Esclerodermia lineal en coup de sabre. Manifestaciones neurológicas, imágenes y revisión / Linear scleroderma en coup de sabre. Neurological symptoms images and review

Introducción. La esclerodermia lineal en ‘golpe de sable’, mejor conocida como esclerodermia lineal en coup de sabre (ELCS), es una enfermedad rara de causa incierta, caracterizada por atrofia focal progresiva craneofacial y, en parte, es diferente al síndrome de Parry-Romberg (SPR). Casos clínicos. Comunicamos tres pacientes con ELCS (2 mujeres y 1 hombre, con un promedio de edad de 40 años). Las manifestaciones neurológicas principales fueron cefalea y crisis convulsivas. Los alteraciones radiológicas, aunque diversas, fueron todas ipsilaterales al ‘golpe de sable’. En una paciente sometida a biopsia se obtuvo evidencia histopatológica de gliosis e infiltrado inflamatorio mixto perivascular. Destacamos en otro paciente la afectación cerebrovascular, dado el hallazgo de un infarto cerebeloso antiguo subclínico y oclusión de la arteria cerebelosa superior en ausencia de otra posible causa. Conclusiones. La ELCS, cuando afecta al sistema nervioso central es heterogénea en su presentación clínica y radiológica. Los estudios de imagen durante el control clínico y los hallazgos histopatológicos apoyan un proceso inflamatorio focal que puede ser progresivo. La afectación arterial se debe probablemente a una vasculopatía inflamatoria no ateroesclerosa, oclusiva y crónica (AU)

Introduction. ‘Sword stroke’ linear scleroderma, which is better known as linear scleroderma en coup de sabre (LSCS), is a rare disease with an uncertain causation that is characterised by progressive craniofacial focal atrophy and is, at least in part, different from Parry-Romberg syndrome (PRS). Case reports. Here, we report on the cases of 3 patients with LSCS (2 females and 1 male, with a mean age of 40 years). The main neurological symptoms were headache and seizures. Although different alterations were observed in the X-ray images, they were all ipsilateral to the coup de sabre. Histopathological evidence for gliosis and mixed perivascular inflammatory infiltrate was found in the study of a biopsy specimen taken from one female. Cerebrovascular involvement was seen in another patient, as highlighted by the observation of an earlier subclinical cerebellar infarct and occlusion of the superior cerebellar artery in the absence of any other possible causation. Conclusions. When it affects the central nervous system, the clinical and radiological presentation of LSCS is heterogeneous. Both the imaging studies carried out during the clinical control and the histopathological findings suggest a focal inflammatory process that can be progressive. The arterial involvement is probably due to a non-atherosclerotic, occlusive and chronic inflammatory disease of the peripheral vessels (AU)

[Linear scleroderma en coup de sabre: neurological symptoms, images and review]. / Esclerodermia lineal en coup de sabre. Manifestaciones neurologicas, imagenes y revision

INTRODUCTION: 'Sword stroke' linear scleroderma, which is better known as linear scleroderma en coup de sabre (LSCS), is a rare disease with an uncertain causation that is characterised by progressive craniofacial focal atrophy and is, at least in part, different from Parry-Romberg syndrome (PRS). CASE REPORTS: Here, we report on the cases of 3 patients with LSCS (2 females and 1 male, with a mean age of 40 years). The main neurological symptoms were headache and seizures. Although different alterations were observed in the X-ray images, they were all ipsilateral to the coup de sabre. Histopathological evidence for gliosis and mixed perivascular inflammatory infiltrate was found in the study of a biopsy specimen taken from one female. Cerebrovascular involvement was seen in another patient, as highlighted by the observation of an earlier subclinical cerebellar infarct and occlusion of the superior cerebellar artery in the absence of any other possible causation. CONCLUSIONS: When it affects the central nervous system, the clinical and radiological presentation of LSCS is heterogeneous. Both the imaging studies carried out during the clinical control and the histopathological findings suggest a focal inflammatory process that can be progressive. The arterial involvement is probably due to a non-atherosclerotic, occlusive and chronic inflammatory disease of the peripheral vessels.

[Severe hemorrhage intestinal in a child with ileal duplication and brief entity review]. / Hemorragia intestinal severa en una niña con duplicación ileal y breve reseña de la entidad.

Intestinal duplications are very uncommon congenital malformations located in the mesenteric edge of the small bowel, particularly in the ileum. Over 60% of the patients become symptomatic during the first year of life whereas the remainder demonstrates symptoms at school age or adulthood. The wide spectrum of symptoms and unspecific signs frequently simulate other diseases. Gastrointestinal hemorrhage is the most noteworthy complication, which can cause severe anemia and shock. In the case we describe an 11-year-old girl experienced massive intermittent intestinal hemorrhage, anemic syndrome and intense abdominal pain over 15 months. In this patient, hemoglobin levels rose up to 6 g/dL necessitating several hemotransfusion. Multiple diagnosis and examinations were carried out until finally the gammagram with Tc99m disclosed an ectopic gastric mucosa. Using a laparotomy an intestinal duplication was found in the terminal ileum. In this paper the intestinal duplication is analyzed and the importance of considering it as a differential diagnosis in any kind of bleeding of the digestive tube is discussed.

