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Biomedical subjects

F Contamin

Publications and source records attributed to F Contamin.

At least 19 recordsLinked to original sources

[A case of acute sensory and autonomic neuropathy with regression].

The authors report the case of a 24 year old man with no previous disease who presented with a severe autonomic neuropathy. This included major gastrointestinal dysfunction characterised by decreased peristalsis without distension and paralysis of the gall bladder, and orthostatic hypotension with a normal cardiac tachycardia reflex. There was an associated sensory neuropathy affecting heat sensitivity without motor dysfunction and an increased CSF protein content. The proprioceptive nerve fibre conduction was decreased but another nerve conduction was normal initially. The mesenteric plexuses examined during sigmoidectomy performed for peritonitis due to multiple bowel perforations caused by fecoliths, showed no significant changes. Peripheral nerve biopsy revealed massive rarefaction of myelinated fibres which were of small diameter, and of the unmyelinated fibres, mainly due to axonal degeneration. Only two similar cases with incomplete recovery were found in the literature. In our case, a complete recovery was observed. The cause of the condition is unknown.

Acute Disease

[Cerebral cavernoma: a rare vascular malformation].

The authors have observed 7 cases of intracranial cavernous haemangioma. A review of the literature shows that the diagnosis is suggested by the occurrence of epileptic seizures, signs of expansive process or meningeal haemorrhage. Computerized tomography displays an area of hyperdensity enhanced by injections of a contrast medium. Surgical excision is mandatory. The post-operative mortality varies with the site of the malformation.

Adolescent

[Oculomotor forms of myasthenia].

The diagnostic difficulty in purely oculo-motor forms of myasthenia can be due to the lack of fluctuation in symptoms, the occurrence of complete spontaneous remissions, lasting several months or years, the absence of a decremental response in muscle potentials as evidenced by classical repetitive stimulation of the motor nerve, and negative responses to pharmacological testing. Thus, several investigative procedures have been developed to aid in the diagnosis of questionable cases. These include electromyography of proximal muscles and single fiber recordings, the dosage of acetylcholine receptors antibodies (specificity of nearly 100 %), and histological examination of neuro-muscular junction (invariably finding specific lesions of motor end plates). In spite of the diffuse pathological process and the possible overlapping with other types of myasthenia, many oculo-motor forms remain strictly localized throughout their course. Furthermore the prognosis of oculo-motor myasthenia is far better than other forms. Several hypotheses exist to explain the peculiarities of ocular myasthenia. Among these explanations are the theories that there are structural factors specific to oculo-motor muscles and that there exist a heterogeneity of acetylcholine receptor antibodies. On the therapeutic grounds, the frequent failure of anticholinesterase agents has been established. Corticosteroids are often very efficacious, but require high maintenance doses. The adverse side effects of such therapy have led some authors to suggest thymectomy as an alternative therapeutic modality.

Adult

[Chronic hepatic encephalopathies. Acquired cerebral degeneration not due to Wilson's disease].

We report on 4 cases of perennial hepatic encephalopathy and review similar published cases. The neurological picture consists of a cerebellar syndrome, both static and kinetic, dysarthria, choreo-athetoid abnormal movements and mental deterioration. Symptoms are permanent and usually worsen progressively. Some patients may present with a myelopathy, either isolated or combined with an encephalopathy. Relevant anatomical alterations, either encephalic or spinal, may be observed similarly in several varieties of liver disease, but in every case the role of portocaval shunts, whether spontaneous or surgically performed, appears essential. Altered results of laboratory studies, such as EEG or ammonemia, are described. Histological changes include a peculiar sort of hyperplasia of the protoplasmic astrocytes, along with a certain amount of neuronal loss. Surmised pathological mechanisms and applied therapy are briefly reviewed. For an appraisal of therapeutic results, perennial hepatic encephalopathies should be set apart from both the acute varieties and the usual chronic variety with its succession of recurrent exacerbations and remissions.

Budd-Chiari Syndrome

[Involvement of the peripheral and pyramidal nervous system in Crohn disease. Determining role of folic acid deficiency].

We report on the case of a 16-year-old male patient who presented with a peripheral neuropathy remarkable by the severity of pain, the proximal involvement and the association with an underlying myelopathy. All these symptoms coincided with an acute exacerbation of Crohn disease (regional enteritis) involving mainly the duodenojejunal segment, and were ascribed to a major folic acid deficiency, with total recovery following supplementation. In this connection we recall the various neurological symptoms induced by folic acid deficiency. We point out how difficult it is to prove the responsibility of such deficiencies in the production of neurologic diseases, mainly because of the possible intrication with other pathogenic factors: combined deficiencies or direct action of the factor responsible for the deficiency on the nervous system. Guidelines for solving these difficulties are suggested.

