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Biomedical subjects

J Emile

Publications and source records attributed to J Emile.

At least 19 recordsLinked to original sources
PubMed2002

[Isolated angiitis of the central nervous system. Report of two cases and review of the literature].

Isolated angiitis of the central nervous system is a rare disease affecting mainly adults of both sex; about 210 cases have been reported. Contrary to other inflammatory arteritis, arthralgia, myalgia, weight loss and fever are exceptional and symptoms are mainly neurologic, but none is specific. The diagnosis is evoked in case of headaches and cognitive impairment, associated or not with multifocal neurologic signs. Evolution is acute, subacute or chronic. Elevated sedimentation rate and cerebrospinal fluid pleiocytosis are present in 2/3 of cases. CT scan and brain MRI generally demonstrate multifocal ischemic lesions involving cortex, white matter, basal ganglia and brainstem. Cerebral arteriography is the key investigation, showing segmental stenoses alternating with fusiform dilatations of blood vessels, which are highly suggestive but not specific. It can be normal and its repetition is then recommended. Certain diagnosis is obtained from cerebromeningeal biopsy, showing a segmental angiitis of small vessels, which is granulomatous in 88 p.100 and non granulomatous in 12 p.100 of cases. The pathogenesis is unknown. Spontaneous evolution is generally fatal. Cyclophosphamide associated with prednisone considerably improves the prognosis, especially when initiated early in the course of the disease.

Adult↗
PubMed2001

Influence of centrality and distinctiveness of actions on script sorting and ordering in patients with frontal lobe lesions.

In order to further investigate script knowledge in patients with frontal lobe lesions (FP), 30 participants with frontal brain damage, 14 controls with posterior brain damage (PC) and 30 normal controls (NC) were asked to sort script actions with various degrees of centrality and distinctiveness according to the script to which they belonged and their order of execution. Actions were given in 3 conditions: scripts with headers (SH), scripts without headers (SwH) and scripts with distractor header (SDH). Results confirmed the dual dissociation we observed in a previous study (Allain, Le Gall, Etcharry-Bouyx, Aubin, & Emile, 1999). Twelve FP made numerous errors in sequencing actions regardless of the conditions, but rejected the irrelevant title. Fourteen FP made few sequencing errors but used the irrelevant title. The last 4 FP performed as well as the PC and NC in every condition. Sorting errors made by the FP mainly concerned actions with low levels of centrality and distinctiveness. These data are consistent with the view that frontal lobe lesions selectively impair the syntactic component of script representation without affecting its semantic component.

Adult↗
PubMed2001

Accuracy of color-Doppler in the quantification of proximal vertebral artery stenoses.

BACKGROUND: Vertebrobasilar (VB) strokes appear to have the same causes as carotid strokes. Obstructive lesions of proximal vertebral arteries probably occur in about 30% of stroke patients. PURPOSE: Our aim was to assess the validity of color Doppler sonography compared to selective intra-arterial angiography in the quantification of proximal vertebral artery stenoses. MATERIALS AND METHODS: A prospective blind study of 316 vertebral arteries was undertaken between 1996 and 1998. One hundred and fifty-eight patients with cerebrovascular disorders without cerebral hemorrhage were studied consecutively by frequency or amplitude color Doppler flow imaging and intra-arterial angiography. The lesions were quantified by morphological and hemodynamic criteria and classified into 6 groups: 0% 207 arteries; 1-29% 32 arteries; 30-49% 29 arteries; 50-69% 13 arteries; 70-99% 23 arteries; 100% 12 arteries. RESULTS: Ten of the 12 occlusions were identified, the 2 false-negatives were due to 2 revascularized vessels. Moderate stenoses (<50%) were differentiated from tight stenoses (>50%) using hemodynamic criteria. The majority of false-negative stenoses (38) in the different groups were related to intrathoracic or very deep origin of the artery, anechogenic stenosis or a tortuous vessel. Stenoses greater than 70% were diagnosed in 71% of cases with a specificity of 99%. The kappa value was 0.80. CONCLUSION: Duplex sonography should be proposed first in VB attacks or stroke to detect and quantify vertebral artery stenoses for surgery and angioplasty.

Arterial Occlusive Diseases↗
PubMed2000

[Design of a force sensor to evaluate the grasping movement].

