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