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Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)

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Progressive Supranuclear Palsy: Diagnosis, Pathology, and Therapy

Part of the book series: Journal of Neural Transmission. Supplementa ((NEURAL SUPPL,volume 42))

Summary

Progressive supranuclear palsy (PSP) is characterized clinically by supranuclear gaze palsy, neck dystonia, parkinsonism, pseudobulbar palsy, gait imbalance with frequent falls and frontal lobe-type dementia. In the advanced typical case, when supranuclear gaze palsy and other main features are present diagnosis is relatively easy. Diagnostic problems, though, are frequent in the early stages due to the variable clinical presentation and in those atypical cases in which gaze palsy does not develop or that present as a severe dementig disorder or as an isolated akinetic-rigid syndrome. In this review we summarize the clinical features of PSP and emphasize those aspects helpful in the differential diagnosis with Parkinsnon’s disease and other motor and cognitive disorders that can pose difficult diagnostic problems. Clinical diagnostic criteria are also discussed and modifications of those currently in used are proposed.

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References

  • Agid Y, Javoy-Agid F, Rubert M, et al (1986) Progressive supranuclear palsy: anatomo-clinical and biochemical considerations. Adv Neurol 45: 191–206.

    Google Scholar 

  • Albert ML, Feldman RG, Willis AL (1974) The “subcortical dementia” of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 37: 121–130.

    Article  PubMed  CAS  Google Scholar 

  • Al-Din ASN, Al-Kurdi A, Al-Salem MK (1990) Autosomal recessive ataxias, slow eye movements, dementia and extrapyramidal disturbances. J Neurol Sci 96: 191–205.

    Article  PubMed  CAS  Google Scholar 

  • Anzil AP (1969) Progressive supranuclear palsy: case report with pathological findings. Acta Neuropathol (Berl) 14: 72–76.

    Article  CAS  Google Scholar 

  • Behrman S, Carroll JD, Janota I, Matthews WB (1969) Progressive supranuclear palsy. Clinico-pathological study of four cases. Brain 92: 663–678.

    Article  PubMed  CAS  Google Scholar 

  • Blin J, Baron JC, Dubois B, Pillon B, Cambon H, Cambier J, Agid Y (1990) Positron emission tomography study in progressive supranuclear palsy: brain hypometabolic pattern and clinicometabolic correlations. Arch Neurol 47: 747–752.

    Article  PubMed  CAS  Google Scholar 

  • Brusa A, Mancardi GL, Bugiani O (1979) Progressive supranuclear palsy 1979: an overview. Italian J Neurol Sci i: 205–222.

    Article  Google Scholar 

  • Cambier J, Masson M, Viader F, Limodin J, Strube A (1985) Le syndrome frontal de la paralysie supranuclĂ©aire progressive. Rev Neurol (Paris) 141: 528–536.

    CAS  Google Scholar 

  • Cruz-Sánchez FF, Rossi ML, Cardozo A, Deacon P, Tolosa E (1992) Clinical and pathological study of two patients with progressive supranuclear palsy and Alzheimer’s changes. Antigenic determinants that distinguish cortical and sub-cortical neurofibrillary tangles. Neurosci Lett 136: 43–46.

    Article  PubMed  Google Scholar 

  • Davis PH, Bergeron C, McLachlan DR (1985) Atypical presentation of progressive supranuclear palsy. Ann Neurol 17: 337–343.

    Article  PubMed  CAS  Google Scholar 

  • De Bruin VMS, Lees AJ, Daniel SE (1992) Diffuse Lewy body disease presenting with supranuclear gaze palsy, parkinsonism and dementia: a case report. Mov Disord 7: 335–358.

    Article  Google Scholar 

  • Dubas F, Gray F, Escourelle R (1983) Maladie de Steele-Richardson-Olszewski sans ophtalmoplĂ©gie: six cas anatomo-cliniques. Rev Neurol (Paris) 139: 407–416.

    CAS  Google Scholar 

  • Duvoisin RC (1987) The olivopontocerebellar atrophies. In: Marsden DC, Fahn S (eds) Movement disorders, vol 2. Butterworth, London, pp 249–271.

    Google Scholar 

  • Duvoisin RC, Golbe LI, Lepore FE (1987) Progressive supranuclear palsy. Can J Neurol Sci 14: 544–547.

    Google Scholar 

  • Fearnley JM, Revesz T, Brooks DJ, Frackowiak RSJ, Lees AJ (1991) Diffuse Lewy body disease presenting with a supranuclear gaze palsy. J Neurol Neurosurg Psychiatry 54: 159–161.