Grasping phenomena are abnormal motor behaviours described after cerebral lesions. They are defined as a stereotyped response, consisting of a progressive forced closure of the patient's hand on the examiner's fingers, when they are slowly moved exerting a strong pressure across the palm in an upward direction. Several types are described. The aim of the study was to develop a force sensor to evaluate the force of grasping in accordance with the force of dragging. The characteristics of this force sensor are presented and the clinical application in two selected patients demonstrated.

Aged↗
PubMed2000

[Incidence of grasping and its relationship to cerebral lesions].

Grasping is associated with frontal lobe pathology. Nevertheless, there is lack of precise anatomical correlations and very few studies are published. The aim of the study was to determine the incidence of the grasping and its relationship to cerebral lesions. We studied 236 patients admitted to the Neurology Department (108 women and 128 men; mean age 65.3), and tested with a standardized procedure (De Renzi and Barbieri, 1992). A score of grasping was determined for each patient. The locations of the cerebral lesions were assessed by two neurologists using the method of Damasio and Damasio (1989). Grasping was found in 38 patients (16.1p.100) with dementia or cerebral damage. In all cases, the lesion affected the frontal lobe. The patients with grasping showed a significant higher number of lesioned areas particularly for the frontal and the parietal regions. The score of right grasping was significantly higher with a lesion in the right paraventricular frontal and in the left parietal paraventricular areas. The score of left grasping was significantly higher with a lesion in the left frontal paraventricular area. These results are discussed in relationship with motor control.

Age Factors↗
PubMed1999

mtDNA haplogroup J: a contributing factor of optic neuritis.

Optic neuritis frequently occurs in multiple sclerosis (MS), and shares several similarities with the optic neuritis of Leber's hereditary optic neuropathy (LHON), which is mainly due to maternally transmitted mitochondrial DNA (mtDNA) mutations. Our report shows for the first time that a mitochondrial DNA background could influence the clinical expression of MS. One European mtDNA haplogroup was found only in MS patients with optic neuritis but not in MS patients without visual symptoms. Therefore, we hypothesize that mtDNA haplogroup J might constitute a risk factor for optic neuritis occurrence when it is coincidentally associated with MS, but not be a risk factor for developing MS per se as suggested previously.

Alleles↗
PubMed1999

Mental representation of knowledge following frontal-lobe lesion: dissociations on tasks using scripts.

Twenty-three frontal-brain-damaged subjects, 10 posterior-brain-damaged subjects and 10 normal controls were compared on script tasks. They were first asked to re-establish the sequential and hierarchical structure of 2 script actions, and then, they were asked to order 2 new scripts which contained aberrant items. Impairments in script information processing were observed only in frontal patients. Of the 23 frontal patient tested 8 made errors in ordering actions, in choosing scenes and in making estimates of action importance in the first task, and they rejected the aberrant elements in the second task. Eleven frontal subjects performed as well as the control subjects in the first task, but used the irrelevant items. The last 4 frontal patients performed as well as the control subjects in both tasks. These results support a possible fractionation of the frontal lobe syndrome.

Adult↗
PubMed1998

Cognitive functions in chronic locked-in syndrome: a report of two cases.

We describe a 38-year-old woman and a 38-year-old man who have been living in a "locked-in" condition 3 and 2 years, respectively. Extensive neuropsychological testing, carried out with a communication aid system (writing apparatus) which exploited the patients residual eye movements, showed preserved cognitive abilities in spite of the long standing de-efferentation.

Adult↗
PubMed1998

A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis. European ALS/IGF-I Study Group.

To test the safety and efficacy of recombinant human insulin-like growth factor-I (rhIGF-I) in ALS, 183 patients from eight European centers were randomized to receive double-blind placebo (n = 59) or rhIGF-I 0.1 mg/kg/day (n = 124) subcutaneously for 9 months. At study completion, the primary efficacy outcome measure (change in disease progression as assessed by the Appel ALS rating scale) showed no significant difference between treatment groups. RhIGF-I appeared to be safe and well-tolerated.

Adult↗
PubMed1997

Extracranial and intracranial vertebrobasilar dissections: diagnosis and prognosis.

