R Palencia

Hospital Clínico Universitario de Valladolid, Valladolid, Castille and León, Spain

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Publications (21)17.15 Total impact


  • No preview · Article · Jan 2011 · Revista de neurologia
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    ABSTRACT: The association of persistent embryonic arteries and the absence of 1 carotid or vertebral arteries with facial or neck hemangioma or vascular malformation have been frequently described. The abnormalities can involve major or minor vessels. Of 22 patients of our series with this neurocutaneous syndrome, 20 had the origin of both anterior cerebral arteries from the same internal carotid artery. Thirteen patients showed absence or hypoplasia of 1 carotid artery and 10 of 1 vertebral artery; 10 showed persistence of the trigeminal artery; 3 had persistent proatlantal artery; 6 showed the absence of the posterior communicating artery; and 4 had hypoplastic posterior cerebral artery. Other less frequent abnormalities were found in 7 patients. Intellectual level of most patients was either borderline or below normal. Abnormalities in the vascularization and perfusion of the frontal lobes may contribute to the borderline or lower mental level of these patients.
    No preview · Article · Oct 2009 · Journal of child neurology
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    ABSTRACT: Plexiform neurofibroma in any location is one of the commonest complications associated with neurofibromatosis type 1 (NF1). Plexiform neurofibroma of the upper eyelid and orbit is usually associated with ipsilateral hemifacial hyperplasia. We present four patients with NF1 and plexiform neurofibroma of the eyelid and orbit associated with hemifacial hyperplasia, who also showed hyperplasia of the unilateral cerebral hemisphere. There are four patients, three females and one male, who consulted because of NF1 with plexiform neurofibroma of upper eyelid and hemifacial hyperplasia. Upper eyelid involvement was observed since birth and progressed during the first years of life. The patients showed normal neurological and mental development without motor or cerebellar disorders. Magnetic resonance studies demonstrated the asymmetric hyperplasia of the ipsilateral hemisphere in all four cases and of the cerebellar hemisphere in one case. The degree of hemispheric hyperplasia was related to the size and extension of the plexiform neurofibroma, as well as to the severity of the hemifacial hyperplasia. In our case which had the plexiform neurofibroma extended to the neck and the upper thorax, the hyperplasia not only affected the cerebral hemisphere but also the ipsilateral cerebellar hemisphere. All parts of the hemisphere showed increased size. The cortex of the entire hemisphere showed normal differentiation of the subcortical white matter. NF1 appears to be related with facial and cerebral ipsilateral hemihyperplasia. The relation between the size and extension of the orbital, eyelid and facial plexiform neurofibroma and the degree of asymmetry of the hemispheric hyperplasia suggest that different influences of a still unknown agent, possibly a gene, obviously related to NF1, causes both the intracranial and extracranial abnormalities.
    No preview · Article · Sep 2006 · Revista de neurologia
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    ABSTRACT: To describe the clinical, diagnostic and therapeutic features of this angiomatous neurocutaneous syndrome, which is the most frequent one, and to report a personal series of 41 patients. Forty one patients--31 females and 10 males--were studied during childhood and then, several patients were followed during many years, which allowed us to learn about the evolution of the abnormalities. The cutaneous lesions were classified as hemangiomas in 30 patients (73%) and as vascular malformations in 11 patients (27%). A cerebellar anomaly (unilateral hemispheric hypoplasia and Dandy-Walker malformation) was seen in 13 patients (31.5%) cerebral cortical dysplasia in 4 patients (10%), aortic arch coarctation in 6 patients (15%), and congenital cardiopathy in 5 patients (12%). The most frequent abnormalities were intracranial and/or extracranial vascular malformations. Persistence of the trigeminal artery was observed in 7 patients (17%), absence or severe hypoplasia of an internal carotid artery in 13 patients (32%), absence of a vertebral artery in 7 patients (17%), hypoplasia of intracranial arteries in 6 patients (15%) and aneurysmal enlargement of carotid or vertebral arteries in 5 patients (12%). Also were observed 4 patients (10%) with intracranial hemangioma, 2 (5%) with hemangioma in mediastinum, and 3 (7.5%) with intestinal hemangioma, all of which disappeared during the first years of life. Aneurysmal enlargement of the carotid and vertebral arteries and intracranial branches also disappeared after a process of progressive narrowing of the arterial lumen that caused complete obstruction of these arteries. At the same time the cutaneous hemangioma regressed. During this process, collateral vascularization through branches of the external carotid artery and of the non-affected branches of the contralateral intracranial arteries developed. This neurocutaneous syndrome is the most frequent one and it is associated with several types of vascular and non-vascular abnormalities which can involve any organ of the body. Internal and external hemangiomas and hemangiomatous lesions progress and tend to regress concomitantly.
    No preview · Article · Aug 2005 · Revista de neurologia
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    ABSTRACT: To present 18 cases of Kabuki make-up syndrome with the associated morphological and neurological alterations. The series consists of 18 patients, 14 males and 4 females, who show the peculiar facial morphology of this disease. Ten cases were studied between 1968 and 1978, before the description of the syndrome. Most of these patients are now over 30 years and two perhaps surmounting the 40, if surviving. The other 8 cases were studied during the last 20 years (a few within the last 3 years). The studies of the patients were anamnesis with genetic history, EEG, image, cytogenetic, laboratory studies, and mental evaluation when the patients collaborated. The most important findings are the peculiar facial characteristics as well as the psychomotor and language retardation, the persistent fingertip pads and clinodactyly that were observed in all patients. Other studies, such as EEG, caryotype, biochemical investigation in blood, urine and CSF (in the studied cases), and the cerebral neuroradiological images (pneumoencephalography, CT or MR) disclosed normal results. None of the patients of this series were neurologically normal. Hyperactivity, respiratory infection and/or otitis were frequently observed. Kabuki make-up syndrome is a dysmorphologic and neurological disease of unknown origin. Its main manifestations involve facial appearance and brain function without anatomical anomalies. None of the patients was sent to the Pediatric Neurology service with the suspicion of Kabuki make-up syndrome.
    No preview · Article · Apr 2005 · Revista de neurologia
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    ABSTRACT: Objective. To show the disorders of the brain cortical development and the possible origin in base to a large series studied in a Pediatric Neurology service. Patients and methods. A series of 144 children with ages ranging between newborn and 12 years was studied from the clinic, image (MR, 3DMR) and evolutive point of views. Results. The diagnosis was: polymicrogyria in 61 cases, lissencephaly in 22, eschizencephaly in 16, heterotopia in 16, cortical dysplasia in 9, hemimegalencephaly in 8, cobblestone in 7, sublobar dysplasia in 3, and 'double cortex' in 2. Mental retardation, motor disorders and epilepsy were the most important anomalies. Conclusions. Actually, the image is the most important study to make the diagnosis of every type of cerebral malformation. However, to know the specific gene that origin every disorder seems to be the most important thing to make the classification of every malformative type and the possible prevention of this pathology.