    Article  PubMed  CAS  Google Scholar 

  • Fisk JD, Goodale MA, Burkhart G, Barnett HJM (1982) Progressive supranuclear palsy: the relationship between ocular motor dysfunction and psychological test performance. Neurology 32: 698–705.

    Article  PubMed  CAS  Google Scholar 

  • Foster NL, Gilman S, Berent S, Sima AAF, D’Amato C, Koeppe RA, Hicks SP (1992) Progressive subcortical gliosis and progressive supranuclear palsy can have similar clinical and PET abnormalities. J Neurol Neurosurg Psychiatry 55: 707–713.

    Article  PubMed  CAS  Google Scholar 

  • Gibb WRG, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112: 1171–1192.

    Article  PubMed  Google Scholar 

  • Goffinet AM, De Voider AG, Gillain C, Rectem D, Bol A, Michel C, Cogneau M, Labar D, Laterre C (1989) Positron tomography demonstrates frontal lobe hypometabolism in progressive supranuclear palsy. Ann Neurol 25: 131–139.

    Article  PubMed  CAS  Google Scholar 

  • Golbe LI, Davis PH (1993) Progressive supranuclear palsy. In: Jankovic J, Tolosa E (eds) Parkinson’s disease and movement disorders. William & Wilkins, Baltimore, pp 145–161.

    Google Scholar 

  • Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC (1988) Prevalence and natural history of progressive supranuclear palsy. Neurology 38: 1031–1034.

    Article  PubMed  CAS  Google Scholar 

  • Grafman J, Litvan I, GĂłmez C, Chase TN (1990) Frontal lobe function in progressive supranuclear palsy. Arch Neurol 47: 553–558.

    Article  PubMed  CAS  Google Scholar 

  • Hughes AJ, Lees AJ, Sterm GM (1991) Challenge tests to predict the dopaminergic response in untreated Parkinson’s disease. Neurology 41: 1723–1725.

    Article  PubMed  CAS  Google Scholar 

  • Hughes AJ, Daniel SE, Kilford L, Lees AJ (1992) Accuracy of diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases. J Neurol Neurosurg Psychiatry 55: 181–184.

    Article  PubMed  CAS  Google Scholar 

  • Jackson JA, Jankovic J, Ford J (1983) Progressive supranuclear palsy: clinical features and response to treatment in 16 patients. Ann Neurol 13: 273–278.

    Article  PubMed  CAS  Google Scholar 

  • Jellinger K, Riederer P, Tomonaga M (1980) Progressive supranuclear palsy: clinico-pathological and biochemical studies. J Neural Transm [Suppl] 16: 111–128.

    Google Scholar 

  • Kish SJ, Chang LJ, Mirchandani L, Shannak K, Hornykiewicz O (1985) Progressive supranuclear palsy: relationship between extrapyramidal disturbances, dementia and brain neurotransmitter markers. Ann Neurol 18: 530–536.

    Article  PubMed  CAS  Google Scholar 

  • Koepper A, Hans M (1976) Supranuclear ophtalmoplegia in olivopontocerebellar degeneration. Neurology 26: 764–768.

    Article  Google Scholar 

  • Kristensen MO (1985) Progressive supranuclear palsy — 20 years later. Acta Neurol Scand 71: 177–189.

    Article  PubMed  CAS  Google Scholar 

  • Lees AJ (1987) The Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). In: Marsden DC, Fahn S (eds) Movement disorders, vol 2. Butterworth, London, pp 272–287.

    Google Scholar 

  • LĂ©ger JM, Girault JA, Bolgert F (1987) Deux cas de dystonie isolĂ©e d’un membre supĂ©rieur inauguant une maladie de Steele-Richardson-Olszewski. Rev Neurol (Paris) 143: 140–142.

    Google Scholar 

  • Lepore FE, Steele JC, Tilson G, Calne DB, Duvoisin RC, Lavine L, McDarby JV (1988) Supranuclear disturbances of ocular motility in Lytico-Bodig. Neurology 38: 1849–1853.

    Article  PubMed  CAS  Google Scholar 

  • Lewis AJ, Gawel MJ (1990) Diffuse Lewy body disease with dementia and oculomotor dysfunction. Mov Disord 5: 143–147.

    Article  PubMed  CAS  Google Scholar 

  • Lezak MD (1983) Neuropsychological assessment. Oxford University Press, New York.

    Google Scholar 

  • Litvan I, Grafman J, GĂłmez G, Chase TN (1989) Memory impairment in patients with progressive supranuclear palsy. Arch Neurol 46: 765–767.

