Valores del protocolo neuropsicológico GERMCIDE en una muestra de sujetos normales / Values of GERMCIDE neuropsychological protocol in a sample of normal subjects

Introducción. El diagnóstico de los pacientes con deterioro cognitivo o demencia exige un enfoque global en el que la exploración neuropsicológica es una pieza clave. Como parte del estudio GERMCIDE (Grupo para el Estudio y Registro Multicéntrico de Casos Incidentes de Demencia en España) se diseñó un protocolo que incluye una valoración de las diferentes funciones cognitivas que con mayor frecuencia se alteran en las demencias (memoria, orientación, lenguaje, praxias, capacidad de abstracción y función ejecutiva). Métodos. Con el objetivo de obtener datos en sujetos normales este protocolo neuropsicológico se aplicó a un grupo de personas mayores de 50 años sin deterioro cognitivo ni demencia. Resultados. Se estudiaron 103 sujetos con edades comprendidas entre 50 y 95 años (media: 73,5; desviación estándar [DE]: '9,3 años); 39 (37,90J0) hombres y 64 (62,1 %) mujeres. La puntuación media en el Mini-Mental State Examination (MMSE) fue de 27/30 (DE: 2,0). En las pruebas de lenguaje y praxias el 900J0 de los sujetos obtuvieron el valor máximo, mientras que en memoria, razonamiento y programación los rendimientos fueron más dispares. Se presenta la puntuación media, DE y distribución en percentiles para cada subtest. Conclusiones. Los valores obtenidos en esta muestra de sujetos normales y su distribución en percentiles pueden ser de gran ayuda para facilitar la interpretación de los hallazgos de la exploración neuropsicológica con el protocolo GERMCIDE en las consultas de neurología general y también en las consultas especializadas en demencia

Introduction. The diagnosis of patients with cognitive deterioration or dementia requires a global approach in which the neuropsychological examination is a key piece. As part of the GERMCIDE study (Group for the Study and Multicenter Registry of Incident Cases of Dementia in Spain), a protocol was designed that included an assessment of the different cognitive functions that are most frequently altered in dementias (memory, orientation, speech, praxis, abstraction capacity and executive function). Methods. In order to obtain data in normal subjects, this neuropsychological protocol was applied to a group of persons over 50 years without cognitive deterioration or dementia. Results. A total of 103 subjects whose ages ranged from 50 to 95 years (mean: 73.5; SD: 9.3 years); 39 (37.9%) men and 64 (62.1%) women were studied. The mean score on the Mini-Mental State Examination (MMSE) was 27/30 (SD: 2.0). In the speech and praxis tests, 90% of the subjects obtained the maximum value, while performances were more unequal in memory, reasoning and programming. Mean score, standard deviation and distribution in percentages for each subtest are presented. Conclusions. The values obtained in this sample of normal subjects and their distribution in percentages may be very helpful to facilitate the interpretation of the findings of the neuropsychological examination with the GERMCIDE protocol in the general neurology clinic visits and also in the specialized visits in dementia

Benefits of cognitive-motor intervention in MCI and mild to moderate Alzheimer disease.

OBJECTIVE: To evaluate the efficacy of a cognitive-motor program in patients with early Alzheimer disease (AD) who are treated with a cholinesterase inhibitor (ChEI). METHODS: Patients with mild cognitive impairment (MCI) (12), mild AD (48), and moderate AD (24) (Global Deterioration Scale stages 3, 4, and 5) were randomized to receive psychosocial support plus cognitive-motor intervention (experimental group) or psychosocial support alone (control group). Cognitive-motor intervention (CMI) consisted of a 1-year structured program of 103 sessions of cognitive exercises, plus social and psychomotor activities. The primary efficacy measure was the cognitive subscale of the AD Assessment Scale (ADAS-cog). Secondary efficacy measures were the Mini-Mental State Examination, the Functional Activities Questionnaire, and the Geriatric Depression Scale. Evaluations were conducted at 1, 3, 6, and 12 months by blinded evaluators. RESULTS: Patients in the CMI group maintained cognitive status at month 6, whereas patients in the control group had significantly declined at that time. Cognitive response was higher in the patients with fewer years of formal education. In addition, more patients in the experimental group maintained or improved their affective status at month 12 (experimental group, 75%; control group, 47%; p = 0.017). CONCLUSIONS: A long-term CMI in ChEI-treated early Alzheimer disease patients produced additional mood and cognitive benefits.

