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1.
Electroencephalogr Clin Neurophysiol ; 96(6): 495-501, 1995 Nov.
Article in English | MEDLINE | ID: mdl-7489670

ABSTRACT

In a group of 10 children (ranging from 5 months to 15 years old) affected by diseases with mitochondrial dysfunction, 4 suffered from mitochondrial myopathy, 4 from mitochondrial encephalopathy and 2 from Friedreich's ataxia. The clinically detectable visual impairment consisted of 3 grey ocular fundi (the other 7 were normal) associated, in 2 subjects, with a mild nystagmus. Electrophysiological assessment, consisting of ERGs and flash VEPs, was systematically performed. The normal ERGs in all subjects confirmed the normal functioning of retinal electrogenesis. In contrast, the VEPs of 6 out of 10 subjects were modified: in 2 of the 4 subjects with mitochondrial myopathy, the VEPs had a hyperamplitude; in the 2 subjects with Friedreich's ataxia, the implicit time of the principal VEP peaks was increased, together with a hyperamplitude in 1 case; lastly, in 2 of the 4 subjects with mitochondrial encephalopathy, the VEPs were altered. These modifications reflected visual pathway conduction disorders with no clinical expression. Various underlying pathophysiological mechanisms possibly responsible for these modifications are discussed.


Subject(s)
Electroretinography , Evoked Potentials, Visual , Mitochondrial Encephalomyopathies/physiopathology , Mitochondrial Myopathies/physiopathology , Adolescent , Child , Child, Preschool , Electrophysiology , Female , Humans , Infant , Male , Time Factors , Visual Pathways/physiopathology
10.
Ophtalmologie ; 3(3): 193-5, 1989.
Article in French | MEDLINE | ID: mdl-2641108

ABSTRACT

A retrospective study of 324 cases of chorioretinal heredodegeneration of all kinds shows an associated pathology in 44% of cases. These associated anomalies point out the need of a complete physical examination, a familial inquest keeping in mind genetic counseling. They are a good model for a high resolution cytogenetic study to find microdeletions which can allow to precise the genome's map.


Subject(s)
Choroid Diseases/genetics , Retinal Degeneration/genetics , Choroid Diseases/diagnosis , Choroid Diseases/pathology , Chromosome Mapping , Cytogenetics , Genetic Carrier Screening , Genetic Counseling , Humans , Pedigree , Retinal Degeneration/diagnosis , Retinal Degeneration/pathology , Retrospective Studies
16.
J Fr Ophtalmol ; 11(8-9): 561-6, 1988.
Article in French | MEDLINE | ID: mdl-3068282

ABSTRACT

46 patients with the association of acute anterior uveitis and the presence of HLA B27 antigen were studied. Ankylosing spondylitis was found in 19 patients (41.3%). Ophthalmoscopy showed a diffuse vitreous haze in 63%, a macular disease in 21.7%, a papillitis in 17.4%. We performed pars plana vitrectomy on 8 patients (10 eyes) with severe posterior uveitis and/or cystoid macular edema. The postoperative follow-up period averaged 14 months with visual acuity improvement in 5 eyes. The surgical indications are described.


Subject(s)
Uveitis, Anterior/complications , Vitreous Body , Acute Disease , Adolescent , Adult , Eye Diseases/etiology , Female , HLA-B Antigens/immunology , HLA-B27 Antigen , Humans , Male , Middle Aged , Spondylitis, Ankylosing/complications , Uveitis, Anterior/immunology , Vitrectomy
18.
J Fr Ophtalmol ; 10(10): 565-73, 1987.
Article in French | MEDLINE | ID: mdl-3443707

ABSTRACT

Surgery was performed in 30 cases to extract foreign bodies located in the posterior segment of the eye, between october 1st, 1985 to november 15th, 1986 in the Hotel-Dieu of Paris Department of Ophthalmology. In 20 cases, the foreign bodies were lead-shot fragments resulting from assaults in 17 cases, from hunting accidents in 3 cases. In 10 cases they were other metallic bodies, resulting from working or pottering accidents. We present the anatomical lesions and the surgical techniques employed, in particular vitrectomy in conjunction with foreign body extraction using the diamond coated jaws forceps. Anatomical results are 83.3% flat retinas. 60% of the patients achieved visual acuity between 0.1 and 1.0. The following points are discussed: true opportunity to perform retinopexy around the retinal tears; problems created by the foreign bodies embedded into the eyeball; operative timing, taking into consideration both the haemorrhagic conditions, i.e. hyphema, choroïdals, massive intra-vitreal bleeding, and the prevention of tractionnal retinal detachment.


Subject(s)
Eye Foreign Bodies/surgery , Microsurgery/methods , Retinal Detachment/prevention & control , Adolescent , Adult , Child , Emergencies , Female , Humans , Male , Microsurgery/instrumentation , Middle Aged , Vitrectomy/methods
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