RESUMEN

D-waves (also called direct waves) result from the direct activation of fast-conducting, thickly myelinated corticospinal tract (CST) fibres after a single electrical stimulus. During intraoperative neurophysiological monitoring, D-waves are used to assess the long-term motor outcomes of patients undergoing surgery for intramedullary spinal cord tumours, selected cases of intradural extramedullary tumours and surgery for syringomyelia. In the present manuscript, we discuss D-wave monitoring and its role as a tool for monitoring the CST during spinal cord surgery. We describe the neurophysiological background and provide some recommendations for recording and stimulation, as well as possible future perspectives. Further, we introduce the concept of anti D-wave and present an illustrative case with successful recordings.

Asunto(s)

Neoplasias de la Médula Espinal , Humanos , Neoplasias de la Médula Espinal/cirugía , Neoplasias de la Médula Espinal/fisiopatología , Monitorización Neurofisiológica Intraoperatoria/métodos , Tractos Piramidales/fisiopatología , Monitoreo Intraoperatorio/métodos , Masculino

RESUMEN

OBJECTIVE: The risk of progression of multiple sclerosis (MS) related to the association of prognostic factors present at disease onset has rarely been explored. We aimed to construct a clinical risk score for MS long-term progression that could be easily applied in clinical practice. PATIENTS AND METHODS: Among 432 patients with MS, 288 patients were selected as a derivation sample for identification of the knowledge prognostic factors more associated with long-term progression. One point was given to each risk factor identified as statistically significant by the adjusted model, and the sum of the points gave the overall risk score. Subsequently the score was applied to the remaining 144 patients to confirm if those with higher scores had reached MS secondary progression. RESULTS: The prognostic factors identified as independently associated with long-term progression were: no specific MS treatment before EDSS 3, age of onset older than 30 years, pyramidal and cerebellar impairment as the first manifestation of disease, time interval between the first and second relapses less than 2 years, and African ancestry. There was no significant difference between expected and observed number of patients in progression (44 vs. 31, p = 0.966), indicating that the score was able to predict the progression in the validation sample. There was no significant difference between patients with low risk (≤ 2 points) (p = 0.98) and high risk (≥ 3 points) (p = 0.48) in the derivation versus validation samples. In the derivation sample, the patients with three or more points had a 2.8-fold increased risk of progression [hazard ratio (HR): 2.8; 95 % confidence interval (CI): 1.2-6.3; p = 0.014). CONCLUSION: The score proposed was capable of predicting long-term MS progression.

Asunto(s)

Esclerosis Múltiple/patología , Adulto , Edad de Inicio , Anciano , Población Negra , Brasil , Cerebelo/fisiopatología , Evaluación de la Discapacidad , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Modelos Estadísticos , Pronóstico , Tractos Piramidales/fisiopatología , Reproducibilidad de los Resultados , Medición de Riesgo , Adulto Joven