Adolescent

[Occlusive arteriopathy of the circle of Willis with outlined "Moya-Moya" network in a woman with migraine under oral contraception. Regressive course].

A 19-year-old woman with migraine under contraceptive therapy had transient right hemiparesis due to a minor lesion in the left hemisphere, which was probably hemorrhagic rather than ischemic. On the left side, angiography showed nearly complete obstruction of the terminal portion of the internal carotid artery, extending to the initial portion of its terminal branches, with an outlined "Moya-Moya" network. On the right side, moderate annular stenosis of the cervical portion of the internal carotid was visible. On subsequent angiographies, done 7 and 19 months later, blood flow was reestablished in the left internal carotid artery as well as in its terminal branches, but with persistent segmental stenosis. Since the stroke, the symptomatology of migrainous attacks has been altered, pointing to a left hemispheric spreading.

Adult

[Incomplete regressive obstruction of the internal carotid artery in a female patient with fibromuscular dysplasia].

The authors report on the case of a 48-year-old woman, with no history of cardiovascular disease, presenting with a progressive right cerebral deficiency syndrome predominating in the parietal region. X-ray, arteriography and CT scan findings led to the diagnosis of right middle cerebral artery ischemic stroke, in the proximal territory, due to a practically complete occlusion of the right internal carotid artery. The patient recovered and a right carotid arteriography performed 8 months after the initial one showed repermeation of the carotid artery, as well as evidence of fibromuscular dysplasia (FMD). The authors then reviewed the literature dealing with cervico-cephalic FMD and concluded that FMD only exceptionally leads to arterial occlusion, whether by arterial dissection, intimal hyperplasia or thrombosis. This case is remarkable by its favorable outcome: the thrombus, which was unquestionably responsible for the clinical picture, dissolved spontaneously.

Arterial Occlusive Diseases

[Association of ankylosing spondylarthritis, cauda equina syndrome and dilatation of the lumbar cul-de-sac with voluminous arachnoid diverticulum. Clinical, radiological and tomodensitometric study of a case].

The authors report a clinical and radiological observation of cauda equina syndrome associated with ankylosing spondylitis. Although only 32 cases have been reported in the literature over the last twenty years, such an association is probably not exceptional. The mode of onset and the course of the neurological symptoms is quite similar from case to case: ankylosing spondylitis preexisting for many years, and inactive when neurological signs develop, the latter usually being very slowly progressive. The usual radiological features, demonstrated by saccoradiculography in the supine position, are posterior diverticulas of the lumbar theca, contiguous to bony erosions of the laminae, well visualized by computed tomography. Pathogenesis remains obscure, and none of the attempted therapies, including surgical procedures, have proved effective.

Aged

[Polyneuropathy complicating Horton's disease (author's transl)].

A patient diagnosed as having Horton's disease presented a complex neurological picture dominated by sensory-motor neuropathies of all four limbs, one year after the appearance of signs of temporal arteritis. No other etiological factor, apart from the Horton's disease, was discovered, and the causal relationship between this disease and the neuropathy is discussed. The possibility of dysimmunity factors being involved in Horton's disease is raised and the resulting therapeutic implications discussed.

Adrenal Cortex Hormones

[Unilateral dysplasia of the circle of Willis with multiple distal stenoses (author's transl)].

Neurological and clinical examinations were found to be normal in a woman aged 40 years who had had a single generalized epileptic seizure. Rediological examinations demonstrated a spiral-shaped calcification above and laterally to the left sella turcica. This corresponded to changes in the C 1 segment of the left carotid sinus, which was partly stenosed distally, with left unilateral abnormalities of the circle of Willis and multiple distal stenoses of the left sylvian and vertebro-basilar arteries. Scanning demonstrated that these had been present for a long time, but it is not possible to establish the diagnosis on an etiological basis as no similar radiological findings have been published.

Adult

[Circulatory disorders induced by amantidine].

Amantadine has been used since 1969 in the treatment of Parkinson's disease. In 1970, were described the special symptoms noted in the lower limbs due to this drug. The authors, after a review of the various disturbances, have studied 10 cases by Capillaroscopy. They emphasize the interest of the study of this abnormality of the micro-circulation, producing vaso-constriction of the arterioles and venules.

Aged