PubMed Health⌕ Search

Biomedical subjects

J M Brucher

Publications and source records attributed to J M Brucher.

138 records · Page 8Linked to original sources

[Progressive multifocal leukoencephalopathy mimicking multiple sclerosis as the sole clinical manifestation of acquired immunodeficiency syndrome].

A 38 year-old wife, of belgian origin and residency, suffered from a left retrobulbar optic neuritis in 1973, with as only sequel dyschromatopsia and central scotoma enlargement. In 1985, 12 years later, she complained of a progressive weakness of the right hand, and developed a spastic tetraplegia within four months. The C.T. Scan showed white matter hypodensities, without mass effect or contrast enhancement, first limited to the left centrum semiovale and later on with multifocal character. Somatosensory evoked potentials after stimulation of the right median nerve demonstrated only lemniscus medialis response. Multiple sclerosis was initially suspected. Analysis of lymphocytes subsets however showed a markedly reduced helper population with as a result a very low H/S ratio, and the serology was found positive for HTLV-III-LAV, thus demonstrating the diagnosis of acquired immune deficiency syndrome (AIDS). Visceral autopsy demonstrated only a CMV pneumonia. Autopsy of the brain showed typical lesions of progressive multifocal leukoencephalopathy (PML). This case is compared with 20 previously published observations of PML associated with AIDS and appears rather unusual due to the association of unfrequent clinical peculiarities: previous, probably coincidental, retrobulbar optic neuritis, female patient, lack of risk factor and clinical symptoms of AIDS. The diagnostic difficulties in the present case are emphasized.

Acquired Immunodeficiency Syndrome↗

[Ultrastructural study of a medullomyoblastoma (author's transl)].

A third case in between twelve reported cases of medullo-myyoblastoma was investigated by electron microscopy. The muscle cells seemed to originate of proliferated endothelial cells. Because of the derivation from two blastodermic layers the tumour was considered a teratoid.

Cerebellar Neoplasms↗

[Anatomo-clinical observation of atherosclerotic subcortical encephalopathy ("Binswanger's disease") with hypodensity of the white matter on brain scan].

A 54 year-old hypertensive female patient presented dementia evolving progressively over a four-year period in association with motor deficiency and asymmetric pyramidal signs. The scan showed diffuse hypodensity of the white matter without enhancement. Brain biopsy indicated a diagnosis of subcortical arteriosclerotic encephalopathy; this was later confirmed by the post-mortem examination. On the strength of data from the literature, the authors draw attention to the frequency of this syndrome in hypertensive patients. They outline the main clinical characteristics and demonstrate the importance of the scan.

Brain↗

[Ultrastructural elementary lesions in muscular pathology (author's transl)].

The authors present and illustrate most of the ultrastructural abnormalities encountered in skeletal muscle in cases of neuromuscular disease. The diagnostic significance of the changes are discussed but emphasis is laid on the nonspecific character of most of them. For diagnostic purposes, examination by light microscopy remains the preferred approach, but electron microscopy enables the nature and origins of the lesions to be better defined and is a very valuable technique in exploring the pathogenesis of muscular diseases.

Glycogen↗

[CAT mirror image in a case of cerebral malignant lymphoma (author's transl)].

Diffuse multicentric sarcomas of the brain, also called cerebral malignant lymphomas, sometimes present symmetrical topography. This is well demonstrated by a CAT mirror image obtained by the authors in the reported case. The diagnostic value of the image is underlined in view of the possible forms of therapy, in particular radiation therapy, applicable to this type of tumour. (Acta neurol. belg., 1980, 80, 86-94).

Aged↗

[Kufs disease with leukoencephalopathy].

We report a case of adult neuronal ceroid lipofuscinosis (Kufs' disease) with leukoencephalopathy on cerebral scan CT and MRI. A 52 year-old woman presented with partial complex epileptic seizure followed by progressive dementia, cerebellar ataxia, pyramidal and akineto-rigid signs and symptoms. After 6 years of evolution, cerebral stereotactic biopsies showed a diffuse gliosis of the white matter, but no clear demyelination. Nerve and glial cells contained numerous PAS+ autofluorescent granules. In the oligodendrocytes and astrocytes of the white matter these granules appeared electronmicroscopically as cytoplasmic osmiophilic lamellar bodies with fingerprint profile combined with some curvilinear and rectilinear aspects. The cortical nerve cells contained granular osmiophilic bodies. This "leukoencephalopathic" variant of Kufs' disease is probably related to the pigmentary type of orthochromatic leukodystrophy, wherein similar inclusions have been only described in the macrophages and glial cells of the white matter.

Biopsy, Needle↗