Abnormal, ubiquitinated cortical neurites in patients with diffuse Lewy body disease.

Although it is known that the severity of dementia in patients with diffuse Lewy body disease is related to cortical Lewy body density, the morphological substrate of dementia in these patients is poorly understood. Vibratome sections processed free-floating for ubiquitin immunohistochemistry in three patients with the common form and one patient with the pure form has shown the presence of large numbers of abnormal, ubiquitinated neurites in the cerebral cortex, mainly cingulate cortex, entorhinal cortex and temporal lobe, and hippocampal complex, regions in which, precisely, Lewy bodies are most abundant. Abnormal neurites are a consistent change which results in abnormal neuronal connectivity. Abnormal cortical neurites, rather than cortical Lewy bodies, may play a significant role in the development and progression of cognitive deficits in patients with diffuse Lewy body disease.

Balint's syndrome. Report of four cases with watershed parieto-occipital lesions from vertebrobasilar ischemia or systemic hypotension.

Four patients with Balint's syndrome secondary to bilateral parieto-occipital ischemic lesions are presented. The pathogenesis of these lesions corresponded to either occlusive arterial disease in the vertebro-basilar system or the effects of systemic hypotension. The clinical features of this syndrome are discussed, along with the pathophysiology and time course of the condition, with particular emphasis on etiological factors, which make of this condition a distinct clinico-pathological syndrome.

[What does the Babinski sign have to offer 100 years after its description?]. / Que nous offre le signe de Babinski cent ans après sa description?

One hundred years after its description, the Babinski sign remains a faithful and precise indicator of dysfunction in the pyramidal tract. Unlike a normal plantar reflex, which is a local reflex (S1), the Babinski sign is an integral part, though with its own specificity and thus value, of the defense reflectivity of the lower limbs. It should be triggered, preferably in the lateral region of the foot, by a non-painful stimulation. The characteristic response is a dorsal flexion of the greater toe by recruitment of the extensor hallucis. The pathological response is related to a dysfunction involving a precise fraction of the pyramidal tract (van Gijn. 1996), responsible for a monosynaptic stimulation of the extensor hallucis motor neurons, but also, via interneurons, for inhibition of the extensor hallucis. Its relationship with defense reflexes is certain (Walshe, etc.) put quite complex. We refer here to the "fanning" sign or Babinski "equivalents", plantar areflexia, peripheral "pseudo-Babinski", responses obtained by stimulating the plantar aspect of the foot in the newborn, and to global hyperflexion. We also discuss the role of the Lundberf dorsal reticulospinal tract.

[Central deafness without aphasic disorders in a patient with bilateral temporal lesions (author's transl)]. / Surdité centrale sans troubles aphasiques par lésion temporale bilatérale.

A case is reported in which there was clinical evidence of complete central deafness without any associated aphasic disorder. A.C.T. scan revealed the presence of bilateral temporal lesions from two arterial ischemic accidents that occurred with an interval of twelve years between them. The lesions were most probably affecting the auditive radiations more than the transverse gyri of Heschl.

[Anatomo-clinical study of Behçet's syndrome with involvement of the central nervous system]. / Etude anatomo-clinique d'un syndrome de Behçet, avec atteinte du système nerveux central.

The authors report a patient with Behçet disease who developed severe central neurological disturbances. The M.R.I. was largely coincident with the distribution of the lesions verified in the neuropathological examination, while CT only shows an hypodense lesion in basal ganglia with contrast enhancement. The neurological symptoms did not respond to a treatment with prednisone and azathioprine. The main histopathological findings are similar to those described by previous observers. Reactive histiocytic infiltration with severe lymphocytic phagocytosis, probably related to immunosuppressor treatment, was also observed.

[Extremely mild forms of multiple sclerosis with very long evolution]. / Sobre las formas de esclerosis multiple de curso extremadamente benigno y prolongado.