    Article  PubMed  CAS  Google Scholar 

  • Maher ER, Smith EM, Lees AJ (1985) Cognitive deficits in the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). J Neurol Neurosurg Psychiatry 48: 1234–1239.

    Article  PubMed  CAS  Google Scholar 

  • Maher ER, Lees AJ (1986) The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 36: 1005–1008.

    Article  PubMed  CAS  Google Scholar 

  • Masliah E, Hansen LA, Quijada S, DeTeresa R, Alford M, Kauss J, Terry R (1991) Late onset dementia with argyrophilic grains and subcortical tangles or atypical progressive supranuclear palsy. Ann Neurol 29: 389–396.

    Article  PubMed  CAS  Google Scholar 

  • Mastaglia FL, Grainger KMR (1975) Internuclear ophtalmoplegia in progressive supranuclear palsy. J Neurol Sci 25: 303–308.

    Article  PubMed  CAS  Google Scholar 

  • Matsuo H, Takashima H, Kishinawa M, Kinoshita I, Mori M, Tsujihata M, Nagataki S (1991) Pure akinesia: an atypical manifestation of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 54: 397–400.

    Article  PubMed  CAS  Google Scholar 

  • Messert B, Van Nuis C (1966) A syndrome of paralysis of downward gaze, dysarthria, pseudobulbar palsy, rigidity of neck and trunk and dementia. J Nerv Ment Dis 143: 47–54.

    Article  PubMed  CAS  Google Scholar 

  • Milder DG, Elliott CF, Evans WA (1989) Neuropathological findings in a case of coexistent progressive supranuclear palsy and Alzheimer’s disease. Clin Exp Neurol 20: 181–187.

    Google Scholar 

  • Morariu MA (1979) Progressive supranuclear palsy and normal-pressure hydrocephalus. Neurology 29: 1544–1546.

    Article  PubMed  CAS  Google Scholar 

  • Mori H, Yoshimura M, Tomonaga M, Yamanouchi H (1986) Progressive supranuclear palsy with Lewy bodies. Acta Neuropathol 71: 344–346.

    Article  PubMed  CAS  Google Scholar 

  • Neville BRG, Lake BD, Stephens R, Sanders MD (1973) A neurovisceral storage disease with vertical supranuclear ophtalmoplegia, and its relationship to Niemann-Pick disease. Brain 96: 97–120.

    Article  PubMed  CAS  Google Scholar 

  • Olson DA, Gearing M, Watts RL, Mirra SS (1992) Clinical-pathological heterogeneity in progressive supranuclear palsy. Ann Neurol 32: 244 (abstract).

    Google Scholar 

  • Perkin GD, Lees AJ, Stern GM, Kocek RS (1978) Problems in the diagnosis of progressive supranuclear palsy. Can J Neurol Sci 5: 167–173.

    Google Scholar 

  • Pfaffenbach DD, Layton OD, Kearns TP (1972) Ocular manifestations in progressive supranuclear palsy. Am J Ophtal 74: 1174–1184.

    Google Scholar 

  • Pillon B, Dubois B, Lhermitte F, Agid Y (1986) Heterogeneity of cognitive impairment in progressive supranuclear palsy, Parkinson’s disease and Alzheimer’s disease. Neurology 36: 1179–1185.

    Article  PubMed  CAS  Google Scholar 

  • Pillon B, Dubois B (1992) Cognitive and behavioural impairments. In: Litvan I, Agid Y (eds) Progressive supranuclear palsy. Clinical and research approaches. Oxford University Press, New York, pp 223–239.

    Google Scholar 

  • Pirozzolo FJ, Jankovic J, Levy JK (1986) Progressive supranuclear palsy: are cognitive and motor deficits related. Neurology 36 [Suppl]1: 308.

    Google Scholar 

  • Probst A (1977) DĂ©gĂ©nĂ©rescence neurofibrillaire sous-corticale sĂ©nile avec prĂ©sence de tubules contournĂ©s et de filaments droits: form atypique de la paralysie supranu-clĂ©aire progressive. Rev Neurol (Paris) 133: 417–428.

    CAS  Google Scholar 

  • Rafal RD, Friedman JH (1987) Limb dystonia in progressive supranuclear palsy. Neurology 37: 1546–1549.

    Article  PubMed  CAS  Google Scholar 

  • Rafal RD, Posner MI, Friedman JH, Inhoff AW, Bernstein E (1988) Orienting of visual attention in progressive supranuclear palsy. Brain 111: 267–280.

    Article  PubMed  Google Scholar 

  • Rascol O, Clanet M, Monstastruc JL, Simonetta M, Soulier-Esteve MJ, Doyon B, Rascol A (1989) Abnormal ocular movements in Parkinson’s disease. Brain 112: 1193–1214.

