Adrenoleukodystrophy: a scoring method for brain MR observations.

Adrenoleukodystrophy: a scoring method for brain MR observations. Research Abstract Details 

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Adrenoleukodystrophy: a scoring method for brain MR observations. Abstract Text:

D J Loes,S Hite,H Moser,A E Stillman,E Shapiro,L Lockman,R E Latchaw,W Krivit,

PURPOSE: To develop a scoring method for brain observations in patients with X-linked adrenoleukodystrophy. METHODS: One hundred seventy-five brain MR scans in 83 male subjects less than 20 years of age with proved biochemical defects were reviewed. A severity score (0 to 34), based on a point system derived from location and extent of disease and the presence of focal and/or global atrophy, was calculated for each exam. RESULTS: Fifty-five of the 83 patients showed MR findings consistent with adrenoleukodystrophy. Two major patterns were observed. A posterior pattern (mean score, 9; range, 0.5 to 25) was present in 80% of patients, and an anterior pattern (mean score, 10; range, 2 to 18) was present in 15% of patients. Serial MR imaging, positive for adrenoleukodystrophy in 34 patients (mean follow-up, 23 months; range, 2 months to 6 years 11 months), showed progressive disease in 52%, progressive disease with subsequent stabilization in 18%, stable disease in 24%, and minimal improvement in 6%. CONCLUSION: The adrenoleukodystrophy MR severity scoring method is a measure that can be used with standard MR images. When used in conjunction with clinical parameters, this scoring method may help define better the natural history of adrenoleukodystrophy and monitor response to developing therapies.

Adrenoleukodystrophy: a scoring method for brain MR observations. Publishing Authors By Initials

DJ Loes,S Hite,H Moser,AE Stillman,E Shapiro,L Lockman,RE Latchaw,W Krivit,

For similar nervous system: central nervous system: spinal cord research abstracts see: nervous system: central nervous system: spinal cord research

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Adrenoleukodystrophy: a scoring method for brain MR observations. Journal Published:

PUBLICATION TYPE: Research Support, U.S. Gov't,

Journal: AJNR. American journal of neuroradiology

VOLUME: 15

Page Numbers: 1761-6

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ISSN: 0195-6108

DAY: 14

MONTH: Oct

YEAR: 1994

Adrenoleukodystrophy: a scoring method for brain MR observations. Information

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LANGUAGE: eng

NlmUniqueID: 8003708

Adrenoleukodystrophy: a scoring method for brain MR observations. Keywords Mesh Terms:

KEYWORDS: Spinal Cord

MESH TERMS: pathology

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Grant and Affiliation Information for Adrenoleukodystrophy: a scoring method for brain MR observations.

AFFILIATION: University of Minnesota Hospital and Clinic, Department of Radiology, Minneapolis 55455.

Country: UNITED STATES

AGENCY: United States NCRR

GRANT: RR00052 000685

ACRONYM: RR

MEDLINETA: AJNR Am J Neuroradiol

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