• Clinical science

Parkinson-plus syndromes

Summary

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 syndromes

Parkinson-plus syndromes have a number of features that differentiate them from Parkinson disease (PD):

References:[1][2]

Dementia with Lewy bodies

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

References:[3][4][5][6]

Multiple system atrophy (MSA)

  • Differential diagnosis
  • Therapy: no causal therapy

References:[7][8][9][10][11][12][13]

Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)

References:[7][14]

Corticobasal degeneration

  • Clinical features
    • Dementia (at any stage)
    • 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.
  • Diagnosis: : asymmetric focal cortical atrophy and bilateral atrophy of the basal ganglia on MRI

References:[7][15]

Important differentiating signs of Parkinson-plus syndromes

Differential diagnoses of Parkinson-plus syndromes
Multiple system atrophy (MSA) Progressive supranuclear palsy (PSP) Corticobasal degeneration (CBD) Dementia with Lewy bodies (DLB)
  • Lewy bodies
  • Autonomic dysfunction with urogenital problems
  • Asymmetric motor symptoms
  • Alien limb phenomenon