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Biomedical subjects

C Vital

Publications and source records attributed to C Vital.

At least 127 records · Page 7Linked to original sources
PubMed1990

[Polyneuropathy in type I multiple endocrine syndrome].

Since 1928, approximately 30 cases of peripheral polyneuropathy consecutive to hypoglycaemia caused by insulinomas or islet cell pancreatic tumours have been published. We report a case of polyneuropathy in a young woman presenting with type I multiple endocrine neoplasia syndrome including multiple insulinomas and parathyroid adenomas. The predominantly motor polyneuropathy was symmetrical and involved both the upper and lower limbs. Biopsy showed muscular atrophy and, in particular non-specific acute axonal lesions. Twelve months after surgery the clinical situation was relatively favourable with resumption of physical activities but persistence of muscular atrophy. Wether hyperparathyroidism was responsible for the neuropathy was uncertain, the subacute post-hypoglycaemia axonal damage being a more likely suspect.

Adenoma↗
PubMed1990

[Non-Hodgkin's lymphoma associated with human immunodeficiency virus infection. Bordeaux experience with 26 cases].

We describe the clinical and histologic features of non-Hodgkin's lymphoma in 26 patients with human immunodeficiency virus 1 (HIV) infection. These represent 10 per cent of AIDS cases recorded in the Bordeaux area. Mean age was 42. Contamination was mostly related to homosexuality (50 per cent) and blood transfusion (27 per cent) with 5 female cases. The initial presentation of lymphoma was extranodal (69 per cent). Lymphoma spread was diffuse (65 per cent), involving the bone marrow (38 per cent), lymph nodes (35 per cent), central nervous system (27 per cent), oral and digestive mucosae (23 per cent), liver (19 per cent) and genital tract (12 per cent). Histologic types were of intermediate or high grade malignancy (88 per cent) with 38 per cent large, non cleaved-cell (centroblastic) subtype. Median survival was 4 months. Lymphoma caused death in 65 per cent of patients and opportunistic infection in 18 per cent. Lymphoma was the first manifestation of HIV infection in 10 patients (38 per cent) and was responsible for AIDS in 14 (54 per cent). Diagnosis of lymphoma could be established at an early stage on extranodal biopsy. In these patients a prolonged disease-free survival was obtained after chemotherapy alone or associated with radiotherapy.

Acquired Immunodeficiency Syndrome↗
PubMed1990

Peripheral neuropathies and lymphoma without monoclonal gammopathy: a new classification.

Recent progress in immunopathological studies of peripheral nerve and lymph node fragments together with 16 personal cases and numerous clinicopathological reports have suggested a new classification of peripheral neuropathies (PN) and lymphomas. These are: (1) PN due to local infiltrations by a T-cell lymphoma: (2) acute polyradiculoneuritis due to active demyelination and associated with infiltrates of a T-cell lymphoma in the epineurium, resembling Marek's disease (which is a T-cell lymphoma); (3) B-cell lymphoma proliferation which may be restricted to or predominate in the peripheral nervous system, with a large clinicopathological heterogeneity ranging from localized forms to ascending polyradiculoneuropathies; (4) angiotropic lymphoma, which is a B-cell lymphoma and may present as an acute mononeuropathy; (5) patients with acquired immunodeficiency syndrome due to lymphomatous infiltrates in the endoneurium, of which 2 cases of PN have been reported; (6) PN associated with organomegaly, endocrinopathy, M-component and skin lesions, certain cases being associated with a plasmocytoma and sometimes Castleman's disease but without any monoclonal gammopathy: (7) classic Guillain-Barré syndrome, prone to develop in patients with extraneural lymphoma but without any lymphomatous infiltrates in the peripheral nervous system; (8) certain cases (4 out of 16 in our series) where there is no clear relationship between PN and lymphoma, and there are mainly features of axonal degeneration. Inflammatory perivascular infiltrates were sometimes present in the epineurium.

Aged↗
PubMed1990

Chronic inflammatory demyelinating polyneuropathy in childhood: ultrastructural features of peripheral nerve biopsies in four cases.

Peripheral nerve biopsies (PNB) from four children suffering from subacute or chronic inflammatory demyelinating polyneuropathy were studied by electron microscopy. Remyelinating features with onion bulb formations, inflammatory cell infiltrates and active demyelinating lesions were strongly suggestive of the disease. In the first case, a second PNB, performed after 7 months of severe subacute course, showed a striking evolution of the lesions. In the second case and in spite of severe neurological symptoms, the PNB was almost normal, suggesting that inflammatory lesions were mainly located in the proximal parts of the nerve. No signs of active demyelination could be seen in the third case but onion bulb formations and inflammatory cell infiltrates were present. In the fourth case, characteristic lesions of active demyelination were associated with a history of familial polyneuropathy; this association suggests an auto-immune process in certain kindreds with hereditary motor and sensory neuropathy.

