[Clinico-pathological and molecular aspects of diagnostic and prognostic value in gliomas]. / Aspectos clinicopatologicos y moleculares de valor diagnostico y pronostico en gliomas.

INTRODUCTION: Diffuse infiltrative gliomas, the most common primary brain tumours, account for almost 80% of malignant brain tumours. 60-70% of gliomas are astrocytic and over 80% of these tumours is considered high grade malignancy (grade III and IV) according to current World Health Organization classification. Infiltrating gliomas include diffuse astrocytomas, oligodendrogliomas and oligoastrocytomas. AIM: To review the clinical and histological features of cerebral gliomas, and molecular alterations that add relevant information for novel approaches in diagnosis, prognosis and treatment. DEVELOPMENT: The current gold standard diagnosis of these tumours relies on histopathological classification, which provides a grading of malignancy as a predictor of biological behaviour. However emerging molecular abnormalities have been discovered in the last years and these molecular changes are playing an increasingly prominent role as predictive biomarkers or in the development of diagnostic and prognostic. Now the neuropathologist is in crossroads between pathology and molecular biology and he plays a significant role in implementation of treatments and/or clinical trials. CONCLUSIONS: The study of proteomics and molecular biomarkers should complement the histopathological analysis and sometimes allows to determine direct or indirect predictive factors as well as the study of affected pathways which may become selective therapeutic targets.

[Reversible segmental cerebral vasoconstriction: the value of duplex transcranial Doppler in its diagnosis and follow up]. / Vasoconstriccion segmentaria cerebral reversible: utilidad del duplex transcraneal en el diagnostico y seguimiento.

INTRODUCTION: Reversible segmental cerebral vasoconstriction, also known as Call Fleming syndrome, is a clinical entity that consists of segmental vasoconstriction of the arteries in the brain, which can course with focal neurological deficits and which is characteristically reversible. Diagnosis and follow up of reversible segmental cerebral vasoconstriction is performed using cerebral arteriography, but no previous evaluation has been conducted of the role that duplex transcranial Doppler can play in this syndrome. CASE REPORT: Here we describe the case of a 57 year old female patient who began suffering from sudden bouts of headaches with no other accompanying clinical features. After ruling out the possibility of a subarachnoid haemorrhage by means of a spinal tap, a suspicion diagnosis of reversible segmental cerebral vasoconstriction was made through duplex transcranial Doppler and this was later confirmed by arteriography, which revealed a segmental stenosis in the upper branch of the left middle cerebral artery. Following treatment with nimodipine, the patient presented a clear clinical improvement and the reversibility of the cerebral vasoconstriction was confirmed by duplex transcranial Doppler. CONCLUSION: Contrast enhanced duplex transcranial Doppler would enable us to obtain an early diagnostic approximation in patients in whom segmental vasoconstriction is suspected; it could also constitute the choice non invasive follow up method in these patients.

[Central nervous system tumours in childhood: their clinical pathological aspects]. / Tumores del sistema nervioso central en la infancia: aspectos clinicopatologicos.

AIMS: Paediatric tumours affecting the central nervous system (CNS) constitute the second most frequent group of tumours at this age. Taking the WHO 2000 classification as our starting point, our intention was to describe the more important clinical and pathological features in the differential diagnosis of the different tumourous entities with the highest incidence in childhood. We highlight, above all, the characteristics that justify the need for a smooth flow of information between neurologists, neurosurgeons, neuroradiologists, neuropathologists and oncologists. We do not deal with familial tumourous syndromes, genetic aspects or clinical information derived from analyses of molecular alterations. DEVELOPMENT: Among CNS tumours, enough age related differences exist to be able to consider those appearing during childhood in their own right. Their topographic specificity is very characteristic and while 50% of them are infratentorial, 90% of those that occur in adults are supratentorial. Embryonic tumours are very frequent in childhood, but rare in adults, and the opposite happens with meningiomas. They are also different as regards their histological features, clinical characteristics, the early tendency to spread throughout the nervous system in the course of the disease and their biological behaviour. These data make us think that, in the pathogenesis of brain tumours in children, the molecular and epigenetic factors involved are different from those at play in the case of adults. CONCLUSIONS: A correct diagnosis requires a multidisciplinary approach and an understanding of the histological criteria and nomenclature by the health professionals involved in treating these patients.

[Usefulness of arteriography with multislice spiral computed tomography in the diagnosis of preocclusive stenosis of the cervical internal carotid artery]. / Utilidad de la arteriografía con tomografía computarizada helicoidal multicorte en el diagnóstico de la estenosis preoclusiva de la carótida interna cervical.

