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J Emile

Publications and source records attributed to J Emile.

At least 37 records · Page 2Linked to original sources

Ultrasonic features of extracranial carotid dissections: 47 cases studied by angiography.

The purpose of this prospective study was to assess the value of continuous wave Doppler velocimetry, standard duplex scanning and color Doppler flow imaging in the diagnosis of carotid dissections. From 1975 to 1993, 42 patients (mean age, 44 +/- 14 years) were admitted to the University Hospital of Angers for a carotid dissection studied first by ultrasonography, then defined by angiography. Five cases were bilateral. Continuous wave Doppler examination revealed signs of severe obstruction of the carotid arteries in 96% of the cases (occlusion, extensive submandibular tight stenoses, significant slowdowns in the carotid and ophthalmic vessels, retrograde ophthalmic blood flow). Standard duplex scanning suggested dissection in 72% of the cases (tapering stenoses or occlusion, segmental ectasis, tubular vessel, peripheral residual channel, or rare irregular "membrane"). Color Doppler flow imaging suggested a dissection in 82% of the cases. This method has the advantage of underlining the peripheral channel, the double lumen, and the dissecting hematoma, which often is hypoechoic. The ultrasonic methods (continuous wave Doppler combined with color Doppler flow imaging) failed only when they are performed late and when moderate or segmental intrapetrosal dissections were present. These ultrasonic investigations would thus appear to be useful for early diagnosis of carotid dissections.

Adult↗

[Doppler transcranial ultrasonography in carotid and vertebral dissections: 36 cases involving angiography].

The aim of this prospective study was to investigate the additional information provided by transcranial pulsed Doppler for the determination of the upper part of dissections of the carotid and vertebral arteries and following-up. Earlier results concerning the role of ultrasound investigations for this indication have been presented elsewhere. Twenty-nine patients were examined (17 females, 12 males; mean age 43 years). All had arterial dissections confirmed by arteriography. There were 24 carotid dissections and 12 vertebral dissections. Investigations were performed during the acute phase and 2 months later. Most of the severe endocranial stenoses were identified by transcranial Doppler: 5 out of 6 severe stenoses of the carotid siphon and 6 out of 7 vertebral stenoses. The haemodynamic effect as measured by sylvian flow was significantly greater (p < 0.01) after a permanent ischaemic event than after temporary events and improved significantly with time (p < 0.008). There was no significant variation in the pulsatility transmission index (PTI), but this index requires a reference artery and is not particularly adapted in cases with bilateral dissection. Collateral perfusion, observed during the acute phase in all cases of occlusion and in 6 of the 8 carotid stenosis, was insufficient in half of the cases. Transcranial pulsed Doppler can be used to guide arteriography and helps determine the length of the dissection. It provides an easy way to assess the haemodynamic consequences of the dissection and is quite useful during follow-up.

Adult↗

[Ultrastructural and immunohistochemical study of the olfactory mucosa in Alzheimer's disease].

The discovery of reliable peripheral markers would be of great interest for the diagnosis of Alzheimer's disease. Interestingly specific lesions of Alzheimer's disease were found in the olfactory areas of the brain. The loss of the detection and the identification capabilities in olfaction suggest a defect in the olfactory mucosa. The ultrastructural study of biopsy of olfactory mucosa from patients suspected to suffer of Alzheimer's disease revealed a degeneration of sustentacullar and olfactory cells as well as an architectural disorganization. No neuropathological characteristic lesion such as Paired Helicoidal Filaments or amyloid fibrils could be evidenced. Immunohistochemical analysis achieved with a polyclonal antiserum raised against A4 protein (fragment 1-28) showed a specific staining in the sustencellular and of the outer part of the olfactory epithelium corresponding to the mucus area, whereas appropriate controls were negative. Our study raised the question of the amyloid protein origin in the olfactory mucosa. Analysis of some more patients will enable us to determine the diagnostic value of this study in Alzheimer's disease.

Aged↗

Uneven distribution of mitochondrial DNA mutation in MERRF dizygotic twins.

A new family of myoclonic epilepsy with ragged-red fibers (MERRF) was studied at clinical, histological, biochemical and molecular genetic levels. There was a remarkable variation in the age of onset, the clinical presentation and the severity of symptoms. Multiple defects affecting respiratory chain complexes I, III and IV were detected in 2 patients. The point mutation at 8344 of the mitochondrial genome was found in all the maternal lineage with a relatively narrow range of variation in the percentage of mutant mitochondrial genomes. The one exception was represented by a set of dizygotic twins, one clinically affected and showing high proportions of mutant mitochondrial DNAs (mtDNAs) in blood cells, while the other was asymptomatic and showed very small amounts of mutant mt-DNAs in blood and skin. This could suggest an early segregation of the mitochondrial genome during ovogenesis.