OBJECTIVES: To compare the diagnosis and prognosis of extracranial versus intracranial vertebral artery dissections without intracerebral haemorrhage. METHODS: Twenty two vertebral artery dissections were defined by intra-arterial angiography and classified in two groups: group 1, nine extracranial dissections (seven patients) and group 2, 13 intracranial dissections (nine patients), involving the basilar artery in five cases. Bilateral dissections were found in 38% of the population. Before angiography, all the patients had been investigated by continuous wave Doppler, colour coded Doppler, and transcranial Doppler. Mean follow up was 44 months. RESULTS: The two most important symptoms of both dissections (81% of patients) were unbearable pain preceding stroke and progressive onset of stroke within a few hours. Severe ultrasonic abnormalities were present in 94% of the patients whereas specific ultrasonic signs (segmental dilation with eccentric channel) were rare (19%) in both groups. Major strokes and brainstem strokes represented respectively 67% and 78% in intracranial versus 43% and 29% in extracranial dissections. Severe sequelae (permanent disabling motor or cerebellar deficit) were more often associated with intracranial (44%) than with extracranial dissections (14%). No recurrence of dissection and no cerebral haemorrhage were found under heparin. Significant factors of poor outcome (P< 0.05) were the initial severity of the stroke and the bilateral location of dissections. CONCLUSION: The combination of a pain and a progressive onset of the stroke, corroborated by ultrasonic findings, could have helped to recognise most of these types of dissections. Intracranial dissections have a poorer prognosis than extracranial dissections.

Adult↗
PubMed1997

Detection of vertebrobasilar intracranial stenoses: transcranial Doppler sonography versus angiography.

Vertebrobasilar intracranial stenoses seem to carry a higher risk of brain stem ischemia than proximal vertebral artery stenoses. Our aim was to assess the value of transcranial Doppler sonography versus angiography in detecting and quantifying these intracranial stenoses. All consecutive patients who underwent transcranial Doppler sonography prior to angiography from 1989 to 1994 and whose sonograms showed a stenosis of greater than 50% of one vertebral artery (21 cases) or of the basilar artery (eight cases) were included in the study. These patients were compared with 60 other consecutive stroke patients studied via transcranial Doppler sonography prior to normal vertebrobasilar angiography The transcranial Doppler sonographic criteria for stenosis were a peak systolic frequency shift greater than 2 KHz. A tight stenosis was identified by this pattern combined with direct and reverse low frequencies of high spectrum energy. The sensitivity of transcranial Doppler sonography using a peak systolic frequency shift in diagnosing stenoses reached 80% and its specificity was 97% if only atheromatous stenoses were considered. The main diagnostic failures concerned bilateral stenoses or contralateral occlusion, tandem lesions, and upper basilar artery stenosis. Transcranial Doppler sonography underestimated the degree of stenosis compared to angiography in 55% of the cases. We conclude that transcranial Doppler sonography is accurate in recognizing a stenosed vessel in the intracranial vertebrobasilar circulation, but if this finding will alter therapy, the examination must be complemented by magnetic resonance angiography.

Adult↗
PubMed1995

Colour Doppler and duplex sonography and angiography of the carotid artery bifurcations. Prospective, double-blind study.

We undertook a prospective double-blind study of 128 carotid artery bifurcations using colour Doppler and duplex sonography and angiography. Sixty-four patients with cerebrovascular events were admitted for angiography. All underwent sonography within 24 h of angiography. Standard duplex sonography and colour Doppler imaging without spectral analysis were performed, on the same device, by two sonographers, using defined morphological and haemodynamic criteria. Digital radiological data on vessel diameter were interpreted independently by two radiologists. The two sonographic methods gave similar grading of stenosis, compared to angiography, with an accuracy ranging from 96% in severe to 83% in minor stenoses. Colour Doppler studies gave better area measurements than standard duplex sonography, except for major stenoses. Discrepancies between ultrasonography and angiography were due mainly to minor stenoses and large plaques of calcification on the vessel walls, which masked very segmental 70% stenoses in 2 cases. Angiography is limited by its own resolution, does not show uncalcified vessel walls and does not give cross-sectional data. It would therefore be inappropriate for showing small plaques, the full extent of ectasia or for defining the carotid bulb accurately. The advantages of colour methods were in investigating sinuous or deep vessels and hypoechoic plaques. Analysis of the residual lumen of a stenosis and its extent could be determined more rapidly. Haemodynamic quantification of stenoses by standard duplex sonography may be difficult because of limited sample volume and error in estimation of angle, whereas colour Doppler allows semiquantitative estimation of haemodynamics.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Flow Velocity↗
PubMed1995

Central sympathetic dysregulation and immunological abnormalities in a case of progressive facial hemiatrophy (Parry-Romberg disease).

