Evolución de tumores hipofisarios en miembros de una familia con síndrome de neoplasia endocrina múltipletipo 1 / Different outcome of pituitary tumors within members of a family with multiple endocrine neoplasia type 1

Se describe la evolución de prolactinomas en 2 pacientes de una misma familia con síndrome de neoplasia endocrina múltiple tipo 1. El diagnóstico se realizó precozmente en dos hermanos con edades de 21 y 14 años, sin síntomas, mediante técnicas de cribado hormonal y genético, y se confirmó mediante resonancia magnética de la hipófisis. Las concentraciones plasmáticas de prolactina se normalizaron en ambos pacientes tras tratamiento con agonistas dopaminérgicos. Sin embargo, mientras en uno de los pacientes la masa tumoral llegó a desaparecer totalmente, en el segundo siguió creciendo por lo que se requirió cirugía transesfenoidal (AU)

[Segmental arteriographic anomalies in migranous cerebral infarct]. / Anomalías arteriográficas segmentarias en el infarto cerebral migrañoso.

A migranous cerebral infarct (ICM) has the appearance of an ischaemic cerebral lesion which occurs during a migraine crisis and is shown by neuroimaging techniques to be in the corresponding vascular area. Four young patients are described. They fulfilled the clinical and neuroimaging criteria for compatibility with ICM and the protocol for the aetiological study of stroke was normal. Angiography ruled out other causes of ischaemia (dissection, fibromuscular dysplasia, etc.) and showed segmental images of 'vasculitis appearance' similar to these described in Call's syndrome, in 'benign intracranial angiopathy' and in other intracranial vasculopathies or criteria of 'reversible segmental cerebral vasoconstriction'. The pathogenic significance, as with migraine, is still unknown.

[Syndromes involving rostral occlusion of the basilar artery: a preliminary study and proposal for a new clinical and neuro- radiological classification]. / Síndromes por oclusión rostral de la arteria basilar: estudio preliminar y propuesta de una nueva clasificación cliniconeurorradiológica.

Occlusion of the top of the basilar artery causes infarctions in supra- and infra-tentorial regions (thalamus, occipito-temporal lobes, rostral trunk and cerebellum) with characteristic clinical and radiological manifestations. We studied 17 patients with this syndrome whose clinical data and neurological images led us to classify them into four groups: type I (2 patients, 12%), showing mainly alterations in consciousness and ocular motricity, and bilateral thalamic infarction; type II (6 patients, 35%), with campimetric manifestations and uni- or bilateral ischemia of the occipito-temporal lobes; type III (5 patients, 29%), with associated corticospinal deficits and lacunar images in the rostral brainstem; and type IV (4 patients, 24%) with symptoms of and neuro-images revealing lesions in the cerebellum. The proposed classification is an attempt to facilitate diagnosis by neuro-imaging and to group patients according to prognosis.

[Syndrome of rostral occlusion of the basilar artery]. / Síndrome de oclusión rostral de la arteria basilar.

Upper occlusion of the basilar artery ("top of the basilar" syndrome) causes ischemics lesions in brainstem, thalamus and occipital and temporal lobes, producing visual, oculomotor and behavioral disorders, with slight motor affectation concomitant. We expose a case with a typical clinical setting and infarctions in thalamus and both occipital lobes with an unusual symmetric distribution.

[Heterotopic gray matter: an infrequent cause of epilepsy]. / Heterotopias de sustancia gris: una causa infrecuente de epilepsia.

Heterotopia of gray matter is a developmental malformation in which ectopic cortex is found in the white matter of the brain. A case of a 27-year-old woman with generalized convulsions is reported, including the results of cerebral computed tomography and magnetic resonance imaging. In this patient, nodular masses of gray matter were present in the periventricular region. We emphasize that magnetic resonance imaging is the current method of choice for diagnosing heterotopic gray matter.