[Cerebral ischemia as first manifestation of paroxysmal nocturnal hemoglobinuria]. / Isquemia cerebral como manifestación inicial de un caso de hemoglobinuria paroxística nocturna.

INTRODUCTION: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disease characterized by chronic intravascular hemolysis, cytopenia and increased tendency to thrombosis. Arterial thrombosis has been observed in patients with PNH but is uncommon compared with venous thrombosis. Cerebral arterial thrombosis has been anecdotally described as the first clinical manifestation of PNH. CASE DESCRIPTION: We present the case of a 48-yearold male who was admitted because of high-grade fever and infarction in the left middle cerebral artery territory. Other associated findings were mild anemia (hemoglobin level: 10.1 g/dl), renal ischemia and intra-abdominal venous thrombosis. Recurrent arterial and venous thromboembolic events were observed during his hospital stay. The diagnosis of PNH was confirmed by flow cytometric analysis. CONCLUSIONS: PNH is a rare cause of cerebral ischemia and it should be considered in stroke patients with abnormal blood findings or marked prothrombotic tendency. We discuss the treatment guidelines and prevention of thrombotic complications.

Isquemia cerebral como manifestación inicial de un caso de hemoglobinuria paroxística nocturna / Cerebral ischemia as first manifestation of paroxysmal nocturnal hemoglobinuria

Introducción. La hemoglobinuria paroxística nocturna (HPN) es una rara enfermedad clonal clínicamente caracterizada por hemólisis crónica intravascular, citopenia y tendencia protrombótica. Las trombosis en el sector arterial han sido observadas en pacientes con HPN, pero son muy poco habituales comparadas con las trombosis venosas. Las oclusiones arteriales tromboembólicas en vasos cerebrales han sido descritas excepcionalmente como manifestación clínica inicial en casos de HPN. Caso clínico. Se describe el caso de un paciente varón de 48 años de edad ingresado por fiebre elevada y un ictus en el territorio de la arteria cerebral media izquierda. Otras manifestaciones asociadas fueron anemia moderada (hemoglobina: 10,1 g/dl), isquemias renales y trombosis de localización abdominal. Durante su ingreso se observaron eventos tromboembólicos recurrentes en los sectores arterial y venoso. El diagnóstico de HPN fue confirmado por citometría de flujo. Conclusiones. La HPN es una causa infrecuente de isquemia cerebral que debe ser considerada en el diagnóstico diferencial de pacientes con ictus y alteraciones hematológicas o estados protrombóticos. Se discuten las pautas de tratamiento y prevención de las complicaciones trombóticas (AU)

Introduction. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disease characterized by chronic intravascular hemolysis, cytopenia and increased tendency to thrombosis. Arterial thrombosis has been observed in patients with PNH but is uncommon compared with venous thrombosis. Cerebral arterial thrombosis has been anecdotally described as the first clinical manifestation of PNH. Case description. We present the case of a 48-year-old male who was admitted because of high-grade fever and infarction in the left middle cerebral artery territory. Other associated findings were mild anemia (hemoglobin level: 10.1 g/dl), renal ischemia and intra-abdominal venous thrombosis. Recurrent arterial and venous thromboembolic events were observed during his hospital stay. The diagnosis of PNH was confirmed by flow cytometric analysis. Conclusions. PNH is a rare cause of cerebral ischemia and it should be considered in stroke patients with abnormal blood findings or marked prothrombotic tendency. We discuss the treatment guidelines and prevention of thrombotic complications (AU)

Apoplejía pituitaria: ¿es una urgencia quirúrgica? / Pituitary apoplexy: is a surgical urgency?

No disponible

Does a relationship exist between carotid stenosis and lacunar infarction?

BACKGROUND AND PURPOSE: The presence of carotid stenosis (CS) in a patient with lacunar stroke is usually considered an indication of atherosclerosis and not directly related to the development of this infarction subtype. This study was designed to determine the relationship between CS and lacunar infarction (LI) and to assess the differences between single and multiple LIs. METHODS: We classified 330 patients with a first-ever cerebral infarction in the carotid territory into LI and non-LI (NLI) groups. In the LI group, patients with a single LI and those with multiple LIs were identified. In this last subgroup, 2 patterns were identified: 1 subtype with lacunar lesions distributed in both cerebral hemispheres, and another with lesions predominantly in 1 hemisphere. RESULTS: In the LI group, isolated CS was significantly more frequent on the homolateral side than on the contralateral side (odds ratio [OR], 5.5; 95% CI, 1.2 to 23; P=0.03). A significant relationship between the pattern of distribution of the infarctions in only 1 hemisphere and homolateral CS >70% was observed (OR, 4.4; 95% CI, 0.9 to 19; P=0.03). In a multivariate analysis, the following variables were found to predict unilateral multiple LI: left ventricular hypertrophy (OR, 9.1; 95% CI, 2.5 to 33.6) and homolateral CS >75% (OR, 14.4; 95% CI, 2.0 to 99.6). CONCLUSIONS: The significant incidence of isolated ipsilateral CS in patients with LI located in the carotid territory and the relationship of CS to ipsilateral multiple LI suggest that CS has a very important role in the development of LI.

