[Surgical treatment of blepharospasm. Report of 2 patients]. / Tratamento cirúrgico do blefaroespasmo essencial. Relato de dois pacientes.

Essential blepharospasm can be approached by several types of treatment: clinical in which the most important is the botulinum toxin with advantages and limitations; and some options of surgical treatment. The surgical technique described by Gillum and Anderson offers an alternative to patients resistant to the clinical treatment or with secondary complications. Two cases are analysed. Myectomy of all accessible orbicularis oculi, procerus and corrugator superciliaris muscle associated with blepharoplastic surgery and frontal lifting was indicated due to poor answer to clinical treatment. The results were satisfactory and both patients returned to their normal activities.

Pontine tegmentum hematoma. Report of a case with pure hemiplegia.

The authors report the case of a 50 year-old hypertensive male patient with a pontine hematoma. The clinical presentation was characterized by pure pyramidal deficit signs (no other signs or symptoms were present). A pure hemiplegia syndrome, although common in supratentorial lesions, is considered to be a rare event in pontine vascular lesions. The pathophysiologic mechanisms of these neurological findings are unclear. The exclusive involvement of the pyramidal tract in this case is likely due to a variation in the vascular anatomy of the pons but, in some cases, a vascular malformation may be the cause.

Parkinson's disease.

Parkinson's disease (PD) is one of the most common neurodegenerative diseases and affects 150 to 200 people per 100,000 population. Rapid advances have been made in the neurobiology of PD; this disorder is now recognized as a model of dysfunction in fronto-striatal circuits expressed by motor and behavioral symptoms. Several clinical and surgical strategies for the management of PD are discussed.

[Dystonia: concepts, classification, and physiopathology]. / Distonias: conceitos, classificação e fisiopatologia.

Since the last decades of the past century there have been several reports in the medical literature describing patients with dystonia. The clinical and phenomenological concepts of dystonic movements have been debated. Presently, dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures. Dystonia is usually classified according to three criteria: distribution, age of onset and etiology. The generalized forms often have their onset at childhood whereas the focal forms are most commonly seen in adults. Dystonic movements and postures can occur during resting or they can be precipitated by voluntary movements or by the adoption of specific posture patterns. The pathophysiologic mechanisms related to the appearance of dystonia are presently unknown. However, there are compelling evidences suggesting the involvement of some basal ganglia nuclei (e.g. putamen and globus palidus) in the development of dystonia.

[Dystonia: therapeutic aspects]. / Distonias: aspectos terapêuticos.

Several approaches have been employed for the treatment of dystonias. Possible specific causes should be searched for and specific treatment should be instituted. Different types of symptomatic treatment are grouped according to the following categories: pharmacological systemic therapy, surgical therapy and botulinum toxin injections in the affected muscles. Each of these approaches has its advantages and limitations. Generalized dystonias should be treated with anticholinergic agents. In some cases, levodopa or other drugs such as dopamine antagonists, baclofen and benzodiazepines should be preferred. Focal dystonias respond dramatically to local injections of botulinum toxin. Over 90% of patients with blepharospasm and 70% of patients with cervical dystonia present a satisfactory response to this procedure. Surgical approaches have been utilized in some cases of generalized (stereotaxic lesions), axial (rhizotomies) and focal dystonias (myectomies and neurectomies) with variable results.

[Juvenile hereditary chorea. Study of a family with recessive pattern of transmission]. / Coréia hereditária juvenil. Estudo de uma família com padrão recessivo de transmissão.

Report of a family in which the parents are consanguineous and healthy and 4 of their 8 children began with involuntary choreic movements at ages 10 to 14. In all cases the clinical manifestations remained stable throughout the 5-year period of observation. There have been no clinical deterioration and intellectual functions were found to be intact. All patients were submitted to neurologic examination, neuropsychological testing, CT-scan, cerebrospinal fluid analysis, electroencephalogram, serum copper and ceruloplasmin among other blood tests. These cases were diagnosed as having "juvenile hereditary chorea" from the typical clinical manifestations and after exclusion of other known causes of chorea. Relevant clinical aspects and possible differential diagnosis are discussed along with some advancing hypothesis concerning its relationship with other hereditary diseases presenting with chorea.

[Hereditary essential myoclonus. Report of a family]. / Mioclonia essencial hereditária. Relato de uma família.

