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An Atypical Case of Progressive Supranuclear Palsy

Published online by Cambridge University Press:  18 September 2015

Andrew J. Gomori  and
Anders A.F. Sima
Andrew J. Gomori
Affiliation:
Sections of Neurology, Department of Medicine and Neuropathology, University of Manitoba and Health Sciences Centre, Winnipeg, Manitoba
Anders A.F. Sima
Affiliation:
Department of Pathology, University of Manitoba and Health Sciences Centre, Winnipeg, Manitoba
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Summary

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We report a 59 year old woman who presented with double vision, nuchal pain and mild dementia. On neurological examination she demonstrated third, sixth and seventh nerve palsies and ataxia. Following intravenous ACTH and oral prednisone therapy she showed a remarkable recovery which left her with only a left facial weakness. She remained well for two years. She then developed bulbar palsy and profound dementia. Pathological examination revealed progressive supranuclear palsy (PSP). This patient demonstrated a greater variability in the course of PSP than has previously been recognized.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 11 , Issue 1 , February 1984 , pp. 48 - 52
Copyright
Copyright © Canadian Neurological Sciences Federation 1984

References

Albert, M.L. (1978). Subcortical dementia. In: Alzheimer’s disease: senile dementia and related disorders (Aging, Vol 7), Edited by Katzman, R., Terry, R.D. and Bick, K.L., New York: Raven Press, pp. 173–180.Google Scholar
Albert, M.L., Feldman, R.G. and Willis, A.L. (1974). The subcortical dementia of progressive supranuclear palsy. J. Neurol. Neurosurg. Psychiatry, 37:121–130.CrossRefGoogle ScholarPubMed
Behrman, S., Carrol, J.D.. Janofa, I., Matthews, W.B. (1969). Progressive supranuclear palsy. Clinico-pathological study of four cases. Brain 92:663–678.Google ScholarPubMed
Bender, M.B. (1980). Brain control of conjugate horizontal and vertical eye movements. Brain, 103:23–69.CrossRefGoogle ScholarPubMed
Blumenthal, H. and Miller, C. (1969). Motor nuclear involvement in progressive supranuclear palsy. Arch. Neurol.. 20:362–367.CrossRefGoogle ScholarPubMed
David, N.J., Mackey, E.A., Smith, J.L. (1968). Further observations in progressive supranuclear palsy. Neurology 18:349–356.CrossRefGoogle ScholarPubMed
Derenzi, E. and Vignolo, L.A. (1969). L-Dopa for progressive supranuclear palsy. Lancet, 2:1359.Google Scholar
Drachman, D.A. (1981). The cholinergic system, memory and aging. In: Brain neurotransmitters and receptors in aging and age related disorders (Aging, vol. 17), Edited by Enna, S.J., Samorajski, T. and Beer, B.New York, Raven Press, pp. 255–268.Google Scholar
Haldeman, S., Goldman, J.W., Hyde, J. and Pribam, H.F.W. (1981). Progressive supranuclear palsy, computed tomography, and response to anti-parkinsonian drugs. Neurology (NY), 31:442–445.Google Scholar
Ishino, H., Higashy, H., Kuroda, S., Yabuki, S., Hayahara, T., Otsuki, S., (1974). Motor nuclear involvement in progressive supranuclear palsy. J. Neurol Sci 22:233–244.CrossRefGoogle ScholarPubMed
Jackson, J.A., Jankovic, J. and Ford, J. (1983). Progressive supranuclear palsy: Clinical features and response to treatment in 16 patients. Ann. Neurol., 13:273–278.CrossRefGoogle ScholarPubMed
Jellinger, K. (1971). Progressive supranuclear palsy (subcortical argyrophilic dystrophy). Acta. Neuropathol., (Berl) 19:347–352.CrossRefGoogle ScholarPubMed
Nuwer, M.R. (1981). Progressive supranuclear palsy despite normal eye movements. Arch. Neurol., 38:784.CrossRefGoogle ScholarPubMed
Perkin, G.D., Lees, A.J., Stern, C.M. and Kocen, R.S. (1978). Problems in the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Can. J. Neurol. Sci., 5:167–173.CrossRefGoogle ScholarPubMed
Pfaffenbach, D.D., Layton, D.D. and Kearns, T.P. (1972). Ocular manifestations in progressive supranuclear palsy. Am. J. Ophthalmol., 74:1179–1184.CrossRefGoogle ScholarPubMed
Powell, H.C., London, G.W. and Lampert, P.W. (1974). Neurofibrillary tangles in progressive supranuclear palsy: electron microscopic observations. J. Neuropathol. Exp. Neurol., 33:98–106.CrossRefGoogle ScholarPubMed
Rafal, R.C. and Grimm, R.J. (1981). Progressive supranuclear palsy: functional analysis of the response to methysergide and antiparkinsonian agents. Neurology (NY), 31:1507–1518.CrossRefGoogle ScholarPubMed
Richardson, J.D., Steele, J. and Olszewski, J. (1963). Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. Trans. Am. Neurol. Assoc. 88:25–27.Google ScholarPubMed
Roy, S., Datta, C.K., Hirano, A., Ghatak, N.R. and Zimmerman, H. (1974). Electron microscopic study of neurofibrillary tangles in Steele-Richardson-Olszewski Syndrome. Acta. Neuropathol. (Berl) 29:175–179.CrossRefGoogle ScholarPubMed
Segarra, J.M. (1970). Cerebral vascular disease and behaviour. Arch. Neurol. 22:408–418.CrossRefGoogle Scholar
Steele, J.C., Richardson, J.C. and Olszewski, J. (1964). Progressive supranuclear palsy: a heterogenous degeneration involving the brainstem, basal ganglia and cerebellum with vertical gaze and pseudo-bulbar palsy, nuchal dystonia and dementia. Arch. Neurol. 10:333–359.CrossRefGoogle Scholar
Steele, J.C. (1972). Progressive supranuclear palsy. Brain, 95:693–704.Google ScholarPubMed
Tellez-Nagel, LandWisniewski, H.M. (1973). Ultrastructure of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome. Arch. Neurol., 27:324–327.CrossRefGoogle Scholar
Tomonaga, M. (1977). Ultrastructure of neurofibrillary tangles in progressive supranuclear palsy. Acta. Neuropathol. (Berl) 37:177–181.CrossRefGoogle ScholarPubMed