Programa de rehabilitación de las capacidades atencionales en pacientes con demencia tipo Alzheimer y enfermedades cerebrovasculares / Program for the rehabilitation of attentional processes in patients with Alzheimer's disease and stroke

En los últimos años, el incremento de la esperanza de vida y el aumento del número de personas que padecen algún tipo de demencia o enfermedad cerebrovascular han hecho patente la necesidad de desarrollar métodos de intervención neuropsicológica. El objetivo principal de esta investigación es la búsqueda de un método de rehabilitación de las capacidades atencionales que se muestre efectivo en pacientes con enfermedad de Alzheimer en estadios iniciales así como en personas que hayan sufrido un accidente cerebrovascular. Para ello se ha diseñado un programa de rehabilitación neuropsicológica que ha sido aplicado a dos muestras: una formada por veintidós personas diagnosticadas de demencia tipo Alzheimer y otra constituida por diez sujetos que habían sufrido un accidente cerebrovascular en los últimos seis meses (AU)

[Antoniana Margarita: Gómez Pereira, Francisco Lobato and the antecedents of cerebral mechanicism during the Spanish renaissance]. / Antoniana Margarita: Gómez Pereira, Francisco Lobato y los antecedentes del mecanicismo cerebral en el Renacimiento español.

The year 2000 is the fifth century of the birth, in Medina del Campo (Vallodolid, Spain) of licenciado Perea (Gomez Perea or Pereira). A man of the Renaissance, he was an outstanding doctor, humanist, theologist, nominalist philosopher, naturalist and practical engineer. He developed the first modern theory of behavior, based purely on mechanicistic principles, describing his ideas in a text known by the curious title of Antoniana Margarita. The objective of this paper is to pay him homage on the fifth centenary of his birth, making a historiographic study of Gomez Perea and his works, with particular emphasis on the ideological basis and its relationship with Renaissance hydraulic engineering, collaborating with his colleague Francisco Lobato, author of one of the only two pretechnological codices of sixteenth century Spain. The book Antoniana Margarita is written in Renaissance Latin and was published in Medina del Campo in 1554. It represents the first truly modern approach to brain function which excludes the providencialist concepts of Galen involving the soul and the spirit, in vogue until then, transmitted through the Arab and Scholastic tradition. Analyzing his theory of the automatism of animals Perea made the first description ever of the reflex arc and the conditioned reflex. He also established a topographical model of the brain in which he sketched the functioning of the prefrontal cortex and neurophysiology of memory. Perea was the immediate forerunner of Neuropsychology and of the methodology and organicist thought which predominates in modern Neurobiology. He was also a visionary of the Evolution of Darwin and of modern aetiology.

[Cerebral ischemia in Rendu-Osler-Weber disease]. / Isquemia cerebral en la enfermedad de Rendu-Osler-Weber.

Neurologic manifestations occur in 8-12% of the patients with Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasia (HHT), principally infectious and hemorrhagic and, less frequently, ischemic ones. More than a half of these neurologic complications are associated with pulmonary arterio-venous malformations (PAVM). The diagnosis of HHT is based on the presence of telangiectases, hemorrhagic events and a family history with an autosomal dominant pattern. We report a case of a patient diagnosed as having HHT with transient ischemic attacks and a PAVM, which was occluded by the use of embolotherapy. Cerebral ischemia in HHT is related to the existence of a PAVM and results from three mechanisms: 1) secondary poliglobulia and hyperviscosity because of the hypoxemia due to a right-left shunt; 2) communication between the airway and the pulmonary circulation during cough access, which produces gas embolism and hemoptysis; 3) and, finally, paradoxical embolism trough the PAVM, the same mechanism proposed to the infectious neurologic manifestations of the disease. When the diagnosis of HHT is suspected, early search and treatment of PAVM, with embolotherapy or surgery, are necessary in order to avoid respiratory problems (hemoptysis, exertional dyspnea, cianosis, clubbing) and neurologic complications.