The occurrence of myoclonic and dystonic movements as an isolated expression of a neurologic condition of hereditary pattern have been scarcely described in literature. For this entity some authors proposed the denomination "hereditary myoclonic dystonia" while others prefer the use of the expression "hereditary essential myoclonus". We present a family in which this unusual association of abnormal movements affected several members in three generations. The propositus patient is a 14-year-old girl who have noticed the dystonic movements by 7 years of age and the myoclonic ones by 13 years of age, with a slow progression. There was no reference about the effect of alcohol (abstemious patient). There was a family history of similar cases. The supplementary investigation (seric dosage of cupper, ceruloplasmine, T3, T4, TSH; acanthocytes search; CSF examination; CT scan and MRI of the head) did not show any abnormality. Clonazepam was the only medication that lead to a clinical improvement, reducing both movements.

[Kinesigenic paroxysmal choreoathetosis. Report of a case treated with carbamazepine]. / Coreoatetose paroxística cinesiogênica. Relato de um caso tratado com carbamazepina.

Paroxysmal choreoathetosis (PC) is a rare entity, and professionals who study movement disorders do not find it easily. Paroxysmal occurrence of dystonic, choreic, athetosic movements is the typical feature of this syndrome. The onset is always subtle and the attacks can last from few seconds to hours. Sporadic cases and more common familial cases have been reported. The therapeutic approach with anticonvulsant drugs like carbamazepine is not always successful. However, there is a good response to this drug in the kinesigenic form of PC. The case of a 21 years old male patient is reported here. The onset occurred during puberty, at 14 years old. Neurologic examination was normal between attacks. Subtle and brief choreic, athetosic and dystonic limb and legs movements precipitated by sudden quick and unexpected passive movements, as by startle, were observed during the attacks. There was no familial history. Routine laboratorial investigation, EEG, CT scan and MRI did not show significant changes. Carbamazepine in low dosages (100 mg/day) brought a complete control of the attacks.

Coreoatetose paroxística cinesiogênica: relato de um caso tratado com carbamazepina / Kinesigenic paroxysmal choreoathetosis: report of a case treated with carbamazepina

A coreatetose paroxística (CP) é entidade rara. Até mesmo profissionais que estudam desordens do movimento näo costumam vê-la com freqüência. A ocorrência paroxística de moviemtnos distônicos, coréicos e atetósicos é a apresentaçäo típica da síndrome. O início costuma ser abrupto e os ataques podem durar de alguns segundos até horas. Casos esporádicos e, mais frequentemente,casos familiares têm sido relatados. A abordagem terapêutica com anticonvulsivantes, como a carbamazepina, nem sempre tem sucesso. Com esta droga, porém, geralmente há boa resposta na variante cinesiogênica da CP. É relatado o caso de um paciente jovem do sexo masculino com essa variante da CP. O início da doença se deu na puberdade. O exame neurológico era normal entre os ataques. A investigaçäo laboratorial, EEG, TC de crânio e RNM de segmento cefálico foram normais. Carbamazepina em doses baixas (100 mg/dia) foi eficaz no manejo dos ataques

[Neurocryptococosis and immunosuppression: experimental model in mice]. / Neurocriptococose e imunossupressão: modelo experimental em camundongos.

In order to accomplish a model for immunosuppression in experimental conditions two lots of 50 young mice were divided into groups of 10, and submitted to dexamethasone ingestion. Two experiments were considered in the study. In the first experiment conditions for immunosuppression were established in 50 mice, by per os supply of several concentrations of dexamethasone in the water given for normal daily intake ad libitum. Criteria of immunosuppression considered include: hair standing on end; leukopenia by decrease of lymphocyte population; spleen atrophy in relation to controls, with severe hypoplasy of all lymphoid structures. In the second experiment another lot of 50 mice in similar conditions established for the first experiment and concerning the immunosuppression were submitted to intraperitoneal inoculation of near 10(6) Cryptococcus neoformans. Culture suspensions of cryptococci isolated from cerebrospinal fluid samples of human neurocryptococcosis cases were used for this purpose. Although fungi could be recognized in lungs, liver and spleen from every mice inoculated, only those with central nervous system involvement (near by an half of all) died. The discussion included considerations about the role of the blood-brain barrier function in the findings registered.

[Demyelinating diseases and the cerebrospinal fluid cytoproteinogram]. / Afecções desmielinizantes e citoproteinograma do líquido cefalorraqueano.

Neuromyelitis of cyanocobalamin deficiency (group 1) post vaccination myelopathy (group 2), multiple sclerosis and neuromyelitis optica (group 3) are studied comparatively as to the respective cerebrospinal fluid changes on cytology, proteins and gamma globulins. Changes were found to be more intense and frequent in the last two groups. Local immunecompetent phenomena are considered in the interpretation of findings and related to modifications that may be induced in myelin macromolecules by factors of diverse order which may be involved in demyelination in each one of the three groups.

