Clinical, pathogenetic and neuropathological correlates in dystonic cerebral palsy.
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Biomedical subjects
Publications and source records attributed to A Brun.
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The time necessary to obtain the immunity of cats against Panleukopenia has been studied by means of a modified live vaccine. This vaccine makes it possible to obtain a very early post-vaccinal immunity: the full immunity is reached 72 hr after the inoculation of the vaccine by the subcutaneous route. Furthermore, we have demonstrated that a sensitive kitten can be admitted in a contaminated environment immediately after vaccination without showing any clinical evidence of the disease.
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In a family of four children, three died within 8 days after birth. The fourth child survived after a critical first week with the same symptoms as the siblings. He was at the age of four found to have non-ketotic hyperglycinemia. The histories and neuropathology of the other children were then reexamined. It seems highly probable that all siblings suffered from non-ketotic hyperglycinemia. This report points to the clinical, biochemical and neuropathological diagnostic problems in this disease and also adds new information on the ultrastructural changes, not hitherto visualized.
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Limbic lobe involvement in presenile dementia was studied from a neuropathological and neuropsychiatric viewpoint. The material consisted of seven cases of Alzheimer's disease, four cases of Pick's disease, and four cases of Jacob-Creutzfeldt's disease. These three groups showed different patterns of distribution of the degeneration characteristic for each group, in particular for the first two. Among the groups, these patterns differed with regard to involvement both of nonlimbic and limbic areas. Thus the Alzheimer group had a mainly temporoparieto-occipital and posterior cingulate gyrus involvement. The Pick group in many respects showed an inverse distribution with frontotemporal and anterior cingulate gyrus accentuation of the damage. Basal temporal limbic areas were involved in both groups. The Jacob-Creutzfeldt group had a less schematic lesion pattern, without involvement of limbic areas. From a neuropsychiatric aspect, these differences were reflected in symptoms that could be referred both to areas spared and those more pronouncedly destroyed by the degenerative process. Thus the Alzheimer group long retained emotional qualities that were lost early in the Pick group. The possible relationship between neurotransmitters and regional accentuation of the degeneration is discussed.
The first Scandinavian cases of Zellweger syndrome (ZS) are described. A brother and sister, children of first cousins, had the typical clinical symptoms and pathological findings. Extensive metabolic studies in the boy were negative. Pipecolic acid in the urine was not elevated. Both children died at 14 weeks of age. Two months earlier the girl had suffered severe intestinal bleeding. Both had pneumocystic carinii pneumonia at autopsy although no evidence of immune deficiency had been found in the boy. The girl had used up her visible iron depots while the boy still had abundant but probably physiologic amounts of hemosiderin in the RES. Most of the cerebral abnormalities are unspecfic and possibly related to anoxia or other causes of delayed maturation. The white matter abnormalities in ZS patients may only be quantitatively different from the common "fatty metamorphosis" in infants. Previously reported ultrastructural abnormalities (absence of peroxisomes and very sparse smooth endoplasmic reticulum, as well as mitochondrial abnormalities) which are possibly unique for ZS, are confirmed. It is stressed that these were seen despite phenobarbital treatment which normally stimulates the formation of smooth endoplasmic reticulum.
1. Rats bred from vitamin B12-depleted dams were fed on a vitamin B12-deficient diet for 12-15 months and developed a severe vitamin B12 deficiency, as judged from methylmalonic acid excretion and tissue vitamin B12 levels at slaughter. Control rats were supplemented with vitamin B12 in the drinking-water. 2. Neurological signs were recorded after 7 months but the motor nerve conduction velocities remained normal. Neuropathological examination revealed mild changes in the peripheral nerves but no changes in the central nervous system. 3. The amounts of total lipids and phospholipids were normal, but in all examined tissues the proportions of pentadecanoate (C15 fatty acid) and heptadecanoate (C17 fatty acid) were considerably increased in vitamin B12 deficiency. 4. 3H2O was incorporated to the same extent into the fatty acids of nervous tissue from vitamin B12-deficient and control rats after 48 h. Less 3H was found in the liver fatty acids of the vitamin B12-deficient rats. 5. Neurological dysfunction can be demonstrated in the vitamin B12-deficient rat; the relation of the biochemical and neuropathological changes to the neurological signs needs further study.
