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Biomedical subjects

P Rondot

Publications and source records attributed to P Rondot.

138 records · Page 8Linked to original sources
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↗
PubMedDate not supplied

[Difficulty in manual prehension under visual control (author's transl)].

Visuomotor ataxia causes difficulty in the prehension of objects under visual control which can occur without paralysis of ocular fixation and spatial agnosia. This can involve the total visual field or the right or left half-fields, and can affect both hands or one only. 1) Unilateral visuomotor ataxia is localized to the two right or left homonymous half-fields and can affect both hands or one only. It is direct when the ataxic hand is on the same side as the affected visual half-field, and crossed when the ataxic hand is contralateral to the affected visual half-field. 2) Bilateral visuomotor ataxia involves the total visual field. Each hand may be ataxic only for the contralateral visual field with a crossed bilateral visuomotor ataxia, or in the homolateral field with a direct bilateral visuomotor ataxia. The anatomical and clinical observations reported imply the existence of visuomotor connections, both direct and crossed, the latter crossing the median line through the corpus callosum.

Adult↗