Haemolytic uraemic syndrome associated with cryptosporidium oocysts.
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Biomedical subjects
Publications and source records attributed to M Berthier.
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A right-handed male patient suddenly noted lower bilateral facial and lingual palsy, and inability to masticate and swallow, but with preserved automatic functions. He was mute, communicating only through writing, but verbal comprehension was normal (aphemia). On anatomopathological examination, an ischemic infarction of the entire right insula, with mild extension to the fronto-temporoparietal operculum was observed. The left hemisphere was normal. The clinical findings suggest a bilateral opercular syndrome due to a right hemisphere lesion and a crossed aphemia.
Twenty-five cases (38%) of ischemic infarction occurred among 65 cases of tuberculous meningitis in patients less than 14 years of age. The male:female ratio was 1.3:1. The most frequent clinical findings were meningeal signs, fever, alteration of consciousness, cranial nerve involvement, seizures, and focal neurologic deficit. Twenty-three patients had anterior circulation infarcts, and two more had infarcts in the vertebrobasilar territories. Distribution of infarcts in the anterior circulation was shown by computed tomography in the territories of the following arteries: lenticulostriate, 10 cases unilateral and 6 bilateral; middle cerebral, 3 cases; internal carotid, 1 case; multiple areas, 3 cases. Of the 25 ischemic infarction cases, 23 (92%) had hydrocephalus, 19 (76%) basal exudates, and 2 (8%) tuberculomas. Outcome was poor since no patient with infarction recovered completely. Six died and bilateral subcortical infarcts led to a considerably higher mortality than unilateral ones, whether cortical or subcortical.
Serum IgG subclass levels were measured by an immunoenzymatic assay with monoclonal antibodies in 225 normal children aged 1 to 17 years. Adult concentrations of IgG1 and IgG3 were reached early (2 to 3 years of age), with mean IgG1 level slightly higher in children than in adults, whereas IgG2 and IgG4 showed a slow increase. Mean IgG2 levels in children aged 13 to 17 were still significantly lower than in adults. The distribution of IgG4 levels in every age group was very heterogeneous, with a fair incidence of subthreshold concentrations especially before 5 years of age. These data must be taken into account when defining IgG subclass deficiencies in children.
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The recent discovery of self-resolving Cryptosporidium infection in immunocompetent patients has aroused growing interest in this parasite, which has thus far been known to affect mainly immunodeficient individuals. Following the hospitalization of two children attending the same day-care center in February 1986 Cryptosporidium oocysts were found in the stools of one of them. This led to an epidemiological investigation to assess the frequency of Cryptosporidium oocysts in the stools of children attending the 7 day-care centers in the city of Poitiers, France. Testing for Cryptosporidium oocysts was performed on formalinized stools, after smear staining by the modified Ziehl-Neelsen procedure. Each positive stool was also submitted to virological and bacteriological examination. Stool specimens from 235 children were studied: 9 (3.8%) of them had Cryptosporidium oocysts in their stools. Four of the children had diarrhea, and 5 (2.2%) were asymptomatic. The existence of healthy carriers should lead to caution in the interpretation of stool parasitology.
We report the case of an 18-month-old boy with many typical Down syndrome features but a normal cytogenetic analysis. High-resolution banding techniques on lymphocytes and fibroblasts of the propositus and his parents did not show any detectable abnormality including that of trisomy 21 mosaicism. However, CuZn superoxide dismutase (CuZn SOD) in the patient's red cells was increased as in trisomy 21. DNA analysis (Southern blots) using a human CuZn SOD probe showed that the genotype of the propositus contained three CuZn SOD genes. In situ hybridization on metaphase chromosomes with the same probe confirmed the gene location in a segment enclosing the distal part of 21q21 and 21q22.1. There was no significant labeling on other chromosomes of the patient. These results indicate that the Down syndrome phenotype of this patient is due to microduplication of a chromosome 21 fragment containing the CuZn SOD gene.
Four cases of intraventricular tuberculoma (IVT) in children are here reported. In none of the patients was there clinical evidence pointing to the intraventricular location. CT scan findings comprised three stages of development, namely: immature, mature and old. Ependymal attachment and asymmetric hydrocephalus were present in three cases, meningitis in two and ependymitis in one. Septum pellucidum traction was clearly observed in two patients, strongly supporting an adhesive process characteristic of intraventricular tuberculosis. Following specific treatment, the tuberculomas remitted partially or entirely.
A severe multimodal neglect syndrome, mutism, oral apraxia and ideomotor apraxia for the right hand suddenly developed in a right-handed male following a right hemisphere (central) stroke. Neuropathologic examination showed an ischemic infarction involving the whole right insula, adjacent white matter, and the inner cortical surface of the right fronto-temporo-parietal operculum. The left hemisphere was spared. It is suggested that damage to the right insula (a polymodal convergence area), and the adjacent white matter may lead to severe neglect. Our case also demonstrates a clear dissociation between dominance for handedness and dominance for kinesthetic motor engrams.
Nine parkinsonian patients with main unilateral symptoms on the right side (RHP) and nine with symptoms on the left side (LHP) were assessed through a comprehensive neuropsychological battery. RHP performed at a lower level than LHP on the WAIS verbal subtests. Although both groups scored poorly on a test of frontal lobe functions, RHP performed significantly lower than LHP. On a line bisection task, LHP showed a mild left hemispatial neglect. In conclusion, mild but significant intergroup differences were observed, tending to correlate with predominantly hemispheric functional deficits.
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Serum IgG subclass levels were determined using a competitive indirect immunoenzymatic assay with monoclonal antibodies in 16 patients with ataxia-telangiectasia. Eight children had IgA deficiency, two had IgG and IgA deficiency and six patients showed no immunoglobulin class abnormality. However, IgG4 and IgG2 levels were undetectable or low in almost every patient. An IgG3 deficiency was associated with the IgG2-IgG4 defect in three patients with undetectable IgA. IgG1 was very low in one patient with a total IgG deficiency. There was no clear correlation between subclass levels and the occurrence of infections.