Summary
The computed tomography (CT) findings in a verified case of neuronal ceroid lipofuscinosis (NCL) are presented. CT revealed diffuse and severe cerebral atrophy, reflected by generalized subarachnoid space enlargement and symmetric ventricular dilation. There was no evidence of abnormalities of the white matter. The CT features in our case of NCL correspond perfectly with the neuropathologic changes of the disease mentioned in the literature. Furthermore, CT is of considerable help in differentiating between those inherited metabolic brain diseases characterized primarily by white matter involvement and those presenting predominantly with changes of grey matter.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Lane, B., Carroll, B. A., Pedley, T. A.: Computerized cranial tomography in cerebral diseases of white matter. Neurology 28, 534–544 (1978)
Robertson, Jr., W. C., Gomez, M. R., Reese, D. F., Okazaki, H.: Computerized tomography in demyelinating disease of the young. Neurology 27, 838–842 (1977)
Heinz, E. R., Drayer, B. P., Haenggeli, Ch. A., Painter, M. J., Grumrine, P.: Computed tomography in white matter disease. Radiology 130, 371–378 (1979)
Buananno, F. S., Ball, M. R., Laster, W., Moody, D. M., McLean, W. T.: Computed tomography in late infantile metachromatic leukodystrophy. Ann. Neurol. 4, 43–46 (1978)
Eiben, R. M., Di Chiro, G.: Computer assisted tomography in adrenoleukodystrophy. J. Comput. Assist. Tomogr. 1, 308–314 (1977)
Duda, E. E., Huttenlocher, P. R.: Computed tomography in adrenoleukodystrophy. Correlation of radiological and histological findings. Radiology 120, 349–350 (1976)
Furase, M., Obayashi, T., Tsuji, S., Miyatake, T.: Adrenoleukodystrophy. A correlative analysis of computed tomography and radionuclide studies. Radiology 126, 707–710 (1978)
Huckman, M. S., Fox, J., Topel, J.: The validity of criteria for the evaluation of cerebral atrophy by computed tomography. Radiology 116, 85–92 (1975)
Friede, R. L.: Developmental neuropathology, pp. 408–423. Berlin, Heidelberg, New York: Springer-Verlag 1975
Batten, F. E.: Family cerebral degeneration with macular change (so-called juvenile form of family amaurotic idiocy). Q. J. Med. 7, 444–454 (1914)
Kristenson, K., Rayner, S., Sourander, P.: Visceral involvement in juvenile amaurotic idiocy. Acta Neuropathol. (Berl.) 4, 421–424 (1965)
Armstrong, D., Dimmitt, S., Van Wormer, D. E.: Studies in batten disease. I. Peroxidase deficiency in granulocytes. Arch. Neurol. 30, 144–152 (1974)
Dekaban, A. S., Herman, M. M.: Childhood, juvenile, and adult cerebral lipodoses. Are these different nosological entities? Arch. Pathol. Lab. Med. 97, 65–73 (1974)
Zeman, W., Siakotos, A. N.: The neuronal ceroid lipofuscinoses. In: Lysosomal and storage diseases. (Eds., H. G. Hers, F. Van Hoof), pp. 519–551. New York: Academic Press 1973
Zeman, W., Dyken, P.: Neuronal ceroid lipofuscinosis (Batten's disease): relationship to amaurotic family idiocy? Pediatrics 44, 570–583 (1969)
Wolman, M.: A histochemical study of various forms of cerebral lipidoses. J. Clin. Pathol. 15, 324–327 (1962)
Haltia, M., Rapoda, J., Santavuori, P.: Infantile type of socalled neuronal ceroid-lipofuscinosis. Histological and electron microscopic studies. Acta Neuropathol. (Berl.) 26, 157–170 (1973)
Haberland, C., Brunngraber, E. G., Witting, L. A., Hof, H.: Late infantile amaurotic idiocy (LIAI). Anatomicochemical report of a case. Neurology (Minneap.) 22, 305–311 (1972)
Herman, M. M., Rubinstein, L. J., McKhann, G. M.: Additional electron microscopic observations on two cases of Batten-Spielmayer-Vogt disease (neuronal ceroid-lipofuscinosis). Acta Neuropathol. (Berl.) 19, 265–270 (1971)
Arimitsu, T., Di Chiro G., Brooks, R. A., Smith, P. B.: Whitegray matter differentiation in computed tomography. J. Comput. Assist. Tomogr. 1, 437–442 (1977)
Seay, A. R., Bray, P. F., Wing, S. D., Thompson, J. A., Bale, J. F., Williams, D. M.: CT scans in Menkes Disease. Neurology 29, 304–312 (1979)
Merland, J. J., Chivas, J., Melki, J. P., Cassan, J. L.: Etude tomodensitométrique dans la maladie de Wilson. Neuroradiology 16, 269–270 (1978)
Boltshauser, E., Spiess, H., Isler, W.: Computer tomography in neurodegenerative disorders in childhood. Neuroradiology 16, 41–44 (1978)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Valavanis, A., Friede, R.L., Schbiger, O. et al. Computed tomography in neuronal ceroid lipofuscinosis. Neuroradiology 19, 35–38 (1980). https://doi.org/10.1007/BF00369086
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00369086