Parkinson-plus syndromes

Last updated: September 6, 2022

Summarytoggle arrow icon

Parkinson-plus syndromes (or atypical parkinsonism) are a group of neurodegenerative diseases that present with parkinsonism and a variety of additional features. Depending on the particular syndrome, a combination of basal ganglia, cerebral cortical, cerebellar, midbrain, and/or brainstem structures are affected. The prognosis is less favorable than in Parkinson disease. Parkinson-plus syndromes should be considered if parkinsonism does not respond to levodopa treatment, if dementia progresses rapidly, or if gait instability occurs early in the course of the disease. In most cases, only symptomatic treatment is possible.

Common characteristics of Parkinson-plus syndromestoggle arrow icon

Parkinson-plus syndromes have a number of features that differentiate them from Parkinson disease (PD). [1]

Dementia with Lewy bodiestoggle arrow icon

Patients with Lewy body dementia have visual hallewynations.

Patients with Lewy-body dementia have an increased risk of life-threatening akinetic crises under antipsychotic treatment.

Multiple system atrophy (MSA)toggle arrow icon

Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)toggle arrow icon

Corticobasal degenerationtoggle arrow icon

  • Epidemiology [11]
    • Incidence: 1 case per 100,000 individuals/year
    • Mean age of onset: 64 years
  • Clinical features
    • Asymmetric motor abnormalities, often initially affecting only one limb
    • Alien limb phenomenon: The patient perceives the affected limb as not belonging to him or her.
    • Dementia (at any stage)
  • Diagnostics: MRI shows asymmetric focal cortical atrophy and bilateral atrophy of the basal ganglia [12]
  • Pathology [11]
  • Prognosis: estimated mean survival is ∼ 6.5 years [11]

Important differentiating signs of Parkinson-plus syndromestoggle arrow icon

Differential diagnoses of Parkinson-plus syndromes
Clinical features MRI Pathology
Macroscopic Microscopic
Multiple system atrophy (MSA)
  • Atrophy of olivopontocerebellar and striatonigral systems
Progressive supranuclear palsy (PSP)
  • Lesions in the following areas:
Corticobasal degeneration (CBD)
Dementia with Lewy bodies (DLB)

Referencestoggle arrow icon

  1. Mitra K, Gangopadhaya PK, Das SK. Parkinsonism plus syndrome: a review. Neurol India. 2003; 51 (2): p.183-188.
  2. Vanacore N, Bonifati V, Fabbrini G, et al. Epidemiology of multiple system atrophy. Neurological Sciences. 2001; 22 (1): p.97-99.doi: 10.1007/s100720170064 . | Open in Read by QxMD
  3. Hot cross bun sign (pons). Updated: January 1, 2017. Accessed: July 13, 2017.
  4. Ahmed Z, Asi YT, Sailer A, et al. The neuropathology, pathophysiology and genetics of multiple system atrophy. Neuropathol Appl Neurobiol. 2012; 38 (1): p.4-24.doi: 10.1111/j.1365-2990.2011.01234.x . | Open in Read by QxMD
  5. Booth TC, Nathan M, Waldman AD, Quigley AM, Schapira AH, Buscombe J. The Role of Functional Dopamine-Transporter SPECT Imaging in Parkinsonian Syndromes, Part 2. Am J Neuroradiol. 2014.doi: 10.3174/ajnr.A3971 . | Open in Read by QxMD
  6. Pure Autonomic Failure. Updated: March 1, 2017. Accessed: July 5, 2017.
  7. Multiple System Atrophy Fact Sheet. Updated: April 22, 2020. Accessed: December 28, 2020.
  8. Constantinides VC, Paraskevas GP, Paraskevas PG, Stefanis L, Kapaki E. Corticobasal degeneration and corticobasal syndrome: A review. Clinical Parkinsonism & Related Disorders. 2019; 1: p.66-71.doi: 10.1016/j.prdoa.2019.08.005 . | Open in Read by QxMD
  9. Corticobasal degeneration. . Accessed: March 11, 2019.
  10. Ferman TJ, Boeve BF. Dementia with Lewy bodies. Neurol Clin. 2007; 25 (3): p.741.doi: 10.1016/j.ncl.2007.03.001 . | Open in Read by QxMD
  11. Mak E, Su L, Williams GB, O’Brien JT. Neuroimaging characteristics of dementia with Lewy bodies. Alzheimers Res Ther. 2014; 6 (2): p.18.doi: 10.1186/alzrt248 . | Open in Read by QxMD
  12. Golbe LI. The Epidemiology of Progressive Supranuclear Palsy. Elsevier ; 2008: p. 457-459

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