[A single deletion of mitochondrial DNA in a Brazilian patient with chronic progressive external ophthalmoplegia].

Introduction: The syndrome of chronic progressive external ophthalmoplegia (CPEO) has been associated to the presence of large deletion, single or multiple, in the mitochondrial DNA of skeletal muscle.

Case Report: We report a sporadic case of chronic progressive external ophthalmoplegia that began at age 19 years and was associated with ragged red fibers in skeletal muscle. Genetic analysis of mitochondrial DNA revealed the presence of a single deletion of 4237 bp that encompasses the nucleotide positions 9486 to 13722, a location that has not been described before, and flanked by a direct repeat sequence.

[Acute myelopathy in a diver caused by decompression sickness. A case description and a survey of the literature].

Introduction: Decompression sickness (DS) is caused when bubbles of an inert gas usually nitrogen, since oxygen is metabolised in the tissues are released into the bloodstream and tissues during fast ascents once the atmospheric pressure is lowered near the surface. Neurological complications are its most serious form of expression and include vertigo, headache, stroke and acute myelopathy, among others. DS that affects the spinal cord is infrequent.

[Treatment of sialorrhoea in neurological diseases with trans-dermic injections of botulinum toxin type A in the parotid glands].

Introduction: Sialorrhoea is a disabling problem in many patients affected by neurological disorders. Anticholinergic drugs often produce side effects in these patients. Botulinum toxin A (TBA) blocks liberation of acetil-choline in motor and autonomic nerve terminals.

[Neurological syndromes associated with the ingestion of plants and fungi with a toxic component (II). Hallucinogenic fungi and plants, mycotoxins and medicinal herbs].

Introduction: A wide range of fungi and medicinal herbs, rich in hallucinogenic substances and widely used for mystic and medicinal purposes, can give rise to neurotoxic symptoms.

Development: We review the toxic syndromes that can arise from the ingestion of hallucinogenic fungi, cacti and plants, together with descriptions of cases of acute poisoning resulting from the use of medicinal herbs and from foodstuffs that are contaminated by mycotoxins. A series of different psychedelic fungi belonging to the Psilocybe, Panaeolus and Stropharia genera contain hallucinogenic alkaloids such as psilocybin.

[Neurological syndromes linked with the intake of plants and fungi containing a toxic component (I). Neurotoxic syndromes caused by the ingestion of plants, seeds and fruits].

Introduction: A wide range of plants, seeds and fruits used for nutritional and medicinal purposes can give rise to neurotoxic symptoms.

Development: We review the neurological pathology associated with the acute or chronic consumption of plants, seeds and fruits in human beings and in animals. Of the plants that can trigger acute neurotoxic syndromes in humans, some of the most notable include Mandragora officinalis, Datura stramonium, Conium maculatum (hemlock), Coriaria myrtifolia (redoul), Ricinus communis, Gloriosa superba, Catharanthus roseus, Karwinskia humboldtiana and Podophyllum pelatum.

["Top-of-the-basilar" syndrome and Chagas' disease].

Introduction: As a chronic chagasic myocardiopathy, Chagas disease (CD) may give rise to cardiac insufficiency, arrhythmias, thromboembolism and stroke. Occlusive vascular disease of the rostral portion of the basilar artery or of its emergent branches may cause ischemia and necrosis in different areas of the midbrain, thalamic nuclei, cerebellum and occipital lobe.

Case Reports: We describe four patients (three males and a female, the average age being 54 years) with positive chagasic serology (indirect immunofluorescence and hemagglutination) and suffering from CD and top of the basilar syndrome.

[Schwartz-Jampel syndrome: a description of two adult siblings].

Introduction: Schwartz Jampel syndrome is a usually recessive disorder that presents myotonia, skeletal (bone dysplasia) and facial (blepharophimosis) anomalies and low height, which is diagnosed in childhood. We report on the clinical, electromyographical and radiological findings of two siblings affected by the disease and who were diagnosed late, in adulthood.

Case Reports: Two siblings, a male aged 39 and a female of 49, the only members of a family of five siblings born to consanguineous parents to be affected, were studied because of deformities in the joints that appeared at the age of nine months and alterations in their gait.

[Nitrous oxide action on the central nervous system: electrophysiological study as a sole agent or a coadjuvant].

Background And Objectives: Nitrous oxide is the most widely used inhalational anesthetic worldwide. Its action mechanism is broadly discussed based on results of experimental studies and clinical evidences. The purpose of this study was to evaluate, through specific monitoring, nitrous oxide electrophysiological action on the central nervous system.

Massive gas embolism during pulmonary nodule hook wire localization.

The locaization of pulmonary nodules by the hook wire technique has allowed accurate resection of small and nonsuperficial pulmonary nodules by video-assisted thoracoscopic surgery. Hook wire localization has been shown efficacious and safe with principal complications limited to pneumothorax, wire dislocation, and localized intrapulmonary hemorrhage. We report a case of massive gas embolism complicating this procedure.

[Superficial siderosis of the central nervous system: an uncommon cause of spastic paraparesia].