RESUMEN

BACKGROUND: Congenital Zika syndrome causes a spectrum of neurological symptoms with varying effects on function that require different therapeutic strategies. To date, this spectrum of effects and its clinical implications have not been completely described. We describe the neurological examination findings in toddlers and preschoolers, including predominant symptom complexes and comorbidities. METHODS: This study is a case-series neurological evaluation of 75 children with congenital Zika syndrome in Campina Grande, Brazil. The study is part of a cohort of children with congenital Zika syndrome that started in 2015 and is still ongoing. Children with Zika virus infection detected during pregnancy (mothers exhibited rash and were followed and diagnosed by fetal ultrasound abnormalities or RT-PCR) or through microcephaly screening after birth, using Intergrowth 21 guidelines, were selected by laboratory and radiological criteria. Children were examined during a 10-day period in September, 2018, and underwent neurological interview, examination, and assessment of functional outcomes and comorbidities. Children were divided in groups of predominant corticospinal or neuromuscular clinical signs and the associations between these groups and clinical comorbidities were assessed. FINDINGS: All of the children recruited to the study from Nov 29, 2015 to Nov 30, 2017 had imaging correlates of congenital Zika syndrome. Children were assigned to groups depending on the signs exhibited, either corticospinal or neuromuscular, with or without dyskinetic signs. 75 children completed the evaluation, 38 (51%) girls and 37 (49%) boys. Median age was 33 months (range 26-40 months; IQR 29-34). Microcephaly was present at birth in 56 (75%) children, and 19 (25%) children were born with normal head circumference, 15 of whom later developed microcephaly. Neurological examination grouped four children as having isolated dyskinetic signs, 48 children were assigned to the corticospinal group and 23 into the neuromuscular group. Dyskinetic findings were present in 30 (40%) children, either alone (four [5%]) or combined with corticospinal (19 [40%] of 48) or neuromuscular (seven [30%] of 23) findings. Comorbidities were highly prevalent, and the neuromuscular group had worse functional outcomes, evaluated by gross motor function (p=0·026), manual abilities (p=0·0013), and communication function (p<0·0005) classification scales, than the corticospinal group, whereas pneumonia (p<0·0005) and urinary tract infections (p<0·0005) were more frequent in the corticospinal group. Cortical hyperexcitability was supported by several clinical correlates, such as early onset epilepsy, persistence of primitive reflexes, and dystonia. INTERPRETATION: We describe distinct neurological profiles in the congenital Zika syndrome spectrum, with functional outcomes tending to correlate with these groups. The clinical division of children based on the disease signs proposed here is supported by the literature on central and peripheral nervous system pathology in congenital Zika syndrome. The high prevalence of dyskinetic symptoms merits special attention. FUNDING: Brazilian National Council for Scientific and Technological Development and by the Coordination for the Improvement of Higher Education Personnel.

Asunto(s)

Discinesias/fisiopatología , Enfermedades Neuromusculares/fisiopatología , Infección por el Virus Zika/fisiopatología , Encéfalo/diagnóstico por imagen , Encefalopatías/diagnóstico por imagen , Encefalopatías/epidemiología , Brasil/epidemiología , Calcinosis/diagnóstico por imagen , Calcinosis/epidemiología , Preescolar , Comorbilidad , Trastornos de Deglución/epidemiología , Discinesias/epidemiología , Epilepsia/epidemiología , Femenino , Humanos , Estudios Longitudinales , Masculino , Malformaciones del Desarrollo Cortical/diagnóstico por imagen , Malformaciones del Desarrollo Cortical/epidemiología , Malformaciones del Desarrollo Cortical/fisiopatología , Microcefalia/epidemiología , Microcefalia/fisiopatología , Enfermedades del Sistema Nervioso/diagnóstico por imagen , Enfermedades del Sistema Nervioso/epidemiología , Enfermedades del Sistema Nervioso/fisiopatología , Examen Neurológico , Enfermedades Neuromusculares/epidemiología , Neumonía/epidemiología , Tractos Piramidales/fisiopatología , Trastornos del Sueño-Vigilia/epidemiología , Tomografía Computarizada por Rayos X , Infecciones Urinarias/epidemiología , Infección por el Virus Zika/congénito , Infección por el Virus Zika/diagnóstico por imagen , Infección por el Virus Zika/epidemiología

RESUMEN

This is a paper about the development of a sensitive examination of subtle motor pyramidal deficits. Jean-Alexandre Barré's life and work are reappraised, and also his milieu prone to increase his clinical skillfulness and sharping many motor maneuvers on subtle motor deficits (1919, 1920, 1937). Giovanni Mingazzini is also remembered by his precursor publication of "small signs" about organic paresis (1913), and by his neuroanatomical background, in an Italian Neuropsychiatric milieu, at the time.

Asunto(s)

Neurología/historia , Paresia/historia , Historia del Siglo XIX , Historia del Siglo XX , Humanos , Tractos Piramidales/fisiopatología

Asunto(s)

Progresión de la Enfermedad , Síntomas Prodrómicos , Tractos Piramidales/fisiopatología , Ataxias Espinocerebelosas/fisiopatología , Estimulación Magnética Transcraneal/métodos , Ataxina-2/genética , Biomarcadores , Estudios Transversales , Cuba , Humanos , Ataxias Espinocerebelosas/diagnóstico , Ataxias Espinocerebelosas/genética

Asunto(s)

Fascia Lata/fisiopatología , Reflejo Anormal/fisiología , Anciano , Humanos , Masculino , Tractos Piramidales/fisiopatología

Asunto(s)

Humanos , Masculino , Anciano , Reflejo Anormal/fisiología , Fascia Lata/fisiopatología , Tractos Piramidales/fisiopatología

