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Biomedical subjects

C Vital

Publications and source records attributed to C Vital.

At least 181 records · Page 10Linked to original sources
PubMed1984

[Sex-linked familial form of progressive spinal amyotrophy in adults].

An X-linked spinal muscular atrophy is reported in one family. Four of the five patients were examined. In three, electromyography, conduction nerve velocities and muscle biopsy were consistent with anterior horn cell disease. Similar families in the literature were reviewed and characteristic data were: 1) adult-onset, 2) proximal, bulbar and facial involvement, 3) prominent perioral fasciculations, 4) frequent association of cramps, tremor and sexual dysfunction (hypogonadism and gynecomastia), 5) very slow progression and favorable prognosis. These characteristics define, among the spinal muscular atrophies, a distinct entity named by several authors Kennedy's disease.

Adult↗
PubMed1984

Occurrence of Renaut's bodies in a peripheral nerve.

We reviewed 1,012 biopsy specimens from the superficial peroneal nerve to assess the occurrence of Renaut's bodies. These structures were first described about 100 years ago, progressively forgotten, and only reexamined ten years ago. They appear as rounded, amorphous structures of the endoneurium and can be mistaken for small infarcts or amyloid deposits. In our biopsy samples, examined during a period of eight years, Renaut's bodies were encountered in 21 cases. Thus, their frequency in that nerve was estimated to be about 2%. The nerve fibers were normal in five cases and variously damaged in the remaining 16. No clinicopathologic correlation could be established. We concluded that Renaut's bodies are probably a normal structure of this peripheral nerve.

Fibroblasts↗
PubMed1983

[Primary non-Hodgkin's malignant lymphomas of the brain. Anatomoclinical and immunopathological study of 21 cases].

Twenty one cases of primary malignant lymphoma of the brain are studied. These tumors very seldom extend from the brain (one case in 21). There were no nodular forms and tumors of high-grade malignancy predominated (Kiel's classification). Immunopathological studies performed in eighteen cases showed immunoglobulin secretions in seven. Unlike other locations of non-Hodgkin lymphoma, prognosis depends on therapy and not on histological type. The outcome was improved by radiotherapy, sometimes combined with chemotherapy. Mean survival time was one month for untreated patients or patients treated only by surgery, and 32 months for patients treated by brain irradiation and chemotherapy.

Adult↗
PubMed1983

Uncompacted myelin lamellae in two cases of peripheral neuropathy.

Peripheral nerve biopsies from two patients with chronic sensorimotor neuropathy were studied. The first case was a non-Hodgkin malignant lymphoma and did not show any dysglobulinemia. The second case had a benign monoclonal gammopathy IgG, Lambda type. Direct immunofluorescence showed no deposits in the first case and slight deposits of anti IgG sera on a few myelinated fibers in the second case. There were numerous fibers showing uncompacted myelin lamellae, 7% in the first case and 4% in the second case. Some of these fibers had axons containing more tubules than filaments. The very few cases reported on neuropathies showing that uncompacted myelin lamellae were frequently associated with dysglobulinemic neuropathy. However, this ultrastructural abnormality of the myelin sheath can be observed without any dysglobulinemia.

Aged↗
PubMed1983

Ultrastructural study of peripheral nerve in arteritic diabetic patients.

The peripheral nerve was taken from 25 diabetic patients with arteritis just before or after amputation of a leg. The ultrastructural study of myelinated fibers showed several alterations. Loss of fibers varied greatly from case to case. This was accompanied in some cases by hypertrophy of the Schwann cells around demyelinated fibers as has been observed in some ordinary diabetic neuropathies. Unusual abnormalities consisted of particular axonal degeneration and evidence of aberrant remyelination. This axonal injury was peculiar because of the accumulation of organelles which tended to transform some of the fibers into enlarged axons. Such a lesion might correspond to a hypoxic axonopathy.

Aged↗
PubMed1983

Nuclear inclusions in oculopharyngeal dystrophy. An ultrastructural study of six cases.

Nuclear inclusions in striated muscle from patients with oculopharyngeal dystrophy have been detected recently. We carried out ultrastructural examinations of biopsy specimens on 5 patients with oculopharyngeal dystrophy and we also reexamined a former case. In these 6 cases we found filamentous inclusions in a few nuclei. These inclusions seem to be characteristic of this disease as they have never been seen elsewhere.

Aged↗
PubMed1983

Amyloid neuropathy and multiple myeloma. Ultrastructural and immunopathological study of two cases.

Report of 2 patients suffering from sensory peripheral neuropathy and multiple myeloma. In 1 case, peripheral neuropathy occurred several months before the appearance of multiple myeloma. A peripheral nerve biopsy exhibited numerous amyloid deposits within the endoneurium. Amyloid deposits have been estimated to occur in 15% of cases of multiple myeloma, but only 4 other detailed observations of amyloid deposits within the endoneurium have been reported in the literature. Other mechanisms might be involved since most peripheral neuropathies associated with multiple myeloma do not exhibit any amyloid deposits.

Amyloidosis↗
PubMed1983

[Allergic granulomatous angiitis (Churg-Strauss syndrome) manifested as polyneuritis. Its relation to panarteritis nodosa].

A 54 year old man with a history of resistant asthma presented with a multiple neuritis of subacute onset and hypereosinophilia. Histology showed necrotizing vasculitis with multinucleated giant cells compatible with Churg and Strauss' syndrome. Ultrastructural examination of nerves and immunopathological tests showed anti IgG serum fixing deposits in the arterial wall. Recovery was complete after corticoid therapy for 20 months. Literature on the Churg and Strauss' syndrome is reviewed and possible clinical, biologic, histopathologic, and physiopathologic relations between allergic granulomatous angiitis and panarteritis nodosa are discussed.