Hemorragia intestinal severa en una niña con duplicación ileal y breve reseña de la entidad / Severe hemorrhage intestinal in a child with ileal duplication and brief entity review

Las duplicaciones intestinales son malformaciones congénitas poco comunes localizadas en el borde mesentérico del intestino delgado, particularmente en el íleon. Más del 60% de los casos son sintomáticas durante el primer año de vida y el resto en escolares y adultos. El amplio espectro de síntomas y signos inespecíficos que presentan, frecuentemente simulan otras enfermedades. Entre las complicaciones destaca la hemorragia gastrointestinal que llega, ocasionar anemia severa y shock, como el caso que describimos de una niña de 11 años de edad, que presentó durante 15 meses severas evacuaciones sanguinolentas intermitentemente acompañadas de dolor abdominal intenso y anemia. Los niveles de hemoglobina llegaron hasta 6 g/dL requiriendo en varias ocasiones hemotransfusión. Múltiples diagnósticos y exámenes fueron realizados y finalmente la gammagrafía con Tc99m mostró mucosa gástrica ectópica; mediante laparotomía se encontró una duplicación intestinal en el íleon terminal. Se analiza esta entidad y su importancia de considerarla en el diagnóstico diferencial en cualquier tipo de sangrado del tubo digestivo (AU)

Intestinal duplications are very uncommon congenital malformations located in the mesenteric edge of the small bowel, particularly in the ileum. Over 60% of the patients become symptomatic during the first year of life whereas the remainder demonstrates symptoms at school age or adulthood. The wide spectrum of symptoms and unspecific signs frequently simulate other diseases. Gastrointestinal hemorrhage is the most noteworthy complication, which can cause severe anemia and shock. In the case we describe an 11-year-old girl experienced massive intermittent intestinal hemorrhage, anemic syndrome and intense abdominal pain over 15 months. In this patient, hemoglobin levels rose up to 6 g/dL necessitating several hemotransfusion. Multiple diagnosis and examinations were carried out until finally the gammagram with Tc99m disclosed an ectopic gastric mucosa. Using a laparotomy an intestinal duplication was found in the terminal ileum. In this paper the intestinal duplication is analyzed and the importance of considering it as a differential diagnosis in any kind of bleeding of the digestive tube is discussedIntestinal duplications are very uncommon congenital malformations located in the mesenteric edge of the small bowel, particularlyin the ileum. Over 60% of the patients become symptomatic during the first year of life whereas the remainder demonstrates symptoms at school age or adult hood. The wide spectrum of symptoms andunspecific signs frequently simulate other diseases. Gastrointestinal hemorrhage is the most note worthy complication, which can cause severe anemia and shock. In the case we describe an 11-year-old girl experienced massive intermittent intestinal hemorrhage, anemic syndromeand intense abdominal pain over 15 months. In this patient,hemoglobin levels rose up to 6 g/dL necessitating several hemotransfusion. Multiple diagnosis and examinations were carried out until finally the gammagram with Tc99m disclosed an ectopic gastric mucosa. Usinga laparotomy an intestinal duplication was found in the terminal ileum. In this paper the intestinal duplication is analyzed and the importance of considering it as a differential diagnosis in any kind of bleeding ofthe digestive tube is discussed (AU)

Neuroprotection in selective focal ischemia in rats by nitrates, an alternative redox manipulation on nitric oxide: experimental model.

The recent advances in the histopathology of ischemia have set forth new proposals, particularly in regard to excitotoxicity by the glutamate receptor, NMDA. The participation of the nitric oxide (NO) in normal and pathological conditions and its relationship with toxicity in ischemia, suggest new alternatives for the modulation of the NMDA receptor REDOX site through its pharmacologic manipulation. This event would potentially limit the consequences of the activation-calcium flow and the production of peroxoinitrite during the ischemic phenomenon. The present work delivers two proposals: 1) A modified technique to the ones that have been described, of endovascular, without craniectomy, for experimental cerebral ischemia in Wistar rats, and with particular harmful effect upon the hippocampus. 2) It promotes the use of nitrates as an additional alternative to other elements, in order to restrict excitotoxicity in the described experimental cerebral ischemia, and paying attention to CA1-CA2 of the hippocampus. This area, specially sensitive to hypoxia-ischemia, offers an excellent study option for focal, experimental, cerebral ischemia associated with toxicity mediated by excitatory amino acids, since it stores an important concentration of NMDA receptors (R1/R2 A) as well as endothelial nitric oxide synthase. Our parameters are supported by quantitative-qualitative cell analysis, and not by the extension of the stroke which offers a more objective perspective upon the assessment of the focal ischemic event. By means of this technique, these results confirm the extent of the ischemic injury to the cell at the level of the hippocampus compared to a control/basal group, P = 0.0006. Furthermore, it suggests a neuroprotective effect of isosorbide dinitrate since it preserves the viable cells, and limits the appearance of hypoxic-ischemic cells at the hippocampus when the middle cerebral artery (MCA) is occluded endovascularly, as compared to the animals with no treatment P = 0.0080. However, other research lines are needed to compare the efficacy of this present work with other therapeutic proposals.