Adolescent↗
PubMed1990

A mitochondrial encephalomyopathy with specific deficiencies of two respiratory chain polypeptides and a circulating autoantibody to a mitochondrial matrix protein.

A 15-yr-old boy with mitochondrial encephalomyopathy and NADH CoQ reductase (Complex I) deficiency is presented. Immunoblotting demonstrated specific deficiencies of the 24 kDa FeS protein of Complex I and subunit II of Complex IV. The patient's serum contained an antibody to a specific mitochondrial matrix polypeptide of apparent Mr 41 kDa. The specific polypeptide deficiencies involve products of nuclear (24 kDa FeS protein) and mitochondrial (subunit II) genes and suggest some intergenomic regulation. The relevance of the circulating antibody to the pathogenesis of the patient's Complex I deficiency is discussed.

Adolescent↗
PubMed1990

Presence of inclusion body myositis-like filaments in oculopharyngeal muscular dystrophy. Ultrastructural study of 10 cases.

Ten cases of oculopharyngeal muscular dystrophy (OPMD) were examined ultrastructurally. Two very different types of filamentous inclusions were observed: (1) Nuclear inclusions composed of 8.5 nm external diameter tubular filaments organized in palisades and similar to those described by Tomé and Fardeau (1980). They have not yet been reported in other muscle diseases. Their presence in 100% of our cases confirms they are the morphological hallmark of OPMD. (2) 16-18 nm external diameter tubular filaments. These were similar to the inclusions observed in inclusion body myositis (IBM) and morphologically very different from the first type. They were randomly dispersed or arranged in bundles near cytoplasmic debris and whorls of membranes. They were found in cytoplasm. Only once were they observed in a nucleus. These inclusions have been described in IBM but also in other diseases. They were found in 80% of our OPMD cases.

Aged↗
PubMed1990

[Subacute familial thalamic atrophy. Memory disorders and complete insomnia].

Thalamic atrophy is rarely primitive. Selective atrophy of the dorso-medial and anterior thalamic nuclei has been reported in a few families. The clinical course is subacute. Symptoms and signs include sleep disorders and intellectual deficit. We report a new family with this uncommon disease and discuss its etiology.

Atrophy↗
PubMed1990

[Malignant primary non-Hodgkin's lymphoma of the central nervous system. Anatomo-clinical analysis of 35 cases].

We have recorded 35 primary central nervous system (C.N.S.) lymphomas from 1982 to 1988 in Bordeaux in order to show their increasing frequency, and clinicopathological features. Mean age was 57 (15-81). Only one patient was immunocompromised. Clinical symptoms consisted mostly of focal neurologic deficits (23 cases) and mental disturbance (16 cases). Diagnosis was made on bioptic material (20 cases mostly stereotaxic), surgery (13 cases) or autopsy (2 cases). Lymphoma was localised in the brain with unifocal (50%), multifocal (30%) or diffuse (20%) involvement at computed tomographic scans. Histopathological features were diffuse infiltration of the cerebral tissue with perivascular clustering. Two particular cases were observed: one with mostly a perivascular pattern affecting the white matter of both cerebral hemisphere; another with amyloidosis deposits. According to Working Formulation, histologic types were of intermediate or high grade of malignancy (66%), with 35% large, non cleaved-cell (centroblastic) subtype. Immunochemistry on paraffin sections with UCHL-1, MB-2, LN-1, L-26 showed that 94% were of B-cell nature and only one probably of T-cell origin.

Adolescent↗
PubMed1990

[Choreo-acanthocytosis].

We report a case of choreo-acanthocytosis in a 33 year-old man with mild chorea of the limbs but no involuntary movements of the face, areflexia, epilepsy and personality changes. Acanthocytosis was 10-20% and creatine-phosphokinase levels were raised. Electrophysiological data were consistent with lower motor neurone dysfunction. Muscle biopsy revealed changes typical of neurogenic atrophy. Nerve biopsy showed a severe loss of large myelinated axons. Electron microscopic examination was in favour of primary axonal damage affecting mainly myelinated fibers and only slightly unmyelinated fibers.

Acanthocytes↗
PubMed1990

[Dysautonomia. A clinical study of a case, ultrastructural data].

