Síndromes postencefalíticos en la literatura médica española / Post-encephalitic syndromes in the Spanish medical literature

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Historia de las algias neuropáticas / History of neuropathic pains

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La “dinámica cerebral” de Justo Gonzalo en la historia / “Brain dynamics” of Justo Gonzalo in history

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[The various meanings of the term 'omnipotence']. / La polisemia del vocablo 'omnipotencia'

DEVELOPMENT: In this paper, we briefly review the characteristics of the how the human psychobiological unit progresses in the early ages. The pre-eminence of the affective axis is underlined, the shades of narcissism, egocentrism and pseudo-omnipotence present in such early stages are highlighted, and emphasis is placed on how the morbid persistence of such shades throughout adult life can distort the subject's evaluations. This is more specifically so if they badly taint the subject's image of God. The author briefly analyzes the organic stratum underlying emotional affective life and discusses the relationships of the joint integration between the brain and the visceral world. The study also includes contributions by leading Spanish thinkers about the serene conception of the omnipotence of God and finishes by comparing the complexity of the brain with the interaction of the galaxies in the universe. CONCLUSIONS: The paper describes the psychobiological structure of the human personality, from its most archaic and childish positions, steeped in egocentrism and pseudo-omnipotence, and points out how they extend into adulthood. The frequency and the risk of projections of God (not abstract, not 'all-embracing') are also stressed, and the most up to date and serene theology is followed. It is pointed out that the complexity of the brain is greater than that of a system of galaxies.

Sobre el diagnóstico clínico de las formas espinales monotópicas de esclerosis múltiple. El valor del signo del abanico en el adulto / Regarding the clinical diagnosis of the "monotopical" spinal forms of multiple sclerosis. The value of the fan sign in the adult

Introducción. Se pretende exponer el valor de la historia y del examen clínico en la orientación diagnóstica de una probable esclerosis múltiple (EM). Caso clínico. Se aporta el estudio de una mujer de 42 años que en el curso de los últimos 10 años ha presentado tres episodios de parestesias, tipo hormigueos, en la extremidad inferior izquierda y algunas otras zonas del mismo hemicuerpo, nunca en el cuello ni en la cabeza, así como fatigabilidad. El examen clínico ambulatorio pone de manifiesto un síndrome de afectación de los segmentos altos de la médula espinal, lateralizado en gran parte a derecha, que dibuja un cuadro de Brown-Séquard, bien notoriamente imperfecto: exaltación de los reflejos musculares clínicos -o profundos-, el más elevado de los cuales era el deltoideo derecho (C5), signo de Babinski bilateral en abanico, Barré de la pierna positivo a derecha, hormigueos con disestesia a izquierda sin nivel preciso. Rendimiento funcional bien conservado comparativamente a la signología. Se orienta clínicamente el caso como una probable EM `monotópica'. Una RM espinal confirma esta impresión clínica. Conclusiones. Se resalta el valor del estudio clínico cuidadoso dirigido al diagnóstico de EM probable. Se resalta el valor de la disociación signología grave frente al rendimiento funcional desproporcionadamente bien conservado, así como la originalidad de la presencia de un signo del abanico bilateral como apoyo de una EM, tratándose de un proceso de la edad adulta (AU)

[On the duality of the sign of fanning of the toes]. / Sobre la probable dualidad del signo del abanico. Hipótesis clinicofisiopatológicas.

Starting from Babinski's original description, the author carries out a revision of semeiological value of the sign "fanning of the toes". It is considered that there are two clinical variants. The first, present in most cases of connatal encephalopathy, is due to involvement of the premotor area, which is usually accompanied by pyramidal, dystonic or athetoid signs. The second variant is present in cases of multiple sclerosis presumably attributable to involvement of the lateral spinal tracts including the reticulospinal one. The author proposes the need of using modern neuroimaging and electrophysiological techniques for further pathophysiological investigation of both clinical variants of the sign.

