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Biomedical subjects

C Vital

Publications and source records attributed to C Vital.

At least 199 records · Page 11Linked to original sources

[Familial malignant carotid body chemodectoma with lymph node metastases. Light and electron microscopy study (author's transl)].

A case of malignant carotid body chemodectoma with neighbouring lymph nodes metastases is reported. The case is noteworthy as it was accompanied by another jugulotympanic chemodectoma. There was also hereditary component as four of the patients forebears had been operated for cervical chemodectomas. An ultrastructural study showed the characteristic secretory granules. 32 other cases of tumors of carotid body and jugular glomus with metastases are reviewed.

Adolescent↗

Ultrastructural study of peripheral nerve in Guillain-Barré syndrome presence of mononuclear cells in axons.

As has been shown during the course of experimental allergic neuritis, in the Guillain-Barré syndrome there is an invasion of the myelin sheath in certain fibres by a mononuclear cell, with an intact axon. In a series of 45 cases we have been able to show this feature in 31 cases. In certain cases the cytoplasm prolongation of the invading cell was seen in the myelin sheath, forcing apart two neighbouring layers. In five cases we have furthermore observed the presence of mononuclear cells located either between the myelin and the axon which is deformed, or on the inside of the axon. Such an anomaly was frequent in one recent case and was occasionally found in four other cases.

Axons↗

Adrenomyeloneuropathy: demonstration of inclusions at the level of the peripheral nerve.

A patients is reported with a progressive neurological disease which started at the age of 19 years and included spasmodic paraplegia, urinary disorders, impotence and neuropathic signs. The neurological syndrome was associated with adrenal failure. The disease led to death within 5 years. In this characteristic picture of adrenomyeloneuropathy, biopsy of peripheral nerve disclosed lamellar inclusions in the Schwann cell cytoplasm. Such structures, which have already been observed in the sural nerve of some adrenoleukodystrophy cases reported previously, are described for the first time in a case of adrenomyeloneuropathy.

Adrenal Insufficiency↗

[Aphasic-type language disorders associated with lesions of the putamen and caudate nucleus: clinicopathological findings in one case (author's transl)].

Language disorders developed in a patient following a subcortical infarct involving the left putamen and caudate nucleus. Language was abundant and fluent, with many perseverations, semantic and ideation incoherences, and all activities concerned with verbal or non-verbal expression were affected. Lesions were present in the head and body of the caudate nucleus, the whole of the putamen, the anterior nucleus of the thalamus and the superior part of the internal capsule. The disorder was unique in relation to disturbances of the aphasic type observed in extensive thalamic lesions, or in transcortical sensorial aphasia. Physiopathological interpretation is based on the functional role of the putamen-caudate system in the regulation of many types of behaviour: animal experiments and results of applying stimuli in humans suggests a release of the inhibition exerted by the caudate nucleus on the frontal cortex. Lack of comparable clinicopathological data implies that other similar cases must be reported before the specificity of the disorder can be confirmed.

Aphasia↗

[Digestive manifestations of dermatomyositis (author's transl)].

The principal digestive anomalies detected by radiological examination in dermatomyositis are the motor and functional consequences of the smooth and striated muscle lesions in these tissues. Vascular changes cause ulcerations, located in any part of the digestive tract, but more frequently in the esophagus, duodenum, and small intestine. These ulcerations sometimes develop into pseudodiverticulae.

Aged↗

Guillain-Barré syndrome and Hodgkin's disease--ultrastructural study of a peripheral nerve.

A 46-year-old male patient developed the Guillain-Barre syndrome and recovered completely within 3 months. He had been treated 5 years previously for Hodgkin's disease, and during the neurological syndrome, relapse of the malignant lymphoma was discovered. On neuro-muscular biopsy, lymphocytes were observed penetrating Schwann cells. These neuropathological aspects confirm the auto-immune character of the nervous involvement.

Hodgkin Disease↗

[Multiple affections of the cranial nerves as a pathognomonic sign of periarteritis nodosa. Relationship with Cogna's syndrome (author's transl)].

A 43-year-old woman was found to have multiple cranial neuritis affections, present as an isolated disorder over a long period, and characterized by bilateral deafness and associated lesions of the VII and V cranial nerves. On investigation, visual disturbances were discovered which were of a transitory nature and made up a total clinical picture suggesting Cogan's syndrome. Neuropathological examination revealed the presence of typical periarteritis nodosa lesions in the cranial nerves. The authors suggest, therefore, that certain cases described as Cogan's syndrome are in fact particular forms of periarteritis nodosa.

Adult↗

[Adrenomyeloneuropathies].

An adrenomyeloneuropathy is observed in a patient died at 24 years old after an illness of five years duration. These case is peculiar by the presence of lamellar cytoplasmic inclusions in the Schwann cells. Adrenomyeloneuropathy is an adult variant of adrenoleucodystrophy. This sex linked recessive disorder is related to an excessive amount of long chain fatty acids.

Adrenal Insufficiency↗

[Ultrastructural study in the diagnosis of various neurolipidoses].

We have studied the sphingolipidosis, principally, ceroid-lipofuscinosis and other complex lipid storage diseases. We examined biopsy tissue of fourteen patients with diseases of the various types given above. The biopsy tissue was usually skin, muscle or peripheral nerve, and occassionally brain or various visceral tissue.

Biopsy↗

[Interest of ultrastructural study of skin and muscle biopsies in inborn storage diseases. A report of 18 cases (author's transl)].

Skin and muscle biopsies were performed in 18 patients affected by various inborn storage disorders: mucopolysaccharidosis (MPS), sphingolipidosis, GM1 gangliosidosis, I-cell disease, ceroid-lipofuscinosis (CLF), adrenoleucodystrophy (ALD) and glycogenosis. In most cases, cutaneous and muscle biopsies demonstrate clearly the presence of storage inclusions in different cell types with great reproducibility and sometimes a specificity for a particular disease. Thus, electron microscopic examinations of skin and muscle, often complementary, can given valuable informations at two levels: - either a morphological confirmation depending on the type of the storage disease: e. g. clear, granular and fuzzy inclusions identified with mucopolysaccharidic material, lamellar and pseudomyelinic figures corresponding to lipidic structures are found in different cell types in MPS, gangliosidosis and sphingolipidosis; - or a diagnostic proof of some lysosomal diseases where there are very specific ultrastructural features such as the curvilinear bodies and fingerprint profiles in CLF or the spicular inclusions in ALD. These informations are the more important as the enzymatic defect is unknown in these inherited disorders. We conclude that the ultrastructural examination of a skin and muscle biopsy are an important tool for the diagnosis of a lysosomal storage disease which avoids any more difficult biopsies as brain, kidney, liver.

Adolescent↗

Epilepsy and agitated delirium caused by an astrocytoma of the amygdala.

The clinical picture of a tumour involving the amygdaloid nucleus was characterized over a period of 6 years by generalised epileptic fits, then a state of agitated delirium with oneirism evolving during 2 months and leading finally to pulmonary embolism. The association of epilepsy and psychiatric disorders is the clinical picture usually observed in tumors of the limbic system but these symptoms are various and unspecific. The conventional neuroradiological procedures often fail to discover the lesion.

Amygdala↗