INTRODUCTION: Distinction between near-occlusion and occlusion of the internal carotid artery (ICA) is relevant because patients can benefit from a surgical treatment in the first case. Digital subtraction angiography (DSA) is the best neuroimaging technique for its diagnosis but the possibility of related neurologic complications suggests the use of non-invasive tests. Ultrasound (US) and magnetic resonance angiography (MRA) have been proposed as alternative techniques of diagnosis. But these can provoke an erroneous diagnosis when a near occlusion of ICA is present. Multislice helicoidal computed tomographic angiography (MHCTA) can play a role in these situations. CASE REPORTS: Case 1: patient diagnosed of occlusion of the left ICA by cervical US. Cerebral magnetic resonance suggested severe stenosis of intracavernous ICA. MHTCA showed carotid permeability. Case 2: patient diagnosed by cervical US of near occlusion and of occlusion/pseudoocclusion by enhanced RMA. MHCTA showed filiform patency of the cervical ICA. Case 3: a cervical US diagnosed moderate stenosis of the right ICA and severe of the left. Transcranial Doppler suggested near occlusion of the right ICA. MHTCA confirmed near occlusion of the right ICA. CONCLUSION: MHCTA correctly diagnosed near occlusion in three patients in which minimally invasive techniques had discrepancies. MHTCA is a new diagnostic technique whose lack of dependence of flow makes a good alternative to confirm findings of US of the cervical ICA.

Adult-onset subacute sclerosing panencephalitis: clinico-pathological findings in 2 new cases.

Subacute sclerosing panencephalitis (SSPE), an uncommon disease usually affecting children and adolescents, is caused by persistent measles infection that progresses to chronic infection with fatal outcome. The debut of this disease in adults is rare, with a small number of cases in the medical literature. This article presents the clinical, radiologic and post-mortem neuropathologic findings in 2 new cases of women with SSPE (1 of them during pregnancy), which showed very atypical clinical characteristics, presentation and evolution. The influence of pregnancy on the course of the disease was unfavorable, in keeping with earlier reports. Our patients showed a very prolonged biphasal clinical course, with a period of disease-free remission that lasted several years. Histological study disclosed features of inflammatory disease associated with others of a neurodegenerative nature, such as the formation of neurofibrillary tangles, which would relate SSPE with other tauopathies.

Síndrome de Eagle: hallazgos por tomografía computarizada / Eagle's syndrome: findings from computerised tomography scans

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[Study of the hypoplasic internal carotid artery by use of multislice spiral computed tomography. Two case reports]. / Estudio de la arteria carótida interna hipoplásica mediante tomografía computarizada helicoidal multicorte. A propósito de dos casos.

INTRODUCTION: Hypoplastic internal carotid artery (HICA) is a rare benign congenital malformation. Its angiographic image string sign is shared by entities that may be either medically or surgically treated. We report two cases diagnosed by helical computerized tomography (HCT). CASE REPORTS: Case 1: woman diagnosed clinically and by magnetic resonance (MR) of Chiari I malformation with associated syringomyelia. An HCT of the craniocervical junction was done to rule out osseous malformations. An hypoplastic posterior fossa (PF) with small right carotid foramen was diagnosed. An arteriography with HCT was done that showed the carotid string sign. Case 2: a 82 years old hypertensive woman with left hemiparesia and homonymous hemianopsia was diagnosed of right temporooccipital infarct. An HCT arteriography disclosed occlusion of the right posterior cerebral artery, calcification of the left carotid siphon and stenosis of the right siphon. Bi and tridimensional reconstructions of the circle of Willis, cranial base and distal cervical carotid arteries showed an hypoplastic right carotid artery and foramen. CONCLUSIONS: Multislice HCT is a recently incorporated diagnostic tool that allows a volumetric study in a short period of time, seconds. An angiographic study can be done intravenously in cases of vascular anomaly suspiction, hypoplastic carotid artery in the reported cases. HCT is the only current imaging tool that can diagnose this anomaly without the aid of other imaging studies.

