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C Vital

Publications and source records attributed to C Vital.

At least 325 records · Page 18Linked to original sources
PubMedDate not supplied

Chronic inflammatory demyelinating polyneuropathy: immunopathological and ultrastructural study of peripheral nerve biopsy in 42 cases.

The authors recently reexamined the peripheral nerve biopsies from 42 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). There were 27 males and 15 females, aged from 9 to 84 years, and 13 had relapses. No patient had vasculitis, monoclonal gammopathy, tumor, diabetes mellitus, Lyme disease, familial neuropathy, HIV, or any other immune deficiency. In the endoneurium, perivascular inflammatory cell infiltrates were present in only one case, but scattered histiocytes marked by KP1 on paraffin-embedded fragments were present in every case and there were no T-lymphocytes. At ultrastructural examination macrophage-associated demyelination was observed in 17 cases, of which 6 had relapses separated by intervals of several months or years. Axonal lesions without associated primary demyelination were observed in 4 cases and 3 of these had relapses. Thirty-two patients had mixed lesions of demyelination and axonal involvement. This study confirms other recent data indicating that in all cases of CIDP, macrophages are present in the endoneurium. Macrophage-associated demyelination is the characteristic feature of demyelinating forms. On the other hand, isolated primary axonal forms, which have been known since 1989, are relatively frequent and prone to relapses.

Adolescent↗
PubMedDate not supplied

Inflammatory demyelinating neuropathy in C57BL/KaLwRij mice.

Neuropathies associated with monoclonal gammopathy (MG) in humans have been extensively studied in the past few years, but experimental models have proved difficult to create. C57BL mice are prone to develop benign MG and it has been reported that some of these mice with benign IgG MG present an inflammatory demyelinating neuropathy (IDN). In order to verify such findings, the serum and the sciatic nerve of the first group of 28 C57BL/KaLwRij mice were examined: none of 10 mice with normal serum showed ultrastructural abnormalities in the sciatic nerve, while lesions of IDN were present in three out of 10 mice with benign IgG MD, in two out of seven with benign IgM MG, and in a mouse with Waldenström-like lymphoma. The second group of animals was studied in the same way; it was composed of seven C57BL mice with transplanted multiple myeloma, and six C57BL mice with Morbus Waldenström-like lymphoma. In none of these animals, which were younger than those of the first group, was any lesion of IDN observed.

Animals↗
PubMedDate not supplied

Adult dementia due to intraneuronal accumulation of ceroidlipofuscinosis (Kufs' disease): ultrastructural study of two cases.

Two cases, a man and a woman, presented the initial signs of progressive dementia at about age 40 years. Aphasia, facial dyskinesias, and ataxia were associated with behavioral disturbances in the first patient. Aphasia, oculomotor disturbances, and ataxia coexisted with dementia in the second patient. In both cases, ultrastructural examination of a cerebral biopsy showed intraneuronal granular osmiophilic deposits mixed with lamellar structures and fingerprint profiles. Such deposits are characteristic of Kufs' disease. In the first patient, brain autopsy was performed 3 years after cerebral biopsy. Our two cases presenting dementia with motor disturbances correspond to type B Kufs' disease.

Adult↗
PubMedDate not supplied

Myelin modifications in 8 cases of peripheral neuropathy with Waldenström's macroglobulinemia and anti-MAG activity.

Characteristic myelin modifications in patients with IgM monoclonal gammopathy and anti-MAG activity have mainly been studied in cases of undetermined significance, but also exist in cases with indolent Waldenström's macroglobulinemia, i.e., when lymphoplasmocytic infiltration in bone marrow is 15% or more, without any visceral involvement. Since 1983, the authors have examined nerve biopsies from 8 cases with Waldenström's macroglobulinelia by direct immunofluorescence examination on frozen sections and ultrastructural examination. At direct immunofluorescence, fixation of anti-IgM serum on myelinated fibers was present in 7 cases. At ultrastructural examination, a widening of some myelin lamellae at the periphery of a few fibers was visible in 8 cases. A few fibers with hypermyelination were present in 5 cases. In 2 of these 5 cases widening of some myelin lamellae was present in numerous fibers, 88% in one of them. Frequently, there was a major widening of some myelin lamellae with dilated lamellae present in the inner part of the myelin sheath. Certain lamellae were more dilated, up to 50 nm. Occasionally, enlarged lamellae were not compacted with each other. The authors also examined nerve biopsies from 36 patients with IgM monoclonal gammopathy of undetermined significance and anti-MAG activity, but found only one case with major widening of some myelin lamellae. Five other cases with major widening of some myelin lamellae, 4 Waldenström's macroglobulinemia and 1 IgM monoclonal gammopathy of undetermined significance, have been reported. Given that demyelinating neuropathies are far more numerous in cases with IgM monoclonal gammopathy of undetermined significance, it is likely that cases of indolent Waldenström's macroglobulinemia are prone to develop major myelin modifications, possibly due to another mechanism, added to the classic anti-MAG activity.

