Trombosis del seno cavernoso en gestantes: a propósito de un caso / Thrombosis of the cavernous sinus in pregnant women: case report

La trombosis de senos venosos cerebrales es una entidad poco frecuente. Puede presentarse con clínica de cefalea, nauseas, vómitos, fiebre, déficits neurológicos focales y incluso el coma. La etiología es multifactorial siendo uno de los factores causantes los estados de hipercoagulabilidad. El embarazo y el puerperio, fisiológicamente, son estados procoagulantes. Presentamos el caso de una gestante de 32 años de 9 semanas que fue ingresada por un cuadro de nausea acompañado de una intensa cefalea, por lo que fue remitida al Servicio de Neurología. La resonancia magnética con angiografía reveló una trombosis de senos venosos cerebrales. Fue tratada con infusión bomba de heparina no fraccionada con mejoría en los síntomas. Tras el parto, prosiguió el tratamiento anticoagulante con heparina de bajo peso molecular a dosis profilácticas durante 6 semanas. En conclusión, resulta muy importante que se produzca un correcto y precoz diagnóstico para el pronóstico de esta enfermedad por lo que debe considerarse esta entidad dentro del diagnóstico diferencial de cefalea intensa en pacientes gestantes

Cerebral venous sinus thrombosis is a rare entity. It can present with symptoms of headache, nausea, vomiting, fever, focal neurological deficits and even coma. The etiology is multifactorial, being one of the factors causing hypercoagulability states. Pregnancy and the puerperium, physiologically, are procoagulant states. We present the case of a pregnant woman of 32 years of 9 weeks who was admitted for a nausea episode accompanied by an intense headache, for which she was referred to the Neurology Service. Magnetic resonance imaging with angiography revealed a cerebral venous sinus thrombosis. It was treated with infusion of unfractionated heparin pump with improvement in symptoms. After delivery, the anticoagulant treatment with low molecular weight heparin at prophylactic doses during 6 weeks. In conclusion, it is very important that there is a correct and early diagnosis for the prognosis of this disease so this entity should be considered within the differential diagnosis of severe headache in patients pregnant women

Brain angiogenic gene expression in fetuses with congenital heart disease.

OBJECTIVE: To assess potential differences in the expression of antiangiogenic and angiogenic factors and of genes associated with chronic hypoxia in cerebral tissue of euploid fetuses with congenital heart disease (CHD) vs those without. METHODS: Cerebral tissue was obtained from 15 fetuses with CHD and 12 control fetuses that had undergone termination of pregnancy. Expression profiles of the antiangiogenic factor soluble fms-like tyrosine kinase-1 (sFlt-1), the angiogenic vascular endothelial growth factor-A (VEGF-A) and placental growth factor (PlGF), and of genes associated with chronic hypoxia were determined by real-time polymerase chain reaction in tissue from the frontal cortex and the basal ganglia of the fetuses. RESULTS: Expression of sFlt-1 was 48% higher in the frontal cortex (P = 0.0431) and 72% higher in the basal ganglia (P = 0.0369) of CHD fetuses compared with controls. The expression of VEGF-A was 60% higher (P = 0.0432) and that of hypoxia-inducible factor 2-alpha was 98% higher (P = 0.0456) in the basal ganglia of CHD fetuses compared with controls. No significant differences were observed between the two groups in the expression of PlGF and hypoxia-inducible factor 1-alpha. CONCLUSION: An overall dysregulation of angiogenesis with a net balance towards an antiangiogenic environment was observed in the cerebral tissue of fetuses with CHD, suggesting that these fetuses may have an intrinsic angiogenic impairment that could contribute to impaired brain perfusion and abnormal neurological development later in life. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients.

OBJECTIVES: To study the clinical spectrum and evolution of Churg-Strauss syndrome in order to assess the clinicopathological features of the disease, the response to treatment and the long-term outcome. METHODS: Thirty-two patients with proven allergic and granulomatous angiitis (Churg-Strauss syndrome) and followed up at a single institution were evaluated. They were recruited between 1977 and 1999 from internal medicine departments. Data were obtained retrospectively from medical files in 15 cases and prospectively, using a standardized form, for the remaining patients. RESULTS: All patients had asthma and hypereosinophilia. The lungs, skin and peripheral nervous system were the organs most frequently involved. Antineutrophil cytoplasmic antibodies with antimyeloperoxidase specificity (MPO-ANCA) were detected in 77.8% of tested patients but they were not useful for monitoring disease activity. Extravascular granulomas were rarely seen in tissue biopsies. Forty per cent of the patients were treated with steroids alone. Immunosuppressive agents were added to the treatment when severe neurological, cardiac or gastrointestinal involvement was present. The outcome and long-term survival were good. Clinical relapse was rare after the first year of therapy. Dysaesthesiae of the distal limbs, neurophatic pain and cardiac failure were the most frequent sequelae. CONCLUSIONS: Churg-Strauss syndrome is a rare disorder characterized by hypereosinophilia and systemic vasculitis occurring in patients with asthma and allergic rhinitis. Vasculitis commonly affects the lungs, skin and peripheral nervous system. Outcome and long-term survival is usually good with steroids alone or in combination with immunosuppressive agents. The syndrome has a low mortality rate compared with other systemic vasculitides.

