[Dialysis dementia. Electroencephalographic study of a case]. / Diálise-demência. Estude eletrencefalográfico de um caso.

A case of a 65 years-old black man with chronic renal failure, treated with chronic haemodialysis, in which a dialysis dementia syndrome developed is reported. In the initial phase the electroencephalogram had the typical burst of sharp and slow waves, waning with haloperidol and diazepam treatment. The withdrawal of this medication induced reappearance of the symptoms and EEG. abnormalities. As soon as the patient came back to the diazepine (potassium clorazepate), the mental symptoms subsided, but few weeks later he died of a cerebral haemorrhage. The electroencephalographic features are described and a brief comment about this disease is made.

[Rett syndrome: report of 7 cases]. / Síndrome de Rétt: relato de 7 casos.

The authors report the clinical and laboratory findings of seven girls with the diagnosis of Rett syndrome seen at the clinic from August 1978 to October 1988. The patient's age varied from two to ten years and they were followed up for 5 years and 9 months in average. The development milestone started to deteriorate between five and twenty-four months. Acquired microcephaly was present in six patients and three cases had hyperventilation. Generalized tonic-clonic seizures were the most frequent type of seizures (6 patients) beginning between one and seven years and effectively controlled with carbamazepine. The EEG's abnormal findings were spike discharges (5 patients) and paroxysmal high-amplitude theta activity (4 patients). The remaining laboratory tests were unremarkable. The pathogenesis of Rett syndrome has not been determined. The clinical evaluation is intended to contribute to the knowledge of that rather uncommon disease.

[Irritative midline and parasaggital foci: a clinical and electroencephalographic correlation]. / Focos irritativos parassagitais e de linha média: correlação clínico-eletrencefalográfica.

We report the clinical and electroencephalographic characteristics of thirteen patients with midline spikes (MS), parasaggital foci (PF), or both. Numerous clinical manifestations, including generalized tonic-clonic, complex partial and partial with secondary generalization could be demonstrated. The pattern of the epileptiform manifestation is unpredictable; however, we find generalized tonic-clonic seizure the commonest type (60%). Unfortunately, this data has no statistic value. Neurologic examination was normal in the majority of the patients. Regarding age, there is a marked predominance of children (84%) with MS and/or PF. Sleep constitutes the main activation method (73%) in the search for this kind of epileptiform activity. In conclusion, although the incidence of the MS/PF is quite low (0.4%), the high epileptogenic potential (70%) of them justifies a careful and adequate evaluation of these regions. Special attention must be paid to the normal sleep complexes, mainly in children, which sometimes can mimic true epileptiform activity.

[West syndrome: clinical and electroencephalographic follow up of 70 patients and response to its treatment with adrenocorticotropic hormone, prednisone, vigabatrin, nitrazepam and valproate]. / Síndrome de West: evolução clínica e eletrencefalográfica de 70 pacientes e resposta ao tratamento com hormônio adrenocorticotrófico, prednisona, vigabatrina, nitrazepam e ácido valpróico.

In a retrospective study we assessed the outcome of the criptogenic and symptomatic forms of West syndrome and evaluated the efficacy of adrenocorticotropic hormone, vigabatrin, prednisone, valproate and nitrazepam in the spasms control. Seventy patients were follwed up by 2 years. Twelve (17%) were criptogenics and 58 (83%) symptomatics. In criptogenic group significantly more patients were in regular school classes and with normal motor development, better control of seizure, less tendency to evoluate to Lennox Gastaut syndrome and 83. 3% had control of spasms (72.4% of patients from symptomatic group had control of spasms). Adrenocorticotropic hormone and vigabatrin were the most efective drugs, with 68.75% and 60% of spasms control, respectivelly, when used as first line of therapy and 75% and 50%, respectivelly, as second line of therapy.

Mitochondrial myopathy and myoclonic epilepsy.

The authors describe a family (mother, son and two daughters) with mitochondrial myopathy. The mother was asymptomatic. Two daughters had lactic acidosis and myoclonic epilepsy, mild dementia, ataxia, weakness and sensory neuropathy. The son suffered one acute hemiplegic episode due to an ischemic infarct in the right temporal region. All the patients studied had hypertension. EEG disclosed photomyoclonic response in the proband patient. Muscle biopsy disclosed ragged-red fibers and abnormal mitochondria by electron microscopy. Biochemical analysis showed a defect of cytochrome C oxidase in mitochondria isolated from skeletal muscle. Several clinical and genetic aspects of the mitochondrial encephalomyopathies are discussed.

Fulminant tropical hepatitis--experimental reproduction.

After a review of the literature on epidemiological, serological and histopathological data concerning so-called "Amazon Black Fever" (Labrea disease), an experimental model of morphological reproduction of the disease is presented. The model is based on the probable causal synergism of Labrea hepatitis, i.e., viral injury plus protein-caloric malnutrition. Mice infected with the Oriboca arbovirus presented classic yellow-fever-like lesions of the liver, with a few malnourished animals showing the characteristic morular changes of the human disease.

Studies on arboviruses--infection of undernourished mice by cocal virus.

Normal and undernourished mice inoculated orally and intraperitoneally with cocal virus show panencephalitis and acute poliomyelitis, which is more accentuated in cases of malnutrition. Speculations are made regarding viral penetration and progression in the nervous system.

Spontaneous amyloidosis in mice with malignant neoplasms.

An outbred strain of Swiss mice carrying malignant neoplasms (48 female with mammary carcinoma, 1 male with osteosarcoma) is described with a high frequency of amyloid deposits selectively located in the ileum. Amyloid deposits were demonstrated by Congo red staining and electron microscopy. Comments are directed toward methods of amyloid detection, as well as pathogenic considerations.

Secondary palatal development in the New Zealand white rabbit: a scanning electron microscopic study.

Examination of surface topography in prefusion stages of secondary palatal development in rabbit embryos reveals a sequence of alterations in the surface cells of the epithelium along the medial margins of the palatal processes. A progressive increase in cellular protrusions resembling lamellipodia and filopodia, as well as cellular necrosis, is observed in those areas that undergo fusion. The changes precede fusion and are restricted to fusion sites. Prior to and at the time of epithelial contact between palatal processes, many long slender cellular protrusions are seen bridging the gap between the approximating tissues. The localization of the epithelial alterations and the appearance of similar cellular morphology in other embryonic epithelial fusion events strongly suggest: either an active role of the epithelial cells in the fusion of the secondary palate, or some common fundamental biochemical events that may facilitate or are responsible for the initial adhesion of such tissues.