Granuloma, Giant Cell↗
PubMed1983

[Osteomalacia. Histologic definition and significance of the histodynamic analysis on undecalcified bone].

Bone histomorphometry has a major significance in metabolic bone diseases, and especially in osteomalacia. This technique needs a strict methodology with quantitative analysis on undecalcified bone and measure of calcification rate. Histologic definition of osteomalacia includes hyperosteoidosis, characterized by thickened osteoid edges, and decrease of calcification rate. Histodynamic analysis on undecalcified bone provides interest in diagnosis, prognosis and supervision of this affection.

Biopsy↗
PubMed1983

[Familial amyloid neuropathies in 3 families of French origin].

Clinical, electromyographic and neuropathological studies were carried out at different stages of evolution of a familial amyloid neuropathy in 6 members of 3 families of French origin. The clinical onset was marked by sensory symptoms and signs in limb extremities, primary manifestations being alterations in pain and thermal sensitivity. This was followed by motor and amyotrophic disorders predominant in the lower limbs. Autonomic nervous system disorders were frequent later. Early electromyographic signs were diminished amplitude and increased duration of sensory potentials. Progression of the disease is shown by the onset of signs resulting from severe axonomyelinic lesions. Neuromuscular biopsy demonstrated the presence of amyloid deposits in the endoneurium in 5 of the 6 cases. Transmission appeared to be dominant autosomal. These cases pertain to group I of the amyloid neuropathies. The axonal lesions marking the onset of the affection could be secondary to biochemical alterations in prealbumin, responsible for amyloid formation. This hypothesis affords a basis for plasmapheresis which has been used in 3 patients.

Adult↗
PubMed1983

[Recurrent cerebral hemorrhage and amyloid angiopathy].

A patient with no previous relevant history presented with recurrent cerebral hemorrhages of which he died 4 years 5 months after the onset. On autopsy, an amyloid angiopathy was present and localised to the cerebrovascular system. Hemorrhages were associated with some small infarcts in the subcortical regions. The leptomeninges and the cortical arteries were mostly involved by an infiltration mainly in the adventitia and media. There were no senile plaques in the cortex. The amyloid deposit present in the senile plaques and in the vessel walls of the amyloid angiopathy could be the result of two distinct pathological processes.

Amyloidosis↗
PubMed1982

Peripheral nerve damage during multiple myeloma and Waldenstrom's macroglobulinemia: an ultrastructural and immunopathologic study.

Peripheral nerve biopsies of 22 patients who were seen with a peripheral neuropathy were studied. On each occasion an ultrastructural study was performed and on 12 occasions an immunopathologic study was done. Ten patients had Waldenström's macroglobulinemia and 12 had multiple myeloma. Cellular infiltrates were observed in three cases of Waldenström's macroglobulinemia by light microscopy. A widening of the myelin sheath, corresponding to an accumulation of macroglobulin, was found in three cases of Waldenström's macroglobulinemia. The presence of abundant deposits of amyloid was noted in one case of multiple myeloma. These diverse features show the various possible mechanisms that can produce peripheral nerve damage during the course of malignant dysglobulinemias. The segmental demyelination is probably of immunologic origin. The axonal lesions cannot be explained satisfactorily except in those rare cases where amyloid deposits are present in the endoneurium.

Adult↗
PubMed1982

[The interest of CSF examinations in the etiological diagnosis of intracranial hypertension (author's transl)].

The authors discuss the case of a 15-year-old girl suffering from intra-cranial hypertension. Neuroradiological examinations do not reveal any obstruction in the cerebral spinal fluid circulation. The CSF cylologic examination was the first to give etiological indications, showing the existence of malignant cells which the cerebral biopsy later enabled us to classify as glioblastoma. This case affords an opportunity to recall the three stages of the diagnosis: first, intra-cranial hypertension; secondly, chronic meningitis; thirdly, cytological difficulties in the examination of CSF in patients having undergone neuro-radiological examinations.

Adolescent↗
PubMed1982

Inclusion body myositis. Clinical, biological and ultrastructural study.

A 48-year-old patient presented for the past 4 years an amyotrophy of the quadriceps and moderate involvement of the truncal and pelvic girdle muscles. The CK level was elevated (10 times the normal rate) and the EMG revealed a fibrillation pattern on relaxation, myotonic bursts on needle insertion and reduced activity during contraction. The histological study of the muscle biopsy showed nuclear cytoplasmic inclusion bodies and pseudo-myelinic membranes. The case was classified in the inclusion body myositis group. Analysis of the other published cases underlines the variety of the clinical, biological and electromyographical aspects and abnormalities.

Biopsy↗
PubMed1982

Abnormal proliferation of intraergastoplasmic microfilaments in myelinated schwann cells: ultrastructural study of two cases.

We report the ultrastructural findings in superficial peroneal nerve biopsies in two patients, one with idiopathic sensory neuropathy and the other with the Guillain-Barré syndrome and Hodgkin's disease. In addition to demyelination, there was an intense proliferation of microfilaments within numerous vesicles of the endoplasmic reticulum of a great number of myelinated Schwann cells. This abnormality does not appear to have been previously reported in the literature. The mechanisms responsible for this finding are unknown.

Aged↗