Sobre la probable dualidad del signo del abanico. Hipótesis clinicofisiopatológicas / On the duality of the sign of fanning of the toes

Partiendo de la descripción por Babinski del fenómeno de la abduction des orteils, el autor procura precisar el valor semiológico del "signo del abanico", en el que tal abducción acompaña a una extensión (flexión dorsal) del dedo gordo. Sugiere considerar dos variantes clinicofisiopatológicas de este signo: 1. Una presente en muchos casos de encefalopatía infantil de instauración connatal o prenatal, con compromiso del área premotora o de sus proyecciones, con la adjunción al síndrome piramidal de rasgos distonicoatetósicos -siquiera mínimos- de índole extrapiramidal.2. Otra, mucho menos frecuente, de aparición en el adulto, casi privativa de algunos casos de esclerosis múltiple, atribuible a la afección difusa de los cordones espinales, incluyendo el compromiso del haz reticuloespinal dorsal, con intensa liberación de los reflejos de defensa.El autor señala la oportunidad de aplicar las metodologías exploratorias actuales a la investigación de tales hipótesis clinicofisiopatológicas (AU)

[Regarding the clinical diagnosis of the monotopical spinal forms of multiple sclerosis. The value of the fan sign in the adult]. / Sobre el diagnóstico clínico de las formas espinales monotópicas de esclerosis múltiple. El valor del signo del abanico en el adulto.

INTRODUCTION: We wish to discuss the value of the clinical history and examination in orientation of the diagnosis of probable multiple sclerosis (MS). CLINICAL CASE: We report the two year study of a woman who over the previous ten years had had three episodes of paraesthesia, with pins and needles in her left leg and other parts of the left side of her body, although never affecting head or neck. She also complained of tiring more than usual. In an outpatient clinic she was found to have a syndrome affecting the upper segments of the spinal cord, mainly involving the right side and resembling an incomplete Brown Sequard type syndrome. There were increased clinical muscle and deep reflexes. The most marked was that of the right deltoid (C5), bilateral fanning of the toes when the Babinski reflex was tested, Barré positive in the right leg, pins and needles and dysaesthesia on the left to an undetermined level. Function was well preserved when compared with the clinical signs found. The case was considered to be of monotopical MS. Spinal magnetic resonance findings confirmed the clinical diagnosis. CONCLUSIONS: We emphasise the value of careful clinical investigation directed towards the diagnosis of probable MS. We draw attention to the diagnostic value of the dissociation between the severe clinical alterations and the functional performance, which was surprisingly well maintained. Also we report the originality of the presence of bilateral fanning sign supporting the diagnosis of MS, occurring in a disease of adult life.

[Frontal lobes: the executive brain]. / Los lóbulos frontales: el cerebro ejecutivo.

OBJECTIVE: To describe the current basic functional neuroanatomy of the frontal lobes and complex cognitive processes associated with this wide brain zone. DEVELOPMENT: We reviewed recent studies with neurofunctional interest. We structured the frontal zones and the cognitive functions more specifically humans, named 'executive functions'. We classified the frontal syndrome into more specific syndromes; and, we reviewed the fronto-cortical and subcortical connections, which are the basis of the frontal zones and functions. CONCLUSIONS: The frontal lobe is not a single anatomical and functional brain region. Regions and fronto-cortical and subcortical circuits within the frontal lobe are associated with motor functions and cognitive processes highly specialized, which may be differently affected.

[Congenital neuromyotonia. Retrospective study of 4 cases]. / Neuromiotonía congénita. Estudio retrospectivo de cuatro casos.

BACKGROUND: Fasciculation, double discharge, myokymia and neuromyotonia are different kinds of involuntary muscular activity that originate in ectopic discharges of the motor axons. Electrophysiological studies are needed in all cases for the diagnosis. Non rigorous electrophysiological studies in some cases is the cause of the historically unclear nosological delimitation of the neuromyotonic syndromes. OBJECTIVE: To report the clinical picture and electrophysiological findings in patients with congenital neuromyotonia. PATIENTS AND METHODS: Four patients with congenital neuromyotonia were studied. Electrophysiological exam included nerve conduction measurements, study of the after-discharges and conventional EMG. Spontaneous discharges were displayed after applying a low pass filter, signal trigger and delay line. RESULTS: In one case positive motor features predominate (continuous muscle fiber activity). On the contrary, two cases, showed neuropathic deficitary signs with a Charcot-Marie-Tooth type II disease phenotype; neuromyotonia was, in both cases, an electrophysiological feature. In the last patient, motor signs were limited to the facial muscles but electrophysiological study discovered generalized neuromyotonia. Treatment with carbamazepine or oxcarbazepine was useful in the four cases. CONCLUSION: Congenital neuromyotonia is a clinically heterogeneous syndrome with uniform electrophysiological features that permit its qualification.