Adult↗

Abnormal expression of actin in lymphocytes of Alzheimer's disease and Down's syndrome patients.

Alzheimer's disease (AD) is a degenerative disorder of the central nervous system accompanied by several immunological disturbances and a number of common features exist between AD and Down's syndrome (DS). High resolution two-dimensional electrophoresis of lymphocyte proteins demonstrates an actin abnormality in AD and DS: a double actin spot instead of the single spot observed in controls. This dual form was studied by pulse-chase experiments and seems to be related to extracellular factors which influence the post-translational modification of actin. These results agree with the immunological disturbances observed in AD and DS, and with the well established hypothesis that AD is a systemic as well as cerebral disease.

Actins↗

[Olfactory mucosa and Alzheimer's disease. Technique for biopsy and ultrastructural study].

The discovery of a reliable peripheral marker would be of a great interest for the early diagnosis of the Alzheimer's Disease. The olfactory deficit and the major histologic changes of the olfactory-related areas of the brain occurring during this disease raised the possibility that the olfactory epithelia could be one of the way of entry of a possible process that still has to be identified. We have developed an instrument and a technique of biopsy of the human olfactory mucosa to search for the presence of characteristics lesions on patients suffering of an Alzheimer's Disease. These small specimens have been prepared for electronic microscopy. The ultrastructural study of a sample of olfactory mucosa has been realised in 9 cases (5 Alzheimer's-4 controls) revealing in 4 patients suspected of an Alzheimer's Disease a complete architectural disorganisation with a destruction of the dendrite of the olfactory cells and a severe degeneration of the sustentacular cells. We did not find any characteristic changes such as Paired Helicoidal Filaments or amyloid fibrils. These results do not presuppose of their eventual presence at a precocious stage of the disease. Further ultrastructural and immunochemical studies carried out with patients at various stages of the disease are necessary in order to confirm this hypothesis.

Aged↗

[Chronic encephalitis with mesencephalic predominance. A clinico-pathologic case].

A 70 year-old woman presented with a progressive supranuclear ophthalmoplegia, with "apraxia" of eyelid opening, axial akinesia and dementia. CT scan showed a mild cortico-subcortical atrophy and there was a high level of immunoglobulins, with an oligoclonal pattern, without cell reaction in the CSF. The patient died two years after the onset. Post-mortem examination, limited to CNS, showed subacute encephalitis confined to the tectal, pretectal, subthalamic areas and to Ammon's horns. These changes and their location were strongly suggestive of polioencephalomyelitis with or without cancer, in which such a prevalent midbrain involvement has been exceptionally described.

Aged↗

[Sensory nerve involvement in X-linked bulbospinal amyotrophy (Kennedy syndrome). Contributions of electrophysiologic and histologic data].

Two new cases of X-chromosome linked bulbospinal muscular atrophy associated with gynecomastia (described by Kennedy in 1968) are reported. In one patient, an electrophysiological study and a muscle nerve biopsy were performed. Motor nerve conduction studies were normal but sensory action potentials were small or unrecordable in the absence of clinical sensory loss. Superficial peroneal nerve biopsy showed axonal lesions. These findings suggest that this disease is not purely due to degeneration of spinal motor neurons and is also associated with an axonopathy.

Electromyography↗

[Clinico-pathologic case of slowly progressive herpes simplex encephalitis without temporal necrosis].

A case of herpes simplex encephalitis (HSE) is reported. The patient experienced short term memory disorders and irritability progressing over 3 months, without seizures or fever. The CSF was normal. CT showed a small low density area in the right posterior orbito-frontal region. At post-mortem examination, one month later, the temporal cortex appeared largely spared by necrosis, which involved the posterior orbito-frontal areas. Cowdry type A inclusions, herpes virus like particles and fluorescent reaction with HSV1 monoclonal antibodies strongly supported the diagnostic. Such atypical cases of long duration have apparently seldom been reported. They suggest that HSE should be considered in the differential diagnosis of a subacute encephalopathy.

Aged↗

[Progressive spinal muscular atrophy and parathyroid adenoma. Clinico-pathologic study of a case].