    Article  PubMed  Google Scholar 

  • Oberts AH (1969) Brain damage in boxers: a study of prevalence of traumatic encephalopathy among ex-professional boxers. London, Pitman.

    Google Scholar 

  • Rosenberg B, Nyhan WL, Day C, Shore P (1976) Autosomal dominance strionigral degeneration. Neurology 26: 703–714.

    Article  PubMed  CAS  Google Scholar 

  • Ross-Russell R (1980) Supranuclear palsy of eyelid closure. Brain 103: 71–82.

    Article  Google Scholar 

  • Sasaki S, Maruyama S, Toyoda Ch (1991) A case of progressive supranuclear palsy with widespread senile plaques. J Neurol 238: 345–348.

    Article  PubMed  CAS  Google Scholar 

  • Schwarz J, Tatsch K, Arnold G, Gasser T, Trenkwalder C, Kirsch CM, Oertel WH (1992) 123-Iodobenzamide-SPECT predicts dopaminergic responsiveness in patients with de novo parkinsonism. Neurology 42: 556–561.

    Article  PubMed  CAS  Google Scholar 

  • Sonies BC (1992) Swallowing and speech disturbances. In: Litvan I, Agid Y (eds) Progressive supranuclear palsy. Clinical and research approaches. Oxford University Press, New York, pp 240–253.

    Google Scholar 

  • Steele JC, Richardson JC, Olszewski J (1964) Progressive supranuclear palsy. A heterogeneous degeneration involving the brainstem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 10: 333–358.

    Article  PubMed  CAS  Google Scholar 

  • Steele JC (1975) Progressive supranuclear palsy. In: Vinken PJ, Bruyn GW, deJong JMB (eds) Handbook of clinical neurology, vol 22. System disorders and atrophies, part II. North-Holland, Amsterdam, pp 217–229.

    Google Scholar 

  • Steele JC, Guzman T (1987) Observations about amyotrophic lateral sclerosis and the Parkinsonism-Dementia complex of Guam with regard to epidemiology and etiology. Can J Neurol Sci 14: 358–362.

    PubMed  CAS  Google Scholar 

  • Tripp JH, Lake BD, Young E, et al (1977) Juvenile Gaucher’s disease with horizontal gaze palsy in 3 siblings. J Neurol Neurosurg Psychiatry 40: 470–478.

    Article  PubMed  CAS  Google Scholar 

  • Troost BT, Daroff RB (1977) The ocular motor defects in progressive supranuclear palsy. Ann Neurol 2: 297–403.

    Article  Google Scholar 

  • Yamamoto T, Kawamura J, Hashimoto S, Nakamura M, Iwamoto H, Kobashi Y, Ichijima K (1990) Pallido-nigro-luysian atrophy, progressive supranuclear palsy and adult onset Hallervorden-Spatz disease: a case of akinesia as a predominant feature of parkinsonism. J Neurol Sci 101: 98–106.

    Article  Google Scholar 

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Authors and Affiliations

  1. Neurology Department, Hospital Clinic, Faculty of Medicine, University of Barcelona, Spain

    Prof. E. Tolosa, F. Valldeoriola & M. J. Marti

  2. Neurology Department, Hospital Clinic, Villarroel 170, E-08036, Barcelona, Spain

    Prof. E. Tolosa

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  1. Prof. E. Tolosa
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  2. F. Valldeoriola
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  3. M. J. Marti
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Editors and Affiliations

  1. Neurology Service, Hospital Clinic, Faculty of Medicine, University of Barcelona, Spain

    E. Tolosa

  2. Neurology Service, Robert Wood Johnson University Hospital, New Brunswick, NJ, USA

    R. Duvoisin

  3. Neurological Tissue Bank, Faculty of Medicine, University of Barcelona, Spain

    F. F. Cruz-Sánchez

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© 1994 Springer-Verlag Wien

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Tolosa, E., Valldeoriola, F., Marti, M.J. (1994). Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). In: Tolosa, E., Duvoisin, R., Cruz-Sánchez, F.F. (eds) Progressive Supranuclear Palsy: Diagnosis, Pathology, and Therapy. Journal of Neural Transmission. Supplementa, vol 42. Springer, Vienna. https://doi.org/10.1007/978-3-7091-6641-3_2

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  • DOI: https://doi.org/10.1007/978-3-7091-6641-3_2

  • Publisher Name: Springer, Vienna

  • Print ISBN: 978-3-211-82541-9

  • Online ISBN: 978-3-7091-6641-3

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