[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

[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.

[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.

[Juvenile amyotrophic lateral sclerosis. Apropos of a case]. / Esclerosis lateral amiotrófica juvenil. A propósito de un caso.

INTRODUCTION: There are scanty reports on juvenile forms of amyotrophic lateral sclerosis, specially amyotrophic lateral sclerosis and deafness, and it is known as Madras pattern of motor neurone disease. CLINICAL CASE: We describe an sporadic case of juvenile amyotrophic lateral sclerosis with deafness in a young person who started with hearing loss at 21 years old, loss of strength in upper limbs and muscular atrophy. He was seen by a neurologist when he was 25 years old, there were evident generalized fasciculation activity in proximal and distal muscles in the four limbs and the tongue, with swallowing troubles, and increased tendon reflexes in lower limbs with abnormal plantar extensor responses. All the paraclinical test were normal, except the electromyogram, showing a classical pattern of lower motor neuron disease, and the auditory brain stem response with absence of the main components of this evoked response, as expression of VIII cranial nerve damage. DISCUSSION: Patients like this one were first described in Madras (India), and the evolution of this kind of juvenile form of amyotrophic lateral sclerosis is chronically progressive and relative benign, in relation to the classical form of amyotrophic lateral sclerosis and other forms of motor neurone disease which begin in childhood, adolescence or young adulthood. CONCLUSION: Its recognition is very important in order to diminish misleading therapies in these patients.

[Bilateral cyclic sciatica caused by endometriosis. Apropos of a case]. / Ciática cíclica bilateral por endometriosis. A propósito de un caso.

INTRODUCTION: Cyclical sciatica due to implantation of endometrial tissue in the sciatic nerve in the region of the sciatic notch is a very unusual cause of sciatica. It occurs in women of childbearing age, as episodes of pain in the distribution of the sciatic nerve, which present in a cyclic manner and coincide with menstruation. If it is not treated, a sensomotor mononeuropathy of the sciatic nerve develops. CLINICAL CASE: The patient had complained of right-sided sciatic pain from the age of 36 years. Over the years a motor deficit had slowly and progressively appeared causing foot drop. The painful crises were related to her menstrual periods. At the age of 44 years a pyramidal muscle syndrome was diagnosed and treated surgically. This was followed by increase in the crises of sciatic pain. A year later, she started to have sciatic pain on the left side, which was similar to that of the right side. The clinical, imaging and electrophysiological findings are reported. The patient improved. She is still being treated with depot medroxyprogesterone and her pain has disappeared. CONCLUSIONS: Cyclical sciatica due to endometriosis is little known and may lead to permanent disability. Computerized axial tomography of the pelvis using contrast material is very useful for diagnosis. The use of depot medroxyprogesterone seems to be a satisfactory treatment in some patients.

[Neurological Behcet syndrome. Presentation of three cases]. / Neurobehçet. Presentación de tres casos.

INTRODUCTION: Behçet's disease is a multisystemic illness in which neurological abnormalities are seen in 5-48% of cases. CLINICAL CASES: We present 3 patients (2 men and 1 women) diagnosed as having Behçet's disease, according to the criteria of the 'International Study Group for Behçet's Disease' of 1990. We describe the clinical findings in these patients, the results of investigations done (MR or CT, evoked potentials-PEV, PESS, PEATC-EEG, ECN, CSF and neuropsychological tests), their course and response to treatment with hyperimmune human gammaglobulim at a dose of 400 mg/kg body weight in 2 patients. All three patients had repeated episodes of meningitis and alterations of the cranial nerves; two patients had epileptic seizures and two had signs of cerebellar disorders and dementia at some time during their illness. One patient died and the other two survived with severe disabilities. Cranial CT showed a cerebral infarct in one patient; evoked potentials showed axon damage in the two patients in whom this was studied. In the only patient in whom ECN was done, signs of axonal polyneuropathy were found. In all three patients there was pleocytosis in the CSF. In the patients in whom hyperimmune human gammaglobulin was given, the results were: one improved after this treatment; in the other there was clinical deterioration, but this was considered to be secondary to a curettage done the day before. CONCLUSION: The presence of neurological findings in Behçet's disease not caused by cerebral vein thrombosis worsens the prognosis of these patients.

[Stroke in a child due to hemoglobinopathy AS]. / Ictus en un niño por hemoglobinopatía AS.

INTRODUCTION: Falciform cell anaemia is a genetically determined haemoglobinopathy which in homozygote form (HbSS) is accompanied by neurological disorders in a quarter of the patients, mainly in the form of cerebral ischemia. Some authors consider the heterozygote (HbAS) form to be asymptomatic, although several such patients have been described with stroke. CLINICAL CASE: A white boy of 5 years of age had a cerebral infarct whilst travelling by car from his home town (222 meters above sea level) to the capital of the country (which is 2,600 meters above sea level). During the journey he complained of headache, vomiting and the onset of right hemiplegia. Angio-MR and CT showed the characteristic features of an infarct. SPECT showed hypoperfusion of left frontal predominance and of the basal ganglia bilaterally, but with left-sided predominance. Electrophoresis studies of his haemoglobin showed the present of AS haemoglobinopathy. We present the results of investigations done and a review of the literature. CONCLUSIONS: AS haemoglobinopathy, together with hypoxia due to altitude and possibly slight dehydration, were probably the causes of our patient's cerebral infarct. We recommended electrophoresis of haemoglobin in all patients (especially children and young adults) who present with stroke.