#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Parkinson’s Disease with a Phenotype of Progressive Supranuclear Palsy – a Case Report


Authors: K. Farníková 1;  J. Ehrmann 2;  L. Tučková 2;  P. Kaňovský 1
Authors‘ workplace: LF UP a FN Olomouc Centrum pro diagnostiku a léčbu neurodegenerativních onemocnění, Neurologická klinika 1;  LF UP a FN Olomouc Ústav patologie 2
Published in: Cesk Slov Neurol N 2011; 74/107(6): 695-699
Category: Case Report

Overview

Current clinical diagnostic criteria for Parkinson’s disease and progressive supranuclear palsy (PSP) are considered relatively safe tools for the clinical differentiation of the two neurodegenerative diseases; neither diagnosis includes the possibility that Parkinson’s disease might duplicate the clinical genotype of PSP. We document the case of a male patient who first manifested parkinsonian signs at the age of 56 years. Parkinsonian syndrome developed over the course of the following three years without tremor, but with typical subcortical dementia, gaze palsy and impairment of conjugated gaze upwards, eyelid opening apraxia and gait apraxia. The disease then progressed to sudden falls, typical Lhermitte’s frontal signs, utilizations and imitations. Response to L-DOPA therapy disappeared relatively quickly. The findings of paraclinical examinations (MRI, electrophysiology) also suggested a diagnosis of PSP. After approximately four years, the patient died of intercurrent disease. Pathological and histopathological examinations and evaluation of the results by two independent pathologists documented and confirmed changes typical of Parkinson’s disease. This case indubitably raises the question of overlap of phenotypes of different types of neurodegenerative diseases with parkinsonian syndrome dominant.

Key words:
Parkinson’s disease – progressive supranuclear palsy – Lewy bodies – synucleinopathy


Sources

1. Hughes AJ, Daniel SE, Ben-Shlomo Y, Lees AJ. The accuracy of diagnosis of parkinsonian syndromes in a specialist movement disorder service. Brain 2002; 125(Pt 4): 861–870.

2. Grambalová Z, Hluštík P, Heřman M, Kaňovský P. Přítomnost tzv. typických MR nálezů u multisystémové atrofie a progresivní supranukleární paralýzy – retrospektivní pilotní studie. Cesk Slov Neurol N 2010; 73/106(5): 538–541.

3. Richardson JC, Steele J, Oslzewski J. Supranuclear ophtamoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of “heterogenous system degeneration“. Trans Am Neurol Assoc 1963; 88: 25–29.

4. Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy: a heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964; 10: 333–359.

5. Ling H, O’Sullivan SS, Holton JL, Revesz T, Massey LK, Williams DR et al. Does corticobasal degeneration exist? A clinicopathological re-evaluation. Brain 2010; 133(Pt 7): 2045–2057.

6. Abhinav K, Marsch L, Crain B, Reich SG, Biglan K. Co-existence of Parkinson’s disease and progressive supranuclear palsy: case report and a review of the literature. Neurol Sci 2011; 32(1): 159–163.

7. Wadia PM, Lang AE. The many faces of corticobasal degeneration. Parkinsonism Relat Disord 2007; 13 (Suppl 3): S336–S340.

8. Wakabayashi K, Takahashi H. Pathological heterogeneity in progressive supranuclear palsy and corticobasal degeneration. Neuropathology 2004; 24(1): 79–86.

9. Mori H, Yoshimura M, Tomonaga M, Yamanouchi H. Progressive supranuclear palsy with Lewy bodies. Acta Neuropathol 1986; 71(3–4): 344–346.

10. Fearnley JM, Revesz T, Brooks DL, Frackowiak RS, Lees AJ. Diffuse Lewy body disease presenting with a supranuclear gaze palsy. J Neurol Neurosurg Psychiatry 1991; 54(2): 159–161.

11. Gearing M, Olson DA, Watts RL, Mirra SS. Progressive supranuclear palsy: neuropathologic and clinical heterogeneity. Neurology 1994; 44(6): 1015–1024.

12. Judkins AR, Forman MS, Uryu K, Hinkle DA, Asbury AK, Lee VM et al. Co-occurrence of Parkinson’s disease with progressive supranuclear palsy. Acta Neuropathol 2002; 103(5): 526–530.

13. Josephs KA, Petersen RC, Knopman DS, Boeve BF, Whitwell JL, Duffy JR et al. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology 2006; 66(1): 41–48.

14. Galpern WR, Lang AE. Interface between tauopathies and synucleinopathies: a tale of two proteins. Ann Neurol 2006; 59(3): 449–458.

15. Strupp M. Corticobasal syndrome: a field of uncertainty. J Neurol 2011; 258(1): 173–175.

Labels
Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 6

2011 Issue 6

Most read in this issue
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#