Summary
We report computed tomography (CT) appearance of two patients with Krabbe's disease. The most common findings included severe brain atrophy: an enlarged frontal extracerebral space, dilatation of ventricles, enlarged cisterns and enlarged cortical sulci. There was low attenuation in the corpus medullaris of the cerebellum, and symmetrical focal hypodensity in the central periventricular white matter. CT at the terminal stage (16 months) showed marked cerebral atrophy including flattening of the heads of caudate nuclei and widening of the third ventricles confirmed by a neuropathological study. There was relatively less low density on the white matter of Krabbe's disease compared with that of other leukodystrophies. These CT findings may be useful in the diagnosis. The relative lack of low density in the white matter of Krabbe's disease might be related to severe gliosis and reduced total lipid contents.
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References
Buonanno FS, Ball MR, Laster W, Moody DM, Molean WT (1978) Computed tomography in late infantile metachromatic leukodystrophy. Ann Neurol 4: 43–46
Duda EE, Huttenlocher PR (1976) Computed tomography in adrenoleukodystrophy. Radiology 120: 349–350
Robertson WC Jr, Gometz MR, Reese DF, Okazaki H (1977) Computed tomography in demyelinating disease of the young. Neurology 27: 838–842
Lane B, Carrol BA, Pedley TA (1978) Computerized cranial tomography in cerebral disease of white matter. Neurology 8: 534–544
Heinz ER, Drayer BP, Haenggel CA, Painter MJ, Crumrine P (1979) Computed tomography in white-matter disease. Neuroradiology 130: 371–378
Statz A, Boltshauser E, Schinzel A, Spiess H (1981) Computed tomography in Pelizaeus-Merzbacher disease. Neuroradiology 22: 103–105
Boltshauser E, Spiess H, Isler W (1978) Computed tomography in neurodegenerative disorders of childhood. Neuroradiology 16: 41–43
Holland IM, Kendall BE (1980) Computed tomography in Alexander's disease. Neuroradiology 20: 103–106
Kendall BE, Kingsley D (1978) The value of computerized axial tomography (CAT) in craniocerebral malformations. Br J Radiol 51: 171–190
Tanaka H, Enomoto F, Oka S, Arima M (1978) Globoid cell leukodystrophy: enzymatic diagnosis in two families, and enzyme replacement therapy. Brain Nerve 30: 727–733
Eto Y, Suzuki K (1971) Brain sphingoglycolipids in Krabbe's globoid cell leucodystrophy. J Neurochem 18: 503–511
Kamoshita S, Rapin I, Suzuki K, Suzuki K (1968) Spongy degeneration of the brain: a chemical study of two cases including isolation and characterization of myelin. Neurology 18: 975–985
Volk BW, Adachi M (1970) Diffuse cerebral sclerosis-Krabbe type. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, Vol 10. North-Holland, Amsterdam, pp 67–93
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Ieshima, A., Eda, I., Matsui, A. et al. Computed tomography in Krabbe's disease: Comparison with neuropathology. Neuroradiology 25, 323–327 (1983). https://doi.org/10.1007/BF00439212
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DOI: https://doi.org/10.1007/BF00439212