The Brazilian database on pregnancy in multiple sclerosis.

OBJECTIVES: To report the results from the Brazilian database on multiple sclerosis (MS) and pregnancy. METHODS: Retrospective data from MS patients who became pregnant at any time of their disease were sent to a Brazilian database, using a specific file for this purpose. RESULTS: Data on 128 women (142 pregnancies) from 30 neurologists working in 21 cities in Brazil were collected. Patients' average age at pregnancy was 29.8 years (range 16-42). EDSS at start of pregnancy was 1.5±1.4; and the relapse rate in the year preceding pregnancy was 1.2±1.5. Exposure to medication at any time during pregnancy was high (69.7%): 48.6% to interferon beta; 14.1% to glatiramer acetate; and 7% to other immunomodulatory and immunosuppressive drugs. There was a significant decrease in relapse rate during pregnancy. The prevalence of complications was relatively low, with 4.9% of obstetric and 1.4% neonatal unfavorable outcomes. CONCLUSIONS: Our patients had low degrees of disability, short histories of disease, high drug exposure, and relatively high relapse rate in the year previous to pregnancy. Obstetric and neonatal outcomes were successful in over 90% of our patients.

Naproxen, clenbuterol and insulin administration ameliorates cancer cachexia and reduce tumor growth in Walker 256 tumor-bearing rats.

Cancer cachexia is characterized by anorexia and intense peripheral catabolism. We examine the potential benefits of combination of different anabolic agents such as insulin and clenbuterol associated to prostaglandin synthesis inhibitor (naproxen) on tumor growth, cachexia and renal function in Walker 256 tumor-bearing rats (WK). Groups were separated into WK, and WK with naproxen (WK N) or naproxen plus clenbuterol (WK NCb) or naproxen plus clenbuterol plus insulin (WK NCbI). Treatment begins at the 4th day after tumor inoculation, at the 14th day they were killed, glycemia, lacticidemia, glycogen content from liver, soleus and gastrocnemius muscles, tumor mass, body weight and kidney function were determined. Glycemia and glycogen content were reduced and lacticidemia increased in WK (p<0.05) as compared to control rats. The glycogen content recovered in all treated groups. Tumor weight was significantly reduced by the different treatments. At the 14th weight change (carcass-initial body weight) in the control increased by 38% and in the WK -2%. Naproxen treatment (WK N) induced an increased by 14%. The inclusion of clenbuterol (WK NCb) and insulin (WK NCbI) by 38 and 41%, respectively. Mean glomerular filtration rate (GFR) increased in the WK (p<0.05) as compared to control, but in the WK NCb the GFR was similar to control. Our results suggest that naproxen is able to reduce tumor growth and its association with insulin and clenbuterol induce mass weight gain and recovery energy fuel.

Amyotrophic lateral sclerosis in Brazil: 1998 national survey.

OBJECTIVES: To assess the epidemiologic characteristics of amyotrophic lateral sclerosis (ALS) in Brazil in 1998. METHOD: Structured Clinical Report Forms (CRFs) sent to 2,505 Brazilian neurologists from January to September 1998 to be filled with demographic and clinical data regarding any ALS patient seen at any time during that year. RESULTS: Five hundred and forty CRFs were returned by 168 neurologists. Data on 443 patients meeting the criteria of probable or definite ALS according to El Escorial definition were analysed: 63 probable (14.2%) and 380 definite (85. 8%). Two hundred and fifty-nine (58.5%) of the patients were male, mean age of onset was 52. Spinal onset occurred in 306 patients (69%); bulbar onset in 82 (18.5%), and both in 52 (11.7%). Twenty-six (5.9%) had a family history of ALS. Two hundred and fifty-nine (58.6%) were seen by private practitioners, and 178 (40. 2%) at a hospital clinic. Age-ajusted incidence shows a peak incidence at the 65-74 years old range. CONCLUSIONS: The disease's characteristics are similar to those described in international studies, except for age of onset (Brazilian patients are younger). This difference is not confirmed when figures are age-adjusted.

