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Biomedical subjects

J R Fève

Publications and source records attributed to J R Fève.

At least 19 recordsLinked to original sources
PubMed2003

Iatrogenic Creutzfeldt-Jakob disease subsequent to dural graft: persisting risk after 1987.

The first case of Creutzfeldt-Jakob disease (CJD) related to the use of a dura mater graft of cadaveric origin was identified in 1987 and this procedure is now considered as one of the main causes of iatrogenic CJD. Although the decontamination procedure for the preparation of graft material was modified, the product was withdrawn from the market in many countries a few years later and replaced by synthetic material. In this context, two patients treated in our institution developed CJD following a cadaveric dural graft performed after cerebral and lumbar trauma. Their clinical presentation, showing predominant cerebellar symptoms, late deterioration and myoclonic jerks, and a rapid disease course until death, was similar to that of previously reported cases involving the iatrogenic form. As the graft for one of the patients was performed in 1991 (several years after modification of the decontamination procedure), this fourth reported case suggests that the risk of iatrogenic CJD may have persisted in some patients treated after 1987, when grafts of cadaveric origin were totally abandoned.

Adult↗
PubMed2002

Decrease in heart ventricular ejection fraction during multiple sclerosis.

Recent studies have shown that mitoxantrone is effective in patients with active multiple sclerosis (MS) and that cardiac monitoring is usually required. However, right and left ventricular ejection fractions (VEFs) have never been studied in MS patients as compared with control subjects. Radionuclide angiocardiography (RA) was performed to assess right and left VEFs at rest in 40 consecutive patients with active definite MS [15 men and 25 women; mean age 33.9 +/- 10 years; mean disease duration 8 +/- 6.5 years; 18 had relapsing-remitting and 22 had secondary progressive forms of the disease; mean Expanded Disability Status Scale (EDSS) score 4.8 +/- 1.9]. The control group consisted of 40 subjects free of neurological or cardiovascular disease (17 men and 23 women; 44.6 +/- 13.4 years of age). The VEF values obtained in the control group defined the normal limits (right VEF 32-54%; left VEF 50-74%). A statistically significant decrease of right (P=0.02) and left (P < 0.0001) VEFs was found in MS patients as compared with control subjects. RA showed pathological results for right (7.5%), left (10%) and both (7.5%) VEFs in 25% of MS patients. No correlation was found between VEF and sex, age, disease duration, disease course, EDSS score or previous treatment. Autonomic impairment, which frequently occurs in MS patients, may have accounted for the decrease in VEFs. Further physiological studies are required to determine factor responsible for the decrease of VEFs in MS.

Adolescent↗
PubMed2002

[Ptosis and mastication disorders revealing concurrent myasthenia gravis and chronic polyradiculoneuritis].

Although myasthenia gravis (MG) has frequently been associated with other autoimmune disorders, it has only rarely been reported in conjunction with diseases of the nervous system. A 74-year-old patient with hypertension suddenly presented left unilateral ptosis and mastication disorders. Clinical examination showed a concomitant loss of strength distally and reduced deep tendon reflex. Electrophysiologic data indicated a diagnosis of MG and chronic inflammatory demyelinating polyneuropathy; acetylcholine receptor antibody was elevated at 4.1 nmol/L (normal < 2 nmol/L). Improvement was rapid after initiation of pyridostigmine in association with corticosteroid (1 mg/kg/day). One month later, the cranial nerve deficit disappeared and strength was normal. It is likely that a basic abnormality of immune regulation was responsible for the emergence of diseases with different clinical presentations, but similar immunopathogenesis. Corticosteroid seemed to be the most effective treatment.

Adrenal Cortex Hormones↗
PubMed2001

[Recurrent syncope and Chiari malformation].

Downward herniation of the cerebellar tonsils through the foramen magnum (Chiari I malformation) is usually revealed by head and neck pain, often associated with brain-stem or spinal cord disturbances. Syncopes are rarely reported and may be difficult to link to their cause when they occur alone. We report two cases with brief and repetitive syncopes revealing a Chiari I malformation. These manifestations may be attributed to transient compression of brain stem or vascular structures at the craniocervical junction, triggered by intracranial pressure increase, as they disappear after posterior fossa decompression.

Budd-Chiari Syndrome↗
PubMed2000

[Late cerebrovascular complications of cardiac transplantation].