Isquemia cerebral en la enfermedad de Rendu-Osler-Weber / Cerebral ischemia in Rendu-Osler-Weber disease

Un 8-12 por ciento de los pacientes con enfermedad de Rendu-Osler-Weber o telangiectasia hemorrágica hereditaria presenta manifestaciones neurológicas, sobre todo, hemorrágicas e infecciosas y, de forma menos frecuente, isquémicas. De ellos, más de la mitad asocian la presencia de malformaciones arteriovenosas pulmonares. El diagnóstico de la enfermedad se basa en la existencia de telangiectasias, fenómenos hemorrágicos y un patrón hereditario autosómico dominante. Se describe el caso clínico de un paciente, diagnosticado previamente de telangiectasia hemorrágica hereditaria, con accidentes isquémicos transitorios y una malformación arteriovenosa pulmonar que fue tratada mediante embolización. La isquemia cerebral en la telangiectasia hemorrágica hereditaria está relacionada con la presencia de una malformación arteriovenosa pulmonar y puede deberse a tres mecanismos: a) hiperviscosidad por poliglobulia secundaria a la hipoxemia provocada por la formación de un cortocircuito derecha-izquierda; b) comunicación de la vía aérea y la circulación pulmonar durante accesos de tos, lo que ocasiona una embolia gaseosa habitualmente acompañada de hemoptisis, y, por último, c) embolia paradójica a través de la malformación arteriovenosa pulmonar, que es, asimismo, el origen de las complicaciones neurológicas infecciosas de la enfermedad. En los pacientes con telangiectasia hemorrágica hereditaria debe investigarse la presencia de malformaciones arteriovenosas pulmonares y llevar a cabo de forma precoz su tratamiento, ya sea intravascular o quirúrgico, con el fin de evitar tanto problemas respiratorios (hemoptisis, disnea de esfuerzo, cianosis o acropaquías) como las posibles manifestaciones neurológicas. (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.

[Lateral sinus thrombosis in the first three months of pregnancy]. / Trombosis del seno lateral durante el primer trimestre del embarazo.

Thrombosis of the cerebral venous sinuses is characterized by clinical pleomorphism and variable pathogenesis. Although there is a relationship with pregnancy and especially with the puerperium, occurrence during the first trimester of pregnancy is unusual and this may effect the diagnosis and treatment. We describe the case of a 33 years-old patient with lateral sinus thrombosis in the first trimester of pregnancy. This was diagnosed and followed-up by magnetic resonance. Investigations as to its aetiology were inconclusive and it followed a benign course without anticoagulation. Thrombosis of the venous sinuses has been found empirically to be related to states of deficiency of S protein, hypercoagulability, anaemia and deshydration during pregnancy, to having previously taken contraceptive pills. Behçet's disease, other coagulopathies, central or systemic vasculitis, collagen disorders, septic and neoplastic disorders and a large number of cases of unknown aetiology. In our patient neurological deterioration secondary to a venous infarct or intraparenchymatous haemorrhage was not seen. There was full recovery and complete spontaneous canalization of the sinus, as shown on posterior cranial angiography. The controversial decision to use heparin in thrombosis of the cerebral venous sinuses should probably depend on the possible risks and benefits in each individual case, taking into account the clinical findings, aetiology and topography of the thrombosed sinus.

[The spectrum of prion pathology broadens: fatal familial insomnia]. / El espectro de la patología prion se amplía: el insomnio familiar fatal.

