Improvement of clinical and MRI findings in a boy with adrenoleukodystrophy by dietary erucic acid therapy. 1992

K Maeda, and Y Suzuki, and S Yajima, and J Asano, and S Yamaguchi, and N Matsumoto, and J Borel, and H W Moser, and T Orii
Department of Pediatrics, Shizuoka Saiseikai General Hospital, Japan.

A 5-year-old boy with adrenoleukodystrophy, with clinical symptoms of visual, mental and motor disturbances which progressed rapidly, was treated with Lorenzo's oil consisting 1 volume of glyceryl trierucate and 4 volumes of glyceryl trioleate. Five months after initiation of this therapy, ability to swallow was enhanced and T2-weighted magnetic resonance imaging of the brain revealed regression of high intensity area of the parieto-occipital white matter.

UI MeSH Term Description Entries
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D001921 Brain The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. Encephalon
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004042 Dietary Fats, Unsaturated Unsaturated fats or oils used in foods or as a food. Dietary Oils,Unsaturated Dietary Fats,Dietary Fat, Unsaturated,Dietary Oil,Fat, Unsaturated Dietary,Fats, Unsaturated Dietary,Oil, Dietary,Oils, Dietary,Unsaturated Dietary Fat
D004338 Drug Combinations Single preparations containing two or more active agents, for the purpose of their concurrent administration as a fixed dose mixture. Drug Combination,Combination, Drug,Combinations, Drug
D004883 Erucic Acids cis-13-Docosenoic Acids. 22-Carbon monounsaturated, monocarboxylic acids. Docosenoic Acids,Brassidic Acids,Acids, Brassidic,Acids, Docosenoic,Acids, Erucic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000326 Adrenoleukodystrophy An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS). Adrenomyeloneuropathy,Schilder-Addison Complex,X-Linked Adrenoleukodystrophy,ALD (Adrenoleukodystrophy),Addison Disease and Cerebral Sclerosis,Bronze Schilder Disease,Melanodermic Leukodystrophy,Siemerling-Creutzfeldt Disease,X-ALD,X-ALD (X-Linked Adrenoleukodystrophy),Adrenoleukodystrophy, X-Linked,Leukodystrophies, Melanodermic,Leukodystrophy, Melanodermic,Schilder Addison Complex,Siemerling Creutzfeldt Disease,X ALD,X ALD (X Linked Adrenoleukodystrophy),X Linked Adrenoleukodystrophy
D014304 Triolein (Z)-9-Octadecenoic acid 1,2,3-propanetriyl ester. Glycerol Trioleate,Trielaidin,Trioleate-Glycerin,Trioleoylglycerol,Trioleyl Glycerol,Glycerol, Trioleyl,Trioleate Glycerin,Trioleate, Glycerol

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