Estudio de la arteria carótida interna hipoplásica mediantetomografía computarizada helicoidal multicorte. A propósito de dos casos / Study of the hypoplasic internal carotid artery by use of multislice spiral computed tomography. Two case reports

Introducción. La hipoplasia de la arteria carótida interna es una malformación congénita de presentación infrecuente. Su presencia se sospecha generalmente en el curso de un estudio angiográfico. La confirmación de la anomalía se realiza demostrando la presencia de un canal carotídeo hipoplásico en la base del cráneo con una tomografía computarizada (TAC). Presentamos dos casos diagnosticados con una tomografía computarizada helicoidal multicorte (TCHM). Casos clínicos. Caso 1: mujer diagnosticada clínicamente y mediante una resonancia magnética (RM) de malformación de Chiari I con siringomielia asociada. Se realizó una TCHM de charnela cervicoccipital para descartar malformaciones óseas. Se diagnosticó como hipoplasia ósea de fosa posterior con canal carotídeo derecho de pequeño tamaño. Se practicó una arteriografía con TCHM que mostró la imagen de una hipoplasia carotídea. Caso 2: mujer hipertensa de 82 años con hemiparesia y hemianopsia homónima izquierdas. La TAC convencional mostró un infarto temporoccipital derecho. Una arteriografía con TCHM mostró una oclusión de la arteria cerebral posterior derecha, calcificación del sifón carotídeo izquierdo y estenosis del derecho. Se realizaron reconstrucciones bi y tridimensionales del polígono de Willis, base del cráneo y carótidas cervicales distales que demostraron una carótida derecha y un foramen carotídeo hipoplásicos. Conclusión. La TCHM es una técnica radiológica de incorporación reciente que permite un estudio volumétrico en un período corto, en segundos. En las situaciones en que se sospecha una anomalía vascular se pueden realizar estudios arteriográficos por vía venosa, y se pueden demostrar las anomalías óseas asociadas con una sola adquisición de datos (AU)

Neonatal tumors of the CNS: a report of 9 cases and a review.

Neonatal central nervous system (CNS) tumors are an uncommon and histologically heterogeneous group of neoplasms with different clinical and biological features from those arising in childhood. We report 9 cases in which a diagnosis of CNS tumor was confirmed by biopsy or autopsy during the years 1982-1997 in the Vall d'Hebrón Children's Hospital, Barcelona. Two cases were fetal tumors detected by fetal sonography, 3 patients were symptomatic in the first days after birth and 4 patients presented initial clinical signs in the first weeks or months of life. Eight lesions were supratentorial and 1 was located in the spinal cord. According to histologic types, there were 2 glioneuronal tumors, 1 anaplastic astrocytoma, 1 choroid plexus carcinoma, 1 immature teratoma, 1 craniopharyngioma, 1 hemangioblastoma, 1 astroblastoma and 1 hemangioendothelioma. Extensive review of the literature indicates that our cases of hemangioblastoma, astroblastoma and hemangioendothelioma are exceptional and one more of the very rare and isolated previously published cases.

[Disseminated acute encephalomyelitis with fatal evolution: clinicopathological study]. / Encefalomielitis aguda diseminada de evolución fatal: estudio clinicopatológico.

INTRODUCTION: Disseminated acute encephalomyelitis is a monophasic demyelinating disease which progresses rapidly and is often fatal. It is an autoimmune condition, mediated by T lymphocytes, in which the immune response is directed against the myelin antigens. CLINICAL CASE: We describe the clinical, radiological and neuropathological findings in the case of a 31 year old woman who, ten days after complaining of a clinical condition of upper respiratory tract inflammation, presented with unilateral focal neurological signs, subsequent rapid deterioration of consciousness and death. On autopsy the neuropathological characteristics of disseminated acute encephalomyelitis were seen. CONCLUSIONS: The relative rarity of this condition at the present time makes clinical diagnosis difficult. The differential diagnosis with other conditions may be difficult also. Thus, this disease often leads to a neuropathological diagnosis.

Encefalomielitis aguda diseminada de evolución fatal: estudio clinicopatológico / Disseminated acute encephalomyelitis with fatal evolution: clinicopathological study

Introducción y caso clínico. La encefalomielitis aguda diseminada es una enfermedad desmielinizante, monofásica, rápidamente progresiva y frecuentemente fatal, de carácter autoinmune mediada por linfocitos T, en la que la respuesta inmunológica se dirige contra los antígenos de la mielina. Describimos los hallazgos clínicos, radiológicos y neuropatológicos en el caso de una mujer de 31 años que tras un cuadro inflamatorio de vías altas presentó, 10 días después, signos neurológicos focales unilaterales y posteriormente afectación rápidamente progresiva del nivel de conciencia que le llevó al éxitus. El estudio autópsico demostró características neuropatológicas de encefalomielitis aguda diseminada. Conclusiones. La relativa poca frecuencia de esta enfermedad en la actualidad y el hecho de que el antecedente infeccioso puede pasar desapercibido, dificultan el diagnóstico clínico. Junto a esto cabe tener en cuenta que el diagnóstico diferencial con otras entidades puede ser difícil. Todo ello hace que esta enfermedad represente con frecuencia un diagnóstico neuropatológico (AU)