Myasthenia gravis and peripheral neuropathy in an Amazon indigenous female.

A 15 year-old female presented with anti-acetylcholine receptor antibody-positive myasthenia gravis and electrophysiological signs of sensory peripheral neuropathy. The repetitive stimulation test showed decremental response of 31% in the median nerve. The clinical picture improved with prostigmine, corticosteroids, plasmapheresis and thymectomy. This is the first case report of an Amazon indigenous patient with myasthenia gravis.

[Possible role of the immune system in lead peripheral polyneuropathy. Case report]. / Possível intermediação do sistema imune na polineuropatia periférica plúmbica. Relato de caso.

This is a case report of a twenty-five years old man who developed, due to lead intoxication, a severe axonal peripheral predominantly motor neuropathy, after a shotgun injury. The projectile was retained in the right hip. Before this diagnosis had been done he was treated with corticosteroids in immunosuppressive doses and showed an improvement, but he had worsened at each attempt to interrupt the drug. Because he had also other signs of lead intoxication, such as abdominal cramps, severe anemia and seizures it was search for the blood levels of lead that was 101.2 micrograms/dl. The patient was treated with calcium disodium edetate and surgical removal of lead fragments. After that he had a good outcome with no need of corticosteroids. It is emphasized the possible relevance of the immune system on the mechanism of plumbic intoxication and the importance of the withdrawal of the lead material retained in joints.

Marcapasso endocárdico em recém-nascido / Endocardial pacemakerin small children

Os autores relatam o caso em que uma criança de 48 horas de vida, portadora de bloqueio atrioventricular (BAV) de grau avançado, bradicardia acentuada e insuficiência cardíaca importante foi submetida com sucesso a um implante de marcapasso endocárdico. Como via de acesso foi utilizada a veia jugular interna, o cabo-eletrodo de fixaçäo passiva foi posicionado no ventrículo direito e uma alça foi efetuada no interior do átrio direito. Com o crescimento da criança, observou-se o progressivo desenrolar do cabo-eletrodo. A freqüência inicial do marcapasso com que se obteve melhor débito cardíaco foi de 120 ppm, valor definido através de ecocardiograma bidimensional. Após 28 meses a criança vem apresentando ótima evoluçäo clínica. O marcapasso atualmente demonstra bom funcionamento, tanto no comando como na sensibilidade, e o cabo-eletrodo apresenta desenrolar suave, acompanhando o crescimento da criança.

[Lafora's disease: a possible diagnosis of juvenile dementia]. / Doença de lafora: uma possibilidade diagnóstica das demências juvenis.

Five patients aged 12 to 16 years old were admitted between 1987 and 1994 at the neurological unit because of uncontrolled epilepsy. They had had a normal development until the adolescence, when a history of poor school performance and memory difficulty started. It is emphasized the clinical sequence of dementia followed by tonic-clonic seizures and myoclonus, the positive family history and the difficult therapeutic management, in spite of multiple anticonvulsant combinations, including sodium valproate and clonazepam. The clinical and laboratory differential diagnosis were discussed to show that similar cases should be submitted to skin biopsy looking for Lafora bodies in apocrine and eccrine glands. However, the liver is considered as the most reliable site for the biopsy, which in our study showed positivity in four out of the five cases.

[Vascular stroke of the brain stem. Study of 21 cases]. / Acidente vascular de tronco encefálico. Estudo de 21 casos.