The occurrence of stroke after the post-operative period of cardiac transplantation is a rare event, and the role of the cardiac transplant in these patients, who often have various vascular risk factors, is unclear. We reviewed the clinical records of 303 consecutive patients with orthotopic cardiac transplantation performed from March 1985 to December 1996 and selected those who developed a stroke over the first 2 months postoperatively, in order to evaluate the frequency and the mechanisms of late cerebrovascular complications. Four patients had presented cerebral infarct and two intracranial hemorrhage. The overall risk of late cerebrovascular complications was 2.6p.100 at 5 years. Ischemic stroke was related to cardiac embolus in one patient, lacunar infarction in another, and was of undetermined etiology in two cases. In addition to vascular risk factors prior to transplantation and the development of intracardiac thrombus, immunosuppressive therapy, the surgical procedure and cardiac rejection may play a role in the occurrence of stroke in such patients.

Aged↗
PubMed2000

[Progressive multifocal leukoencephalopathy and pulmonary sarcoidosis].

An association of progressive multifocal leukoencephalopathy (PML) and sarcoidosis in a 47 year-old-woman is reported. This is the third case in which PML has been diagnosed by PCR. Clinical, biological and radiological features were in agreement with previous findings in immunologically suppressed patients. JC virus should be systematically detected by PCR in blood and cerebrospinal fluid (CSF) in patients with sarcoidosis presenting neurological and radiological PML manifestations.

Anti-Inflammatory Agents↗
PubMed1995

[Anarthria, progressive apraxia and extrapyramidal syndrome: an uncommon clinical form of corticobasal degeneration? A case studied by HMPAO Tc99m single-photon emission tomography].

A 48-year-old man presented with impaired joint movement and buccofacial apraxia. The disease progressed for six years associating an akineto-hypertonic syndrome, marked anarthria, saliva incontinence, bi-opercular syndrome, bucco-facial apraxia, severe global gestual apraxia and a frontal syndrom. Oculo-motricity and gait were normal. Magnetic resonance imaging of the brain demonstrated fronto-parietal atrophy and HMPAO Tc99 tomography revealed hypoperfusion of the cortex clearly predominating in the left parietal region. These particular findings with predominantly intense joint involvement is similar to the clinical picture in corticobasal degeneration--subcortical signs (progressively uncontrollable hypertonia) together with cortical signs (severe gestual apraxia). The neuroradiological imaging and functional results also suggest a degenerative process.

Apraxias↗
PubMed1995

[Frontal type dementia and amyotrophic lateral sclerosis. 3 cases with (HmPAO Tc99m) single-photon emission tomography study].

Three cases of rapid onset neuropsychological frontal dementia preceded the development of sporadic amyotrophic lateral sclerosis by 12 to 24 months. HmPAO Tc99m scintigraphy demonstrated hypoactivity in the cortex, predominantly in the frontal region. Three hypotheses are discussed: 1) coincidence between two degenerative diseases, Alzheimer's disease or Pick's disease and ALS; 2) an amyotrophic form of Creutzfeld Jakob disease; 3) pre-senile dementia associated with a motoneuron disease, a clinical pathology entity recently described by Mitzuyama.

Aged↗
PubMed1994

[Molecular genetic study of a family with Kennedy syndrome including a symptomatic heterozygote].

Four men and one woman of the same family with Kennedy-type-bulbo-spinal amyotrophy have been followed up for 7 to 20 years. The genetic marker: insertion of repeated sequences of trinucleotide Cytosine-Adénine-Guanine described by Fischbeck and La Spada in Nature (1991), in the coding region of the androgen receptor gene, on the long arm of X chromosome, has been demonstrated here by DNA extraction and PCR amplification.

Adult↗
PubMed1994

[Leptomeningeal gliomatosis. 2 clinicopathological cases].

Leptomeningeal gliomatosis is a diffuse glial infiltration of the subarachnoid space. It is primary and very rare when primary astrocytoma arises in the leptomeninges from heterotopic neuroglial tissue; it is secondary and more frequently reported when associated with a medullar or cerebral intraparenchymal astrocytoma and secondary involvement of the leptomeninges. Primary and secondary forms are difficult to differentiate before neuropathological examination. The authors report 2 anatomo-clinical cases of leptomeningeal gliomatosis in adults, with clinical courses of 6 months and 40 days respectively. The initial clinical picture was aseptic chronic or subacute meningitis. Cytologic examinations of the cerebrospinal fluid (CSF) showed moderate lymphocytosis, with elevated protein and low glucose levels, without abnormal cells. On case 2 CT scan and in case 1 spinal MRI isolated diffuse meningeal contrast enhancement was present, without intraparenchymal lesion. The neuropathological study revealed a diffuse astrocytoma glial leptomeningeal tumour with a focal involvement of the central nervous system (spinal cord in one case, temporal lobe in the other). In conclusion, an isolated aseptic lymphocytosis meningitis with meningeal abnormal signal may reveal leptomeningeal gliomatosis. Neuropathological examination can distinguish primary from secondary forms.

