusion (ILVO) was determined and their characteristics and outcome are described. METHODS: Data from a population-based, prospective, externally audited registry of all stroke patients treated with intravenous thrombolysis (IVT) and endovascular therapies in Catalonia from January 2011 to December 2013 were used. Patients with a baseline TACI and initial stroke severity measured by the National Institute of Health Stroke Scale (NIHSS) ≥ 8, evaluated less than 4.5 h post-onset, for whom a vascular study prior to treatment was available (n = 1070) were selected. Clinical characteristics, outcome and radiological data for patients treated with IVT alone (n = 605) were compared between those with detected ILVO (n = 474) and non-ILVO patients (n = 131). RESULTS: A total of 1070 patients met study criteria; non-ILVO was found in 131 (12.2%). Analysing the 605 patients treated only with IVT, no significant differences were found between non-ILVO and ILVO patients in age, sex, risk factors, time-to-treatment and type of radiological studies performed. Although non-ILVO patients had lower initial stroke severity (P < 0.001) and a better prognosis (P = 0.001), 51.3% had a poor outcome and 16% were deceased at 90 days. In 66.4% of patients without ILVO, a recent anterior territorial infarct was detected. CONCLUSIONS: Intracranial artery patency was observed in 12.2% of TACI patients evaluated within 4.5 h. Although absence of ILVO was associated with slightly better prognosis, more than half had a poor outcome at 3 months.

Recomendaciones del Doppler transcraneal en el diagnóstico de la muerte encefálica / No disponible

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Heterogeneidad topográfica del síndrome de Foix-Chavany-Marie / Heterogeneous topography of Foix-Chavany-Marie syndrome

Introducción. El síndrome de Foix-Chavany-Marie (FCM) fue descrito como una diplejía labio-facio-faringo-laringogloso- masticatoria con disociación automático-voluntaria del movimiento. Se correlaciona habitualmente con lesiones corticales bilaterales con afectación de ambos opérculos (síndrome biopercular). Describimos tres pacientes con síndrome de FCM asociado a lesiones isquémicas en dos topografías atípicas: a) subcortical bilateral, y b) opercular unilateral. Casos clínicos. Paciente 1: varón de 66 años que consultó por paresia facial, lingual y faríngea bilaterales junto con anartria de inicio ictal. En la exploración destacaba la presencia de disociación automático-voluntaria de la motilidad facial. La resonancia magnética (RM) craneal mostró un infarto reciente subcortical izquierdo, así como múltiples lesiones isquémicas antiguas subcorticales contralaterales. Paciente 2: varón de 61 años, diabético, que consultó también por anartria y parálisis facial y lingual bilaterales con disociación automático-voluntaria junto con alteración de la deglución de inicio brusco. La RM craneal mostró la presencia de una única lesión isquémica opercular izquierda. Paciente 3: varón de 36 años que consultó por aparición brusca de disartria grave, disfagia, diplejía facial con disociación automático-voluntaria y pérdida de fuerza de la extremidad superior izquierda. La RM craneal mostró un infarto opercular derecho sin lesiones contralaterales. Conclusiones. El síndrome de FCM no se asocia únicamente a lesiones operculares bilaterales, sino que además puede deberse a lesiones subcorticales bilaterales e incluso a lesiones corticales unilaterales

Introduction. Bilateral facio-pharyngo-laryngo-glosso- masticatory palsy with automatic-voluntary dissociation is known as Foix-Chavany-Marie (FCM) syndrome. It is usually due to bilateral cortical lesions involving both anterior opercula (biopercular syndrome). We describe three patients with FCM syndrome associated with ischemic lesions in two atypical localizations: a) bilateral subcortical infarcts, and b) unilateral opercular infarct. Cases report. Patient 1, a 66 year old male, was admitted for a sudden onset of anarthria and facial, lingual and masticatory paralysis. Neurological examination revealed automatic-voluntary dissociation of facial motility. MRI showed an acute left subcortical infarct and multiple bilateral subcortical ischemic lesions. Patient 2, a 61 year old male, also suffered a sudden onset of anarthria, with bilateral facial and lingual paralysis and automatic-voluntary dissociation together with sudden onset swallowing alteration. MRI showed a single ischemic lesion involving the left operculum. Patient 3, a 36 year old male, presented sudden onset of dysarthria, dysphagia and bilateral facial palsy with automatic-voluntary dissociation and loss of force in left upper limb. MRI showed an acute right opercular infarct and no contralateral lesions. Conclusions. FCM syndrome is not only due to bilateral opercular lesions but can also be seen in bilateral subcortical and even unilateral opercular lesions

[Heterogeneous topography of Foix-Chavany-Marie syndrome]. / Heterogeneidad topográfica del síndrome de Foix-Chavany-Marie.