Aged↗
PubMedDate not supplied

Subacute inflammatory polyneuropathy: two cases with plasmacytoid histiocytes in the endoneurium.

Inflammatory polyneuropathies are mainly known by their acute form, Guillain-Barré syndrome, but there are also chronic cases and all are considered as having an autoimmune mechanism. In each form, peripheral nerve biopsies show scattered macrophages in the endoneurium and in certain cases macrophages invade the Schwann cell cytoplasm and destroy the myelin sheath. In rarer cases there is a primary axonal degeneration. The authors studied two chronic cases, which both exhibited mixed primitive axonal and demyelinating lesions, with peculiar histiocytes in the endoneurium. These rounded cells were characteristically well marked by KP1 but showed well-developed rough endoplasmic reticulum cysternae at ultrastructural examination. Such plasmacytoid histiocytes have mainly been studied in subacute lymphadenopathies and have been only briefly illustrated in a few cases of peripheral neuropathies due to Lyme disease or HIV infection. The two cases reported here had no associated diseases and probably correspond to a peculiar subacute autoimmune reaction.

Adult↗
PubMedDate not supplied

[Anatomoclinical study of 29 cases of acute necrotizing encephalitis. Demonstration of herpes virus in 24 cases].

The authors studied the cerebral lesions in 29 cases of acute necrotizing encephalitis. Electron microscopy showed the presence of typical herpes virus particles in 24 cases. In 16 of them the virus had been isolated from specimens of the brain taken by biopsy and in 8 other cases from autopsy specimens. In 3 acute cases the search for the virus was negative and it was not carried out in the two cases with prolonged evolution. Only 6 patients have survived. Some peculiar varieties have beeen observed, i.e. two adolescents had temporal and almost entirely unilateral lesions, and two aged females had severely hemorrhagic necrotic areas which could have been confounded with vascular lesions. In these two later cases typical particles were present. Finally, one case showed within the same nuclei at once typical herpes virus particles and other viral structures of vermicular form, the origin of which could not be precised.

Adolescent↗
PubMedDate not supplied

Chorea-acanthocytosis: neuropathology of brain and peripheral nerve.

We report the neuropathological data from a familial case of chorea-acanthocytosis with central and peripheral nervous system involvement. At the age of 34, the patient underwent a peripheral nerve biopsy which was analyzed by light- and electron microscopy. These studies showed a selective reduction in the large diameter myelinated fibre population, with several clusters of regeneration. Remyelinating fibers surrounded by flattened Schwann cell processes were also present. The patient died at the age of 44, and post-mortem macroscopic examination of the brain showed marked atrophy of the caudate. Histological examination of paraffin sections showed almost complete depletion of neurons in the caudate, with severe astrocytic gliosis. The putamen and pallidum were slightly less severely depleted of neurons, but with marked astrocytic gliosis. Diffuse mild gliosis was also evidenced, by immunohistochemistry with anti-GFAP, in the thalamus and subcortical white matter.

Adult↗
PubMedDate not supplied

[Drachman's "ophthalmoplegia plus" or Kearns and Shy syndrome. Apropos of 2 cases with mitochondrial abnormality ultrastructurally demonstrated in the muscle].

Two typical cases of the Kearns and Shy syndrome are reported. Some of the authors have previously published observations on simple ocular myopathy and oculo-pharyngeal dystrophy associated with abnormalities of the mitochrondria in skeletal muscle cells. This disease, which is still called "ophthalmoplegia plus", combines a progressive external ophthalmoplegia, muscular defects and varied neurological signs with other symptoms particularly retinal, endocrine and cardiac. Histo-enzymological and ultra-structural studied of a fragment of skeletal muscle confirm the presence of mitochondrial anomalies.

Adult↗
PubMedDate not supplied

[Ultrastructural study of the oculomotor muscle in 2 familial cases of Steinert's disease].