Arachnoid↗
PubMed1993

Electrophysiological study of a case of diffuse loss of sweating with segmental hyperhidrosis associated with areflexia.

Electrophysiological investigations were carried out on a 43 year-old man with segmental hyperhidrosis in three radicular territories, generalized anhidrosis elsewhere and areflexia. A recurrent labial herpetic rash and a history of sweating disorders in his grandfather were the only associated clinical data. Sympathetic skin response was found to be absent in anhidrotic territories, including the hands and feet, although it was present in hyperhidrotic territories. Peripheral adrenergic sympathetic fibers evaluated by photoplethysmography were normally responsive, as were visceral vegetative regulations involved in cardiovascular control during postural changes or exercise. The present case is compared to the previously described Ross Syndrome, associating segmental hypohidrosis, areflexia and tonic pupils. Our results and observations in the literature are consistent with the occurrence of lesions affecting postganglionic cholinergic fibres of the sympathetic system.

Adult↗
PubMed1993

Pentoxifylline inhibits experimental allergic encephalomyelitis.

Pentoxifylline, a widely used methylxanthine, has been proven to inhibit the production and action of the cytokine TNF alpha. Since it has been suggested that TNF alpha is the major cytokine involved in the pathogenesis of multiple sclerosis, we tested pentoxifylline for its capacity to prevent experimental allergic encephalomyelitis (EAE). 26 Lewis rats with acute EAE were treated with either pentoxifylline or saline. The pentoxifylline treated rats showed a significantly lower incidence of clinical signs as well as significantly lower histological inflammation. The exact mechanism of this preventive effect remains to be clarified but it might be mainly related to inhibition of TNF alpha release from central nervous system macrophages.

Animals↗
PubMed1989

[Ischemia of the anterior horn of the spinal cord].

Ischemia of the motoneurons in the anterior horn is a well known pathological entity. Their clinical signs and symptoms are similar to those of amyotrophic lateral sclerosis. Evidence by selective angiography of angiomas of the spinal cord or compression or deviation of Adamkiewicz artery may be suggestive of an initial vascular lesion. Various data (knowledge of development or lesions during experimental ischemia, selective electrophysiologic analysis of anterior horn neurons, evidence of precise circumstances of spinal vascular disorder or spinal arteriography) suggest that anterior horn ischemia is a multiple aspect phenomenon. Our 4 cases illustrate this hypothesis and demonstrate under confirmed vascular circumstances the different clinical aspects of anterior horn ischemic lesions. In addition to typical amyotrophic paralysis unusual or misleading symptoms may occur such as claudication, paroxysmal contractures or progressive spastic paraparesis. Investigations required and possible treatment of the lesions are simplified by awareness of these various clinical aspects.

Adolescent↗
PubMed1982

[Rigid-spine syndrome in a female patient (author's transl)].

A case of rigid spine syndrome in a woman is reported. There were a diffuse myopathic process, with atrophy and mild weakness not involving the face and a major rigidity of the spine. Contractures were present as well as a pure restrictive respiratory failure. Heart-rythm disorders and prolapse of the mitral valve were present. Histological features of a deltoid muscle biopsy were slight necrosis, lack of fibrosis and major disproportion in fiber-types. There were a high rate of fiber I and absence of fiber IIB. This case was similar to others described as Dubowitz's rigid spine syndrome. The histological features belonged to the second neuropathological group of cases, with disproportion in fiber-types. The rigid spine syndrome may be considered as a clinically definite disease and distinguished from other myopathies with orthopedic deformations. It should not be confused with arthrogryposis multiplex. The disease is probably autosomic recessive.

Adult↗
PubMed1977

[Anatomy and physiology of the muscles and median frenum of the upper lip. Initial results of selective electromyography].

Usually when reference is made to the Orbicularis oris muscle of the upper lip only the horizontal bands of muscle are mentioned. This muscle also comprises oblique fibres (Orbicularis externus) that are of very great importance in lip movements. The balance of the lip derives from the activity of the two types of muscle fibers. The lip can thus perform, at the same time, a movement of compression of the underlying structures and projection movements whereby these structures are freed. These different modes of action are well demonstrated by selective electromyography. There is also, in the middle of the upper lip, a medial cellulo-fibrous septum forming a prolongation of the septum and penetrating into the median interincisive suture and ending at the frenulum of the upper lip. This medial septum seems to play a very important physiological role in the growth of the premaxilla.

Electromyography↗