[Corticospinal dysfunction in patients with primary and amyotrophic lateral sclerosis]. / Disfunción corticoespinal en pacientes con esclerosis lateral primaria y amiotrófica.

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.

Sindrome de Guillain-Barre / Guillain-Barre's syndrome

Objetivo. Hacer una revisión de este trastorno, poniendo énfasis en los cuidados intensivos del paciente con síndrome de Guillain-Barré (SCB) grave. Desarrollo. El SGB es una polineuropatía inflamatoria autoinmune, que puede originar cuadriparesia, fallo respiratorio, disfunción autonómica, así como otras complicaciones graves, que pueden influir notablemente en la evolución del enfermo. Por tal motivo, los pacientes con SGB grave requieren atención en unidades de cuidados intensivos (UCI), en donde también pueden surgir nuevas complicaciones además de las ya anteriormente mencionadas. El neurólogo que pretenda tratar integral y continuamente a estos enfermos deberá estar familiarizado con la terapia antimicrobiana, nutrición del enfermo crítico, equilibrio hidromineral, ciertos aspectos sobre ventilación mecánica, y las indicaciones y complicaciones de la plasmaféresis y de la infusión de inmunoglobulinas. Conclusiones. Con los cuidados intensivos disponibles actualmente, la evolución de estos enfermos suele ser excelente (recuperación en más de un 80 por ciento de los casos), aunque en algunos pacientes persiste cierto grado de paresia residual. Atendiendo a que el SGB es un proceso en gran medida autolimitado, se suele plantear que el cuidado esmerado y diario de estos enfermos y sus complicaciones en la UCI contribuye a la buena evolución de un paciente individual, tanto o más que la terapia inmunomoduladora

[Guillain Barre syndrome]. / Síndrome de Guillain-Barré

OBJECTIVE: To review about this disorder, with emphasis on the intensive care of severe Guillain Barr syndrome (GBS). DEVELOPMENT: GBS is an acute immune mediated inflammatory polyneuropathy that may lead to quadriparesis, ventilatory failure, and autonomic dysfunction but also to many general medical problems that have great bearing on outcome. Therefore severe GBS patients require admission into an intensive care unit (ICU), where in addition to the disorders mentioned before, other complications can arise. The neurologist who plans to deal comprehensively with these patients must be familiar with therapy for infections, nutrition, fluid management, and selected aspects of pulmonary medicine as well as the indications for and complications of plasma exchange and gammaglobulin infusion. CONCLUSIONS: With modern intensive care support, the outcome is excellent (>80% recovery), although in many cases a persistent residual paresis occurs. Because GBS is largely self limited, the skill daily cares of these patients in an ICU contributes as much, or more, to the overall outcome of an individual patient as do specific immune therapies.

Neurorrehabilitación en la esclerosis múltiple / Neurorehabilitation in multiple sclerosis

No disponible

Mieloneuropatía óptica epidémica cubana: discrepancias en relación con una posible etiología vírica / Cuban epidemic optic myeloneuropathy: discrepancies regarding its possibly viral aetiology

No disponible

[Intensive multifactorial rehabilitation in patients with multiple sclerosis]. / Rehabilitación multifactorial e intensiva en pacientes con esclerosis múltiple.

INTRODUCTION: Studies have been published recommending rehabilitation in multiple sclerosis (EM), but in practice this is often not indicated. PATIENTS AND METHODS: We made a retrospective analysis of 41 patients diagnosed as definitely having EM (clinical form of relapse remission, in phase of remission) according to the criteria of Poser et al. These patients carried out a programme of intensive multifactorial rehabilitation of 41 hours per week with a minimum duration of 10 days and a maximum of 178 days. There were classified on the EDSS scale of Kurztke and Hanser s ambulatory index on admission and when their treatment had finished. These results were compared using the Wilcoxon test for paired series and we analysed the relation between the time of treatment and final score on the scales applying the Spearman s multiple range correlation test. RESULTS: The differences were statistically significant between the scores for initial and final evaluations of the Kurztke and Hauser scales (Z: 3.17, p: 0.001475 and Z: 3.29, p: 0.000983 respectively). No correlation was shown between total duration of treatment or total duration of the disorder and the final score on the scale (p > 0.05). CONCLUSION: Intensive multifactorial rehabilitation treatment may have a positive effect in patients with Em with the clinical form of relapse remission, according to the scales applied. We therefore recommend their indication in patients during the remission phase.

Sobrediagnóstico en la epidemia de neuropatía de Cuba / Overdiagnosis in the epidemic of neuropathy in Cuba

No disponible

[Bioethical considerations in the approach to patients with amyotrophic lateral sclerosis]. / Algunas consideraciones bioéticas en el abordaje de los pacientes con esclerosis lateral amiotrófica.