[Pain]. / El dolor.

This text reports the 'introduction remarks' of Professor Ochoa at the inauguration of a Symposium on Pain at the Spanish Neurology Society. Profoundly, but as clearly as possible, the author expounded on the nociceptors to the cerebral cortex, the structures involved in senso-perception--and emotion--which are to be taken into consideration. Some basic facts are: 1. The functional characterization of different nociceptors. 2. The existence of usually 'silent' nociceptors which are only activated under certain conditions. 3. The enumeration of the zones of the posterior horn which are 'specific' for nociception: lamina I and the external zone of lamina II. 4. The great feature of lamina V, with a population of convergent neurones, which receive both tactile and nociceptive stimuli. 5. The contrast between the postero-ventral thalamic nucleus (touch, pain, etc.) and the internal or 'medial' thalamic sectors, which are involved in the most crude and aggravating pain and in the course of impulses which predispose to pathological emotional experiences. This thalamic sector does not generate pain when stimulated under normal conditions, but may do so when the patient has pain of central origin, in which case the condition--sometimes complex--of desafferentization may be assessed. We point out that both the classically named causalgia and the central algias (thalamic, due to pathology of the brainstem and cortico-subcortical lesions) have similar aspects: burning pain with paroxystic exacerbations, sensory defects, hyperpathia and adodynia. We also mention the complex inhibitory effects of impulses conducted by large diameter myelinated fibres or nociceptive effects at different levels. Reference was not made to centrifuge systems (endogenous opiates etc.) which inhibit pain. References 1, 3, 6 and 9 include a considerable quantity of bibliography.

[Delayed central pain associated with a sub-insular hematoma followed by parieto-occipital tumor. Favorable effects of chronic stimulation of the ventral posterior thalamic nucleus]. / Dolor central retardado asociado a hematoma subinsular seguido por tumor parietoccipital. Efecto favorable de la estimulación crónica del núcleo VPL talámico.

INTRODUCTION: Déjerine and Roussy reported thalamic syndrome, chronic pain after a vascular lesion in the thalamus, in 1906. Posterior clinical observations allowed know that the same clinical picture can be observed after lesions in other parts of the central nervous system. Due to the fact that the more frequent etiology is vascular, nowadays the term central poststroke pain syndrome is preferred. CLINICAL CASE: We report a patient who suffered a left subinsular hematoma when he was 62 years old. Four years later he started complaining burning constant pain in the right side of the body with crisis of lancinating pain. Also allodynia was observed in the face and right arm. MRI disclosed a necrotic lesion at the level of the left subinsular region. CONCLUSIONS: The lancinating pain and the allodynia were properly controlled by deep brain stimulation with an electrode placed stereotactically at the level of VPL nucleus of the left thalamus. Five months later there was a recurrence of the pain, a CT disclosed a tumour in the parietal region with an important shift of the midline and migration of the electrode out the thalamus. A biopsy disclosed tumoral necrosis. The pathophysiology of the central poststroke pain and effectivity of the deep brain stimulation in this cases are discussed.

[Procedural learning and neurological disorders]. / Aprendizajes procedimentales y enfermedades neurológicas.

INTRODUCTION AND DEVELOPMENT: We review the current state of learning procedures in six neurological conditions: amnesia, Alzheimer-type dementia, Huntington's disease, Parkinson's disease, progressive supranuclear paralysis and cerebellar pathology. In each condition the conservation or deterioration of different types of ability is specified: motor and percepto-motor abilities were evaluated using different tests such as following a rotating disk or signal, the labyrinth test, percepto-motor adaptation test or tests using paradigms of time of serial reaction; percepto-cognitive abilities evaluated by 'mirror-reading' and cognitive abilities evaluated by the Tower of Hanoi, of Toronto, or of London. CONCLUSION: Most of the papers published describe conservation of learning procedures in amnesic syndromes, relatively conserved in Alzheimer's disease and relatively deteriorated in Huntington's disease, in progressive supranuclear paralysis and in cerebellar dysfunction.