RESUMEN

OBJECTIVE: We used an assembly of electrodes C3 and C4-Cz in order to activate the motor cortical area of the corticobulbar tract to elucidate the motor-evoked potential of the contralateral mentalis muscle. METHOD: We compared this setup to that of an assembly with electrodes C5 or C6-Cz using a train of electrical pulses and a single electrical pulse. This analysis was made in 23 consecutive patients who underwent several varied surgeries and were prospectively operated on at Santa Paula Hospital between January and June 2011. RESULTS: The results showed that the assembly with C5 or C6-Cz produced a multisynaptic motor-evoked potential in the contralateral mentalis muscle in 86.9 % of the patients, whereas 82.6 % of patients stimulated at points C3 or C4-Cz presented the same response. However, both assemblies showed similar behavior with the use of a single electrical pulse for peripheral contralateral nerve stimulation. CONCLUSION: We concluded that the C5 or C6-Cz assembly was similar to C3 or C4-Cz in obtaining a multisynaptic response in the contralateral mentalis muscle, although it required less intensive stimulation than the C3 or C4- Cz assembly.

Asunto(s)

Estimulación Eléctrica/métodos , Potenciales Evocados Motores/fisiología , Músculos Faciales/fisiopatología , Nervio Facial/fisiopatología , Monitoreo Intraoperatorio/métodos , Tractos Piramidales/fisiopatología , Adulto , Anciano , Estimulación Eléctrica/instrumentación , Electrodos , Músculos Faciales/inervación , Nervio Facial/anatomía & histología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Monitoreo Intraoperatorio/instrumentación , Adulto Joven

RESUMEN

UNLABELLED: Charcot-Marie-Tooth (CMT) disease is a hereditary neuropathy of motor and sensory impairment with distal predominance. Atrophy and weakness of lower limbs are the first signs of the disease. It can be classified, with the aid of electromyography and nerve conduction studies, as demyelinating (CMT1) or axonal (CMT2). OBJECTIVE: Clinical and neurophysiological investigation of a large multigenerational family with CMT2 with autosomal dominant mode of transmission. METHOD: Fifty individuals were evaluated and neurophysiological studies performed in 22 patients. RESULTS: Thirty individuals had clinical signs of motor-sensory neuropathy. Babinski sign was present in 14 individuals. Neurophysiological study showed motor-sensory axonal polyneuropathy. CONCLUSION: The clinical and neurophysiological characteristics of this family does not differ from those observed with other forms of CMT, except for the high prevalence of Babinski sign.

Asunto(s)

Enfermedad de Charcot-Marie-Tooth/fisiopatología , Tractos Piramidales/fisiopatología , Adolescente , Adulto , Anciano , Enfermedad de Charcot-Marie-Tooth/genética , Niño , Electromiografía/métodos , Femenino , Ligamiento Genético/genética , Humanos , Masculino , Persona de Mediana Edad , Linaje , Fenotipo

RESUMEN

Charcot-Marie-Tooth (CMT) disease is a hereditary neuropathy of motor and sensory impairment with distal predominance. Atrophy and weakness of lower limbs are the first signs of the disease. It can be classified, with the aid of electromyography and nerve conduction studies, as demyelinating (CMT1) or axonal (CMT2). OBJECTIVE: Clinical and neurophysiological investigation of a large multigenerational family with CMT2 with autosomal dominant mode of transmission. METHOD: Fifty individuals were evaluated and neurophysiological studies performed in 22 patients. RESULTS: Thirty individuals had clinical signs of motor-sensory neuropathy. Babinski sign was present in 14 individuals. Neurophysiological study showed motor-sensory axonal polyneuropathy. CONCLUSION: The clinical and neurophysiological characteristics of this family does not differ from those observed with other forms of CMT, except for the high prevalence of Babinski sign.