    No preview · Article · Aug 2003
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    ABSTRACT: To show the disorders of the brain cortical development and the possible origin in base to a large series studied in a Pediatric Neurology service. A series of 144 children with ages ranging between newborn and 12 years was studied from the clinic, image (MR, 3DMR) and evolutive point of views. The diagnosis was: polymicrogyria in 61 cases, lissencephaly in 22, eschizencephaly in 16, heterotopia in 16, cortical dysplasia in 9, hemimegalencephaly in 8, cobblestone in 7, sublobar dysplasia in 3, and 'double cortex' in 2. Mental retardation, motor disorders and epilepsy were the most important anomalies. Actually, the image is the most important study to make the diagnosis of every type of cerebral malformation. However, to know the specific gene that origin every disorder seems to be the most important thing to make the classification of every malformative type and the possible prevention of this pathology.
    No preview · Article · Aug 2003 · Revista de neurologia
  • R Palencia
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    ABSTRACT: The brain needs a constant supply of oxygen and nutrients from blood. This fact explains its tight dependence on the cardiovascular system as well as the after effects of heart disease on the central nervous system. This study intends to describe the main conditions in which a neurological disorder appears secondarily to or coincidentally with heart disease. Methods. A bibliographical review of publications of neurological disorders related to heart disease is carried out. The main cardiac diseases that can induce neurological disorders include: disturbances of the cardiac rhythm, congenital heart diseases, and complications of cardiac catheterisation and reparative surgery. Likewise, other conditions congenital or acquired in which neurological and heart disorders coexist must be considered. Medical practice confirms that heart diseases account for different neurological conditions that can manifest by a very variable clinical expressivity.
    No preview · Article · Aug 2002 · Revista de neurologia
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    ABSTRACT: We describe a series of 13 patients, ten males and three females, with ages ranging between three and fourteen years, who show unilateral polymicrogyria (in the left cerebral hemisphere in four cases and in the right in nine). The first and the main clinical alteration in all 13 cases was hemiparesis that did not change along the years. Ten patients had seizures that were completely controlled in seven. Three cases never presented seizures. Spontaneous threat of abortion or arterial hypertension during the first five months of pregnancy occurred in five cases. Weight less than 3 kg at birth was observed in six cases. The only patient who shows severe mental retardation associated with deafness is a girl who had infantile spasms at 20 days of age and presents duplication of a short arm of the chromosome X. Conventional magnetic resonance (MR) studies, performed in four cases did not disclose polymicrogyria but only an enlarged cortex that was diagnosed as cortical dysplasia. Three-dimensional MR (3DMR) images are very important not only to see the polymicrogyria, but also its extension and severity, especially if, moreover the axial, coronal and sagittal views, oblique, frontal, occipital, basal and superior images of the hemispheric cortical surface are performed.
    No preview · Article · Aug 2001 · Brain and Development
  • R Palencia
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    ABSTRACT: To carry out, by means of a literature review, an update of the entities that can be included within the group of benign partial epilepsies of childhood. Among partial epilepsies with onset in the first stages of life, a group extended in the last years with a favourable course and a trend to reverse, even spontaneously, has been identified. Some of these entities have a genetic origin but we do not know the mechanisms by which these epilepsies show a self limited course, which have given rise to the denomination of epilepsy that comes and goes; nevertheless, an evolution to other more complex forms is also possible. Benign partial epilepsies of childhood constitute a wide group of conditions of varied semiology, usually with a good prognosis even without treatment. Occasionally, these epilepsies may show a more unfavourable course with a worsening, in spite of medication, and the appearance of neurologic and neuropsychologic disorders. All these aspects must be known and considered by the physician in charge of these patients management.
    No preview · Article · Jan 2001 · Revista de neurologia
  • R Palencia
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    ABSTRACT: INTRODUCTION: Data coming from the literature regarding the frequency of epilepsies in childhood are different, depending on the methodology used, and it is noteworthy that there exist very few studies conducted among us. DEVELOPMENT: Factors influencing the disparities of the figures found in the literature about incidence and prevalence of epilepsies are analyzed; among them, the diversity of the diagnostic criteria employed (sometimes not reported, other times imprecise or with inclusion of non epileptic episodes), the methodology used in the study for data collection (hospitals, surveys, reviews, follow-ups), and the study population are prominent. Annual incidence rates that have been published range between 11/100,000 and 145/100,000, being the majority between 27/100,000 and 70/100,000. Estimations about prevalence range between 0.8/1,000 and 49/1,000, which in the school population among us means 5.7/1,000.
    No preview · Article · Jul 2000 · Revista de neurologia
  • R Palencia
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    ABSTRACT: The presence of myoclonus in a patient has different meanings: there exist myoclonus without encephalopathy or epilepsy (sleep myoclonus), encephalopathies with non-epileptic myoclonus (Kinsbourne's syndrome), encephalopathies with epileptic myoclonus (progressive or not), epileptic encephalopathies with myoclonic seizures (the classic West and Lennox-Gastaut syndromes) and myoclonic epilepsies. Main types of myoclonic epilepsies (benign childhood myoclonic epilepsy, severe-polymorphic-myoclonic epilepsy, juvenile myoclonic epilepsy, childhood familial myoclonic epilepsy, benign reflex myoclonic epilepsy, as well as progressive myoclonic epilepsies, among which progressive myoclonic epilepsy (PME) type 1 (Unverricht-Lundborg's disease) and PME2 (Lafora type) are prominent, whereas other entities, such as those related to neuronal ceroid lipofuscinosis or mitochondrial cytopathies are discussed more briefly) are reviewed, analyzing clinical, EEG and therapeutic issues, while the most recent contributions in the field of genetics are considered. Myoclonic epilepsies constitute a very heterogeneous type of epilepsy, both in their origin and in their prognosis, with favorable forms of course along with other progressive and refractory forms with an ill-fated prognosis.
    No preview · Article · Jul 2000 · Revista de neurologia
  • R Palencia · M I Sinovas
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    ABSTRACT: The object of this study was to determine the prevalence of migraine in school children in our area, since few such studies have been done. The study was carried out by means of a questionnaire given to school children aged between 6 and 14. We selected a representative sample of the population, the size of which was determined by accepting as a reference value the 4% mentioned by Bille. The questionnaire was drawn up after a search of the literature for similar questionnaires and a preliminary trial on children known to suffer from migraine (giving a specificity of 96.6% and a sensitivity of 96.5%). Using the criteria of Vahlquist there was a prevailence of 7.0% and with IHS of 6.7%. There was a predominance (not significant) of females (54.4%). In 89.1% there was a positive family history (parents and/or siblings) of migraine.
    No preview · Article · Dec 1997 · Revista de neurologia
  • R Palencia · M I Sinovas

