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Titolo:
ARRESTED CEREBRAL ADRENOLEUKODYSTROPHY - A CLINICAL AND PROTON MAGNETIC-RESONANCE SPECTROSCOPY STUDY IN 3 PATIENTS
Autore:
KORENKE GC; POUWELS PJW; FRAHM J; HUNNEMAN DH; STOECKLER S; KRASEMANN E; JOST W; HANEFELD F;
Indirizzi:
UNIV GOTTINGEN,DEPT PAEDIAT & NEUROPAEDIAT,ROBERT KOCH STR 40 D-37075GOTTINGEN GERMANY MAX PLANCK INST BIOPHYS CHEM,BIOMED NMR FORSCH GMBH D-3400 GOTTINGEN GERMANY UNIV GOTTINGEN,INST HUMAN GENET D-3400 GOTTINGEN GERMANY UNIV HOMBURG,DEPT PAEDIAT D-6650 HOMBURG GERMANY
Titolo Testata:
Pediatric neurology
fascicolo: 2, volume: 15, anno: 1996,
pagine: 103 - 107
SICI:
0887-8994(1996)15:2<103:ACA-AC>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
X-LINKED ADRENOLEUKODYSTROPHY; GENE; ADRENOMYELONEUROPATHY; THERAPY; ACIDS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
18
Recensione:
Indirizzi per estratti:
Citazione:
G.C. Korenke et al., "ARRESTED CEREBRAL ADRENOLEUKODYSTROPHY - A CLINICAL AND PROTON MAGNETIC-RESONANCE SPECTROSCOPY STUDY IN 3 PATIENTS", Pediatric neurology, 15(2), 1996, pp. 103-107

Abstract

We report three unrelated boys with X-linked adrenoleukodystrophy with onset of typical neurological symptoms of cerebral adrenoleukodystrophy between the age of 7 and 11 years, In contrast to the expected rapid progression, these patients showed an apparent arrest of initial neurological deterioration for subsequent periods of 5-12 years, Repeated neuroimaging revealed no progression of demyelination, Despite regional variability of demyelination, proton magnetic resonance spectroscopy revealed a specific metabolic pattern in all patients, with only moderate reduction of N-acetylaspartate, normal or reduced choline-containing compounds, normal or enhanced myo-inositol and no detectable lactate, which differs from findings in progressive cerebral adrenoleukodystrophy which usually exhibits a severe reduction of N-acetylaspartate and marked increases of choline-containing compounds, myo-inositol, and lactate. The ability to identify this newly described subgroup of patients with cerebral adrenoleukodystrophy is important for medical advice and planning of therapy.

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Documento generato il 07/07/20 alle ore 15:29:55