INTRODUCTION: The small group of prion diseases, caused by accumulation in the brain of an abnormal protein characterized by its aggregation and relative resistance to proteases (the PrPSc) in man is comprised of Creutzfeldt-Jacob disease (CJE), the Gerstmann-Straussler-Scheinker syndrome, kuru and the newest addition which is fatal familial insomnia (FFI). DEVELOPMENT: FFI is a hereditary condition with dominant autosomal transmission, characterized clinically by progressive insomnia, dysautonomy, changes in the circadian rhythm of hormone secretion, motor signs and slight to moderate deterioration of cognition. The usual age of onset is between 40 and 60 years, and the course of the illness lasts between 7 and 18 months. The histopathological changes, involving neurone loss and reactive gliosis, particularly affect the anteroventral and dorsomedial thalamic nuclei. These lesions lead to insomnia and to autonomic and endocrine disorders. To a lesser extent and degree, lesions are seen in other thalamic nuclei, the cerebral cortex, inferior olives and the cerebellum. FFI and some families with CJE have the same mutation of the codon 178 of the protein prion gene (gene PRNP) with substitution of aspartic acid by asparagine. Polymorphism of codon 129, which codifies methionine or valine determines the development of the clinical and neuropathological phenotype of FFI or CJE respectively. CONCLUSIONS: The description of FFI and the detection of PrPSe in familial cases of diffuse subcortical gliosis has indicated the possibility that there may be other familial or non-familial neurodegenerative diseases caused by prions.

[Kinematic deterministic chaos of fluids and fractal geometry in the carotid system]. / Caos determinista, cinemática de fluidos y geometría fractal en el sistema carotídeo.

INTRODUCTION: Atherosclerosis is a generalized vascular disorder which tends to be localized to specific arterial territories. At the bifurcation of the carotid artery there is a marked predisposition to form plaques of atheroma on the postero-external wall. This tendency is due to the kinematics of fluids and their particular morphological characteristics which are unique in the vascular system. The carotid tree is a physical, non-lineal or in 'non-equilibrium', dynamic system which depends on the fluctuating contribution of energy from the cardiac cycle. It has fractal geometry which follows the Law of Biology of maximum efficiency with a minimum of effort. DEVELOPMENT: The complexity of the relationship between the haemo-rheological and anatomical factors, and the periodic oscillation of flow does not permit use of simple models and classical determinist equations to describe idealized systems of continuous movement and Newtonian fluids. On the contrary, since we are considering a complex dissipative dynamic system. It has marked intrinsic operational freedom adapting its responses to external disturbances well, thus determining vasculo-cerebral autoregulation. The theories of Determinist Chaos and of the Science of Complexity imply the existence of emerging properties which exceed those of the individual elements in the dynamic systems in non-equilibrium, which tend to function in the 'frontier of chaos' at the critical points of phase transition. The carotid tree has non-linear properties, appearance of order and fractal 'sibisemejanza'. Pseudo-chaotic vortices appear--in regions of phase transition between laminar flow and turbulence--with the emergence of a 'strange attractor' near to the postero-external wall of the bulb. CONCLUSIONS: The anatomical and kinematic complexity of the system, together with the irreversibility of the second Law of Thermodynamics, lead to a long-term tendency towards the appearance of a region of stagnant flow with increased Entropy in the territory of the strange attractor which determines--as an inevitable long-term outcome--the tendency to the appearance of atherosclerosis at this particular point.

[Untreatable hiccup as un unusual sign in a rostral occlusion syndrome of the basilar artery]. / Hipo intratable como manifestación inusual de un síndrome de oclusión rostral de la arteria basilar.

Hiccoughs (singultus) is a habitual physiological phenomenon, the persistence of which might indicate neurological and extraneurological multiple lesions. Its activation involves starting up numerous central and peripheral mechanisms which have yet to be clearly determined. Hiccoughs results from an intermittent myoclonus of the diaphragm, reflex in origin, with unknown authentic cause or physiological significance. It has been suggested that, more than just an abnormal reflex, it could be a type of myoclonus brought on by repeated activity of the 'solitary inspiratory nucleus', by releasing control of the inhibiting-activating upper nervous system activity. We present the case of a patient with uncontrollable hiccoughs lasting over six years as a result of paramedial bilateral thalamic ischaemia (rostral occlusion syndrome of the basilar artery type 1) of cardioembolic origin (auricular fibrillation) secondary to hyperthyroidism.

[Wilson disease: a new case treated with trientine]. / Enfermedad de Wilson: a propósito de un caso tratado con trientine.