[Myelopathies: behavior of cerebrospinal fluid cells and proteins]. / Mielopatias: comportamento das células e proteínas do líquido cefalorraqueano.

Data on cerebrospinal fluid (CSF) cells and proteins (total proteins and gamma globulins content) are reviewed in 287 patients with myelopathies. Clinical data on these patients were reported. Results obtained are distributed according to diagnoses and to time of disease (tables 1 and 2). Informations obtained through the study show that signalization of CSF system is related to the episode responsible by the disease, when the episode is singular. Repeated episodes or a progressive evolution are more apt to produce a maintained signalization of CSF cyto-protein dual. In this way data on infectious myelopathies, multiple sclerosis and neuromyelitis optica are compared to those found in post-vaccination, post-infection and post-intoxication myelopathies, as well as to those found in B-12 deficiency and myelopathies of vascular origin. Primary myelitis are evaluated in report to this comparison. It is shown that changes in the CSF cyto-protein dual are more related to those found in multiple sclerosis and neuromyelitis optica when chronic primary myelitis is considered. In acute and sub-acute primary myelitis they are related to those found in post-vaccination, post-infection and post-intoxication myelopathies.

[Primary acute and subacute meningomyeloradiculopathies: study of the cerebrospinal fluid]. / Meningomielorradiculopatias agudas e sub-agudas primárias: estudo do líquido cefalorraqueano.

Forty four patients with primary acute and subacute meningomyeloradiculopathies were studied in respect to the evolutive aspects of the cerebrospinal fluid (CSF) cell-protein dual in several periods of the disease. The tendency of CSF hypercytosis to normal values of cell count occurred in most cases (96%) at the end of the period in which the study was performed (60 days). The CSF protein levels had a similar but slower behavior pattern when compared with hypercytosis. The comparative analysis of clinical improvement of two groups of patients (treated and not treated with ACTH or corticosteroids) showed a statistically significant difference between on the two groups favouring the former. This fact enhances the possibility that an auto-immune process plays an important role in cases of primary meningomyeloradiculopathies.

Mielopatias: comportamento das celulas e proteinas do liquido cefalorraqueano. / Myelopathies: behavior of cells and proteins of the cerebrospinal

Analise do comportamento do dual citroproteico do liquido cefalorraqueano em 287 pacientes com mielopatias relacionadas a processos etiologicos diversos. Os dados sao distribuidos em funcao do diagnostico e do tempo de doenca a ocasiao do exame. Considerando os mecanismos fisiopatologicos envolvidos em cada grupo e no decurso da doenca, sao feitas avaliacoes sobre os aspectos imunopatologicos envolvidos

Meningomielorradiculopatias agudas e sub-agudas primarias: Estudo do liquido cefalorraqueano. / Acute and subacute primary meningomyeloradiculopathies: a study of the cerebrospinal fluid

Foram estudados 44 casos de meningomielorradiculopatias primarias agudas e sub-agudas com relacao ao perfil evolutivo da citologia e da concentracao proteica do liquido cefalorraqueano nas diversas fases da doenca. A evolucao da pleocitose para niveis normais ou proximos da normalidade ocorreu na maioria dos casos (96%) ao termino do periodo de estudo (60 dias). A hiperproteinorraquia evoluiu com padrao semelhante, embora de modo mais lento. A analise comparativa desses dados a evolucao clinica dos pacientes, inclusive dos efeitos beneficos da terapeutica por ACTH ou corticosteroides em um grupo deles, sugere a importamcia de fatores autoimunes na deflagracao da doenca

Afeccoes desmielinizantes e citoproteinograma do liquido cefalorraqueano. / Demyelinating diseases and cerebrospinal fluid cytoproteinogram

Estudo do comportamento da citologia, das proteinas totais e das globulinas gama em pacientes com esclerose multipla e neuromielite optica, mielopatia pos-vacinacao e neuromielopatia da deficiencia de cianocobalamina. A expressividade das alteracoes e maior nos dois primeiros grupos que no terceiro, tanto em frequencia, como em intensidade. Os resultados sao interpretados quanto aos conhecimentos sobre a desmielinizacao e a presenca de macromoleculas, modificadas ou nao, no LCR na vigencia desses tipos de processos e a respectiva habilidade em deflagrar resposta imunocompetente