    No preview · Article · Jul 1997 · Anales espanoles de pediatria
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    ABSTRACT: To describe the vascular and nonvascular intracranial and extracranial anomalies associated with hemangiomas and vascular malformations of the face, neck, and/or chest. Seventeen patients had a physical examination and imaging studies consisting of one or more of the following: pneumoencephalography, conventional carotid and vertebral arteriography, CT, MR imaging, and MR angiography. Conventional arteriography revealed persistence of the trigeminal artery in 5 cases, absence of internal or external carotid and/or vertebral arteries in 11 cases, persistence of intervertebral arteries in 1 case, deformities of the aortic arch in 3 cases, and anomalies of the intracranial arteries in 3 cases. MR angiography revealed persistence of the trigeminal artery in 1 case in which conventional arteriography failed to show the malformation, and permitted visualization of narrowing of the intracranial arteries. CT and MR imaging showed a cerebellar anomaly in 8 cases and cerebral cortical dysplasia with cerebral hemispheric hypoplasia in 1 case. Vascular and nonvascular anomalies appeared ipsilateral to the external vascular abnormalities in most cases. This study demonstrates the association of cutaneous angiomas with anomalies affecting intracranial and extracranial arteries, the cerebellum, and, less frequently, the cerebral hemispheres and aortic arch. This association constitutes a relatively frequent neurocutaneous disorder, which we call the cutaneous hemangioma-vascular complex syndrome.
    Preview · Article · Apr 1996 · American Journal of Neuroradiology