A biopsy specimen of an extra-ocular muscle from a brother and sister affected by typical familial Steinert's disease was examined by electron microscopy. Considerable disorganisation of the contractile element was observed with a gross accumulation of abnormal mitochondria. This was a very different appearance to the findings observed in skeletal muscle in Steinert's disease and much closer to the findings in skeletal muscle in certain 'ocular' myopathies. One cannot fail to be impressed by the discordance between the appearance of the muscular disorganisation and the rarity of clinical oculomotor disturbances in this disease.

Female↗
PubMedDate not supplied

[Peripheral neuropathies i- Waldenstrom's disease. Histological and ultrastructural studies of 5 cases].

In four cases it was the existence of peripheral neuropathy which led to the discovery of macroglobulinemia within an interval from 1 to 11 years. The treatment of the disease also markedly improved the peripheral neuropathy in three cases. Two patients died. Two of the biopsies of the peripheral nerve showed infiltration by atypical lymphocytes. Ultrastructural changes in the five cases were variable. Marked axonal involvement in the two cases with cellular infiltration. In one of these two cases it was combined with segmental demyelinization. This lesion was clearly predominant in the three other cases. In one of them demyelinization occurred in a very peculiar way with dilation of the internal part of the mesaxone going on in the most peripheral layers of the myelin. Discussion of the mechanisms of the involvement of the peripheral nervous parenchyma in Waldenström's disease. Cellular infiltrations, amorphous deposits, changes in the capillaries and macroglobuline may occur.

Adult↗
PubMedDate not supplied

[Role of folic-acid deficiency in deficiency diseases of the nervous system. Apropos of 12 cases including an anatomo-clinical case].

The authors report 12 cases of neurological syndromes due to folic acid deficiency, due in 8 cases to chronic alcoholism. In 5 cases there was polyneuritis, 3 cases had cerebellar atrophy, whilst 4 patients had subacute combined degeneration of the cord. Folic acid deficiency occurred alone in five cases out of twelve, as in 3 cases vitamin B1 deficiency was associated, and in four cases there was malabsorption of vitamin B12. A neuropathological study of these cases showed: 1) moderate involvement of the mamillary tubercles as observed in deficiency encephalopathies. 2) severe peripheral nerve involvement especially of axonal type. 3) involvement of the anterior horns of the spinal cord with appearances of central chromatolysis and a few atrophic neurones.

Adult↗
PubMedDate not supplied

Morphological study of the aging human peripheral nerve.

The superficial peroneal nerve from 46 elderly patients was studied by light and electron microscopy. Like most elderly people, these patients displayed evidence of varying degrees of peripheral nervous system dysfunction, but they were not suffering from any disease known to alter the peripheral nerve. A quantitative study showed that myelinated fiber loss predominated in the large diameter group. In the majority of cases, clusters of regenerating myelinated fibers were numerous. On the other hand, Wallerian-like degeneration aspects, segmental demyelination figures and axonal organelle accumulation were relatively rare. Unmyelinated fibers were also notably damaged in most cases. There was moderate reduplication of endoneurial vascular basement membrane.

Aged↗
PubMedDate not supplied

Guillain-Barré syndrome: an ultrastructural study of peripheral nerve in 65 patients.

65 biopsies of peripheral nerve from patients suffering from Guillain-Barré syndrome were studied by electron microscopy. In 48 cases there was macrophagic invasion of the Schwann cells of certain myelinated fibers, and in 32 of these cases some myelin sheaths were stripped away by an elongated macrophagic process. Vesicular disruption of the myelin sheath was observed in only 8 cases and in less than 1% of the myelinated fibers. Uncompacted myelin lamellae were observed in a few myelinated fibers. These ultrastructural lesions are analysed and commented on with a view to selecting patients who are to undergo plasma exchange.

Biopsy↗
PubMedDate not supplied

Quantitative, histological and ultrastructural studies of peripheral nerve in arteriosclerotic non-diabetic patients.

The superficial peroneal nerve was taken from 12 arteriosclerotic non-diabetic patients just after amputation of a leg. Preparations of teased fibers were performed in 8 cases. Specimens were studied by light and electron microscopy in all cases. Histograms of myelinated fibers in transverse semi-thin sections showed that depletion of myelinated fibers varied from case to case, with no selective vulnerability in either the large or the small diameter group. There was a dramatic loss of myelinated fibers in only one case. No real nerve infarct was observed. In most cases, regions of Wallerian-like degeneration were prevalent; however, myelino-axonal changes were severe only in a few cases. Axons with organelle aggregates were seen in some cases. Figures of segmental demyelination were not numerous in this series. Unmyelinated fibers were also damaged, and the degree of involvement differed from case to case. Pathological changes observed in this study confirm the relative resistance of peripheral nerve to ischemia.

Aged↗