A case of hemifacial atrophy (Parry-Romberg disease) is discussed. Electrophysiological and immunological studies were performed. Electromyography, blink reflex and trigeminal evoked potential abnormalities indicate that the brain stem may be implicated in the aetiology of the disease. Immunological evidence favoured this possibility and demonstrated possible involvement of the noradrenergic system. Hyperactivity of the brain stem sympathetic centres, possibly caused by an autoimmune process, may be the primary cause of the cutaneous and subcutaneous atrophy in Parry-Romberg disease.

Adolescent↗
PubMed1995

Comparison of subcutaneous sumatriptan with usual acute treatments for migraine. French Sumatriptan Study Group.

246 migraine patients (International Headache Society definition, 1-6 severe attacks per month) were randomised into a multicentre, cross-over study comparing subcutaneous (s.c.) sumatriptan 6 mg administered by an auto-injector (Glaxo device) with usual acute migraine treatments. Patients were treated for 2 months or up to 12 attacks, and then crossed over to the alternative treatment for the same duration. Usual treatments were: analgesics (including combinations), 49%; ergotamine, 24%; NSAIDs 19%; DHE, 7%. Rescue medication was allowed 2 h after the first dose. Headache was assessed on a 4-point self-rating scale (0: none, 1: mild, 2: moderate, 3: severe). Other migraine symptoms were assessed as present or absent. Quality of life was assessed before the study and at the end of each treatment period. Two hundred and seventeen patients were eligible for the cross-over analysis. At 2 h post-dosing, an average of 78% of attacks per patient were successfully relieved (grade 3 or 2 to 1 or 0) by s.c. sumatriptan, compared with 34% for the usual treatments (p < 0.001) and 63% of attacks per patient were completely relieved (grade 0) by s.c. sumatriptan compared with 15% for the usual treatments (p < 0.001). Sumatriptan-treated patients used rescue medication for 19% of their attacks, compared to 59% for comparator drugs (p = 0.001). Results for patient preference were: s.c. sumatriptan, 85%; usual treatments, 10%; no preference, 5% (p < 0.001). Sumatriptan was significantly superior to comparator drugs for all other efficacy end-points (p < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Effect of subclavian syndrome on the basilar artery.

Fifty-five patients with a permanent or intermittent subclavian steal syndrome demonstrated by continuous wave Doppler were included in a prospective study: 25 patients without vertebro-basilar symptoms, 8 symptomatic patients with defined vertebro-basilar symptoms and 22 with hemodynamic vertebro-basilar occurrences. The basilar artery velocity was recorded by Transcranial Doppler Sonography in baseline conditions, and after a hyperaemia test to the upper limb. A spontaneous, incomplete basilar steal was diagnosed in seven patients, and a complete basilar steal in one patient, (14.5% of the cases). After hyperaemia test, 18 other incomplete basilar steal were observed. The occurrence of a basilar steal was higher in the vertebro-basilar group (57% of the cases) especially in 7 of the 8 cases with defined vertebro-basilar symptoms; it was lower in the patients without vertebro-basilar occurrences (36% of the cases). This basilar steal was also seen in five of the six symptomatic patients with opposite vertebral artery stenosis above a 50% diameter. Transcranial Doppler Sonography could help to define a subgroup of subclavian steal syndrome with a high risk of strokes.

Adult↗
PubMed1994

Hereditary neuralgic amyotrophy and hereditary neuropathy with liability to pressure palsies: two distinct clinical, electrophysiologic, and genetic entities.

Hereditary neuralgic amyotrophy (HNA) is an autosomal disease characterized by painful episodes of brachial palsy. The presence of tomacula in some patients suggested that HNA might be genetically related to hereditary neuropathy with liability to pressure palsies (HNPP), caused by point mutations in the PMP22 gene or deletion of the region containing this gene. In a clinical, electrophysiologic, and molecular study of two families with HNA, we show that the PMP22 gene is not deleted, duplicated, or mutated in HNA and that the disease is not linked to any other gene in the HNPP deleted region. We conclude that HNA and HNPP are distinct genetic entities.

Adolescent↗