Primer homotrasplante fetal homotópico de estriado para el tratamiento de la enfermedad de Huntington / First homotypic fetal homotransplant of striate in the treatment of Huntington's disease

El uso de injertos cerebrales, útiles para restaurar la función en modelos animales de efermedad de Huntington (EH), se aplicó en una mujer de 37 años de edad con EH moderada a grave, de nueve años de evolución. Se trasplantaron los dos estriados de un feto humano de 13 semanas de edad gestacional en cuatro cavidades hechas en la pared ventricular del núcleo caudado derecho. Diez meses después de la cirugía, las evaluaciones neurológicas y neuropsicológica de la paciente revelaron la estabilización de su sintomatología y de la mayor parte de sus indicadores neuropsicológicos. Hubo mejoría moderada de los movimientos coréicos, principalmente los de la cara; de la capacidad para definir y expresar ideas en forma oral y escrita; de su agilidad articulatoria, así como en sus actividades cotidianas y en su comportamiento social. Ocurrió deterioro leve de la postura y la marcha; además se deterioraron su sistema sacádico y nistagmo optocinético, así como sus funciones visoespaciales y visoperceptuales

Pseudodemencia y jubilación / Pseudodementia and disability

Se presenta la experiencia en 10 pacientes referidos para evaluar una posible invalidez por la Superintendencia de Administradoras de Fondos de Pensiones, en los cuales se formula el diagnóstico clínico de pseudodemencia. Con este diagnóstico se rechazó la jubilación. El seguimiento de 8 casos mostró que su invalidez (sólo un caso dudoso) era persistente a pesar de no existir una ganancia secundaria evidente. Se concluye que debe aceptarse la realidad de esta invalidez y que debería modificarse la legialación al respecto

Parafasia / Paraphasia

Las parafasias descritas tradicionalmente en la semiología de afásico como propias y con carácter de autenticidad, deberían ser en la actualidad, o considerarse como un componente de la condición afásica, pero de más significación y de mayor extensión compleja. Aparece característicamente en el estado de reorganización del proceso lenguaje-habla destruido primero por la enfermedad; es transitoria y con diferencias auditivas y afasiológicas en relación a la etapa de transición entre el acmé de enfermedad y el inicio y primeras etapas de la reconstitución. Mantienen una cierta estructura diferente según la forma básica de la supresión del habla. No son elementos pasivos y permiten una diferenciación diagnóstica según que los defectos radiquen en las fases fonético-fonémica, palabras y nivel sintagmático. En esta comunicación damos un conjunto de diversas formas, no sólo considerando las estructuras clásicas sino también las relacionadas o derivadas del criterio fenomenológico, que justamente muestra otras posibilidades patogénicas explicativas

El test del reloj: una evaluación cognitiva rápida y sensible al deterioro incipiente / The clock setting test: a quick cognitive assessment sensitive to early deterioration

El test del reloj, utilizado inicialmente como evaluación visuomotora, es de fácil y rápida administración y sirve como indicador de deterioro demencial. Se compararon 15 pacientes con enfermedad de Alzheimer (EA) incipiente (MMSE 23,71 ñ 3,02; GDS 3,50 ñ .76) y 15 controles apareados por edad y educación respecto a la ejecución del reloj (comando y copia), planificación de la hora, lectura horaria, tamaño y ubicación de las agujas. Los enfermos calificaron peor en reloj de comando (6.07 ñ 1.70 vs controles = 9,50 ñ .97 p<.0004); graficación de la hora por código lectoescrito numérico (p<.001) y coloquial (p<.05); ubicación (Chi2 = 10.29 p<.0004), y tamaño de las agujas (Chi2 = 13.89 p<.0001). La planificación horaria en posición 2:50 y 8:40 demostró ser una variable capaz de discriminar el 85% de población en normales y deteriorados leves. Se concluye que las variables consideradas demostraron ser sensibles al deterioro incipiente. Las correlaciones sugirieron que los procesos cognitivos involucrados en esta prueba serían la visuespacialidad, la praxia constructiva, el contenido y el procesado del código específico de la hora, los procesos de transcodificación y la programación frontal

