Abstract
We describe a family with hereditary adultonset leukodystrophy with sparing of the optic radiations, shown clinically, neuroradiologically and electrophysiologically. All five affected members developed their leukodystrophy in their fourth decade and clinical features steadily progressed. The sparing of the optic radiations is not typical of other leukodystrophies. We suggest that this is a new clinical entity.
Similar content being viewed by others
References
Myrianthopoulos NC (1981) Leukodystrophy and demyelinating disorders. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, vol. 42. North-Holland, Amsterdam, pp 481–500
Campp CD, Loewenberg K (1941) An American family with Pelizeus-Merzbacher disease. Arch Neurol Psychiatry 45: 261–264
O'Neill BP, Moser HW, Saxena KM, Marmion LC (1984) Adrenoleukodystrophy: clinical and biochemical manifestations in carriers. Neurology 34: 798–801
Moser HW, Singh I, O'Neill BP (1984) Adrenoleukodystrophy: survery of 303 cases: biochemistry, diagnosis, and therapy. Ann Neurol 16:628–641
Kurtzke JF (1983) Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology 33: 1444–1452
Eldrige R, Anayotos CP, Schlesinger S, Cowen D, Bever C, Patronas N, McFarland H (1984) Hereditary adult-onset leukodystrophy simulating chronic progressive multiple sclerosis. N Engl J Med 311:948–953
Shwankhaus JD, Patronas D, Dorwart R, Eldrige R, Schlesinger S, McFarland H (1988) Computed tomography and magentic resonance imaging in adult-onset leukodystrophy. Arch Neurol 45: 1004–1008
Cutler JR, Aminoff M, Brant-Zawadzi M (1988) Evaluation of patients with multiple sclerosis by evoked potentials and magnetic resonance imaging: a comparative study. Ann Neurol 20: 645–648
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Abe, K., Ikeda, M., Watase, K. et al. A kindred of hereditary adult-onset leukodystrophy with sparing of the optic radiations. Neuroradiology 35, 281–283 (1993). https://doi.org/10.1007/BF00602615
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00602615