The author presents the clinical history of three patients with a clinical picture of multiple sclerosis with a very long evolution and with a typical but mild symptomatology. In each case the diagnosis was corroborated by a MR imaginary. In the patient with a longer evolution the first symptoms, very smooth, appeared in 1943, when he was 19 years old, and the first clinical exploration was performed in 1945. We made the last control of this man in January, 1996. The other two patients were also controlled this same month. In conclusion, the author stressed the need of having always in mind the possibility of a "very benign" form of evolution of multiple sclerosis.

[Introduction to neuropsycholinguistics]. / Introducción a la neuropsicolinguística.

The authors summed up the evolution of the current neuropsicolinguistics and of its origins. They proposed a general definition of the aphasic disorders in relation to the psycholinguists capacities involved. The paper finish with a brief reference to the variety of types of the aphasics disorders according with its fluent or non-fluent caracter.

[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.

[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.

[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.

[Cerebral evoked potentials and semantic categorization]. / Potenciales evocados cerebrales y catagorización semántica.

INTRODUCTION: Cerebral evoked potentials represent variations in the electrical activity of the nervous system, excited by a sensory stimulus and which are recorded on the surface. They may be classified as endogenous or exogenous. Amongst the late endogenous evoked potentials (EEP) we may emphasize N400 which seems to represent the linguistic management wave, particularly for semantics. OBJECTIVE: To observe the EEP recorded throughout the process of carrying out a task involving sequential semantic categorization. PATIENTS AND METHODS: Nine healthy subjects carried out a task based on a paradigm of sequential reaction time (RT), similar to that designed by us in 1994. The data recorded were the RT and EEP at the level of the electrodes Fz, Cz and Pz in five lots of stimuli (in the first four lots the same list of words were repeated and in the fifth and final lot a different, new list appeared). CONCLUSIONS: It was seen that only N400 varied during the procedure. This variation was related to familiarity with the task. That is to say that the amplitude of N400 was reduced when the same words were repeated and increased when new words appeared for semantic classification. The reduction in parallel, although not significant for RT, allowed the reduction in N400 alone to be followed whilst the task was carried out. It may be the electrophysiological marker of the process of learning semantic categorization.

[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.

[Memory and learning: 'experience' and 'skill' of the brain]. / La memoria y el aprendizaje: 'experiencia' y 'habilidad' en el cerebro.

OBJECTIVE: To describe the current typology and different processes involved in memory and learning, as well as adequate tests in the diagnosis of the mnesic disorders. DEVELOPMENT: We reviewed the most recent studies about functional and lesional neuroanatomy of memory and learning and their neurophysiological bases (cellular and biochemical), with special emphasis in studies published in the three last years. We structured a typological classification, we expose the processes involved in short-term and long-term memory, we detailed the mnesic processes of declarative and implicit type, and we expose profiles of amnesias frequent in the clinical neurology and neuropsychology. CONCLUSIONS: Memory is not a diffuse and unitary process in our brain, neither amnesia is an absolute loss of memory. The multidimensional combination of two temporary memories (short- and long-term) and three mnesic processes ('working memory', explicit and implicit memory-learning) increases our capacity to memorize and learn, and it allows us to store the information in distinctive periods, with different mechanisms and covering different necessities. Patients with amnesia exhibit distinctive profiles of mnesic processes affected.

[Procedural learning in aphasic patients]. / Aprendizaje procedimental en un grupo de afásicos.

OBJECTIVE: This study concerned the procedural learning of semantic categorization. PATIENTS AND METHODS: The procedure used was a serial reaction time in an group of aphasic patients. RESULTS: Group of aphasic patients performed slower reaction times with more errors than the controls, but everyone manifested a decreasing reaction time, even during new semantic material categorization. CONCLUSIONS: Our results showed that the semantic categorization could be facilitated by procedural learning. Furthermore, this study suggest that procedural learning of semantic categorisation is at least relatively independent from cerebral structures usually involved a group of aphasic patients.