Estudio de la lateralidad mnésica episódica en las lesiones isquémicas temporales internas / A study of mnemanic lateralitation in internal temporal ischemic lesions

Fueron evaluados neurológica y neuropsicológicamente 6 pacientes con lesiones isquémicas temporales internas tres derechas y tres izquierdas. La evaluación cognitiva global mostró en las izquierdas normalidad de todos los parámetros estudiados y en las derechas deterioro del recuerdo y de la visuoconstrucción. En el examen más detallado del sistema mnésico episódico encontramos que las lesiones izquierdas tienen un deterioro de la memoria verbal y las derechas compromiso global pero predominando en la memoria visual. Esta asimetría verbal/visual de la memoria episódica de largo plazo se corresponde con la lateralidad hipocámpica. La falla de la memoria verbal en las lesiones derechas aparecería por un trastorno gestáltico de la organización temporo-espacial que perturbaría la estructuración de la imagen de memoria

Estudio de la lateralidad mnésica episódica en las lesiones isquémicas temporales internas / A study of mnemanic lateralitation in internal temporal ischemic lesions

Fueron evaluados neurológica y neuropsicológicamente 6 pacientes con lesiones isquémicas temporales internas tres derechas y tres izquierdas. La evaluación cognitiva global mostró en las izquierdas normalidad de todos los parámetros estudiados y en las derechas deterioro del recuerdo y de la visuoconstrucción. En el examen más detallado del sistema mnésico episódico encontramos que las lesiones izquierdas tienen un deterioro de la memoria verbal y las derechas compromiso global pero predominando en la memoria visual. Esta asimetría verbal/visual de la memoria episódica de largo plazo se corresponde con la lateralidad hipocámpica. La falla de la memoria verbal en las lesiones derechas aparecería por un trastorno gestáltico de la organización temporo-espacial que perturbaría la estructuración de la imagen de memoria

El test del reloj: una evaluación cognitiva rápida y sensible al deterioro incipiente / The clock setting test: a quick cognitive assessment sensitive to early deterioration

El test del reloj, utilizado inicialmente como evaluación visuomotora, es de fácil y rápida administración y sirve como indicador de deterioro demencial. Se compararon 15 pacientes con enfermedad de Alzheimer (EA) incipiente (MMSE 23,71 ñ 3,02; GDS 3,50 ñ .76) y 15 controles apareados por edad y educación respecto a la ejecución del reloj (comando y copia), planificación de la hora, lectura horaria, tamaño y ubicación de las agujas. Los enfermos calificaron peor en reloj de comando (6.07 ñ 1.70 vs controles = 9,50 ñ .97 p<.0004); graficación de la hora por código lectoescrito numérico (p<.001) y coloquial (p<.05); ubicación (Chi2 = 10.29 p<.0004), y tamaño de las agujas (Chi2 = 13.89 p<.0001). La planificación horaria en posición 2:50 y 8:40 demostró ser una variable capaz de discriminar el 85% de población en normales y deteriorados leves. Se concluye que las variables consideradas demostraron ser sensibles al deterioro incipiente. Las correlaciones sugirieron que los procesos cognitivos involucrados en esta prueba serían la visuespacialidad, la praxia constructiva, el contenido y el procesado del código específico de la hora, los procesos de transcodificación y la programación frontal

Neurotoxicity of acrylamide and 2,5-hexanedione in rats evaluated using a functional observational battery and pathological examination.

