Abstract
A 46 year old woman had a relapsing-remitting course of hemiparesis, disorientation, paraparesis and seizures, followed by progressive dementia, spasticity and ataxia. Computed tomography at onset showed a parietotemporal hypodense area with diffuse mottled enhancement obliterating the lateral ventricle. Subsequent scans demonstrated symmetric periventricular non-enhancing hypodensities, progressive ventricular enlargement and atrophy. Adult metachromatic leukodystrophy was diagnosed on the basis of low leukocyte arylsulphatase A level and metachromatic material accumulation at neural nerve biopsy.
Full text
PDF



Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Austin J., Armstrong D., Fouch S., Mitchell C., Stumpf D., Shearer L., Briner O. Metachromatic leukodystrophy (MLD). 8. MLD in adults; diagnosis and pathogenesis. Arch Neurol. 1968 Mar;18(3):225–240. doi: 10.1001/archneur.1968.00470330015001. [DOI] [PubMed] [Google Scholar]
- Barnes D. M., Enzmann D. R. The evolution of white matter disease as seen on computed tomography. Radiology. 1981 Feb;138(2):379–383. doi: 10.1148/radiology.138.2.7455118. [DOI] [PubMed] [Google Scholar]
- Bosch E. P., Hart M. N. Late adult-onset metachromatic leukodystrophy. Dementia and polyneuropathy in a 63-year-old man. Arch Neurol. 1978 Jul;35(7):475–477. doi: 10.1001/archneur.1978.00500310077017. [DOI] [PubMed] [Google Scholar]
- Finelli P. F. Metachromatic leukodystrophy manifesting as a schizophrenic disorder: computed tomographic correlation. Ann Neurol. 1985 Jul;18(1):94–95. doi: 10.1002/ana.410180117. [DOI] [PubMed] [Google Scholar]
- Hoes M. J., Lamers K. J., Hommes O. R., ter Haar B. Adult metachromatic leukodystrophy. Arylsulphatase-A values in four generations of one family and some reflections about the genetics. Clin Neurol Neurosurg. 1978;80(3):174–188. doi: 10.1016/s0303-8467(78)80039-0. [DOI] [PubMed] [Google Scholar]
- Manowitz P., Kling A., Kohn H. Clinical course of adult metachromatic leukodystrophy presenting as schizophrenia. A report of two living cases in siblings. J Nerv Ment Dis. 1978 Jul;166(7):500–506. doi: 10.1097/00005053-197807000-00005. [DOI] [PubMed] [Google Scholar]
- Reider-Grosswasser I., Bornstein N. CT and MRI in late-onset metachromatic leukodystrophy. Acta Neurol Scand. 1987 Jan;75(1):64–69. doi: 10.1111/j.1600-0404.1987.tb07891.x. [DOI] [PubMed] [Google Scholar]
- Skomer C., Stears J., Austin J. Metachromatic leukodystrophy (MLD). XV. Adult MLD with focal lesions by computed tomography. Arch Neurol. 1983 Jun;40(6):354–355. doi: 10.1001/archneur.1983.04050060054009. [DOI] [PubMed] [Google Scholar]
- Tagliavini F., Pietrini V., Pilleri G., Trabattoni G., Lechi A. Adult metachromatic leucodystrophy: clinicopathological report of two familial cases with slow course. Neuropathol Appl Neurobiol. 1979 May-Jun;5(3):233–243. doi: 10.1111/j.1365-2990.1979.tb00622.x. [DOI] [PubMed] [Google Scholar]
- Waltz G., Harik S. I., Kaufman B. Adult metachromatic leukodystrophy. Value of computed tomographic scanning and magnetic resonance imaging of the brain. Arch Neurol. 1987 Feb;44(2):225–227. doi: 10.1001/archneur.1987.00520140083022. [DOI] [PubMed] [Google Scholar]