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A doença de Charcot-Marie-Tooth (CMT) é uma neuropatia hereditária de acometimento sensitivo e motor de predomínio distal. Atrofia e fraqueza em membros inferiores são os primeiros sinais da doença. Pode ser classificada, com auxílio da eletroneuromiografia, em desmielinizante (CMT1) ou axonal (CMT2). OBJETIVO: Investigação clínica e neurofisiológica de família com portadores de CMT2 de herança dominante. MÉTODO: Foi feita avaliação neurológica de 50 indivíduos e eletroneuromiografia em 22 pacientes. RESULTADOS: Trinta indivíduos tinham sinais clínicos de neuropatia sensitivo-motora. Sinal de Babinski estava presente em 14 indivíduos. A eletroneuromiografia demonstrou polineuropatia axonal sensitiva e motora. CONCLUSÃO: As características clínicas e neurofisiológicas desta família não se diferem das observadas em outras formas de CMT, exceto pela alta prevalência de sinal de Babinski.

Asunto(s)

Adolescente , Adulto , Anciano , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedad de Charcot-Marie-Tooth/fisiopatología , Tractos Piramidales/fisiopatología , Enfermedad de Charcot-Marie-Tooth/genética , Electromiografía/métodos , Ligamiento Genético/genética , Linaje , Fenotipo

RESUMEN

Experimental studies in nonhuman primates have questioned the selectivity of pyramidal tract damage in giving rise to the classical pyramidal syndrome in humans, characterized by permanent spastic hemiplegia (PSH). According to this view, concomitant injury of extrapyramidal pathways is necessary for the development of both hemiplegia and spasticity. In this study we used conventional magnetic resonance imaging and diffusion tensor imaging tractography to characterize the anatomical correlates of PSH in a patient with a rare and discrete unilateral lesion of the medullary pyramid. Our findings support the hypothesis that damage confined to the medullary pyramid/pyramidal tract is sufficient to produce PSH. In contrast to nonhuman primates, the human 'pyramidal' and 'pyramid' syndromes are equivalent clinico-anatomic concepts.

Asunto(s)

Hemiplejía/patología , Tractos Piramidales/patología , Anciano , Imagen de Difusión por Resonancia Magnética/métodos , Femenino , Hemiplejía/fisiopatología , Humanos , Imagenología Tridimensional , Masculino , Persona de Mediana Edad , Pruebas Neuropsicológicas , Tractos Piramidales/fisiopatología , Síndrome

RESUMEN

PURPOSE: The main objective of this study was to verify, in one hundred in-patients from the Serviço de Clínica Médica do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro who did not have a history of clinical symptoms of pyramidal disfunction, the presence of the Babinski and Chaddock signs. As a secondary objective, we looked for a prevalence of one of the signs over the other, and the influence of the head position regarding the obtained responses. METHOD: The patients were examined while supine with their heads in three different positions. RESULTS: Out of the one hundred patients, ten of them (10%) showed hallux extension uni or bilateral. The Babinski sign was positive 18 times (40%), and the Chaddock sign was positive 27 times (60%). CONCLUSION: The Chaddock sign occurred more frequently than the Babinski sign, the abnormal reflex occurred twice as much on the left foot than the right, and apparently there was no interference regarding the head position in relation to the obtained results.

Asunto(s)

Tractos Piramidales/fisiopatología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Cabeza , Humanos , Masculino , Persona de Mediana Edad , Enfermedades del Sistema Nervioso/diagnóstico , Enfermedades del Sistema Nervioso/fisiopatología , Postura , Reflejo Anormal/fisiología , Reflejo de Babinski/diagnóstico , Reflejo de Babinski/fisiopatología

RESUMEN

OBJETIVO: Verificar a presença dos sinais de Babinski e de Chaddock em cem pacientes sem história ou indícios clínicos de comprometimento da via piramidal, internados no Serviço de Clínica Médica do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro. Como objetivos secundários, observar a possível prevalência de um sinal sobre o outro, assim como a influência da posição da cabeça sobre as respostas obtidas. MÉTODO: Cada um dos sinais foi pesquisado por um único autor, utilizando o mesmo instrumento, estando os pacientes em decúbito dorsal e com a cabeça em três posições. RESULTADOS: Em dez pacientes (10 por cento) obteve-se a resposta de extensão do hálux uni ou bilateral. O sinal de Babinski apresentou-se 18 vezes (40 por cento) e o sinal de Chaddock 27 vezes (60 por cento). CONCLUSÃO: O sinal de Chaddock foi o mais freqüente. As respostas anormais ocorreram duas vezes mais à esquerda e, aparentemente, não houve interferência da posição cefálica em relação às respostas obtidas.