The clinical effects of two neurotoxicants, acrylamide and 2,5-hexanedione, were compared in rats using a functional observational battery (FOB), which includes a series of home cage and open-field observations, sensorimotor measurements, and physiological parameters. Neurotoxicity was assessed weekly in adult male Long-Evans rats after initiation of IP administration of 9 doses of acrylamide (12, 15, or 50 mg/kg given 3 times a week) and 28 doses of 2,5-hexanedione (150, 225, and 350 mg/kg given daily). Using the FOB, it was possible to detect differences in neurotoxic effects of these two chemicals. Acrylamide significantly affected home cage posture, foot splay and time on the rotarod, whereas 2,5-hexanedione altered hindlimb grip strength and the approach response. Both compounds caused changes in ability to walk, right, and maintain agility on a rotarod within 21 days from initiation of toxicant administration. In addition, both compounds caused dose-dependent decreases in weight gain. Neuropathic changes were detectable at the highest dosages at 21 days in acrylamide-treated rats and at 28 days in rats treated with 2,5-hexanedione. Administration of acrylamide also decreased activities of neural esterases. This study indicated that the FOB could be used to detect evidence of neurotoxicity in rats treated with acrylamide and 2,5-hexanedione, with alterations evident even before pathological changes were induced by 2,5-hexanedione.

Sequelae of haemolytic uraemic syndrome.

Twenty two patients with previous episodes of haemolytic uraemic syndrome (HUS) were investigated for evidence of deficits in cognitive, behavioural, and academic function. Patients were pair matched with 22 controls for age (+/- 1 year), gender, and socioeconomic status. HUS patients had numerically lower cognitive and achievement scores and higher behavioural problem ratings than their controls on every measure. None of the group differences was significant at the 0.01 level. Significance values between 0.10 and 0.01 were obtained for the Wechsler full scale and verbal intelligence quotient scores and for several of the achievement measures and behaviour ratings. These results were conservatively interpreted as trends and are considered to provide preliminary indications of a post-HUS deficit in behaviour, verbal intelligence, and the verbally based skills of reading comprehension and vocabulary. The findings provide interim guidelines for follow up care but require confirmation and elaboration in a larger study.

Research and clinical criteria for development of neurobehavioral test batteries.

Because neuropsychological testing can detect subtle changes in central nervous system function resulting from occupational and environmental exposure to toxic chemicals, it has been widely used in behavioral neurotoxicologic investigations. However, work in this field often has ignored the distinction between clinical and research testing when applying these assessment techniques. Experimental studies generally compare groups of subjects on specific outcome measures, whereas clinical work usually is focused on diagnosis and treatment of individual patients. Therefore, the inclusion criteria applicable to the selection of neuropsychological test batteries are different in research and clinical settings. Issues germane to test selection in research settings include sensitivity to neurotoxins, psychometric standards, sensitivity to central nervous system dysfunction, overview of cognitive functions, sampling of cognitive processing, sampling of output modalities, and examination of theoretical constructs. The usual questions asked in the clinical setting can be addressed most efficiently when the following issues are considered in test selection: sensitivity to specific toxicant exposure, estimation of native ability patterns, differential diagnosis, developmental specificity of tests and exposure effects, and description of patterns of cognitive strengths and weaknesses.

[Memory deficit in Parkinson's disease. Precocious aging of recall processes]. / Déficit mnésique au cours de la maladie de Parkinson. Vieillissement accéléré des processus de rappel.

In this study, the various components of the memory process were analysed in 25 non-demented parkinsonian patients (PP). A battery of tests was used to explore words, drawings and semantic organization of items. Results were compared with young (n = 22) and elderly (n = 11) healthy controls. Scores were correlated with the characteristics of Parkinson disease. Recall of words and drawings was significantly disturbed in PP. In contrast, the recognition of drawings and faces was not impaired. A high degree of interindividual difference in performance was observed; it was strictly correlated with age but not with the features of parkinsonism. A specific pattern of memory impairment can be described in parkinsonism, which would suggest and support the theory that different pathogenic mechanisms are involved in ageing and in parkinsonian patients.

Comparison of screening approaches.