We report a new case of non-familial dysautonomia. The patient was a boy with no known Jewish ancestry in whom psychomotor retardation in early infancy failed to lead to specialized evaluation. Regressive episodes of ataxia developed at the age of three and infrequent generalized seizures occurred between four and seven. Diagnosis was first considered at the age of seven after neuroparalytic keratitis developed and rapidly became bilateral. This case has remained highly unusual throughout the course since none of the commonly reported complications (swallowing disorders, aspiration pneumonia, and dysautonomic "attacks") has occurred. No life-threatening manifestations have developed. Peripheral nerve biopsy specimens showed that myelinated fibers, especially of large diameters, were abnormally scarce, and that the histogram failed to exhibit the normal bimodal aspect. Ultrastructural studies also disclosed a marked reduction in the caliber of unmyelinated fibers, whose axons were flattened or occasionally missing. No evidence of regeneration was found. The atypical clinical features in our patient are discussed.

Ataxia↗
PubMed1990

[Muscle and brain biopsy in a case of mitochondrial encephalomyopathy. Demonstration of a mitochondrial vasculopathy].

The authors report the case of a 14-year-old boy who presented with shunted growth and low weight gain, asthenia with fatiguability on effort, clinical signs of myopathy, ataxia, epilepsy, deafness, loss of visual acuity and reduced intellectual capacities. Muscle biopsy revealed numerous ragged-red fibres and mitochondrial anomalies at ultrastructural examination. Cerebral biopsy showed spongiform changes. Both muscular and cerebral biopsies showed vascular lesions: swollen endothelial cells. Mitochondria increased in number with concentric whorls of cristae were observed in pericytes and smooth muscle cells. This mitochondrial vasculopathy could explain the neurological symptoms.

Adolescent↗
PubMed1989

Polyneuropathy associated with IgM monoclonal gammopathy. Immunological and pathological study in 31 patients.

Quantitative, immunopathological, light and electron microscopic studies of superficial peroneal nerve biopsies from 31 patients with IgM monoclonal gammopathy were carried out. Six patients had Waldenström's macroglobulinemia and 25 had IgM monoclonal gammopathy of undetermined significance. Serum samples from 28 of these patients were assayed for anti-myelin-associated glycoprotein (anti-MAG) activity. Anti-MAG activity was found in 25 of the samples. There was a relationship between the widening of some myelin lamellae observed on ultrastructural examination and the serum anti-MAG activity (23 cases). Immunopathological examination showed IgM binding to myelin sheaths in 17 cases.

Aged↗
PubMed1989

Congenital hypomyelination with axonopathy.

We report a case of congenital neuropathy taking a rapidly fatal clinical course. Ultrastructural features of the peripheral nerve were unusual with sparsity of myelinated fibres, lack of "onion bulb" formation and presence of axonal damage. This case is compared with previously reported cases of early infantile neuropathy.

Axons↗
PubMed1989

Acute mononeuropathy with angiotropic lymphoma.

Angiotropic lymphoma can present as a vascular disease in the central nervous system. The patient described in this report had a sudden pain in the region of the right superficial peroneal nerve and a nerve biopsy showed tumoral cells in the lumen of most small blood vessels. This pathology, first described in the skin as malignant angioendotheliomatosis, can be compared with the occurrence of multiple emboli in the vasa nervorum. In recent cases, tumoral markers have evidenced a lymphomatous origin, generally of the B type.

Acute Disease↗
PubMed1989

Chronic relapsing idiopathic polyneuropathy with primary axonal lesions.

Idiopathic polyradiculoneuropathy with primary axonal lesions is rarely encountered. Two cases are reported with a chronic relapsing course and a fatal outcome. Neuropathological examination of biopsied peripheral nerve in the two patients and in a necropsy case showed loss of myelinated fibres, but neither active demyelination nor inflammatory cells were observed. Acute and chronic relapsing axonal polyradiculoneuropathies appear to be two clinical forms of a peculiar entity different from GBS.

Axons↗
PubMed1989

[Malignant transformation of an intracranial epidermoid cyst].

We report the case of a 56-year-old man who underwent removal of an intracranial epidermoid cyst 16 years ago. The cyst recurred 12 years later and was once again almost completely removed. A new recurrence 4 years later proved to be due to malignant change in the form of squamous cell carcinoma. Only 17 other cases of malignant change in an intracranial epidermoid cyst have been reported. Moreover, three other cases presented as primary intracranial squamous carcinoma.

Brain Diseases↗