["Stroke code". Shortening the delay in reperfusion treatment of acute ischemic stroke]. / "Código Ictus" y tiempos de latencia en el tratamiento de reperfusión durante la fase aguda del ictus isquémico.

BACKGROUND: "Stroke Code" is a system for the rapid identification, pre-notification and transport of acute ischemic stroke patients. The objective of this study was to evaluate the impact of delay reduction for thrombolytic therapy in these patients. SUBJECTS AND METHODS: We evaluated acute ischemic stroke patients admitted in the emergency unit within the first 6 hours after onset of symptoms and included into reperfusion clinical trials. We compared the delay for initiating reperfusion treatment related to the activation or not of the stroke code. RESULTS: From 454 patients evaluated, 25% were admitted to the hospital in less than 6 hours from stroke onset. 59% of these patients were candidates for reperfusion treatment. "Stroke Code" was activated in 13 (55%). We observed a significant reduction in the delay since the onset of symptoms in relation to "Stroke Code" activation or not (mean X [SD]): emergency room arrival: 49.6 (48) vs 80 (48) min; Stroke Team evaluation: 65.3 (57) vs 133.6 (58) min; CT scan performing: 86.2 (60) vs 171.8 (62) min; Start of treatment; 212.9 (51) vs 287.3 (59) min. CONCLUSIONS: "Stroke Code" activation reduced in 50% pre- and in-hospital delay to start reperfusion treatment in acute ischemic stroke patients.

Cyclosporine A in atopic dermatitis.

Atopic dermatitis is a chronic pruritic dermatosis that requires individualized treatment. Recent studies have shown that low-dose oral treatment with cyclosporine A may be useful in more severe forms. We report our experience with two patients who had severe atopic dermatitis. Both had high IgE concentration and satisfied Hanifin and Rajka's diagnostic criteria for atopic dermatosis. We began oral treatment at a daily dosage of 5 mg/kg divided into two doses. We monitored cyclosporine concentration in whole blood, as well as kidney and liver function. Contraindications for cyclosporine A were excluded. Clinical improvement commenced between the first and third week of treatment and was marked in one case and partial in the other. Improvement did not correlate with a reduction in serum IgE concentration. Side effects were mild (hypertrichosis, slight trembling, and gingival hypertrophy). On discontinuing treatment, clinical manifestations reappeared. We conclude that cyclosporine A may be an effective alternative for some forms of atopic dermatitis resistant to other treatments.

[Idiopathic inflammatory myopathy. Analysis of 31 patients]. / Miopatía inflamatoria idiopática. Análisis de 31 pacientes.

A series of 31 patients suffering idiopathic inflammatory myopathy (IIM); we describe the extramuscular manifestations, specially pulmonary, the association to neoplasia, the histopathological characteristics, and their response to treatment. Fourty three percent of IIM patients presented a pulmonary involvement, 9% presented an associated neoplasia. The histopathological study allowed us to clearly differentiate dermatomyositis and polymyositis within IIM. 65% of patients initially responded to glucocorticoids and the most usefull therapeutic alternatives were azatioprine and cyclosporin-A.

Prolonged remission after cyclosporine therapy in pemphigus vulgaris: report of two young siblings.

We report the clinical evolution of two young siblings with severe pemphigus vulgaris treated with cyclosporine for 30 and 12 months, respectively. One was resistant to treatment with high-dose corticosteroids and azathioprine. A good clinical response was achieved in both cases. No major side effects were observed. The patients have remained disease free for more than 20 months after stopping cyclosporine therapy.

Kienböck's disease and antiphospholipid antibodies.

To evaluate the pathogenetic role of antiphospholipid antibodies in Kienböck's disease, we performed a clinical and laboratory study in a series of 16 patients. Antiphospholipid antibodies detected by means of the lupus anticoagulant and anticardiolipin antibody test were found in three of them. No other risk factors were present in these patients. We discuss the possible significance of these findings.