Unilateral seizures: hemiconvulsions-hemiplegia syndrome (HH) and hemiconvulsions-hemiplegia-epilepsy syndrome (HHE).
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Biomedical subjects
Publications and source records attributed to M Bureau.
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Ultrasonographic examination of the liver segment I, based on limiting vascular ans fissural elements, requires detailed knowledge of hepatic anatomical and organizational features. Findings are of two-fold interest for pre-operative assessment. Firstly, as a complement to essential arteriography it can establish the integrity of the segment when considering hepatectomy for liver tumors, and secondly it enables application of modifications in techniques for portocaval anastomoses. The data obtained can also assist diagnosis in cases of cirrhosis and Budd-Chiaris syndrome.
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The authors have studied 119 cases of partial epilepsies, selected from the patients treated in the Centre Saint-Paul between 1966 and 1970 according to the following criteria: seizures beginning before the age of 12; electro-clinical evolution known about for at least 5 years (mean duration: 13 years 3 months). The cases are separated in 4 groups according to the severity of epilepsy: (1) complete recovery and withdrawal of treatment; (2) no seizure with treatment; (3) persisting seizures with treatment; (4) frequent and severe seizures with treatment. The severe epilepsies are characterized by an early commencement of fits (before 3 years), associated symptoms of encephalopathy, lack of familial antecedents, association of several types of seizures, or the existence of a particular critical semiology (either complex partial motor seizures, or succession of simple motor and complex semiology, sudden fall, or reflex triggering). However, it is also necessary to consider the syndromic grouping of signs which is of greater value than a simple analysis of benignity or malignancy factors.
The authors report observations on 7 children who have shown an early epilepsy characterized only by brief myoclonic fits, with generalized spikes and waves on the EEG. This type of epilepsy had a favorable evolution in all cases under treatment (usually VPA). In 2 cases, rare grand mal seizures appeared between 9 and 12 years. The authors discuss the situation of this epilepsy among all the myoclonic childhood epilepsies reported in the literature. They conclude that it is an early type of primary generalized epilepsy.
A case of isolated necrotizing, granulomatous hepatitis of Brucella origin is discussed, together with 6 similar cases reported in the published literature. The clinical picture is dominated by the history of the patient (occupation, geographical location), fever, right hypochondrial pain, and altered general condition. Radiological examination shows characteristic calcifications. Diagnosis is confirmed by serology, Brucella suis usually being involved. Pathological appearances are typical : large foci of hepatic necrosis surrounded by marked inflammatory zones with histiocytic cells arranged in palisades. The only possible treatment is surgical.
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Thermodynamic variables characterizing the transport of water and urea across Dog pulmonary capillary membrane were obtained in vivo by means of a pulse transient osmotic method. Results were inconsistent with the hypothesis of a homoporous membrane. When data were interpreted in terms of an exclusive water channel and of a shared pore pathway, the value of the pore reflexion coefficient was close to zero for urea.
The evolution of 15 patients initially evaluated during Phase One of the Quebec Cooperative Study of Friedreich's ataxia has been studied approximately three years later. It is concluded that the deterioration of cardio-pulmonary function in Friedreich's ataxia is multifactorial. The neuromyopathy (or the underlying metabolic or cellular defect) appears to be the main contributing factor to the deterioration of cardio-pulmonary function, which is exacerbated by the scoliosis and varying severity of the cardiomyopathy.
Unilateral or bilateral electrographic temporal discharges at 5 Hz were analyzed in relation to their morphological characteristics, their topography, and their evolution in II subjects. The clinical context, in which there was no evidence of epileptic disorder, was also analyzed. These discharges were compared to other infraclinical critical phenomena reported in the literature, and related to similar manifestations described by the Gibbs "school" as "Psychomotor variant type of discharge", or by Hughes group as "Rhythmic mid Temporal discharges".
The authors describe the electro-clinical state of four children having a type of epilepsy clinically characterized by rare partial motor seizures and frequent absences. From E.E.G. point of view they had focal (mainly frontal) and diffuse abnormalities. Such diffuse abnormalities became continuous during slow sleep, thus realizing an electrical status epilepticus. During such status partial subclinical seizures were recorded. At the onset such cases have electroclinical features resembling those observed in the form of epilepsy so-called "benign partial epilepsy of children with rolandic or mid-temporal foci". All cases, however, have behavioural problems (instability, desorientation) and decreased school performances. The epilepsy evolution, however, is favourable and such form should consequently be distinguished from the Lennox-Gastaut Syndrome.
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Acute emphysematous cholecystitis is a rare condition. Its preoperative diagnosis is based upon radiological studies which should include a film in the dorsal decubitus position, a lateral and a film in lateral decubitus with the ray horizontal. The presence of air within the gall bladder lumen; and in a part or all of the wall. Additional findings: the presence of air in the peribiliary space; the presence of gas within the biliary tract, rarely reported, is of special interest in the case reported here. Gangrenous cholecystitis must be differentiated essentially from: 1 degree an internal biliary fistula 2 degrees air in a neighbouring organ 3 degrees lipomatosis.
Partial volume measurements have been performed for human hemoglobin, both on the oxygenated and deoxygenated forms. Density measurements (by pycnometry) give vHbO2 = 0.752 +/- 0.002 and vHb =0.753 +/- 0.006 for the partial specific volume and do not distinguish between the two different structures. Differential measurements, by dilatometry, however, show a signigicantly higher molal volume (of about 50 cm3/mol hemoglobin tetramer) for the deoxy over the oxygenated from at pH 7. The same reaction, at pH 9, gives a much smaller increase or even a decrease of volume. The different volume changes at pH 7 and at pH 9 are not due to the so-called Bohr ionization but to the weakening, at pH 9 compared to pH 7, of stabilising salt linkages in the deoxy structure.
In three awake dogs in a hypobaric chamber at 140 m and at 3550 m, resting ventilation, pulmonary gas exchanges, respiratory gases and pH of the arterial blood, acid-base status in the cerebrospinal fluid (csf), and ventilatory responses to transient O2-inhalation were studied before (intact) and after chronic bilateral carotid body denervation (cbd). 1. The hypoxic chemoreflex drive of ventilation was reduced by about half in cbd dogs. 2. At low altitude, sino-carotid body denervation resulted in hypoventilation and respiratory acidosis in the arterial blood and csf. 3. At high altitude, initial hypoxic hyperventilation, and the related alkalosis in blood and csf, occurred within 30 min in intact dogs, but was not observed in cbd ones. 4. Further increase in ventilation was achieved upon 3 hrs of altitude exposure in intact animals, while a delayed hyperventilation occurred after 24 hrs in cbd ones. 5. Neither in intact nor in cbd dogs, the ventilatory changes at altitude were related to the changes in csf pH. It is concluded that the rate of ventilatory acclimatization to altitude is dependent upon the strength of the arterial chemoreceptor drive. Integrity of this chemoreflex drive of breathing is essential in determining the eupneic level of ventilation and normal acid-base status of the blood and csf at low altitude and at high altitude.