[Neuro-Behçet: clinicopathological findings in an autopsy case]. / Neurobehçet: hallazgos clinicopatológicos de un caso de autopsia.

INTRODUCTION: Behçet's disease (BD) was first described by Hulusi Behçet in 1937 as a triad of oral aphthae, genital ulcers and uveitis. It affects most of tissues and organs without exception and has a prevalence of neurologic involvement between 5 and 30%. Histopathological research of autopsy cases is limited, even though the cause of death is confirmed in this study. CASE REPORT: A 30-years-old man without prior medical history of interest debuting with a BD with progressive neurological manifestations and fatal evolution. The autopsy showed, in gross examination, oral and scrotal ulcers. Moreover, the brain revealed a marked cerebral edema, opaque leptomeninges and hemorrhagic infarction of the left frontal lobule extended to the homolateral ventricular cavity. In the brain stem, multiples hemorrhagic infarctions and hemorrhage in the fourth ventricle. The microscopic examination showed a generalized perivascular lymphocytic and neutrophilic inflammation in small and medium vessels with intense diapedesis bleeding. This phenomenon was predominant in brain stem. CONCLUSIONS: The necropsy studies of patients with neuro-Behcet's disease are scarce. We report the clinicopathological findings in a patient with BD and neurological involvement, in which stands out a cerebral vascular affectation predominant in brain stem.

Neurobehçet: hallazgos clinicopatológicos de un caso de autopsia / Neuro-behçet: Clinicopathological findings in an autopsy case

Introducción. La enfermedad de Behçet (EB) la describió por primera vez en 1937 Hulusi Behçet como la tríada deúlceras orales, úlceras genitales y uveítis. Involucra a casi todos los tejidos y órganos sin excepción, y la tasa de afectación del sistema nervioso central es del 5 al 30%. Los estudios histopatológicos de casos de necropsias son escasos, a pesar de que la causa de muerte se constata en este estudio. Caso clínico. Varón de 30 años que comenzó con EB con afectación neurológicade evolución progresiva y fatal. El estudio autópsico mostró en el examen externo úlceras orales y escrotales. El encéfalo presentaba marcado edema cerebral, meninges opacas y un infarto hemorrágico frontal izquierdo con extensión hacia el ventrículo lateral homólogo, y el tronco del encéfalo, infartos hemorrágicos confluentes y hemorragia del cuarto ventrículo. En el examen de los órganos restantes destacaban múltiples infartos pulmonares bilaterales. El estudio histológico del encéfalo mostró, de forma generalizada, predominante en el tronco del encéfalo, vasos de pequeño y mediano calibre rodeados por una corona de linfocitos y neutrófilos, con intensa extravasación eritrocitaria perivascular. Conclusiones. Los estudios denecropsias de pacientes con neurobehçet son escasos. Presentamos los hallazgos clínicos y patológicos de un paciente con EB con afectación neurológica, en el que destaca una afectación vascular cerebral predominante en el tronco del encéfalo

Introduction. Behçet’s disease (BD) was first described by Hulusi Behçet in 1937 as a triad of oral aphthae, genital ulcers and uveitis. It affects most of tissues and organs without exception and has a prevalence of neurologic involvement between 5 and 30%. Histopathological research of autopsy cases is limited, even though the cause of death is confirmed in thisstudy. Case report. A 30-years-old man without prior medical history of interest debuting with a BD with progressive neurological manifestations and fatal evolution. The autopsy showed, in gross examination, oral and scrotal ulcers. Moreover, the brain revealed a marked cerebral edema, opaque leptomeninges and hemorrhagic infarction of the left frontal lobuleextended to the homolateral ventricular cavity. In the brain stem, multiples hemorrhagic infarctions and hemorrhage in the fourth ventricle. The microscopic examination showed a generalized perivascular lymphocytic and neutrophilic inflammationin small and medium vessels with intense diapedesis bleeding. This phenomenon was predominant in brain stem. Conclusions. The necropsy studies of patients with neuro-Behçet’s disease are scarce. We report the clinicopathological findings in a patient with BD and neurological involvement, in which stands out a cerebral vascular affectation predominant in brain stem

[Is it too late to start orthopedic treatment for idiopathic scoliosis with Risser scores of 4?]. / Est-il trop tard pour entreprendre un traitement orthopédique pour scoliose idiopathique à Risser 4 ?

Orthopaedic treatment for idiopathic scoliosis in adolescence is indicated with a curve of > or =20 degrees and a Risser score< or =4, because progression of curve is low with Risser scores >4. We present the case of a young man with a left lumbar idiopathic scoliosis (T12-L4) with a curve of 10 degrees, which was stable from 13 years (Risser 0) to 16 years old (Risser 4). The scoliosis progressed quickly after a Risser score of 4 was achieved. The man had been wearing a lift on the left foot since he was 13, because of a leg length discrepancy, and had been under clinical and radiological monitoring. When the boy reached 17 years, the scoliosis rapidly progressed, to a curve of 22 degrees and a Risser score of >4. The scoliosis was effectively treated with a Boston brace. At 20 years, the Risser score was 5, and the left lumbar curve was 13 degrees after discontinuing the use of the brace. To our knowledge, no scientific reference indicates a time limit to orthopaedic treatment for idiopathic adolescent scoliosis. Despite the experts' recommendations, a brace might be indicated with a Risser score > or =4 to stop the progression of the curve.

[Sudden hearing loss and uveitis as a form of presentation of neurosarcoidosis]. / Sordera súbita y uveítis como forma de presentación de neurosarcoidosis.

Sarcoidosis is a multisystem granulomatous disease of unknown cause with frequent pulmonary, ocular, and lymphatic system manifestations. Central nervous system involvement, although rare (1 to 5%), most commonly affects the cranial nerves. Cranial nerve VIII abnormalities occur in 20% of these patients and are associated with other neuropathies and organ or system involvement. We report a case of neurosarcoidosis that presented as isolated sudden sensorineural hearing loss and uveitis.

AMO array multifocal versus monofocal intraocular lenses: long-term follow-up.

PURPOSE: To assess prospectively the visual outcomes of multifocal and monofocal intraocular lens (IOL) implantation in selected patients with a minimum follow-up of 18 months. SETTING: Department of Ophthalmology, National Health Service (INSALUD). Puerta de Hierro University Clinic, Madrid, Spain. METHODS: This prospective study comprised two groups of 50 eyes each: one with AMO Array multifocal IOLs and the other with monofocal IOLs. Main outcome measures were Snellen and Regan 96% distance visual acuities; Jaeger and decimal near acuities; Regan contrast sensitivity test at 96, 50, 25, and 11%; brightness acuity test (BAT) on distance acuity at Regan 96%; depth of focus measured with the Regan 96% distance test; defocusing from +3s to -4s; subjective evaluation of optical side effects. RESULTS: The differences between groups in mean distance vision and BAT results were not statistically significant Contrast sensitivity results at 96 and 50% were similar, but there were significant differences of 0.94 and 1.14 lines on the 25 and 11% charts, respectively (P < .01). Near vision without addition in the multifocal group was significantly better and depth of focus significantly wider than in the monofocal group (P < .001). CONCLUSION: With appropriate patient selection, the AMO Array IOL provided distance acuity and contrast sensitivity similar to that of the monofocal IOLs and excellent intermediate vision. Near vision, without spectacles, with the multifocal lens was functionally acceptable although short of refractive power for best acuity results.