  • No preview · Article · Dec 1990 · Anales espanoles de pediatria
  • R Palencia

    No preview · Article · Dec 1990 · Anales espanoles de pediatria
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    ABSTRACT: Aim. To present 18 cases of Kabuki make-up syndrome with the associated morphological and neurological alterations. Case reports. The series consists of 18 patients, 14 males and 4 females, who show the peculiar facial morphology of this disease. Ten cases were studied between 1968 and 1978, before the description of the síndrome. Most of these patients are now over 30 years and two perhaps surmounting the 40, if surviving. The other 8 cases were studied during the last 20 years (a few within the last 3 years). The studies of the patients were anamnesis with genetic history, EEG, image, cytogenetic, laboratory studies, and mental evaluation when the patients collaborated. The most important findings are the peculiar facial characteristics as well as the psychomotor and language retardation, the persistent fingertip pads and clinodactily that were observed in all patients. Other studies, such as EEG, caryotype, biochemical investigation in blood, urine and CSF (in the studied cases), and the cerebral neuroradiological images (pneumoencephalography, CT or MR) disclosed normal results. None of the patients of this series were neurologically normal. Hyperactivity, respiratory infection and/or otitis were frequently observed. Conclusion. Kabuki make-up syndrome is a dysmorphologic and neurological disease of unknown origin. Its main manifestations involve facial appearance and brain function without anatomical anomalies. None of the patients was sent to the Pediatric Neurology service with the suspicion of Kabuki make-up syndrome. (REV NEUROL 2005; 40: 473-8)
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  • R Palencia

    No preview · Article · · Revista de neurologia
  • R Palencia
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    ABSTRACT: INTRODUCTION AND DEVELOPMENT: Although the presentation of paroxystic episodes usually suggests epilepsy, there are many varied clinical conditions which occur periodically and paroxystically and are due to different causes. The pediatrician has to remember this in order not to make an erroneous diagnosis and so as to avoid unnecessary administration of antiepileptic drugs (which will be of no benefit), and also to avoid the repercussions which the diagnosis of epilepsy may have in family and social affairs. Paroxystic episodes related to anoxia/hypoxia are, amongst non-epileptic disorders, some of the commonest occurring in infancy and may have different degrees of importance: together with transitory, benign conditions such as apnea of crying there are serious and even potentially life-threatening events such as some syncopes of cardiac origin. CONCLUSION: We review the main situations included in this section.
    No preview · Article · · Revista de neurologia