Parkinson's Plus Syndrome

FACTS

  • The terms “parkinsonism” or “parkinsonian” are not diagnoses, but rather meaning slow, stiff, or tremor-like movements, affected speech, and change in gait or walking pattern.
  • There are many causes of parkinsonism, including Parkinson’s disease (PD) and certain gastrointestinal and psychiatric medications.
  • Parkinson-plus syndromes are distinct neurodegenerative diseases that resemble PD but have some additional challenging features and different changes in the brain.
  • These disorders typically do not respond to levodopa, the mainstay of PD treatment.
  • Parkinson-plus syndromes tend to have a poor prognosis.

TYPES

Progressive supranuclear palsy (PSP) 

  • Symmetric slowness and stiffness with very early loss of balance and subsequent falls
  • Impaired eye movements that cause double vision
  • Tremors usually not present
  • Often stiff, rigid neck
  • Spontaneous, unprovoked laughing and crying
  • Possible shrinkage in the midbrain

Multiple System Atrophy (MSA) 

  • Frequent and severe drops in blood pressure, which cause fainting spells
  • Incontinence
  • MSA-P (parkinsonian variant) – A slow and stiff subset
  • MSA-C (cerebellar variant) – A subset with poor coordination and slurred speech
  • Possible shrinkage in the cerebellum

Parkinson’s Disease or Lewy body Dementia (PDD or LBD)

  • Appears as a cross between Alzheimer’s and Parkinson’s
  • Cognitive problems include:
  • Memory loss
  • Visual hallucinations
  • Delusions
  • Frequent changes in level of alertness
  • Physical signs of Parkinsonism including slow movement, stiffness, and tremors develop a year or more after the cognitive issues begin
  • Can be overly sensitive to medications

Corticobasal syndrome or corticobasal degeneration

  • Also called useless hand syndrome
  • Rigidity and jerking movements on one side of the body
  • May experience alien limb phenomenon where one arm or one leg will move without conscious control of the individual
  • Possible shrinkage in various lobes of the brain resulting in difficulty with language, visuospatial function, or behavior

Vascular Parkinsonism

  • Symptoms result from multiple small strokes in the basal ganglia
  • Multiple small strokes may precede the signs and symptoms of Parkinsonism
  • Usually affect both sides of the body
  • Usually no tremors
  • Sometimes there is lower body Parkinsonism, in which only the gait is affected but the upper body is spared
  • Often resembles PSP (described above)
  • Brain scans show multiple tiny strokes scattered throughout the basal ganglia

Wilson’s disease

  • Rare disorder of copper metabolism causing accumulation of copper in the brain, liver, and eyes
  • Important to screen anyone younger than 40 with a movement disorder, because it may be curable
  • Symptoms are a combination of:
    • Movement disorder
    • Cognitive decline
    • Severe psychiatric problems
  • Symptoms include:
    • Slowness
    • Stiffness
    • Tremors
    • Uncoordinated movement
    • Abnormal twisting muscle contractions
  • Diagnosed with blood and urine tests, and an eye exam to look for copper deposits