INTRODUCTION: The traditional doctor-patient relation has become a great bioethical challenge due to the advances in science in recent years. This is particularly true when patients suffer diseases such as amyotrophic lateral sclerosis (ALS), a neurodegenerative disease with a relentless course and in spite of modern treatment 50% of the patients die within three years of first having symptoms of the disease. It therefore causes great psychological and social impact. OBJECTIVE: To analyze the great bioethical challenge which arises when diagnosing and treating a patient with ALS. DEVELOPMENT: In this paper we analyze the doctor-patient relationship, the principles of doing no harm and of being beneficial, and more modern concepts such as informed consent, biomedical investigations and euthanasia, as well as the importance of palliative medicine and rehabilitation to alleviate suffering and improve quality of life. Biomedical investigations should conform to the relevant national and international rules. We discuss the right of patients to be given truthful information. CONCLUSIONS: We recommend better training of doctors in all aspects of attention to these patients, with emphasis on the diagnosis and importance of rehabilitation, palliative medicine and the management of psychological aspects. Biomedical investigations should fulfil current regulations. We recommend discretion, complete or partial, with regard to information given to the patients and their relatives so as not to cause despair.

[The importance of multifactorial rehabilitation treatment in amyotrophic lateral sclerosis]. / Importancia del tratamiento rehabilitador multifactorial en la esclerosis lateral amiotrófica.

INTRODUCTION: The treatment of amyotrophic lateral sclerosis (ALS) is still a major challenge. Rehabilitation treatment is scarcely considered and its usefulness in these patients continues to be controversial. PATIENTS AND METHODS: A multidisciplinary team made up of neurologists, physiotherapists, logopaedists, defectologists, psychologists and specialist physicians treated six patients with ALS in an intensive rehabilitation programme of 41 hours per week for four weeks. We follow certain basic principles in rehabilitation including: 1. Treatment by a multidisclipinary team; 2. Treatment tailored to the individual; 3. Avoidance of muscle fatigue and vigorous exercise, and 4. Intensive treatment with carefully measured amounts of different activities (logophoniatrics, occupational therapy, psychology, physical therapy, etc.) to avoid fatigue. The patients fulfilled the criteria of E1 Escorial for the diagnosis of definite ALS, and gave their informed consent to undergo the treatment. Forced Vital Capacity (FVC) and ALS Functional Rating Scale (ALSFRS) tests were done on all patients at the beginning and end of the treatment. The Wilcoxon test for paired series, comparing scores at the start and finish, were done in each case. RESULTS: In all patients the FVC and ALSFRS improved after the treatment. The results were statistically significant (Z: 2.2013; p= 0.027) on the Wilcoxon paired series test and no complications were seen. CONCLUSIONS: Intensive, multifactorial rehabilitation treatment for four weeks improved the FVC and ALSFRS in all patients with ALS and no complications were seen. Until there is a curative treatment for ALS, multifactorial rehabilitation remains the best hope for these patients.

Algunas consideraciones bioéticas en el abordaje de los pacientes con esclerosis lateral amiotrófica / Bioethical considerations in the approach to patients with amyotophic lateral sclerosis

Introducción. La tradicional relación médico-paciente se ha convertido con el desarrollo científico de las últimas décadas en un gigantesco reto bioético, especialmente cuando los seres humanos padecen enfermedades como la esclerosis lateral amiotrófica (ELA), enfermedad neurodegenerativa de curso inexorable, en la que, a pesar de los tratamientos actuales, mueren el 50 por ciento de los pacientes en los tres primeros años del inicio de los síntomas, por lo que produce un enorme impacto psicológico y social. Objetivo. Analizar el gran reto bioético que ocurre al diagnosticar y tratar a un paciente con ELA. Desarrollo. En el presente artículo se analizan la dinámica de la relación médico-paciente, los principios de no maleficencia y beneficiencia, y temas más recientes como el consentimiento informado, las investigaciones biomédicas y la eutanasia, al igual que la importancia de la medicina paliativa y la rehabilitación para aliviar los sufrimientos y mejorar la calidad de vida. Las investigaciones biomédicas deben cumplir las reglamentaciones nacionales e internacionales existentes al respecto. Se discute el derecho de los pacientes a la veracidad de la información. Conclusiones. Se recomienda una mejor formación de los médicos en todos los aspectos concernientes a estos pacientes, donde se haga énfasis en el diagnóstico y la importancia de la rehabilitación, medicina paliativa y en el manejo de los aspectos psicológicos. Las investigaciones biomédicas deben cumplir las reglamentaciones existentes. Se recomienda tener en ocasiones reserva absoluta o relativa de la información con respecto a los pacientes y a algunos familiares, para no provocar la desesperanza (AU)