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Biomedical subjects

J P Bernier

Publications and source records attributed to J P Bernier.

At least 19 recordsLinked to original sources
PubMed1995

[Significance of polyglucosan bodies in neuropathology. Clinico-pathologic study of seven cases].

Seven observations, having in common an accumulation of polyglucosan bodies in the nervous system, are reported. In 2 cases of adult polyglucosan body disease, characteristic polyglucosan bodies were found on the peripheral nerve biopsies, and also on the cerebral biopsy in one of these patients who was demented. The 5 other cases presented medically intractable temporal lobe epilepsy. Recovery was obtained by a temporal lobectomy in which a massive accumulation of polyglucosan bodies was discovered. These personal observations are discussed with regard to other conditions characterized by accumulation of polyglucosan bodies in the nervous system. The non specificity of these formations is emphasized.

Adult↗
PubMed1990

Paraneoplastic encephalomyelitis and subacute dysautonomia due to an occult atypical carcinoid tumour of the lung.

A case of paraneoplastic encephalomyelitis and subacute pandysautonomia associated with an occult atypical carcinoid tumour of the lung is described. The main clinical features were lethargy, impaired memory, constipation, and orthostatic hypotension. Neurological investigation was unremarkable except for mononuclear pleocytosis and increased protein level in the cerebrospinal fluid (CSF). Tests of autonomic function revealed a low plasma norepinephrine level, a marked drop of blood pressure (BP) to vertical tilt and Valsalva maneuver, and a marked rise of BP to dilute norepinephrine infusion. A few days prior to death, the patient became hypothermic and had repeated episodes of respiratory arrest associated with transient atrioventricular block on the electrocardiogram (ECG). A polysomnographic study confirmed a sleep apnea syndrome. Autopsy revealed an atypical carcinoid tumour in one tracheobronchial lymph node, widespread lymphocytic infiltrates and loss of neurons in the cerebral, cerebellar and brainstem grey matter, the spinal cord and roots, and the paravertebral sympathetic ganglia as well as microglial and astrocytic proliferation in the central nervous system.

Aged↗
PubMed1990

A craniometric study of the C. de Lange syndrome (CdLS).

After a short review of our method, we compared the results of the radiographs of 18 cases of CdLS to the results of our normal material. All of our normal statistical data was obtained from AP and lateral view radiographs at a distance of 1 m, in the prevailing conditions of everyday practice in a radiological department. We studied quantitative changes such as microcephaly and brachycephaly, and for the first time we introduced qualitative changes such as occipital rotation and the study of obliquity of the foramen magnum (line BAO). We found microcephaly in all our cases of CdLS and brachycephaly in only 72%. Furthermore, we found a lack of positive occipital rotation in all our cases of CdLS and the line BAO turned posteriorly with O higher than BA. The same conditions were observed on our normal material only in an early postnatal stage.

Cephalometry↗
PubMed1985

The statistical analysis of distribution of blood deposits in arterial prostheses after short-term exposure to reduced blood flow in dogs: a computerized study using radioisotopes.

This study was undertaken to analyse the distribution pattern of blood elements deposits in 15 cases of vascular prostheses submitted, ex vivo, to reduced blood flow in a canine model. Autologous blood platelets labeled with Indium-111 and human 125 Iodinated fibrinogen were injected into the animals. Ten minutes later, the blood flow in the aorta was reduced to 50 ml/min and the prostheses (Woven DeBakey, 30 cm long and 6 mm internal diameter), were implanted as substitutes for the infrarenal abdominal aorta. The blood flow was then reestablished to 50 ml/min and controlled throughout the entire experiment. When a thrombosis occurred, or after 4 hours of low blood circulation, the prosthesis was collected, weighted and chemically fixed, and the radioactivity was measured by means of a two-channel gamma scintillation counter. These data were then formatted and processed by a special computer program. Final values were processed for graphical representation and analysed statistically. Results show a lack of correlation between weight of thrombotic matrix, platelet accumulation, and fibrin deposits retained from one animal to the other. There is, though, an appearance of a complex interrelationship between the mean pattern of longitudinal distribution of these elements, which shall be qualified as contagious distal accumulation of the deposits. The influence of each animals' blood characteristics, as evaluated by means of regression analysis, account for 40 to 60% of the interindividual variance in blood deposits. Data corrections following evidence of undesirable influences due to uncontrolled parameters are presented. This experimental method permits simultaneous graphical representation of the distribution of several elements in the thrombotic matrix, and at the same time an elaborate numerical analysis of their longitudinal distribution and total accumulation. The method is easy to use, does not require sophisticated equipment, and is less expensive than scintigraphy.

Animals↗
PubMed1982

Pathogenesis of respiratory insufficiency in myotonic dystrophy: the mechanical factors.

We have previously shown that the chemosensitivity of the respiratory centers is well preserved in myotonic dystrophy but that the ventilatory output is reduced. The present study was designed to determine at which degree of ventilatory performance weakness and fatigability of the respiratory muscles are interfering with ventilation and which mechanical factors contribute to the tachypnea of patients with myotonic dystrophy at rest and during low ventilatory output. We studied 10 patients with the disease and 10 normal control subjects. The strength of respiratory muscles was assessed by measurements of maximal pressure-volume diagrams generated against airway occlusion. Performance was evaluated during 1-min maximal voluntary ventilation (1-min MVV) test, during 7-min 7% CO2 breathing and during quiet breathing. Occlusion pressure (P0.1) in patients at rest was slightly higher than in control subjects, and during CO2 breathing, it was similar to that of control subjects. Maximal static pressure was reduced in patients to an average of 35% of that of control subjects. During the 1-min MVV test, there was a 50% reduction in esophageal and transdiaphragmatic pressure output (Pes, Pdi) in patients, resulting in similar reduction in ventilation (VE) and patients had rapid cycles of alternating dominant thoracic and abdominal volume displacements (Vrc/Vabd) suggesting respiratory muscle fatigue. During the 3- to 4-fold increase in breathing drive induced by hypercapnia, pressure output and the Vrc/Vabd were identical in both groups. However, ventilation was reduced in patients who had tachypneic respiration. In patients, tachypnea was also observed during quiet breathing. This tachypnea was associated with higher impedance of the respiratory system (Zrs) in patients and identical impedance of the lung (ZL) in both groups. In addition, Pdi during tidal volume was significantly higher in patients. These data demonstrate that the ventilatory output in out patients was altered predominantly by weakness and fatigability of the respiratory muscles during high ventilatory performance and by increased impedance of the respiratory system at lower degrees of ventilation.

Adolescent↗
PubMed1977

[Progressive multifocal leucoencephalopathy. Observation with predominant pontocerebellar lesions and association with congenital immune deficiency].

A young woman presented a mixed congenital and familial immunodeficiency syndrome consisting in an absence of IgA and lowered levels of IgG and IgM, with a defect in cellular immunity. She had a mild malabsorption syndrome with slight alterations of the jejunal mucosa. Non-caseating tuberculoid granulomata were found in skin lesions, in lymph nodes and in the spleen. At age 27 the patient died of a neurological disease of 4 months duration. Autopsy revealed a very widespread demyelinating process involving mainly the right cerebellar hemisphere but also most of the pons and left cerebellum, with the typical morphologic characters of PML. In the hemispheres lesions were limited to microscopical "microglial nodules" with discrete demyelination. A review of 86 published cases of PML revealed 9 other cases in which lesions showed a strong predilection for the subtentorial territories. This sampling allows for tha assumption that some 11% of the cases of PML have this particular lesion distribution. Other pertinent features of this case are briefly discussed.

Adult↗