Parkinsonian Syndromes




    Parkinson Disease

    • Difficult diagnosis
      • Response to levodopa challenge. (side effects, poor Sn and Sp)
      • Physical Exam the best for initial dx.
    • Pathology:
      • Depletion of brain stem dopaminergic neurons in the substantia nigra (they are pigmented neurons) and appearance of intracytoplasmic inclusions called Lewy bodies.
      • Suspected causes: viral infections, environmental toxins, oxidative stress, heredity.
      • Symptoms appear when 70-80% of dopamine is lost.
    • Classic: TRAP
      • Tremor at rest
      • Bradykinesia
      • Akinesia (Rigidity)
      • Postural Instability
    • Most frequent misdiagnosis:
      • Progressive Supranuclear Palsy
      • Multisystem Atrophy (MSA)  [ Includes:
        • Shy-Drager syndrome
        • Olivopontocerebellar atrophy
        • Striatonigral degeneration
      • Dementia with Lewy Bodies
    • Parkinson's Disease vs. Parkinsonism:
      • Parkinsonism is 2 or more of (Tremor, Rigidity, Bradykinesia)
      • Parkinson Disease is a form of primary or idiopathic parkinsonism.


      • Nonspecific generalized malaise, easy fatigability, subtle personality changes. (years before main symptoms).
      • Other secondary manifestations:
        • Disordered sleep (42%)
        • Constipation (50%)
        • Pain (50%)
        • Depression (40%)
        • Dementia (20%)
      • Signs begin unilaterally --> progress asymmetrically.
    • TREMOR (75%)
      • Sx:
        • 75% of PD pts.
        • Usually at rest in upper extremity, visible oscillations 4-6 Hz
        • Disappears during sleep and movement.
        • Increases during emotional distress/anxiety.
        • "Pill-rolling" quality. (index finger flexes/extends against thumb).
      • Involuntary stiffness of skeletal muscles.
      • Electromyogram: alternating discharge pattern in opposing muscle groups (i.e. triceps+biceps)
      • Resistance to movement can be smooth or interrupted.
      • Cogwheeling: jerky motion of limbs as constant force is applied.
      • Spasticity: selective increase in tone of flexor muscles in arms and extensor in legs (NOT in PD!)
      • Slowing of active movement or slowness in initiating movement. 
      • Initial surge of motor activity is inadequate, movements are fragmented into incremental steps.
      • Changes in gait and balance.
      • Short, shuffling + festination.
      • Loss of arm movements. (walks with arms straight down).
      • Postural reflex mechanisms lost, --> pts have tendency to fall.



    • Levodopa/Carbidopa  (Sinemet) 100/25mg formulation 1/2 TID x7d
      • Increase to 1tic and judge effect after 2 weeks. (titrate up by 1/2 tab increments)
      • "Fail medication" if tried 1000mg of L-dopa/day  (Carbidopa 65mg/day)
      • As time goes on --> benefit becomes shorter
    • Dopamine Agonists
      • Don't work as well, S/E: nausea, psychotic, hallucinate, edema, impulse control.
      • Pramipaxole, 


    • By definition:
      • 2 or more are present:
    1. Tremor
    2. Rigidity
    3. Bradykinesia
    • Primary Parkinsonism (aka Idiopathic) => Parkinson Disease
    • Secondary parkinsonism
      • May persist for months after discontinuation of causing drugs.
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