James Pitágoras de Mattos

Spinal cord compression due to extramedullary hematopoiesis in the proliferative phase of polycythemia vera

Extramedullary hematopoiesis is a common accompaniment of a variety of hematologic diseases such as hereditary spherocytosis, thalassemia and myelofibrosis. The association of extramedullary hematopoiesis with polycythemia vera in the proliferative phase is much less usual. We report a patient who presented with paraplegia due to spinal cord compression; clinical investigation revealed a paravertebral hematopoietic tumor, and the diagnosis of polycythemia vera was then established.

Involuntary movements and AIDS: report of seven cases and review of the literature

We studied 1086 AIDS patients in the last six years. Of these 389 (35.82%) had neurological manifestation and 7 (1.8%) male patients had abnormal involuntary movements (parkinsonism in 3, hemichorea-hemiballism in 2, spinal myoclonus in 1 and rubral tremor in another). All patients were men, 5 white and 2 black. Four were homosexual, 2 drug-users and 1 bisexual. The mean age was 33.14 years. The time between AIDS diagnosis and the onset of movement disorders was 23.8 months in 5 patients and in 2 it was the first symptom. The parkinsonian patients did not show any opportunistic infection in connection with the neurological symptoms but in the remaining four cases this relationship was suggested. The data showed that not only the opportunistic infection but also the AIDS virus may play an important role on the development of involuntary movements.

Paroxysmal dystonia and neuromyelitis optica

Paroxysmal dyskinesias (PD) are thought to be rare movement disorders. The overwhelming majority of reported cases are primary. Secondary PD has seen reported to occur in some conditions, mainly in multiple sclerosis and head trauma. The anatomic origin of the lesion is also rarely seen at the spinal cord. Our objective was to describe four patients with paroxysmal dystonia secondary to spinal lesions during the recovering phase of a neuromyelitis optica (NMO) bout. In the reviewed literature, we do not find any report of PD related to NMO.

Parkinsonism and AIDS: a clinical comparative study before and after HAART.

In 2002, after analyzing 28 HIV-positive patients with movement disorders we emphasized the decreasing not only of Parkinsonism but also of other involuntary movements in HIV patients in the last few years. The objective of this study is to compare the clinical results between HIV-positive patients with Parkinsonism before and after HAART. In 14 years (1986-1999) 2,460 HIV-positive patients were seen in our Hospital 14 (0.6%) of which presented with Parkinsonism. Eight years after (2000-2007) 970 HIV positive patients were seen and only two (0.2%) had Parkinsonism. We conclude that after the introduction of HAART there was an evident decrease in AIDS-related Parkinsonism.

Diagnóstico diferencial dos tremores

After the review of the literature the author presents the semiologic basis for the differential diagnosis of rest and action tremors. The parkinsonian, severe essential, rubral, tardive, postural, kinetic and the task or position-specific tremors are mainly analysed.

Toxina botulínica no blefaroespasmo, no espasmo hemifacial e na distonia cervical: resultados em 33 pacientes

The effects of botulinum toxin type A were studied in 33 patients with dystonia (12 blepharospasms, 10 hemifacial spasms and 11 spasmodic torticollis). A rate scale was used to evaluate the severity of the dystonic movements, before and two weeks after each injection. Among blepharospasm patients, eight were female and four were male; the mean age was 57.7 years; the mean time of the disease duration was four years. Three had familial history for similar disease; nine were essential and three had used neuroleptic drugs (tardive dystonia). The mean dose used was 51.3 U, with a mean time of beneficial effects of 2.8 months. For 22 injections and reinjections, 14 (63.7%) showed an excellent result, five (22.7%) good and three (13.6%) null. In the hemifacial spasm group, eight were female and two male; the mean age was 52.6 years; the mean time of the disease duration was 7.4 years; eight were essential and two post-paralytic. The mean dose used was 32 U. From the total of 15 injections and reinjections, all of them (100%) had an excellent result, with a mean time of beneficial effect of 3.4 months. Among the cervical dystonic patients, eight were male and three female; the mean age was 44.2 years; the mean time of the disease duration was 12.2 years; six had essential dystonia, three had used neuroleptic drugs and two had familial history for similar disease. The mean dose used was 238.6 U, with the mean duration of effect of 3.5 months. From the total of 20 injections and reinjections, 18 (90%) had good result, one (5%) mild and one (5%) null.(ABSTRACT TRUNCATED AT 250 WORDS)

Mielopatia transversa e lupus eritematoso sistemico: registro de um caso e revisão da literatura

: A case of transverse myelopathy in systemic lupus erythematosus with subacute onset and fatal course is reported. A review of the literature is done and clinical, laboratorial, therapeutics and pathological aspects are discussed. The authors call attention to the poor results of therapy and discuss the participation of immunological factors leaving to demyelination which are, aside the classical vascular involvement, the probable mechanisms in the neurological manifestations of systemic lupus erythematosus. The authors believe that, in their case, such a demyelinative mechanism was responsible for the clinical picture, although a necroscopic examination was not allowed.A case of transverse myelopathy in systemic lupus erythematosus with subacute onset and fatal course is reported. A review of the literature is done and clinical, laboratorial, therapeutics and pathological aspects are discussed. The authors call attention to the poor results of therapy and discuss the participation of immunological factors leaving to demyelination which are, aside the classical vascular involvment, the probable mechanisms in the neurological manifestations of systemic lupus erythematosus. The authors believe that, in their case, such a demyelinative mechanism was responsable for the clinical picture, although a necroscopic examination was not allowed.

Síndrome de Klippel-Trenaunay-Parkes-Weber com angiomatose medular

A case of Klippel-Trenaunay-Parkes-Weber syndrome associated with spinal cord angioma is reported. The author points out that it was found only one reference in literature with such association.

Novos agonistas dopaminérgicos

Apresentamos breve revisao da literatura sobre os agonistas dopaminergicos. Referimos os cinco receptores conhecidos e onde estao localizados, as vantagens e as desvantagens de sua utilizacao nos pacientes com a doenca de Parkinson.Introduzidos com o objetivo principal de controlar as limitacoes da levodopa, aumentando a janela terapeutica, analisamos a farmacocinetica, a eficacia e os efeitos colaterais da cabergolina, do ropinirole e do pramipexole.

Doença dos tiques: aspectos genéticos e neuroquímicos atuais

We review historical, conceptual, clinical and diagnostic criteria as well as present genetic and neurochemical factors of tic disorders. Epidemiologic data suggest that tic is an organic disease with autosomal dominant transmission. We emphasize immunohistochemical studies particularly related to the dopaminergic, noradrenergic and serotonergic systems. These modulate the activity of the cortico-striato-thalamocortical circuits implicated in both Tourettes syndrome and obsessive-compulsive disorder.We review historical, conceptual, clinical and diagnostic criteria as well as present genetic and neurochemical factors of tic disorders. Epidemiologic data sugest that tic is an organic disease with autosomal dominant transmission. We emphasize imunohistochemical studies particularly related to the dopaminergic, noradrenergic and serotonergic systems. These modulate the activity of the cortico-striato-thalamocortical circuits implicated in both Tourettes syndrome and obsessive-compulsive disorder.