INTRODUCTION: Bilateral facio-pharyngo-laryngo-glosso-masticatory palsy with automatic-voluntary dissociation is known as Foix-Chavany-Marie (FCM) syndrome. It is usually due to bilateral cortical lesions involving both anterior opercula (biopercular syndrome). We describe three patients with FCM syndrome associated with ischemic lesions in two atypical localizations: a) bilateral subcortical infarcts, and b) unilateral opercular infarct. CASES REPORT: Patient 1, a 66 year old male, was admitted for a sudden onset of anarthria and facial, lingual and masticatory paralysis. Neurological examination revealed automatic-voluntary dissociation of facial motility. MRI showed an acute left subcortical infarct and multiple bilateral subcortical ischemic lesions. Patient 2, a 61 year old male, also suffered a sudden onset of anarthria, with bilateral facial and lingual paralysis and automatic-voluntary dissociation together with sudden onset swallowing alteration. MRI showed a single ischemic lesion involving the left operculum. Patient 3, a 36 year old male, presented sudden onset of dysarthria, dysphagia and bilateral facial palsy with automatic-voluntary dissociation and loss of force in left upper limb. MRI showed an acute right opercular infarct and no contralateral lesions. CONCLUSIONS: FCM syndrome is not only due to bilateral opercular lesions but can also be seen in bilateral subcortical and even unilateral opercular lesions.

[Wallenberg's syndrome as the presenting symptom of a cavernous angioma]. / Síndrome de Wallenberg como forma de presentación de un angioma cavernoso.

INTRODUCTION: Cavernous angiomas account for 5-13% of all vascular malformations. In 75% of cases they are situated in the posterior fossa and up to 30% are associated with abnormal venous drainage. The main complication is haemorrhage; the presence of a neurological focus without radiological evidence of bleeding is very rare. CASE REPORT: We report the case of a 54-year-old male with cardiovascular risk factors who presented symptoms that progressively deteriorated over a 72-hour period involving the left lower cranial nerves, sensory impairment and coordination disorder, compatible with Wallenberg's syndrome. Two computerised axial tomography scans of the brain were normal and so a tentative diagnosis of ischemic stroke in progression was proposed. Five days later, magnetic resonance imaging (MR) revealed the presence of a venous angioma and associated abnormal venous drainage. CONCLUSIONS: Cavernous angiomas present a dynamic balance between intracavernous bleeding and thrombosis, with very slow venous blood flow. Upsetting this balance leads to an increase in the intracavernous pressure and involvement of the surrounding tissue, with no radiological expression of bleeding. In these cases MR scanning helps to distinguish between a vascular malformation with reduced blood flow and a clinical picture of ischemic stroke of an arterial origin.

Síndrome de Wallenberg como forma de presentación de un angioma cavernoso / Wallenberg's syndrome as the presenting symptom of a cavernous angioma

Introducción. Los angiomas cavernosos representan el 5-13 por ciento del total de malformaciones vasculares. En el 75 por ciento de los casos se encuentran en la fosa posterior, y hasta en el 30 por ciento, asociado a un drenaje venoso anómalo. La principal complicación son las hemorragias; es excepcional la presencia de focalidad neurológica sin evidencia radiológica de sangrado. Caso clínico. Presentamos un caso de un varón de 54 años con factores de riesgo cardiovascular, que muestra un cuadro progresivo en 72 horas de duración de afectación de los pares craneales bajos izquierdos, déficit sensitivo y alteración de la coordinación compatible con un síndrome de Wallenberg. Con la tomografía axial computarizada craneal en dos ocasiones normal, se realizó la orientación diagnóstica de ictus vertebrobasilar en progresión. Cinco días después, la resonancia magnética (RM) demostró la presencia de un angioma venoso y un drenaje venoso anómalo asociado. Conclusión. Los angiomas cavernosos presentan un equilibrio dinámico de sangrado y trombosis de intracavernoma, con un flujo venoso muy lento. Una rotura en este equilibrio supone un aumento de la presión de intracavernoma y la afectación del tejido circundante, sin expresión radiológica de sangrado. La RM ayuda en estos casos a diferenciar una malformación vascular de flujo lento de un cuadro ictal isquémico de origen arterial (AU)

