Abstract
The authors report the case of two brothers suffering from Adrenoleukodystrophy. CT indicated the possibility of ALD, confirmed in the second case by biochemical examinations. The usefulness of CT scanning in all patients with progressive behavioral changes is emphasized and its diagnostic validity in ALD is confirmed. The clinical data, the negativity of laboratory results for other neurodegenerative diseases and the CT scan with parietal and occipital hypodensity constitute very characteristic evidence for ALD.
Sommario
Gli Autori riportano il caso di due fratelli affetti da Adrenoleucodistrofia. La diagnosi venne sospettata in base ad un esame TC e successivamente confermata nel secondo dei due pazienti attraverso l'esame biochimico. Viene sottolineata l'importanza dell'esame TC in soggetti che manifestano turbe comportamentali e, inoltre, viene confermata la validità diagnostica della TC nella ALD. Il quadro clinico, la negatività di alcuni esami di laboratorio per altre malattie neurodegenerative e il reperto della TC, con ipodensità delle regioni parietale ed occipitale, forniscono un quadro molto caratteristico per la diagnosi di ALD.
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References
Duda E.E., Huttenlocher P.R.:Computed tomography in adrenoleukodystrophy: Correlation of radiological and histological findings. Radiology, 120:349–350, 1976.
Eiben R.M., Dichiro G.:Computer-assisted tomography in adrenoleukodystrophy. J. Comput. Assist. Tomography, 1:308–314, 1977.
Greenberg H.S., Halverson D., Lane B.:CT scanning and diagnosis of adrenoleukodystrophy. Neurology, 27:884–886, 1977.
Igarashi M., Schaumburg H.H., Powers J., Kishimoto Y., Kolodny E., Suzuki K.:Fatty acid abnormality in adrenoleukodystrophy. J. of Neurochem., 26:851–860, 1976.
Kendall B.E., Kingsley D.P.E.:The diagnostic and prognostic significance of CT in neurodegenerative, metabolic and leukodystrophic diseases of childhood. In: Choices and characteristics in CT. Proceedings of the 8th Congress of the European Society of Neuroradiology. Strasburg 7–8 Sept. 1979, pp. 65–80. Kugler Publications Amsterdam 1980.
Lane B., Carrol B.A., Pedley T.A.:Computerized cranial tomography in cerebral diseases of white matter. Neurology, 28:534–544, 1978.
Menkes J.H., Corbo L.M.:Adrenoleukodystrophy. Accumulation of cholesterol esters with very long chain fatty acids. Neurology, 27:928–932, 1977.
Moser H.W., Moser A.B., Kawamura N., Murphy J., Milunsky A., Suzuki K., Schaumburg H., Kishimoto Y.:ALD: Elevated C 26 fatty acid in cultured skin fibroblasts;Annals of Neurology. In press. 7: 542–549, 1980
Quisling R.G., Andriola M.R.:Computed Tomography evaluation of the early phase of adrenoleukodystrophy. Neuroradiology, 17:285–288, 1979.
Ramsey R.B., Banik N.L., Davison A.N.:Adrenoleukodystrophy brain cholesterol esters and other neutral lipids. J. of Neurol. Sci., 40:189–196, 1979.
Schaumburg H., Powers J.M., Suzuki K., Raine C.S.:Adrenoleukodystrophy: (Sex-linked Schilder disease). Ultrastructural demonstration of specific cytoplasmic inclusions in the central nervous system. Arch. of Neurol., 31:210–213, 1974.
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Pavone, L., Pero, G., Fiumara, A. et al. Adrenoleukodystrophy. Validity of CT in the diagnosis. Ital J Neuro Sci 3, 71–74 (1982). https://doi.org/10.1007/BF02043350
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DOI: https://doi.org/10.1007/BF02043350