Hepatolenticular degeneration, also known as Wilson's disease (WD), is an infrequent hereditary disorder which is transmitted in recessive autosomic fashion: its genetic defect is to be found in the long branch of chromosome 13 (13q14.3) and allows disorder to take place which has not been sufficiently clarified, in the bilious excretion of the copper (Cu) which is deposited in an anomalous manner on a level with different organic tissues, giving rise to characteristic clinical manifestations which are, basically, of a neurological, hepatic, psychiatric and ocular nature. We present the case of a young patient whose case began, four years ago, with depressive-type manifestations, with diagnosis only being made now. Our opinion on the early detection of asymptomatic patients is commented on, along with that concerning the effectiveness and safety of therapeutic alternatives to D-penicilamine.

[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.

[Benign evolution of a spontaneous vermian hematoma using conservative treatment]. / Evolución benigna de un hematoma vermiano espontáneo con tratamiento conservador.

Spontaneous intracerebellar haematomas are a relatively unusual cause of haemorrhagic stroke. The uncommon variety of vermian haematomas (5%) represents a serious diagnostic and treatment challenge and its outcome is generally poor, being the result of rapid elevation of intracranial pressure and brainstem compression. A rare case of spontaneous vermian haematoma with benign course without surgery is reported, including the results of neuroimaging tests.

[Clinicopathological study of a case of oculopharyngeal muscular dystrophy]. / Estudio clinicopatológico de un caso de distrofia muscular oculofaríngea (DMOF).

One case of oculopharyngeal muscular dystrophy is reported, in a 75 year old white male with manifest family history of palpebral ptosis. He displayed bilateral ptosis and dysphagia. Diagnosis was carried out after clinical, electrical and bioptic procedures. Data obtained have been discussed with previous reports in the literature. It was necessary to perform a cricopharyngeal myotomy to overcome the malnutrition secondary to his swallowing problem.

[Pseudomeningocele after laminectomy]. / Pseudomeningocele postlaminectomía.

Pseudo-meningocele post-laminectomy is a non-frequent complication of this surgical technique. It is usually diagnosed by myelography; the CT scan has helped to detect more cases. We present a case studied by CT and RMN and there are no existing previous publications on the case confirmed by RMN. The relation between the findings and the clinic as well as the therapy still on discussion.

[Charcot-Marie-Tooth: a family case report]. / Charcot-Marie-Tooth: aportación de una familia.

Three cases in a Spanish family are presented, showing diagnosis criteria of hereditary motor-sensitive neuropathy (Charcot-Marie-Tooth disease) type I: suggestive symptoms, neurological and anatomopathological findings. The current classification of this pathology.

[Monomelic segmental amyotrophy: a Spanish case involving the leg]. / Amyotrophie spinale partielle monomélique. Un cas espagnol affectant un membre inférieur.

A 65-year old male presented with selective amyotrophy of the right lower limb which, after a progressive course of some months, had stabilized 12 years previously. Physical examination showed amyotrophy involving the gastrocnemius and the quadriceps extensor femoris without sensory impairment. EMG suggested motor neuron disease. This case meets Hirayama and Serratrice's criteria for benign chronic monomelic amyotrophy. We support the hypothesis of a casual vascular factor, as proposed by Hirayama.

[Unusual neurologic manifestation of Graves-Basedow disease]. / Manifestación neurológica inusual en la enfermedad de Graves-Basedow.

A 74 year old, male patient had an acute, painful bilateral exophtalmos, ptosis, ophtalmopathy and vision impairment. The CT-scan showed a thickness of the extrinsic ocular muscles which oriented the diagnosis of Graves-Basedow disease, later confirmed by the laboratory test. We highlight the rarity of the acute onset contrary to the slowly progressive ophtalmopathy frequently seen in this type of thyrotoxicosis, as well as the importance of the orbital CT-scan for an early diagnosis. We review the pathogenic theories of the process with its important immunologic back-ground, taking part the humoral and the cellular immunity associated with the HLA-B35 haplotype commenting on the good response to high dosage of steroids, later combined with bromocriptine.