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Titolo:
Choreic movements and MRI abnormalities in the subthalamic nuclei reversible after administration of coenzyme Q10 and multiple vitamins in a patient with bilateral optic neuropathy
Autore:
Chariot, P; Brugieres, P; Eliezer-Vanerot, MC; Geny, C; Binaghi, M; Cesaro, P;
Indirizzi:
Hop Henri Mondor, Dept Pathol, F-94010 Creteil, France Hop Henri Mondor Creteil France F-94010 Pathol, F-94010 Creteil, France Hop Henri Mondor, Dept Neuroradiol, F-94010 Creteil, France Hop Henri Mondor Creteil France F-94010 oradiol, F-94010 Creteil, France Hop Henri Mondor, Dept Neurol, F-94010 Creteil, France Hop Henri Mondor Creteil France F-94010 Neurol, F-94010 Creteil, France Hop Henri Mondor, Dept Ophthalmol, F-94010 Creteil, France Hop Henri Mondor Creteil France F-94010 thalmol, F-94010 Creteil, France Univ Paris 12, Creteil, France Univ Paris 12 Creteil FranceUniv Paris 12, Creteil, France
Titolo Testata:
MOVEMENT DISORDERS
fascicolo: 5, volume: 14, anno: 1999,
pagine: 855 - 859
SICI:
0885-3185(199909)14:5<855:CMAMAI>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
MITOCHONDRIAL-DNA MUTATION; SYDENHAM CHOREA; DISEASE; IDEBENONE; DYSTONIA; RECOVERY; GENE; PLUS;
Keywords:
chorea; MRI; optic neuropathy; Leber hereditary optic neuropathy; mitochondria; coenzyme Q10;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
35
Recensione:
Indirizzi per estratti:
Indirizzo: Chariot, P Hop Henri Mondor, Dept Pathol, F-94010 Creteil, France Hop Henri Mondor Creteil France F-94010 94010 Creteil, France
Citazione:
P. Chariot et al., "Choreic movements and MRI abnormalities in the subthalamic nuclei reversible after administration of coenzyme Q10 and multiple vitamins in a patient with bilateral optic neuropathy", MOVEMENT D, 14(5), 1999, pp. 855-859

Abstract

A 37-year-old man developed choreic movements of the limbs over a few months. His medical history included bilateral visual loss detected at the age of 9 and worsening at age 20. Visual field testing showed a central scotoma. Fundus examination showed atrophy of the optic disks and narrowing of vessels. The diagnosis of Leber hereditary optic neuropathy (LHON) was considered. There was no family history of visual loss or movement disorders. Blood lactate:pyruvate ratio was moderately elevated. Skeletal muscle biopsy was normal. Magnetic resonance imaging showed bilateral hypointense lesions on T1-weighted sequences in the subthalamic nuclei and in the lateral part of the substantia nigra. Linear hyperlucencies in the pyramidal tract facingthe lateral part of the ruber nuclei were also demonstrated on T2-weightedsequences. Nine LHON-associated mutations were ruled out by RFLP analysis. Treatment with 250 mg coenzyme Q10 per day and multiple vitamins was initiated. Gradual recovery in movement disorders occurred over 1 year. Lactate to pyruvate ratio normalized. No change of visual function was observed. Onmagnetic resonance imaging performed 3 years later, lesions of the subthalamic nuclei almost completely disappeared. We think the patient might have an unusual, genetically uncharacterized mitochondrial disorder, combining optic neuropathy and chorea.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/09/20 alle ore 02:48:58