Introduction: Superficial siderosis of the central nervous system (CNS) is an uncommon neurological condition, characterized clinically by cerebellar ataxia, neurosensorial deafness, anosmia, myelopathy and cognitive deterioration. It is associated with the deposition of haemosiderin in the subpial layers of the brain, cerebellum and spinal cord, following chronic bleeding (often clinically silent) in the subarachnoid space. Histopathologically there is gliosis, neurone loss and demyelination of the CNS.

[Stroke of cardioembolic origin in Chagas disease].

Introduction: American trypanosomiasis, Chagas disease is caused by Trypanosoma cruzi. Between 10% and 30% of infected persons develop the chronic form, with predominance of the cardiac and gastrointestinal forms. Chagas myocardiopathy leads to congestive heart failure, dysrhythmias and thromboembolic phenomena, and may cause strokes.

Forced oscillation technique to evaluate tracheostenosis in patients with neurologic injury.

Study Objectives: To determine the utility of forced oscillation technique (FOT) for measuring pulmonary resistance and reactance in patients with central nervous system injuries, for detection and follow-up of posttracheostomy tracheal stenosis.

Design: Case series.

Setting: A rehabilitation hospital, Brasilia, Distrito Federal, Brazil.

[Neurological anthropology among the Kamayura Indians of the Alto Xingu].

Introduction: The Kamayura tribe is made up of 300 persons living in the Alto Xingu in Mato Grosso, Brazil. Their traditional system of health care is based on the pajé, a witch doctor who uses plants and prayers for treatment.

Material And Methods: Field work was done in the Kamayura village holding successive interviews with the chief and the pajé Takumá to obtain information regarding the neurological disorders found there, the indigenous beliefs regarding illness (natural or based on witch-craft), their classification and traditional treatment based on plants.

The benefit of pulmonary metastectomy for bone and soft tissue sarcomas.

The outcome of patients having metastectomy for pulmonary metastases from locally controlled primary malignancy of bone and soft tissue of the limbs is compared to a similar group deprived of such therapy. Forty patients receiving pulmonary metastectomy and 18 patients who were candidates for, but did not receive, metastectomy were compared by age, sex, histology, number of metastases, synchronous and metachronous presentation of metastases and the use of chemotherapy. The only variable correlated with survival was metastectomy.

[Devic's neuromyelitis optica. Presentation of 2 new cases and review of the bibliography].

Devic's neuromyelitis optica is a clinical entity characterized by severe transverse myelitis, acute unilateral or bilateral optic neuropathy, no clinical involvement beyond the spinal cord or optic nerves and a monophasic or recurrent evolution. We report two cases, both female, affected by spinal cord and visual symptoms suggesting Devic's neuromyelitis. First patient, a 30 y.

[Type I neurofibromatosis presenting as a progressive cervical myelopathy. The first case reported in Kaxinawa Indians].

Introduction: Type I neurofibromatosis is an autosomal dominant disorder with an estimated prevalence of 1/3,000. Half of the new cases are due to mutations; their penetration is complete in adults and may affect any ethnic group. We describe the first reported case of type I neurofibromatosis in a Kaxinawa Indian from the state of Acre, Brazil, in whom the presenting symptoms were of progressive tetraparesis due to multiple paraspinal neurofibromata.

[Potentially treatable subacute forms of infection due to the HTLV-1].

Introduction: Tropical spastic paraparesis due to HTLV-I virus is diagnosed at very advanced stages, when there is spinal atrophy present and so only symptomatic treatment can be given. Early diagnosis of HTLV-I infection in unusual syndromes and the use of corticosteroids may help to slow the development of the disease.

Clinical Cases: We describe two Brazilian patients who developed symptoms due to HTLV-I present for less than one year: subacute myelopathy with a sensory level and an ataxic pyramidal syndrome associated with axonal neuropathy, which partly improved after treatment with corticosteroids.

[Spastic paraparesis due to long term consumption of wild cassava (Manihot esculenta): a neurotoxic model of motor neuron disease].

Introduction: Cassava (Manihot esculenta) is the basic foodstuff of more than 500 million persons in developing countries. Its edible root contains a glucoside with a high cyanogenic content, linamarina, which is hydrolysed in the human intestinal tract by the resident microbial flora, with liberation of HCN. Inadequate preparation and cooking followed by consumption whilst half-raw, especially in diets based almost exclusively on cassava for a long period of time, may lead to a neurological syndrome of damage to the upper motor neuron and the appearance of spastic paraparesia.

[Thyrotoxic periodic paralysis. A report of 2 cases].

Introduction: Thyrotoxic hypokalemic periodic paralysis is characterized by recurrent episodes of motor weakness of variable intensity associated with thyroid overactivity. It is usually associated with low plasma potassium levels and is often precipitated by physical activity or ingestion of carbohydrates.

Clinical Cases: We describe two men, aged 33 and 50, who complained of several episodes of muscular paralysis in the context of previously undiagnozed hyperthyroidism and associated with low plasma potassium levels.

[Measurement of the quality of life in stroke survivors].

Objective: The aim of this article is to describe methods of evaluation of the quality of life in relation to health after a stroke, methodological considerations and criteria for the use of scales.

Development: The terms affected person, disability and handicap are defined. We discuss the indices of functional evaluation used in the study of strokes: activities of daily life (ADL), instrumental activities and the generic health questionnaires used to study quality of life.