Lymphocyte chromosomes were studied in 10 female patients with Alzheimer's disease, including 5 cases with positive heredity for organic dementia. The chromosomes were analyzed by means of the Giesma banding technique. All patients had a normal karyotype with a completely normal banding pattern in all metaphases analyzed. It may be concluded that chromosomal changes demonstrable with methods available today are not involved in the causation of Alzheimer's disease, whether sporadic or with positive heredity for organic dementia starting in the presenile or senile periods.
Six cases of fibromuscular dysplasia of the cervical and cephalic portions of the internal carotid arteries, including their intracranial branches are reported. It should perhaps be pointed out that one of the cases was from the Sudan. As far as we know, the condition has never before been reported in a male African. The condition was associated with an intracranial aneurysm in four of our cases. To our knowledge only three autopsied cases of fibromuscular dysplasia involving intracranial arteries are on record. In our six cases the diagnosis was based on angiographic evidence, and three of the cases, two with intracranial involvement, were verified post mortem.
This paper deals with the comparative development of active immunity in young pigs which were born from unvaccinated sows and from recently vaccinated sows. The development of immunity is expressed by the neutralizing serum antibody titer and protection against a pathogenic virus. The results show that: --the post-vaccinal increase in antibodies is inversely proportional to the titer of passive antibodies at the time of vaccination, --protection at the end of economic life depends on the antibody titer at the time of the challenge, --young pigs born from a mother which is not immune can be vaccinated starting at the age of 7 days, --the percentage of protection at the end of economic life of vaccinated baby pigs with colostral antibodies ranges from 50% when the vaccination was carried out at the age of 7 days to 100% when the vaccination occurs at the age of 2 months.
In view of the advantages and disadvantages of the different rabies vaccines, classified according to the production method of the virus and according to their nature - activated or inactivated - the choice of an activated vaccine produced on cell cultures is particularly justified for developing countries. The production of this type of vaccine, as well as the methods and results of the activity and innocuity controls are shown in detail. The interest of combined vaccines for certain developing countries, particularly the combined vaccine rabies/foot-and-mouth disease for ruminants, is stressed. The use of this type of vaccine on a vary large scale (more than 20 million doses up to this date) has given complete satisfaction. It should be noted that the same principles have been successfully applied to the realisation of a vaccine for human use, which can be administered before as well as after contamination.
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EEG, regional cerebral blood flow (rCBF) and neuropathological autopsy data were studied in 17 patients with presenile dementia. RCBF was measured in 14 cases using the intra-arterial-133-Xenon clearance technique. The neuropathological study included semi-serial whole brain microscopical sections in which the severity and regional distribution of the neuronal degeneration was studied, particularly in the cortex and the brain stem. There were two main diagnostic groups, seven cases of Alzheimer's disease and five cases with a cortical atrophy of the Pick (DFT) type. Both groups showed fronto-temporal cortical degeneration. In addition the Alzheimer group showed severe postcentral and posterior-temporal loss of neurons. There were also three cases with degenerative changes, suggesting Jacob-Creutzfeldt's disease. The Alzheimer cases had EEG abnormalities which progressed slowly. In contrast, the Pick (DFT) patients had normal EEGs which remained so even late in the course when the signs of dementia were marked. A positive correlation between rCBF reduction in postcentral areas (as evidence of neuronal loss in these regions) and an increase of EEG abnormality could be established. Degenerative changes confined to frontal and anterior-temporal cortical regions with concomitant flow reduction, on the other hand, appeared to leave the alpha rhythm essentially undisturbed. Finally, the relationship between bifrontal delta-episodes in EEG and neuropathological evidence of brain stem lesions was confirmed.
Brain proteins were analyzed in supra- and infratentorial structures of 6 patients dying from liver failure. An equal number of patients, lacking any evidence of liver disease or neurological disorder, served as controls. The results were related to regional light microscopic findings. Hepatic coma was associated with a marked reduction of soluble brain proteins, particularly in areas of grey matter. The protein loss is probably neuronal and may be secondary to abnormalities in glial function. Implications for the pathogenesis of hepatic encephalopathy are discussed.
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