Abstract

Case Report

A case report of Multi System Atrophy (MSA) with cross over features of Progressive Supranuclear Palsy (PSP)

Judah Rajendran R*, Ramesh Kannan and Hannah Ranjee Prasanth

Published: 13 September, 2021 | Volume 5 - Issue 2 | Pages: 019-022

We describe an interesting case of Multi System Atrophy who had cross over features of progressive supranuclear palsy along with classical clinical findings which led to the diagnosis.

Read Full Article HTML DOI: 10.29328/journal.ijceo.1001038 Cite this Article Read Full Article PDF

Keywords:

Progressive supranuclear palsy; Parkinson’s disease; Diplopia; Vertical gaze palsy; Multisystem atrophy

References

  1. Respondek G, Levin J, Höglinger GU. Progressive supranuclear palsy and multiple system atrophy: clinicopathological concepts and therapeutic challenges. Curr Opin Neurol. 2018; 31: 448-454. PubMed: https://pubmed.ncbi.nlm.nih.gov/29746401/
  2. Schrag A, Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet. 1999 20; 354: 1771-1775. PubMed: https://pubmed.ncbi.nlm.nih.gov/10577638/
  3. Bower JH, Maraganore DM, McDonnell SK, Rocca WA. Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990. Neurology. 1997; 49: 1284-1288. PubMed: https://pubmed.ncbi.nlm.nih.gov/9371909/
  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. PubMed: https://pubmed.ncbi.nlm.nih.gov/14107684/
  5. Joutsa J, Gardberg M, Röyttä M, Kaasinen V. Diagnostic accuracy of Parkinsonism syndromes by general neurologists. Parkinsonism Relat Disord. 2014; 20: 840-844. PubMed: https://pubmed.ncbi.nlm.nih.gov/24816002/
  6. Cosseddu M, Benussi A, Gazzina S, Manes MA, Dell'Era V, et al. Natural history and predictors of survival in progressive supranuclear palsy. J Neurol Sci. 2017; 382: 105-107. PubMed: https://pubmed.ncbi.nlm.nih.gov/29111000/
  7. Glasmacher SA, Leigh PN, Saha RA. Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry. 2017; 88: 402-411. PubMed: https://pubmed.ncbi.nlm.nih.gov/28250027/
  8. Schrag A, Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet. 1999; 354: 1771–1775. PubMed: https://pubmed.ncbi.nlm.nih.gov/10577638/
  9. Takanashi M, Ohta S, Matsuoka S, Mori H, Mizuno Y. Mixed multiple system atrophy and progressive supranuclear palsy: a clinical and pathological report of one case. Acta Neuropathol (Berl). 2020; 103: 82–87. PubMed: https://pubmed.ncbi.nlm.nih.gov/11837750/
  10. Gearing M, Olson DA, Watts RL, Mirra SS. Progressive supranuclear palsy: neuropathologic and clinical heterogeneity. Neurology 1994; 44: 1015–1024. PubMed: https://pubmed.ncbi.nlm.nih.gov/8208392/
  11. Dickson DW, Lin WL, Liu WK, Yen SH. Multiple system atrophy: a sporadic synucleinopathy. Brain Pathol. 1999; 9: 721–732. PubMed: https://pubmed.ncbi.nlm.nih.gov/10517510/
  12. Bower JH, Maraganore DM, McDonnell SK, Rocca WA. Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976–1990. Neurology. 1997; 49: 1284–1288. PubMed: https://pubmed.ncbi.nlm.nih.gov/9371909/
  13. Winter Y, Spottke AE, Stamelou M, et al. Health-related quality of life in multiple system atrophy and progressive supranuclear palsy. Neurodegener Dis. 2011; 8: 438-446. PubMed: https://pubmed.ncbi.nlm.nih.gov/21576919/
  14. Glasmacher SA, Leigh PN, Saha RA. Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry. 2017; 88: 402-411. PubMed: https://pubmed.ncbi.nlm.nih.gov/28250027/
  15. Multiple System Atrophy Research Collaboration. Mutations in COQ2 in familial and sporadic multiple system atrophy. N Engl J Med. 2013; 369: 233-234. PubMed: https://pubmed.ncbi.nlm.nih.gov/23758206/
  16. Scholz SW, Houlden H, Schulte C, Sharma M, Li A, et al. SNCA variants are associated with increased risk for multiple system atrophy. Ann Neurol. 2009; 65: 610-614. PubMed: https://pubmed.ncbi.nlm.nih.gov/19475667/
  17. Sailor A, Schlz SW, Nalls MA, Schulte C, Federoff M, et al. A genome- wide association study in multiple system atrophy. Neurology. 2016; 87: 1591-1598. PubMed: https://pubmed.ncbi.nlm.nih.gov/27629089/
  18. Vilarino-Guell C, Soto-Ortolaza Al, Rajput A, Mash DC, Papapetropoulos S, et al. MAPT H1 haplotype is a risk factor for essential tremor and multiple system atrophy. Neurology. 2011; 76: 670-672. PubMed: https://pubmed.ncbi.nlm.nih.gov/21321341/
  19. Labbe C, Heckman MG, Lorenzo-Betancor O, Murray ME, Ogaki K, et al. MAPT haplotype diversity in multiple system atrophy, Parkinsonism Relat Disord. 2016; 30: 40-45. PubMed: https://pubmed.ncbi.nlm.nih.gov/27374978/
  20. Donker Kaat L, Boon AJ, Azmani A, Kamphorst W, Breteler MMB, et al. Familial aggregation of parkinsonism in progessive Supranuclear Palsy. Neurology. 2009; 73: 98-105. PubMed: https://pubmed.ncbi.nlm.nih.gov/19458322/
  21. Heckman MG, Brennan RR, Labbe C, Soto AI, Koga S, et al. Association of MAPT subhaplotypes with Risk of Progressive Supranuclear Palsy and Severity of Tau Pathology. JAMA Neurol. 2019; 76: 710-717. PubMed: https://pubmed.ncbi.nlm.nih.gov/30882841/
  22. Im SY, Kim YE, Kim YJ. Genetics of Progressive Supranuclear Palsy. J Mov Disord. 2015; 8: 122-129. PubMed: https://pubmed.ncbi.nlm.nih.gov/26413239/

Similar Articles

Recently Viewed

Read More

Most Viewed

Read More

Help ?