Asunto(s)

Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Tractos Piramidales/fisiopatología , Cabeza , Enfermedades del Sistema Nervioso/diagnóstico , Enfermedades del Sistema Nervioso/fisiopatología , Postura , Reflejo Anormal/fisiología , Reflejo de Babinski/diagnóstico , Reflejo de Babinski/fisiopatología

RESUMEN

Inherited prion diseases are characterized by mutations in the PRNP gene encoding the prion protein (PrP). We report a novel missense mutation in the PRNP gene (resulting in a G114V mutation in PrP) in members of a Uruguayan family with clinical and histopathologic features of prion disease. Affected individuals were characterized by an early age at onset, initial neuropsychiatric symptoms, late dementia with prominent pyramidal and extrapyramidal symptoms, and long disease duration.

Asunto(s)

Amiloide/genética , Encéfalo/fisiopatología , Predisposición Genética a la Enfermedad/genética , Mutación/genética , Enfermedades por Prión/genética , Precursores de Proteínas/genética , Adolescente , Adulto , Edad de Inicio , Sustitución de Aminoácidos/genética , Biopsia , Encéfalo/metabolismo , Encéfalo/patología , Aberraciones Cromosómicas , Análisis Mutacional de ADN , Demencia/genética , Demencia/patología , Demencia/fisiopatología , Progresión de la Enfermedad , Resultado Fatal , Femenino , Lóbulo Frontal/metabolismo , Lóbulo Frontal/patología , Lóbulo Frontal/fisiopatología , Pruebas Genéticas , Humanos , Masculino , Trastornos de la Personalidad/genética , Trastornos de la Personalidad/patología , Trastornos de la Personalidad/fisiopatología , Enfermedades por Prión/patología , Enfermedades por Prión/fisiopatología , Proteínas Priónicas , Priones , Tractos Piramidales/metabolismo , Tractos Piramidales/patología , Tractos Piramidales/fisiopatología , Uruguay

RESUMEN

Corticoespinal dysfunction is a common finding in primary and amyotrophic lateral sclerosis (PLS and ALS). AIM. To compare the behaviour of motor evoked potentials (MEP) with transcranial magnetic stimulation (TMS) in patients with ALS and PLS. It was performed a retrospective analysis of MEP recordings of 11 patients with PLS and 10 patients with sporadic ALS. Central motor conduction time and amplitude ratio were the selected variables for the statistical analysis of MEP from abductor pollicis brevis and tibialis anterior muscle from the four limbs, using non-parametric methods. As a general observation there was a high incidence of abnormal recordings in both groups of patients; in 30 percent of recording from ALS patients response to TMS was absent, but only the 4,5 percent in the group of PLS had the same characteristic. In PLS patients abnormal central motor conduction time was the most frequent finding, as it was the low amplitude ratio in ALS patients; both variables showed statistically significant differences between groups (Kruskall-Wallis, H = 6,32, p = 0,011; and Kruskall-Wallis, H = 5,777, p = 0,0163, respectively). Corticoespinal dysfunction has different characteristics in ALS and PLS patients, and the analysis of MEP could add useful information for differential diagnosis of these diseases(AU)

Asunto(s)

Humanos , Masculino , Femenino , Persona de Mediana Edad , Esclerosis Amiotrófica Lateral/fisiopatología , Potenciales Evocados Motores , Conducción Nerviosa/fisiología , Tractos Piramidales/fisiopatología

RESUMEN

INTRODUCTION: Corticoespinal dysfunction is a common finding in primary and amyotrophic lateral sclerosis (PLS and ALS). AIM. To compare the behaviour of motor evoked potentials (MEP) with transcranial magnetic stimulation (TMS) in patients with ALS and PLS. PATIENTS AND METHODS: It was performed a retrospective analysis of MEP recordings of 11 patients with PLS and 10 patients with sporadic ALS. Central motor conduction time and amplitude ratio were the selected variables for the statistical analysis of MEP from abductor pollicis brevis and tibialis anterior muscle from the four limbs, using non-parametric methods. RESULTS: As a general observation there was a high incidence of abnormal recordings in both groups of patients; in 30% of recording from ALS patients response to TMS was absent, but only the 4.5% in the group of PLS had the same characteristic. In PLS patients abnormal central motor conduction time was the most frequent finding, as it was the low amplitude ratio in ALS patients; both variables showed statistically significant differences between groups (Kruskall-Wallis, H = 6.32, p = 0.011; and Kruskall-Wallis, H = 5.777, p = 0.0163, respectively). CONCLUSION: Corticoespinal dysfunction has different characteristics in ALS and PLS patients, and the analysis of MEP could add useful information for differential diagnosis of these diseases.

