Search PubMed⌕ Search

Biomedical subjects

F Dubas

Publications and source records attributed to F Dubas.

85 records · Page 5Linked to original sources
PubMed1984

[Adrenomyeloneuropathy. 4 cases developing with the features of familial spasmodic paraplegia].

Four cases of adrenomyelopathy, the adult form of adrenoleukodystrophy, three of which were familial, were reported. The neurological disease consisted of progressive spasmodic paraplegia, sphincter disorders, peripheral neuropathy, occasional deep sensibility impairment and psychiatric disturbances in one case. Adrenal insufficiency was confirmed after the appearance of the neurological signs in two cases; in another case it had been diagnosed in childhood. Gonadal insufficiency was present in all cases and clinically apparent in two patients. Detailed genetic studies were carried out in two patients of the same family. Histocompatibility tests showed A2 and B15 antigens in three out of four cases. The diagnosis was confirmed in three cases by measurement of serum long chain fatty acid with increased C26 levels and C26/C22 ratios. Peripheral nerve biopsy in 1 patient showed characteristic inclusion bodies in the Schwann cells. The authors discuss the relationship between ALD and familial spasmodic paraplegia, the mode of genetic transmission of the disease and the diagnostic value of neuromuscular biopsy and measurement of serum long chain fatty acid levels.

Adrenoleukodystrophy↗
PubMed1984

[Adie's syndrome and polyneuropathy: apropos of a new case and a case of pupillotonia, polyneuropathy and generalized amyloidosis sparing the nerve].

The association of Adie's syndrome and of neuropathy of chronic evolution is not a frequent occurrence; first made known by Togi in 1967, four cases have been described ever since. The authors report two new observations: one concerns a man showing a unilateral tonic pupil associated to sensory neuropathy, raised C.S.F. protein, rarefaction of myelinated fibres with schwannian proliferation, without amyloid deposit. The other is that of a woman affected with bilateral pupillotonia, motor neuropathy of the four limbs and generalized amylosis. If the first observation is comparable to the five cases reported, the second is more questionable: the absence of amyloid deposit on three nerve biopsies and at the ciliary ganglion level does not absolutely exclude the possibility of amyloid neuropathy.

Adie Syndrome↗
PubMed1983

[Steele-Richardson-Olszewski disease without ophthalmoplegia. 6 clinico-anatomic cases].

Six clinico-pathological cases (4 males and 2 females) with a Parkinson-like syndrome which lasted an average of 5 1/2 years are reported. The average age at death was 73 years. Neuropathological features were similar to those described in post encephalitic Parkinson's syndrome and in Steele-Richardson-Olszewski disease. However, neurofibrillary tangles were less numerous; lesions in tectal, periaqueductal and reticular structures were less severe. Furthermore, the lesions seemed more pronounced in the substantia nigra, the globus pallidus and the nucleus subthalamicus, giving the appearance of a systematic pallido-luyso-nigral atrophy. None of these patients had a history of encephalitis. Clinical examination did not reveal the dystonic rigidity in extension of the neck nor the characteristic ophthalmoplegia of progressive supranuclear palsy. These 6 cases are compared to 10 cases of progressive supranuclear palsy typical both on clinical and pathological grounds. They could be considered as a particular form of Steele-Richardson-Olszewski disease without ophthalmoplegia.

Aged↗
PubMedDate not supplied

Acute delirium, delusion, and depression during IFN-beta-1a therapy for multiple sclerosis: a case report.

Adverse effects of interferon (IFN) treatment are common, and efforts to minimize these reactions are of considerable importance. IFN-beta-1a is an established therapy for patients with relapsing-remitting multiple sclerosis (MS). Its psychiatric side effects are debated and not yet fully established. The authors report here the case of a patient on IFN-beta-1a therapy for MS who developed acute delirium, delusion, and depression that ceased with treatment discontinuation. Although he had a history of recurrent major depressive disorder, his prior psychiatric illness had followed a course that was clinically independent of other signs of MS. This observation points out psychiatric vulnerability of patients taking IFN-beta-1a therapy for MS and suggests that IFN-beta-1a may induce or exacerbate preexisting psychotic symptoms.

Adjuvants, Immunologic↗
PubMedDate not supplied

Intravascular malignant lymphomatosis (so-called malignant angioendotheliomatosis): a case confined to the lumbosacral spinal cord and nerve roots.

Intravascular malignant lymphomatosis (IML), so-called malignant angioendotheliomatosis, was found in lumbosacral spinal cord and nerve roots of a 78-year-old women who died one month after the onset of symptoms. With regard to the majority of the 37 reviewed neurological cases in the literature, this report is unusual in that the disease was exclusively localized in the spinal cord and systemic involvement was absent. The usual clinical hallmark of the disease is a subacute dementia or encephalopathy, often associated with focal neurological signs, culminating in death within several months. The pathological features of IML characteristically include multiple small foci of necrosis of the whole brain, caused by occlusion of small vessels by noncohesive neoplastic cells and secondary changes of the vascular wall. All organs may be involved, especially the skin and the adrenals, sometimes with tumoral formations. Despite the fact that lymphoid tissues are usually spared, recent reports and the present case strongly suggest a lymphoid rather than endothelial origin of the malignant cells. The pathogenesis of this mainly intravascular lymphoma remains obscure.

Aged↗
PubMedDate not supplied

[Adrenomyeloneuropathy: clinical, genetic and morphologic features. Apropos of 5 cases].

The authors describe five cases of adrenomyeloneuropathy, an adult form of adrenoleukodystrophy. Three of the cases were related. The clinical picture comprises progressive spasmodic paraplegia, sphincter disorders, peripheral neuropathy, sometimes hollow foot and posterior columns disorders. In one case psychic disorders were observed. Biochemical analyses confirmed or revealed adrenal insufficiency in four cases and gonadal insufficiency in three cases. A detailed genealogical study was made in two patients belonging to the same family. Histocompatibility tests revealed the presence of A2 and B15 antigens in three cases. Diagnosis was confirmed in three cases by plasma determination of long-chain fatty acids, with an increase in the level of C26 acids and the C26/C22 ratio; biopsy of the peripheral nerve in one patient revealed inclusions characteristic of Schwann's cells. Symptomatological links between ALD and familial spasmodic paraplegia, the mode of genetic transmission of the disease, the contribution of neuromuscular biopsy to the diagnosis and the therapeutic prospects of a diet low in long-chain fatty acids are discussed.

Adrenoleukodystrophy↗