Neurobehavioral techniques have been used extensively in animal toxicology studies because, in many cases, such procedures are designed to evaluate neurobiological functions thought to be affected in chemical-exposed humans, e.g., changes in sensorimotor function. Procedures used to identify or screen for the presence of neurotoxicity are usually designed to test large numbers of animals and are not considered to be as sensitive to subtle effects as more specialized tests for neurobiological dysfunction. For purposes of screening, the use of a functional observational battery (FOB) is now generally accepted. In general, FOB evaluations in animals are similar to clinical neurological examinations in humans in that they rate the presence and, in some cases, the severity of behavioral and neurological signs. A number of batteries containing different observations and measurements have been developed in several laboratories for rodents, dogs, and non-human primates. Frequently, the FOB is used in conjunction with other measures of neurotoxicity, i.e., neuropathology or sensory evoked potentials. FOB used in screening typically assess several neurobiological domains including neuromuscular (i.e., weakness, incoordination, abnormal movements, gait, motor seizures, myoclonia, rigidity and tremor), sensory (i.e., auditory, visual and somatosensory) and autonomic (i.e., pupil response, salivation) functions. Most FOB used for screening do not assess cognitive function (i.e., learning and memory). FOB evaluations can yield important information concerning dose-response characteristics and data on the onset, duration and persistence of an effect. FOB should be able to differentiate neurotoxicants from non-neurotoxicants and neurotoxicants having different mechanism(s) or site(s) of action.

Wisconsin Card Sorting Test performance in schizophrenia: remediation of a stubborn deficit.

OBJECTIVE: Schizophrenic patients typically perform poorly on the Wisconsin Card Sorting Test, which is a putative index of prefrontal functioning. The authors attempted to remediate the deficits of schizophrenic patients on this measure by giving detailed instructions and monetary reinforcement. METHOD: Forty-six inpatients with chronic schizophrenia and 20 control subjects with other psychiatric illnesses were given the Wisconsin Card Sorting Test under four conditions that varied in monetary reinforcement and level of instructions. The schizophrenic patients were given the Brief Psychiatric Rating Scale (BPRS) and three information processing measures (the Continuous Performance Test, Span of Apprehension, and Pin Test). RESULTS: Schizophrenic patients performed worse than psychiatric control subjects across most conditions. Monetary reinforcement had little effect on performance, but detailed instructions significantly improved the scores for both groups. When instructions were withdrawn and monetary reinforcement was maintained, both groups continued to show improved performance over baseline. Symptoms were not significantly associated with Wisconsin Card Sorting Test performance. One measure (the Pin Test) correlated significantly with performance on the Wisconsin Card Sorting Test. CONCLUSIONS: The results suggest the importance of combining motivational with instructional factors for training psychiatric patients in problem solving.

[Psychological evaluation of the effects of chronic occupational exposure of paint shop workers to the mixture of organic solvents]. / Psychologiczna ocena skutków przewleklego narazenia zawodowego lakierników pracujacych w stycznosci z mieszanina rozpuszczalników organicznych.

The aim of the study was to evaluate the psychological effects of chronic occupational exposure to the mixture of organic solvents containing xylene, toluene, n-butanol, butyl acetate, n-butyl acetate, ethyl acetate and cyclohexanone defined by MAC values. Thirty-four paint shop workers and 34 controls, matched according to sex, age, education and the type of work performed, were examined. The Neurobehavioral Core Test Battery (NCTB) was used in the study. In the exposed workers, a longer simple reaction time was observed, as well as reduced manual skills and impaired precision of the hand movements.

Neuropsychological characteristics of right hemisphere damage: investigation by attention tests, concept formation and change test, and self-evaluation task.