Asunto(s)

Esclerosis Amiotrófica Lateral/fisiopatología , Potenciales Evocados Motores/fisiología , Conducción Nerviosa/fisiología , Tractos Piramidales/fisiopatología , Anciano , Femenino , Humanos , Magnetismo , Masculino , Persona de Mediana Edad , Músculo Esquelético/fisiología , Estudios Retrospectivos

RESUMEN

OBJECTIVE: Posteroventral pallidotomy (PVP) has proved to be an effective method for the treatment of Parkinson's disease. However, data on bilateral procedures are still limited. To assess the effects of bilateral globus pallidus (GPi) lesion and to compare it with a combination of unilateral GPi lesion plus contralateral GPi stimulation (PVP+PVS), an open blind randomised trial was designed. METHODS: A prospective series of patients with severe Parkinson's disease refractory to medical treatment, and severe drug induced dyskinesias, were randomised either to simultaneous bilateral PVP or simultaneous PVP+PVS. All patients were assessed with the core assessment programme for intracerebral transplantation (CAPIT), and a comprehensive neuropsychological and neuropsychiatric battery both before surgery and 3 months later. RESULTS: The severe adverse effects found in the first three patients subjected to bilateral PVP led to discontinuation of the protocol. All three patients developed depression and apathy. Speech, salivation, and swallowing, as well as freezing, walking, and falling, dramatically worsened. By contrast, all three patients undergoing PVP+PVS had a significant motor improvement. CONCLUSION: Bilateral simultaneous lesions within the GPi may produce severe motor and psychiatric complications. On the other hand, a combination of PVP+ PVS significantly improves parkinsonian symptoms not associated with the side effects elicited by bilateral lesions.

Asunto(s)

Terapia por Estimulación Eléctrica/métodos , Lateralidad Funcional/fisiología , Globo Pálido/fisiopatología , Globo Pálido/cirugía , Procedimientos Neuroquirúrgicos/métodos , Enfermedad de Parkinson/fisiopatología , Enfermedad de Parkinson/terapia , Tractos Piramidales/fisiopatología , Anciano , Trastornos de Deglución/etiología , Trastorno Depresivo/etiología , Método Doble Ciego , Femenino , Humanos , Masculino , Procedimientos Neuroquirúrgicos/efectos adversos , Enfermedad de Parkinson/psicología , Enfermedad de Parkinson/cirugía , Estudios Prospectivos , Trastornos del Habla/etiología , Síndrome

RESUMEN

Twenty-six patients with syringomyelia were studied with polysomnography to determine the frequency of periodic limb movements (PLM) and its relationship to the presence of a Chiari anomaly, the severity of corticospinal tract involvement, and localization of the syrinx. Sixteen patients showed PLM in stages I and II of non-REM sleep and three PLM also while awake. There were no statistically significant differences in overall disability, corticospinal signs, presence of an associated Chiari anomaly, and disease duration between patients with and without PLM, although there was a trend for patients with PLM to have more severe disease. There was preservation of the lumbosacral enlargement of the spinal cord by the syrinx in all patients with PLM. The latency delay between lower and upper limb muscles was suggestive of conduction along propriospinal pathways. Syringomyelia may lead to an abnormal state of spinal hyperexcitability favoring the appearance of PLM. Detailed magnetic resonance image studies of patients with different localizations of the syrinx cavities may help to determine which tracts are involved in the production of PLM.