A prospective study was designed to follow patients with syndromes of brain stem during 12 months (in 1991-1992). The aim was to correlate clinical and radiologic findings and comparing them with the classical descriptions of brain stem syndromes. Twenty one consecutive patients were admitted at the Hospital de Base do Distrito Federal. The diagnosis and the follow up were carried out by neurological examination and neuroradiologic images: CT (100%), angiography (24%, 5) and MRI (5%, 1). Hypertension and old age were the most important risk factors and hemorrhage at pons with extension to midbrain was the most frequent finding. Coma at admission was associated to a poor outcome. The outcome was defined by the Glasgow Outcome Scale: 28% rated 5, 24% rated 4, 5% rated 3, 28% rated 2 and 14% rated 1. Our results show that the clinical presentation of ischemic strokes and hemorrhages of the brain stem very frequently follow a mixed pattern, which do not conciliate with the classical of these syndromes.

[Hemifacial spasm and basilar impression associated with Arnold-Chiari deformity. Report of a case]. / Espasmo hemifacial e impressão basilar associados a malformação de Arnold-Chiari. Relato de caso.

The authors report a case of symptomatic basilar impression and Arnold-Chiari malformation being presented as the first symptom of hemifacial spasm. The surgical treatment of the malformation resulted in improvement of the clinical manifestation with reduction of the hemifacial spasm. The need for the aetiological therapy for the hemifacial spasm is emphasized, before symptomatic treatment with botulinum toxin is tried.

[Correlation of HLA and myasthenia gravis in a Brazilian population]. / Estudo da correlação HLA e miastenia gravis em uma população brasileira.

Based on the fact that Brazilian ethnic groups are mixed and therefore different from caucasian, chinese or japanese populations the authors studied the HLA antigen distribution in 37 myasthenic patients. The control group consisted of 69 healthy individuals of the same population studied in the same laboratory. The antigens HLA B8 showed the highest relative risk for female, younger than 40 years old, with thymic hyperplasia. Also the HLA B8 and A1 showed high frequency for black myasthenic patients.

[Familial myasthenia gravis: a case report in identical twins]. / Miastenia grave familiar: relato de caso em gêmeas idênticas.

To the best of our knowledge this is the seventh case report of monozygotic twins both affected by myasthenia gravis (MG). The monozygotism was proven by sex identity, blood group and HLA determinations ('O' Rh+, A2, A19.2, B40, CW3). One paternal aunt was also affected and the three cases have high titles of anti-acetylcholine receptor antibodies and anti-striated muscle antibodies, which indicate an acquired form of MG. After several myasthenic crises the twins are now doing well with corticosteroid therapy. The paternal aunt has a more benign form of MG, with ocular and limb involvement (grade IIa of Osserman) and was submitted to thymectomy. The authors discuss the use of corticosteroid for the infantile form of MG instead of thymectomy, based on the immaturity of the immune system in the young age. The use of other immunosuppressive drugs is not advisable because of potential hazardous development of neoplasms.

[Myasthenia gravis: treatment with thymectomy, corticoids and plasmapheresis]. / Miastenia grave. Tratamento com timectomia, corticóide e plasmaferese.

This paper reviews a 12 year experience with myasthenia gravis management (surgical and drug therapy). A total of 27 patients were submitted to thymectomy, and 12 out of 13 showed fairly good results with this only form of therapy (15.3% with complete remission, 46.15% with marked improvement and 30.7% with moderate improvement). The other 14 need either a combination of surgery and plasmapheresis or corticosteroids with the cummulative results of: 14.8% of remission (4 out of 27), 74% of improvement (20 out of 27), 7.4% of worsening (2 out of 27) and 3.7% without change (1 out of 27). Two other patients not submitted to surgery showed either a stable state of their symptoms or a mildly worsening. Another eight patients not submitted to surgery could not be bollowed up. The authors also conclude by the validity of the use of plasmapheresis in myasthenic crises leading to a transient relief of the symptoms and suggest the use of corticosteroids as a second choice, due to their undesirable side effects and difficulty in their reduction and elimination without worsening the symptoms. Other immunosuppressive drugs could be used in cases in which those above cited therapies showed unsuitable results.

Chronic progressive external ophthalmoplegia. I. A quantitative histochemical study of skeletal muscles.