Neuropsychological characteristics of right (non-dominant) hemisphere damage were investigated by attention tests, a concept formation and change test, and a self-evaluation task on a total of 126 brain damaged subjects (66 right hemisphere damaged and 60 left hemisphere damaged). Common response patterns specific to subjects with right hemisphere lesions were studied by three attention tests (audio-motor method, cancellation test, and set dependent activity test). Those with right hemisphere lesions showed a large number of responses (excessive response) and low proportion of correct responses (low hit rate, qualitative deterioration of response), indicating a tendency toward randomness. A concept formation and change test, the new modified Wisconsin Card Sorting Test, was performed to investigate the handling of higher concepts. Only the frequency of difficulty of maintaining set (DMS) was significantly higher in those with right hemisphere lesions. Problems concerning attention and attitude toward the tests, i.e. random attitude were thought to exist in those with right hemisphere damage. Self-evaluation and corrective ability were studied on the self-evaluation task using the audio-motor method. Feedback was less effective in subjects with right hemisphere lesions, and they tended to underestimate their errors. These results were consistent with personality characteristics such as the lack of a serious attitude and poor volition for treatment, which are frequently observed in clinical settings. The above are considered basic, common characteristics of right hemisphere damage. These findings may partially explain the phenomenological characteristics of right hemisphere damage described by various investigators.

Neuropsychological assessment for detecting adverse effects of volatile organic compounds on the central nervous system.

Because there are no direct biological markers for the substances implicated in indoor air exposure, it is impossible to directly measure if an individual or group of individuals has been exposed to a potentially neurotoxic substance in the workplace. Behavioral changes may be the earliest and only manifestation of central nervous system (CNS) effects and are often too subtle to be revealed by routine physical or neurological examination. Neuropsychological techniques are sensitive to subtle behavioral/cognitive changes that can result from exposure to neurotoxins. These techniques consist of oral and written tests that are administered by a trained examiner on a one-to-one basis. In general, a wide variety of cognitive domains are evaluated. The typical battery generally includes assessing orientation, attention, intelligence, language, visual memory, verbal memory, perception, visuoconstruction, simple motor speed, psychomotor speed, and mood. As with most assessment techniques, the neuropsychological methods have limitations. One major drawback is the availability of appropriate norms that are used to compare the results of a specific individual. Because these tasks are greatly affected by age, intelligence, and in some instances sex, the availability of appropriate norms is mandatory to determine if the CNS has been effected. Although neuropsychological tests are sensitive to the presence of CNS involvement, they are not specific. Patterns of performance seen with specific instances of neurotoxic exposure may also be seen with a number of other diseases of the CNS such as dementia, cerebrovascular disease, hydrocephalus, or normal aging. In addition, neuropsychiatric symptoms such as anxiety and/or depression are often manifested as cognitive difficulties that will mimic the cognitive dysfunction seen with toxicity of the CNS.(ABSTRACT TRUNCATED AT 250 WORDS)

[Visual-spatial function in cerebral palsy and proposal of a new test computer-assisted]. / Função vísuo-espacial em paralisia cerebral e proposta de novo teste com a ajuda de computador.

The first idea of this study is that there may be a disturbance of the visuo-spatial function in cerebral palsy. This idea is based upon the theories of Luria and Piaget. The second idea of this study is that the computer can help the investigation of visuo-spatial function. With the intention of evaluating the spatial function in cerebral palsy, the literature related to these themes was revised. We justify the proposal of a new test to study visuo-spatial function. This test uses straight lines in four different orientations and the subject has to recognize and arrange them individually or sets of them. This was done through the use of pencil and paper, and computer. The results confirm that there is a spatial disturbance and that a better understanding of the subject's difficulties was made possible through the use of the computer and the Logo computer language.

Funçäo vísuo-espacial em paralisia cerebral e proposta de novo teste com a ajuda de computador / Visual-spatial function in cerebral palsy and proposal of a new test computer-assisted

Os autores apresentam o estudo da funçäo vísuo-espacial em uma criança com paralisia cerebral (PC). Apoiados nas teorias de Luria e Piaget, justificam a existência de tal distúrbio em PC. A criança-propósito deste estudo apresentou elementos teórico-práticos que embasaram esta idéia. Face às limitaçöes dos testes disponíveis, propomos novo teste aplicável tanto em papel quanto em computador. Trata-se de quatro linhas dispostas equidistantemente em diferentes inclinaçöes. A simplicidade do teste permitiu seu uso em computador, o que ampliou e aprofundou a investigaçäo, acrescentando novos elemento ao estudo