Asunto(s)

Malformación de Arnold-Chiari/diagnóstico , Síndrome de Mioclonía Nocturna/diagnóstico , Polisomnografía , Siringomielia/diagnóstico , Adolescente , Adulto , Anciano , Malformación de Arnold-Chiari/fisiopatología , Electromiografía , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Bulbo Raquídeo/patología , Bulbo Raquídeo/fisiopatología , Persona de Mediana Edad , Neuronas Motoras/fisiología , Síndrome de Mioclonía Nocturna/fisiopatología , Tractos Piramidales/patología , Tractos Piramidales/fisiopatología , Tiempo de Reacción/fisiología , Fases del Sueño/fisiología , Médula Espinal/patología , Médula Espinal/fisiopatología , Siringomielia/fisiopatología

RESUMEN

En 1896 Babinski describe por primera vez el fenómeno de los dedos. En este primer trabajo simplemente decribe la extensión de todos los dedos con el estímulo nociceptivo de la planta del pie. Es hasta 1898 que específicamente describe la extensión del primer dedo con la estimulación del borde lateral de la planta. Babinski probablemente no estaba enterado que E. Remak, un médico alemán, había descrito previamente el signo. En su tercer artículo de 1903, Babinski discute el tema y concluye que si otros autores descritos el reflejo anormal antes que él, lo había encontrado de una manera fortuita y no reconocieron su valor semiológico. Babinski probablemente descubrió su signo por una combinación de casualidad, observación cuidadosa e intuición. También tuvo en mente la aplicación práctica del signo, particularmente en el diagnóstico diferencial con la histeria y en el área médica-legal. Varias de las observaciones y mecanismos fisiopatológicos propuestos por Babinski son válidos actualmente. Babinski reconoce, desde 1896, que el reflejo era parte de la sinergia del reflejo flexor. Observa que varios pacientes durante las primeras horas de una lesión cerebral o espinal aguda del reflejo extensor. Reconoce que la mayoría de los pacientes con el reflejo anormal tienen debilidad de dedos y tobillo. Encuentra una ausencia de correlación entre los reflejos miotáticos hiperactivos y la presencia de la extensión del primer dedo, y descubre que no todos los pacientes con hemi o paraplejia tienen el signo. Pensaba, erróneamente, que algunos sujetos normales podían tener la extensión del primer dedo. Su sueño de una aplicación práctica del signo se consiguió completamente. El motto de Babinski fue observatio summa lex. Quizá no hay mejor aforismo en la neurología clínica

Asunto(s)

Humanos , Adulto , Trastornos Cerebrovasculares/fisiopatología , Enfermedades de la Médula Espinal/fisiopatología , Medicina Legal/historia , Historia de la Medicina , Historia del Siglo XIX , Neuronas Motoras/fisiología , Enfermedades del Sistema Nervioso/fisiopatología , Neurología/historia , Tractos Piramidales/fisiopatología , Reflejo de Babinski

RESUMEN

In 1896 Joseph Francois Felix Babinski described for the first time the phenomenon of the toes. In his first paper he simply described extension of all toes with noxious stimulation of the sole of the foot. It was not until 1898 that he specifically described the extension of the hallux with stimulation of the lateral border of the sole. Babinski was probably not aware at the time that E. Remak, a German physician, had previously described the sign. In his third paper of 1903 Babinski concludes that if other authors had described the abnormal reflex before him, they found it fortuitously and did not realize its semiologic value. Babinski probably discovered it by a combination of chance, careful observation and intuition. He also had in mind practical applications of the sign particularly in the differential diagnosis with hysteria and in medico-legal areas. Several of his observations and the physiopathological mechanism proposed by him are still valid today. He realized since 1896 that the Babinski reflex was part of the flexor reflex synergy. He observed that several patients during the first hours of an acute cerebral or spinal insult had absent extensor reflexes. He realized that most patients with the abnormal reflex had weakness of the toes and ankles. He found a lack of correlation between hyperactive myotatic reflexes and the presence of an upgoing hallux. He discovered that not all patients with hemiplegia or paraplegia had the sign. He thought erroneously that some normal subjects could have an upgoing toe. His dream of a practical application of the sign has been fully achieved. The motto of Babinski was Observatio summa lex. Perhaps there is no better dictum in clinical neurology.

Asunto(s)

Neurología/historia , Reflejo de Babinski , Adulto , Trastornos Cerebrovasculares/fisiopatología , Medicina Legal/historia , Historia del Siglo XIX , Historia del Siglo XX , Humanos , Recién Nacido , Neuronas Motoras/fisiología , Enfermedades del Sistema Nervioso/fisiopatología , Tractos Piramidales/fisiopatología , Reflejo de Babinski/fisiología , Enfermedades de la Médula Espinal/fisiopatología