[Hashimoto's encephalopathy]. / Encefalopatía de Hashimoto.

Hashimoto's autoimmune thyroiditis is a common cause of thyroid disease. Neurological dysfunction related to thyroid hypo or hyperfunction is well known. Not so is autoimmune thyroid disease-associated or Hashimoto's encephalopathy, which includes different neurological manifestations appearing in the context of autoimmune thyroiditis with normal hormone levels. Around fifty cases have been reported since the first description by Brain in 1966. Pathogenesis is unknown, although the most accepted theory points out to an autoimmune cerebral dysfunction. There are two different clinical presentations. The vasculitic type is characterized by relapsing-remitting stroke-like episodes. The diffuse-progressive type shows insidious cognitive impairment, confusion, psychosis, somnolence and coma. Cerebrospinal fluid is abnormal in more than 80% of patients, with high protein levels and mononuclear pleocytosis. Steroids are the treatment of choice, although favourable evolution have been reported spontaneously or after thyroxine treatment.

Encefalopatía de Hashimoto / Hashimoto's encephalopathy

La tiroiditis autoinmune de Hashimoto es una causa común de enfermedad tiroidea. Las complicaciones neurológicas asociadas a hipo o hiperfunción tiroidea son bien conocidas. No lo es tanto la denominada encefalopatía autoinmune distiroidea o encefalopatía de Hashimoto, que incluye diferentes cuadros neurológicos que aparecen en el seno de una tiroiditis autoinmune y no se acompañan de una alteración en los valores de las hormonas tiroideas. Se han comunicado alrededor de 50 casos desde su descripción por Brain en 1966. Su patogenia es desconocida, aunque la teoría más aceptada es una afección cerebral autoinmune. Existen dos formas de presentación más o menos diferenciadas. La forma vasculítica se caracteriza por episodios seudoictales y su curso clínico es recidivante-remitente. La variante difusaprogresiva se caracteriza por un inicio insidioso, con deterioro cognitivo acompañado de confusión, psicosis, somnolencia o coma. El líquido cefalorraquídeo (LCR) es anormal en el 80 per cent de los pacientes. El hallazgo más frecuente es una hiperproteinorraquia moderada, que puede acompañarse de pleocitosis mononuclear. Aunque se han comunicado varios casos con evolución favorable espontánea o tras la administración de tiroxina, el tratamiento de elección son los esteroides (AU)

[Lacunar infarcts. A comparative study of risk factors and carotid stenosis]. / Infartos lacunares. Estudio comparativo de factores de riesgo y estenosis carotídea.

OBJECTIVE: In order to evaluate the pathogenic role of carotid atheromatosis and risk factors for the lacunar type of cerebral ischaemia a comparative analysis was made between a group of patients with this type of stroke and other with non-lacunar infarctions. Unlike previous works, patients with clinical-radiological criteria for ischaemia in the carotid territory were included in both groups. METHODS: Ninety-eight patients with a first episode of stroke were selected prospectively. Strokes were classified as lacunar (52 patients) and non-lacunar (46 patients). The following risk factors were evaluated: age, sex, increased blood pressure, left ventricle enlargement, smoking, alcohol intake, lipid profile, history of previous transient stroke, arterial retinopathy, diabetes, ischaemic heart disease and presence of peripheral arteriopathy. The narrowing degree of the carotid artery was determined by means of Doppler ultrasonography. RESULTS: Significant differences were found for the presence of two variables: hypertensive retinopathy was more prevalent in the lacunar group (p = 0.003) and history of transient ischaemic stroke was recorded more frequently in association with non-lacunar infarction (p = 0.01). A 50%-70% degree of narrowing in the upper carotid artery was observed more frequently in association with non-lacunar infarctions (p < 0.001) in the ipsilateral artery to the symptomatic hemisphere. In the heterolateral artery the narrowing degree was similar for both groups (p = 0.87). CONCLUSIONS: Lacunar and non-lacunar infarctions share the same risk factors; nevertheless, the distribution of atheromatous lesions was different. The presence of hypertensive retinopathy is an excellent marker for perforant arterial disease. The carotid stenosis is an unusual pathogenic mechanism for the development of ischaemic lacunar syndrome with an appropriate injury visualized by CT.