A 82 year-old man died 6 years after the onset of a progressive spinal muscular atrophy. Post-mortem examination disclosed a parathyroid adenoma. Weakness and wasting were prominent in the proximal lower limbs. There were no fasciculations. Involvement of the medulla was mild and late. These clinical features were also present in 16 reported cases, which were improved by treatment of primary or secondary hyperparathyroidism. Our patient differs by the involvement of the hand muscles and the loss of tendon reflexes. Neuropathological study, as in one other reported case, showed a loss of anterior horn cells. Such cases underline that calcium metabolism must be studied in syndromes of spinal muscular atrophy.

Adenoma↗

[Long-term study of 20 cases of proximal stenosis of the middle cerebral artery. Role of transcranial Doppler ultrasound].

The validity of transcranial Doppler (tD) was evaluated for the identification of stenoses of the middle cerebral artery (MCA). Twenty patients with atheromatous stenosis of the proximal MCA of more than 30% (3 of these cases with stenosis of more that 75%) had repeated transcranial Doppler between 1975 and 1987 and conventional angiography. No patient had carotid stenosis nor embolus-producing cardiopathy. Ten patients had had infarcts or transient ischemic attacks (symptomatic group). Ten patients were asymptomatic (asymptomatic group). Cerebral infarcts in the MCA territory occurred in 1 patient of each group and 1 additional patient in each group had an infarct in another territory. Follow-up was possible in 15 patients. The stenosis remained unchanged in 14, occlusion occurred in 1. The results of transcranial Doppler were compared to those of angiograms in this series and in an additional series of 40 patients who had had a normal angiogram. Sensitivity of transcranial Doppler was 60% (75% when stenosis was over 50%). Specificity was 95%. Comparison with angiography findings showed, among causes of failure of tD, analysis of the artery over too short a segment or kinked artery of very long stenoses, or too decreased blood flow. The failure rate of tD due to failure of bone penetration by ultrasounds was 5%. This new reproducible, non-invasive method appears to be able to detect MCA stenoses of 50% or more and help in follow-up.

Adult↗

[Ultrasonic study of 22 cases of carotid artery dissection].

The purpose of this study was to assess the value of duplex scanning and continuous wave Doppler velocimetry in the diagnosis and follow-up of ICA dissections. Between 1975 and 1988, 20 patients (11 women and 9 men; mean age 45 years) were admitted to the University Hospital of Angers for dissection of the ICA confirmed by angiography. The dissections were unrelated to direct cervical injury and were unilateral in 18 cases and bilateral in 2 cases. Six patients experienced transient cerebral ischaemic accidents (later completed in 2 cases) and 2 patients merely complained of ocular disorders of sympathetic origin. Fourteen patients were treated with heparin. All patients were examined by continuous wave Doppler ultrasound followed by duplex scanning. These examinations were performed 10 days on average before angiography. Continuous wave Doppler revealed signs of obstruction of the ICA in 95% of the cases: occlusion in 4 cases, tight stenosis in 13 cases and marked slowing of blood flow in the carotid and ophthalmic arteries in 4 cases. The acoustic signs of high or extensive ICA stenosis with reduced or retrograde ophthalmic artery blood flow were fairly suggestive of dissection. These results were completed by mode B which showed signs of dissection in 61% of the cases (tapering stenosis or occlusion, tubular ICA, separation of the vascular walls on rare occasions) and excluded atheromatous lesions in 81% of the patients. The tubular ICA image being non-specific was interpreted in relation to the clinical context and haemodynamic data, after discussion and exclusion of fibromuscular dysplasia, intracranial carotid stenosis causing severe reduction of blood flow and the exceptional hypoplasia of the ICA.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Singular clinical form of intramedullary cavity revealed by a loss of proprioceptive sensation in the upper limbs].

A 47-year-old woman developed a progressive sensory deficit of the upper limbs. She lost perception of joint position, vibratory sense, skin-writing and stereognosis. Repeated tests by several examiners, up to 10 years after the onset, demonstrated normal pain and temperature sensation. The right hypoglossal nerve was involved at a late stage of the disease. CT and MRI showed an intramedullary cavity from C1 to T7. Somato-sensory evoked potentials from stimulation of the median nerves confirmed the bilateral impairment of the lemniscal pathways.

Arm↗

An ELISA technique for the measurement of C1q in cerebrospinal fluid.

Determination of the C1q content of cerebrospinal fluid (CSF) may be of value in understanding the immunological reactions occurring within the central nervous system (CNS). A double sandwich ELISA method has been developed for the detection of C1q in human serum and CSF. It uses polyclonal antibodies and is sensitive in the nanogram range. The mean concentrations of C1q were determined to be 127 micrograms/ml in serum and 0.4 microgram/ml in CSF. These results suggest that increased levels of C1q in the CSF play a role in some neurodegenerative disorders.

Adolescent↗