This study quantitates the major morphological and cytochemical changes in limb muscle biopsies from 37 patients with the syndrome of chronic progressive external ophthalmoplegia (CPEO). The aim was to assess the value of limb muscle biopsy in the diagnosis of this syndrome; to define the myopathological changes and to determine whether there were any specific clinico-pathological correlations. Patients were divided into three clinical groups--11 patients with CPEO with facial and/or limb muscle weakness; 10 with CPEO with facial and/or limb muscle weakness and a positive family history; 16 with CPEO with one or more of the following: pigmentary retinopathy, cerebellar ataxia, pyramidal signs and peripheral neuropathy. The following parameters were measured: the proportions of histochemical fibre types, the muscle fibre areas and the percentage of muscle fibres showing increased oxidative enzyme activity. Pooled results for each of the clinical categories were compared. Statistical analysis of fibre areas and the percentage of fibres with increased oxidative enzyme activity, showed that group 2 differed from the others (p less than 0.05). Patients in group 2 showed the highest incidence of type 1 fibre hypertrophy, type 2A atrophy and the lowest incidence of fibres with increased oxidative activity. Fibre type disproportions occurred in all three groups but the differences were not significant.

Chronic progressive external ophthalmoplegia. II. A qualitative and quantitative electronmicroscopy study of skeletal muscles.

This study quantifies the major electron microscopic changes in limb muscle biopsies from 31 out of 34 patients with the syndrome of chronic progressive external ophthalmoplegia. Patients were divided into three clinical groups -- A) 10 sporadic cases with muscle weakness only; B) 9 familial cases with muscle weakness only; C) 15 cases with muscle weakness and one or more of the following features: pigmentary retinopathy, cerebellar ataxia, pyramidal signs and peripheral neuropathy. Electron microscopic mitochondrial abnormalities were found in all groups (8 patients from group A, 3 from group B, 14 from group C). Quantitative measurements of certain muscle fibre constituents, using a point-counting technique, revealed decreased myofibril volume-fractions and increased volume-fractions of mitochondria, glycogen and lipid in some biopsies from each group. Mitochondrial volume-fractions correlated positively with lipid content, the proportion of type 1 fibres, and the percentage of fibres with increased oxidative enzyme activity. The three groups defined clinically showed no significant differences in terms of the relative proportions of these measured constituents.

[The use of corticosteroids in Guillain-Barré syndrome: study of 51 cases]. / O uso de corticosteróides na síndrome de Guillain-Barré: estudo de 51 casos.

This is a retrospective analysis of 51 patients admitted to the Hospital de Base do Distrito Federal (Brasília, Brazil), with the Guillain-Barré syndrome, from 1974 to 1984. Eighteen patients were treated with corticosteroids (prednisone or dexamethasone), whereas 33 did not receive such treatment. The clinical course (degree of recovery, sequelae, complications, causa mortis) and the length of the hospital stay were compared in these two groups. It is concluded that the use of corticosteroids did not have a major effect on the clinical course of the illness. Moreover, the only two cases of relapses occurred in patients that had been treated with corticosteroids.

[Bilateral palpebral ptosis with unilateral paralysis of upward gaze: a case report]. / Ptose palpebral bilateral com paralisia unilateral do olhar vertical para cima: registro de caso.

The authors report a case of bilateral ptosis with miosis, paralysis of upward gaze and of smooth pursuit movements to the right, as well as a slowly regressive state of lassitude. This syndrome followed a haemorrhagic brain stem infarction. After a review of the pathophysiology of ptosis and of vertical eye movements, together with analysis of data provided by computerized tomography studies--since the case was not fatal--explanations are offered for each clinical sign and it is proposed that the lesion, a paramedian, slightly deviated to the right haemorrhagic focus in the midbrain, with a diameter of 15 mm, might have involved several structures, namely the rostral interstitial nucleus of the medial longitudinal fasciculus, the mesencephalic reticular formation, as well as cortico-nuclear and sympathetic pathways.