Introduction. Cavernous angiomas account for 5-13% of all vascular malformations. In 75% of cases they are situated in the posterior fossa and up to 30% are associated with abnormal venous drainage. The main complication is haemorrhage; the presence of a neurological focus without radiological evidence of bleeding is very rare. Case report. We report the case of a 54-year-old male with cardiovascular risk factors who presented symptoms that progressively deteriorated over a 72-hour period involving the left lower cranial nerves, sensory impairment and coordination disorder, compatible with Wallenberg’s syndrome. Two computerised axial tomography scans of the brain were normal and so a tentative diagnosis of ischemic stroke in progression was proposed. Five days later, magnetic resonance imaging (MR) revealed the presence of a venous angioma and associated abnormal venous drainage. Conclusions. Cavernous angiomas present a dynamic balance between intracavernous bleeding and thrombosis, with very slow venous blood flow. Upsetting this balance leads to an increase in the intracavernous pressure and involvement of the surrounding tissue, with no radiological expression of bleeding. In these cases MR scanning helps to distinguish between a vascular malformation with reduced blood flow and a clinical picture of ischemic stroke of an arterial origin (AU)

[Cerebral venous thrombosis as the presenting symptom of systemic lupus erythematosus]. / Trombosis venosa cerebral como presentación de lupus eritematoso sistémico.

INTRODUCTION: Cerebral venous thrombosis (CVT) is an infrequent process in systemic lupus erythematosus. We report the case of a female patient whose initial manifestation of lupus was a CVT. CASE REPORT: A 30-year-old female who presented headaches and diminished visual acuity; on exploring the patient bilateral papilloedema was found. Magnetic resonance imaging revealed the presence of a venous thrombosis in the superior and transversal longitudinal sinus. Complementary explorations showed high levels of antinuclear antibodies with leukopenia and proteinuria. Antiphospholipid antibodies were negative. Following treatment with anticoagulants, the patient's condition improved both clinically and radiologically. Months later a biopsy was performed and revealed a grade IV diffuse glomerulonephritis. CONCLUSIONS: In systemic lupus erythematosus, phenomena such as CVT can be the initial form of presentation of the disease. The presence of antiphospholipid antibodies plays a partial role in CVT; other phenomena, such as inflammatory processes, should also be taken into account.

Trombosis venosa cerebral como presentación de lupus eritematoso sistémico / Cerebral venous thrombosis as the presenting symptom of systemic lupus erythematosus

Introduction. Cerebral venous thrombosis (CVT) is an infrequent process in systemic lupus erythematosus. We report the case of a female patient whose initial manifestation of lupus was a CVT. Case report. A 30-year-old female who presented headaches and diminished visual acuity; on exploring the patient bilateral papilloedema was found. Magnetic resonance imaging revealed the presence of a venous thrombosis in the superior and transversal longitudinal sinus. Complementary explorations showed high levels of antinuclear antibodies with leukopenia and proteinuria. Antiphospholipid antibodies were negative. Following treatment with anticoagulants, the patient’s condition improved both clinically and radiologically. Months later a biopsy was performed and revealed a grade IV diffuse glomerulonephritis. Conclusions. In systemic lupus erythematosus, phenomena such as CVT can be the initial form of presentation of the disease. The presence of antiphospholipid antibodies plays a partial role in CVT; other phenomena, such as inflammatory processes, should also be taken into account (AU)

Introducción. La trombosis venosa cerebral (TVC) no es un proceso habitual en el lupus eritematosos sistémico. Presentamos una paciente cuya manifestación inicial del lupus fue una TVC. Caso clínico. Paciente de 30 años de edad que presenta cefalea y disminución de la agudeza visual; en la exploración destaca un papiledema bilateral. La resonancia magnética realizada demuestra la presencia de una trombosis venosa en el seno longitudinal superior y transverso. Las exploraciones complementarias mostraron altas concentraciones de anticuerpos antinucleares con leucopenia y proteinuria. Los anticuerpos antifosfolípidos fueron negativos. Tras un tratamiento anticoagulante, mejoró clínica y radiológicamente. Meses más tarde se realizó una biopsia, que demostró una glomerulonefritis difusa grado IV. Conclusión. En el lupus eritematoso sistémico, fenómenos como la TVC pueden ser la forma de manifestación inicial de la enfermedad. La presencia de anticuerpos antifosfolípidos participa de forma parcial en la TVC; deberían considerarse otros fenómenos, como los inflamatorios (AU)